patient_id
int64 0
167k
| patient_uid
stringlengths 8
10
| PMID
int64 1.34M
35M
| file_path
stringlengths 31
35
| title
stringlengths 7
377
| patient
stringlengths 55
95.4k
| age
stringlengths 14
33
| gender
stringclasses 2
values | relevant_articles
stringlengths 15
20.5k
| similar_patients
stringlengths 2
1.26k
|
|---|---|---|---|---|---|---|---|---|---|
165,100 | 3883382-1 | 24,427,506 | noncomm/PMC003xxxxxx/PMC3883382.xml | An Unusual Case of Peters Plus Syndrome with Sexual Ambiguity and Absence of Mutations in the B3GALTL Gene | The patient presented here is a 4 year-old girl of healthy consanguineous parents. Her younger sister was also healthy. Prenatal echography revealed short limbs. The PPS was evoked at birth and the patient was addressed for facial dimorphisms. Clinical investigations including karyotype, pediatric and ophthalmologic exams were performed after informed consent. At birth, she was underweight 2660 g (-1.6 SD), her length was 46 cm (-1.4 SD), and occipitofrontal circumference (OFC) 34 cm (±0 SD). Apgar score was 8/10. Neonatal exams revealed complete bilateral cleft lip and palate, large anterior fontanel, hypertelorism and a coccygeal dimple suggesting vertebral schisis. She also had disproportionate short stature and facial dysmorphism including dolichocephaly, round face, small nose with reverted nostrils and erased and long philtrum. Short limbs in the proximal segment and brachydactyly with bilateral clinodactyly of the fifth finger were also seen. Transfontanel ultrasound imaging showed quadri ventricular hydrocephaly and microcephaly caused by agenesis and atrophy of corpus callosum. Ophthalmologic exam revealed bilateral central corneal opacity without buphthalmos or congenital glaucoma.\nIn addition to these typical features for PPS, the patient showed sexual ambiguity without urogenital anomalies. In fact, she had hyper pigmented labia majora with pseudo scrotal aspect without clitoris hypertrophy, but she had two distinct (urethral and vaginal) orifices. Abdomen ultrasound showed a morphologically normal uterus with normal size, but renal pyelo-calicial dilation on left side was appreciated. Chromosome analysis on lymphocytes revealed a normal 46, XX karyotype. At the age of 4 years, the patient's weight was 11 kg (-2SD), length 78 cm (-3SD) and OFC 47 cm; without delayed psychomotor development.\nB3GALTL is the only gene known to be associated with Peters Plus syndrome, and most affected individuals tested to date are homozygous for a hot spot splice mutation in the acceptor splice site of intron 8 (c.660 + 1G > A). Sequencing of the 15 coding exons and flanking introns boundaries of the B3GALTL gene showed that the patient is B3GALTL mutation-negative. But we cannot exclude the presence of splice site mutation distant from the coding sequence, or mutation in the regulatory sequences of the gene. In addition, screening of the three intragenic SNP(s) which are described to be associated with B3GALTL gene mutations showed that the patient is heterozygous for two SNP (rs9315120, rs877103) and homozygous for the rs877104 one (). These results suggest but not confirm the exclusion of the B3GALTL gene in this case (). | [[4.0, 'year']] | F | {'16909395': 1, '21378754': 1, '6443615': 1, '22759511': 1, '19118497': 2, '30693145': 2, '20584037': 1, '20451863': 1, '24427506': 2} | {'6334245-1': 1, '2635348-1': 1} |
165,101 | 3883395-1 | 24,403,620 | noncomm/PMC003xxxxxx/PMC3883395.xml | Airway management in cervical spine ankylosing spondylitis: Between a rock and a hard place | A 69-year-old gentleman presented with 3 year history of progressive dysphagia. His past medical history was significant for AS with previous anaesthetics notable for failed direct laryngoscopy and considerably difficulty with fibreoptic orotracheal intubation.\nThe patient's presenting dysphagia was first evaluated by the ear nose and throat (ENT) surgeon whose awake flexible fiberoptic nasoendoscopy revealed a significant bulging of the posterior pharyngeal wall into the airway at the level of the epiglottis. A plain X-ray and computed tomography scan of the spine demonstrated significant anterior cervical osteophytes from C3 to C5 with the largest osteophyte of size equivalent to the diameter of the vertebral body at level C3 and C4 [Figure and ]. Patient was referred to the Spine Surgery Unit and he was scheduled to undergo removal of the exuberant anterior cervical osteophytes for symptomatic relief of his dysphagia. The surgeons requested that an elective pre-operative tracheostomy be avoided, if possible as this would interfere with the planned surgical approach through an incision through the anterior triangle of the neck. In the Pre-anaesthetic Clinic, after careful evaluation and discussion with the patient, an awake fibreoptic intubation was planned.\nAfter an appropriate period of fasting, patient was brought to the OR with both the ENT and Spine Surgery teams in attendance. After standard monitoring was instituted, in the semi-sitting position; airway topicalization with 2% lidocaine spray was attempted. A right superior laryngeal nerve block and trans-tracheal injection were performed with 2 ml each of 2% lidocaine. Patient was placed supine, but as his head did not touch the mattress (phantom pillow sign); a stack of flannel in the configuration of a ramp was placed under his head, neck, and torso.\nA modified nasal trumpet (MNT) was prepared by inserting a 15 mm universal connector from a 7.0 mm ID tracheal tube into the flanged end of a 30Fr nasal airway [simulated in ]. This was lubricated and inserted into the left nares. Oxygen (3 l/min) was delivered through this airway using the anaesthetic breathing circuit and capnography confirmed a patent airway and adequate spontaneous breathing. Into the other nares, the split nasopharyngeal airway (SNPA) was inserted. The SNPA is a 32Fr nasal airway, which had previously been cut spirally [simulated in ]. A standard adult fibreoptic bronchoscope (5.5 mm) was loaded with a size 7.0 mm ID reinforced tracheal tube (LMA Fastrach tube, Vitaid, Toronto, Canada). The bronchoscope was introduced through the SNPA and the large posterior pharyngeal wall bulge was evident. Advancing and rotating the SNPA caudad and posterior to the bony bulge was required to facilitate visualisation of the vocal cords,caudad and posterior to the bony bulge. The vocal cords were sprayed with 2cc of 2% lidocaine and the bronchoscope passed into the trachea without resistance. The SNPA was withdrawn out of the nares and peeled off the bronchoscope. The lubricated tracheal tube was then threaded off the bronchoscope into the trachea. Patient tolerated the procedure well and after confirmation of the appropriate position of the tracheal tube, anaesthesia was induced intravenously and then maintained with sevoflurane in an air-oxygen mixture using controlled ventilation.\nThe surgeons performed a left-sided anterior neck approach to the cervical spine and then proceeded to dissect the pharynx off of the osteophytes. The dissection was carried down to the pre-vertebral fascia covering the osteophytes. They removed the anterior osteophytes from C3 to 5. After removal of the bony elements, the pharynx was once again evaluated to make sure there was no soft-tissue pathology. The neck was closed in layers. Throughout the surgery, all monitored parameters remained stable.\nAt the end of surgery, the need for a definitive postoperative airway was discussed. The possibility of pharyngeal oedema and anticipated difficult airway due to the AS were considered and though leaving the trachea intubated was considered, it was decided that the patient would be best served by an elective tracheostomy. After the oxygen delivery was reduced to FiO2 of 0.3, a tracheostomy was performed and the tracheal tube was removed. He woke up breathing comfortably and was monitored in the Post-Anaesthetic Care Unit overnight. The tracheostomy was decannulated on the 3rd post-operative day and he was discharged on the 5th post-operative day. When followed-up 6 weeks later, he continued to be doing well - his dysphagia partially resolved and he was able to swallow semi-solid food. | [[69.0, 'year']] | M | {'16829671': 1, '19393122': 1, '11049921': 1, '20428989': 1, '14656788': 1, '8425244': 1, '11812720': 1, '8311302': 1, '16548876': 1, '20532081': 1, '16076927': 1, '19413825': 1, '9890380': 1, '32605592': 2, '19772826': 1, '17884706': 1, '24403620': 2} | {'7325286-1': 1} |
165,102 | 3883396-1 | 24,403,621 | noncomm/PMC003xxxxxx/PMC3883396.xml | Anaesthetic management in a child with an atypical triad for reconstructive scoliosis surgery | An 11-year-old, 26-kg female patient was admitted in orthopaedic unit with complains of backache, difficulty in walking and deformity of spine and was diagnosed case of G6PD deficiency and CP. She had severe thoracolumbar scoliosis (Cobb's angle - 55°) and was planned for surgical correction of the deformity. She had a history of premature delivery with low birth weight (1500 g) and epilepsy on oral carbamazepine for the last 5 years. On chest auscultation, bilateral coarse crepitations in lower zones and a pansystolic murmur in precordial area were heard.\nPulmonary function tests (PFT) and echocardiography with colour Doppler were advised, but the patient was not able to perform PFT. Echocardiography showed moderate pulmonary artery hypertension (pulmonary artery pressure - 32 mmHg) with left ventricular ejection fraction of 70%and grade-3 mitral regurgitation. Patient was scheduled for surgery after optimizing chest condition with full course of antibiotics and bronchodilators. On the day of surgery, infective endocarditis prophylaxis was instituted (amoxicillin 1 g PO and Inj.Gentamicin 40 mg 1 h prior to the surgery) in view of severe regurgitant lesion.\nAfter obtaining written informed parental consent the child was transferred to the operating room and all baseline monitors (non-invasive blood pressure, continuous electrocardiogram, oxygen saturation-pulse oximeter and bispectral index) were attached. Child was pre-medicated with glycopyrrolate (125 μg IV) and fentanyl (60 μg IV). Anaesthesia was induced with propofol (60 mg IV) and muscle relaxation was achieved by administration of atracurium besylate (15 mg IV). Trachea was intubated with cuffed endotracheal tube and anaesthesia was maintained with rate controlled infusion of propofol (6 mg/kg/h, following the 10-8-6 regimen to achieve effective plasma concentration).[] Atracurium (0.5 mg/kg/h) and fentanyl (1 μg/kg/h) with 50% O2 in air. Left radial artery and ultrasound guided right internal jugular vein cannulation were carried out for continuous haemodynamic and central venous pressure monitoring respectively. Peripheral nerve stimulator was applied and urinary catheter and nasopharyngeal temperature probe were inserted. Appropriate measures to maintain normothermia were employed including warming blankets and warm fluids infusion. Intraoperatively, wake-up test was performed successfully. Tranaexemic acid (loading dose of 10 mg/kg just before surgical incision followed by continuous infusion of 1 mg/kg/h until closure of skin incision) was also administered to minimise intra-operative blood loss. Surgery lasted for nearly 7 h with approximate blood losses of 1200 ml, which was adequately replaced.\nPost-operatively, patient was transferred to intensive care unit (ICU) for elective ventilation in view of hypothermia (32°C) following prolonged surgery, which was not responding to aforementioned warming measures. Patient was electively ventilated for next 24 h on continuous infusion of fentanyl (2 μg/kg/h IV) and dexmedetomidine (0.2 μg/kg/h IV). Trachea was extubated next day uneventfully. On day 3, patient was shifted to high dependency unit and subsequently discharged on the 7th day without any complication. | [[11.0, 'year']] | F | {'20407351': 1, '3259089': 1, '15675216': 1, '17631035': 1, '26543477': 1, '15644748': 1, '21821507': 1, '16310661': 1, '16225031': 1, '19571298': 1, '24403621': 2} | {} |
165,103 | 3883397-1 | 24,403,622 | noncomm/PMC003xxxxxx/PMC3883397.xml | Perioperative management of liver transplantation with concurrent coronary artery disease: Report of two cases | A 62-year-old man (156 cm, 48 kg), scheduled to undergo LRLT for cryptogenic ESLD (Child-Turcotte-Pugh score 9, MELD 12), was decompensated with hepatic encephalopathy, ascites and hepatorenal syndrome. He also had hypertension and diabetes mellitus. He suffered cardiac arrest lasting approximately 30 seconds during large volume paracentesis 8 months ago. A quicker decompression probably induced asystole. Post-cardiac arrest, there was no neurological deficit. He had effort tolerance of NYHA class II. Preoperative echocardiogram showed ejection fraction (EF) of 55% with no pulmonary hypertension. Dobutamine stress echocardiogram was negative for inducible ischemia. Preoperative angiography revealed 30% block in proximal left-anterior descending artery (LAD), 100% block in posterior descending artery with good collateralization. The patient was on metoprolol, terlipressin, aspirin and insulin. Airway examination was normal. Preoperative laboratory values are summarized in . Aspirin was discontinued 7 days before surgery while metoprolol was continued.\nAnaesthesia was induced with fentanyl 200 mcg, propofol 70 mg and intubation was facilitated with rocuronium 50 mg. Anaesthesia was maintained with 1-1.5% isoflurane in air/oxygen and fentanyl at 2 mcg/kg/h. Intraoperative monitoring included a five-lead electrocardiogram (ECG) with ST trends, invasive arterial blood pressure; EV1000® (version-1.2)/flotrac® (Edward life sciences, USA) for CO, SVR, systolic volume variation (SVV) and central venous pressure (CVP) and pulmonary artery catheter (PAC) for mean pulmonary artery pressure (MPAP) and pulmonary capillary wedge pressure (PCWP). Thromboelastograhy, PT/INR, haemoglobin, platelet count and ABG were monitored at regular intervals.\nThe intraoperative goals for mean arterial pressure (MAP), heart rate (HR), MPAP, CO, SVR and urine output were 65-75 mmHg, 60-90 beats/min, 15-20 mmHg, 6-10 l/min, 500-800 dyne.s/cm5 and 0.5 ml/kg/h, respectively. They were maintained with intravenous fluids along with noradrenaline and terlipressin infusions.\nInferior vena cava (IVC) was partially clamped during hepatic vein reconstruction. Transient fall in MAP at reperfusion was managed with boluses of noradrenaline and phenylephrine. No intraoperative ST-segment changes were detected.\nIntraoperative haemodynamic variables are summarized in . Total blood loss was approximately 1500 ml. The patient was shifted to liver transplant ICU and extubated 12 hours later. Antiplatelet therapy and metoprolol were restarted 48 hours after the surgery. Twelve-lead ECG 12 hourly and Troponin-I level 24 hourly were monitored for the first 5 days. The patient was shifted from ICU on the 10th day and discharged on the 16th postoperative day. | [[62.0, 'year']] | M | {'9346688': 1, '19100436': 1, '9039930': 1, '19752326': 1, '22429362': 1, '17033479': 1, '17456488': 1, '2401460': 1, '11699035': 1, '15629509': 1, '15488059': 1, '16382461': 1, '24403622': 2} | {'3883397-2': 2} |
165,104 | 3883397-2 | 24,403,622 | noncomm/PMC003xxxxxx/PMC3883397.xml | Perioperative management of liver transplantation with concurrent coronary artery disease: Report of two cases | A 55-year-old man (167 cm, 93 kg), scheduled to undergo LRLT for hepatitis B associated ESLD (Child-Turcotte-Pugh score 9, MELD 15), was decompensated with ascites and oesophageal variceal bleed. CAD was diagnosed when he had an episode of myocardial infarction 7 months ago. Subsequently, he underwent percutaneous intervention (PCI). Drug eluting stent was placed in LAD and bare metal stents were placed in D1 and D2 artery. He was on aspirin and clopidogrel (75 mg each). He remained asymptomatic subsequently. He also had hypertension and diabetes mellitus. Aspirin was stopped 2 months later in the view of repeated malaena. Preoperative echocardiogram showed EF of 42% with no pulmonary hypertension. During exercise stress echocardiogram, he achieved 90% of target HR but EF reduced from 42% to 35% and the mid-anterior septum and distal-inferior wall became akinetic. He became breathless 6 minutes after exercise and his functional capacity was rated good (METs-7, NYHA class II). Preoperative angiography revealed patent stent in LAD, blocked stent in D1 and D2 artery. Left circumflex artery was 10% blocked, while right coronary artery was 40% blocked. No further intervention was deemed necessary by cardiologist. The patient was on bisoprolol, torsemide, atorvastatin, clopidogrel and insulin. Clopidogrel was stopped 1 week prior to surgery and enoxaparin 60 mg OD was started. Airway examination was normal. Preoperative laboratory reports are summarized in .\nEnoxaparin was stopped 12 hours prior to surgery and bisoprolol was continued. Anaesthesia was induced with fentanyl 300 mcg, thiopentone 175 mg and intubation of trachea was facilitated with rocuronium 100 mg. Anaesthesia was maintained with 1-1.5% isoflurane in air/oxygen and fentanyl at 3 mcg/kg/h. Intraoperative monitoring and goals were same as in case 1. In addition, defibrillator pads were applied on chest. A 6 Fr sheath introducer was placed into left femoral artery for emergent establishment of intra-aortic balloon pump; however, the requirement did not arise.\nIntraoperative haemodynamic variables are summarized in . During the later part of the dissection phase, despite high dose of noradrenalin infusion, terlipressin infusion was started to maintain MAP and SVR. IVC was partially clamped during hepatic vein reconstruction. Before reperfusion, portal flush was used to avoid post-reperfusion syndrome (PRPS). A transient fall of MAP was treated with boluses of noradrenaline, phenylephrine and soda-bicarbonate. No intraoperative ST-segment changes were detected. Total blood loss was approximately 1200 ml. The patient was shifted to liver transplant ICU and extubated 6 hours later. Antiplatelet therapy and bisoprolol were restarted 1 day after the surgery. His postoperative recovery was uneventful. Postoperative monitoring was similar to the first patient. The patient was shifted from ICU on the 8th day and discharged on the 16th postoperative day. | [[55.0, 'year']] | M | {'9346688': 1, '19100436': 1, '9039930': 1, '19752326': 1, '22429362': 1, '17033479': 1, '17456488': 1, '2401460': 1, '11699035': 1, '15629509': 1, '15488059': 1, '16382461': 1, '24403622': 2} | {'3883397-1': 2} |
165,105 | 3883398-1 | 24,403,623 | noncomm/PMC003xxxxxx/PMC3883398.xml | Airway management in Escobar syndrome: A formidable challenge | An 1-year-old female child diagnosed with Escobar syndrome since birth, weighing 10 kg was posted for bilateral hamstring to quadriceps transfer. Pre-operative examination revealed multiple joint flexion contractures. Airway examination showed adequate mouth opening, whereas neck extension was limited due to flexion contracture over neck. Systemic and haematological examinations were unremarkable. The lateral X-ray of the neck showed that there was no cervical spine fusion. The child also had atrial septal defect at birth, which was now closed, with normal electrocardiography (ECG) and echocardiography findings. After adequate nil per oral, patient was shifted to the operation theatre (OT) without premedication. Difficult airway cart was already kept ready in the OT. Preinduction monitors were five lead ECG, pulse oximetry and non-invasive blood pressure. Child was induced by inhalation induction with 6-8% sevoflurane and 100% oxygen. After adequate depth of anaesthesia, intravenous (IV) cannulation was carried out in the right upper limb. After checking ability to mask ventilate, 0.1 mg IV glycopyrrolate, 10 μg IV fentanyl, 5 mg IV propofol were given to increase depth of anaesthesia. Child was paralyzed with 5 mg IV atracurium, and mask ventilation was carried out with 2-3% sevoflurane and oxygen. After 3 min, intubation was attempted with conventional laryngoscopy. Intubation was unsuccessful twice wherein only the tip of epiglottis could be visualized even after optimal external laryngeal manipulation. Further attempts at conventional laryngoscopy were abandoned considering possibility of airway trauma and edema. Patient's saturation was maintained around 96-98% during this period. A size 1.5 proseal laryngeal mask airway (PLMA) was secured in place and capnogram was obtained. Ryles tube was inserted through the gastric channel to deflate the stomach. Even though there was optimal chest expansion, it was accompanied by an increase in end tidal CO2 values to about 55-60 mmHg after 5 min of controlled ventilation. Furthermore, as the surgery was expected to last about 3 h, we decided to proceed with PLMA-assisted fibreoptic guided endotracheal intubation. A 4.0 mm internal diameter uncuffed portex endotracheal tube (ETT) was railroaded over the bronchoscope of 2.4 mm outer diameter and then the bronchoscope was introduced through the lumen of PLMA into the trachea. The ETT was then advanced through the lumen of the PLMA into the trachea and tip position was confirmed above the carina by visualization through bronchoscope. Equal bilateral air entry was confirmed by auscultation. The PLMA with ETT in situ was fixed as a single unit []. Anaesthesia was maintained with 66% nitrous oxide, 33% oxygen and sevoflurane 1.5-2%. With adequate ventilation, the ETCO2 of 35-37 mmHg was maintained throughout surgery. Caudal analgesia was given after careful positioning with 10 mL of 0.2% ropivacaine and 10 μg clonidine. Surgery was carried out in supine position with application of tourniquet. Rest of the intraoperative period was uneventful. At the end of the procedure, child was reversed, and when she was fully awake and breathing adequately, the PLMA and ETT were removed as a single unit after oral and gastric suctioning. Child was shifted to a high dependency unit for monitoring. | [[1.0, 'year']] | F | {'18751807': 1, '21258974': 1, '3430553': 1, '23293383': 1, '21814339': 1, '2063909': 1, '30636811': 1, '3621655': 1, '27375397': 2, '28928596': 1, '19319089': 1, '21541234': 2, '8694333': 1, '18184253': 1, '8280738': 1, '24403623': 2} | {'4916826-1': 1, '3085429-1': 1} |
165,106 | 3883399-1 | 24,403,624 | noncomm/PMC003xxxxxx/PMC3883399.xml | Anaesthetic management of two different cases of mediastinal mass | An 8-year-old boy weighing 16 kg scheduled for median sternotomy for excision of anterior mediastinal mass had intermittent fever for 3 months and dry cough for 2 weeks associated with breathlessness on exertion. Chest X-ray, computer tomography (CT) scan, magnetic resonance imaging (MRI) and echocardiogram were performed to assess the mass and its compression on vital structures. Biochemical investigations were normal.\nIntravenous (I.V.) access was established in the right and left hands with a 22G I.V. cannula in addition to a femoral vein catheter for central venous access. The child was pre-medicated and induced with 5 mg/kg of thiopentone I.V. After confirming mask ventilation, suxamethonium 1.5 mg/kg was used for intubation. Intubation was performed with a 5.5-mm I.D. cuffed reinforced oral endotracheal tube connected to Bain's system. Anaesthesia was maintained with N2O:O2 (50:50) and sevoflurane 0.8-2.0%. Relaxation was achieved with vecuronium bromide 0.1 mg/kg. Analgesia was achieved with I.V. morphine 0.1 mg/kg. Normocapnia was maintained with suitable ventilatory frequency and tidal volume. Five hundred millilitres of Ringer's lactate was administered over 2 h.\nHeart rate, systolic blood pressure, diastolic blood pressure, mean arterial pressure, oxygen saturation, end-tidal carbon-di-oxide (EtCO2) and electrocardiography (ECG) with ST segment analysis were recorded. Anterior mediastinal mass was removed. Minimal compression was noted on innominate vein and addressed intra-operatively. Sternum was closed in midline. The patient was electively ventilated in a paediatric Intensive Care Unit (ICU) for 3 h, reversed and extubated. He was haemodynamically stable. Later, he had an uneventful course in the hospital before discharge. | [[8.0, 'year']] | M | {'4087108': 1, '11571906': 1, '4575847': 1, '972103': 1, '16317755': 1, '2350036': 1, '29416489': 1, '7091715': 1, '17211158': 1, '12722093': 1, '9044140': 1, '2764625': 1, '7175638': 1, '14717880': 1, '6546480': 1, '34898707': 1, '24403624': 2} | {'3883399-2': 2} |
165,107 | 3883399-2 | 24,403,624 | noncomm/PMC003xxxxxx/PMC3883399.xml | Anaesthetic management of two different cases of mediastinal mass | A 16-year-old female weighing 37 kg, with dyspnoea on exertion that worsened in the supine position, had productive cough, intermittent fever and dysphagia for solid food for 1 month; hence, she was referred to a pulmonologist for management of her condition. On treatment with antibiotics and bronchodilators, the breathlessness reduced and she was able to lie supine without discomfort. Her haemoglobin improved from 7.3 gm% to 11.5 gm% after transfusion of 2 units of packed red blood cells (PRBCs). Her pulse rate was 110/min and her blood pressure was 110/60 mmHg. On examination, her trachea was central and the apical impulse was in the 5th left intercostal space in the mid-axillary line and there was reduced air entry in the right supraclavicular, infraclavicular, mammary and subscapular areas and dullness on percussion. Clinically, the other systems were normal. ECG showed sinus tachycardia. The chest X-ray showed mediastinal widening to the right of the midline obscuring the right cardiac border [].\nFibreoptic bronchoscopy (FOB) performed pre-operatively by a pulmonologist with the aid of oral local anaesthetic gargle and spray showed no extrinsic compression on the airway. CT scan of the thorax revealed a lobulated heterogeneous solid cystic mass on the right side of the superior mediastinum, extending from the inlet of the thorax to the level of the upper part of the right atrium. The mass showed fat, fluid, soft tissue and calcification. Spirometry showed severe restrictive and obstructive lung disease with peak expiratory flow rate of 26%. Arterial blood gas indicated mild hyperventilation.\nAfter taking informed high-risk consent, the patient was scheduled for right lateral thoracotomy and excision of tumour. She was given Tab. Alprazolam 0.25 mg and Tab. Ranitidine 150 mg previous night with nil-per-oral orders.\nVenous cannulation was obtained with a 16G peripheral I.V. cannula. The right femoral vein and the left femoral artery were also cannulated. Inj Ondansetron 0.1 mg/kg and Inj Glycopyrrolate 0.02 mg/kg were administered before induction. Pre-oxygenation with 100% oxygen for 3 min was followed by induction with sevoflurane 8%. After confirmation of mask ventilation, the child was intubated with a 26Fr left-sided double-lumen tube (DLT). Air entry was bilaterally equal inspite of blocking bronchial or tracheal lumen. The tube was then removed and the patient was ventilated using a face mask with 100% O2. Three attempts of intubation with the 26Fr left-sided DLT failed to achieve single lung ventilation. As the patient started desaturating, ventilation with 100% O2 and intubation with a 6.00 mm cuffed oral endotracheal tube was performed. Bilateral air entry was not appreciated. As the patient developed bradycardia, Inj. Atropine 0.02 mg/kg was then administered, but there was no improvement. The patient responded to Inj adrenaline (1 mg/kg) I.V. and developed tachycardia (150-170 bpm). Because of the non-availability of a reinforced tube, the 6.0 mm tube (already in situ) was removed as it had failed to establish air entry, and was reintroduced this time with a stylet. This attempt with a stylet was successful at negotiating the compressive obstruction and establishing ventilation, although insufficiently. While the immediate danger of desaturation was addressed, adequate ventilation was not achieved.\nTo achieve adequate ventilation, the 6.0 mm tube was replaced with a 7.0 mm tube using a tube exchanger, after making sure that the existing tube tip was indeed at the mid-tracheal position with the aid of a flexible FOB.\nAfter 10 min, the patient developed pulmonary oedema for which Inj Furosemide 40 mg I.V. was given and pulmonary oedema was seen to resolve. While risks versus benefit of proceeding with the surgery (in view of pulmonary oedema having occurred, although transiently and the possible need for elective ventilation – should the need arise) were being deliberated by the surgical and the anaesthesiology teams, the patient was placed in a left lateral position for better ventilation. When surgery resumed, the patient was positioned for right lateral thoracotomy. Anaesthesia was maintained with O2, N2O, sevoflurane and Inj Atracurium. Intra-operatively, there was one episode of hypotension, which was managed with Inj Noradrenaline infusion. As blood loss was 350 mL, one unit of PRBC was transfused. EtCO2 was high (60-76 mmHg.) until excision of the tumour. After excision of the tumour, the heart rate and EtCO2 improved (36-48 mmHg). The patient was ventilated in the ICU for 24 h. Inotropic support was tapered on the second day and patient was extubated on the third post-operative day. She had an uneventful course and got discharged after 10 days. Histopathology confirmed the mass as benign mature teratoma. | [[16.0, 'year']] | F | {'4087108': 1, '11571906': 1, '4575847': 1, '972103': 1, '16317755': 1, '2350036': 1, '29416489': 1, '7091715': 1, '17211158': 1, '12722093': 1, '9044140': 1, '2764625': 1, '7175638': 1, '14717880': 1, '6546480': 1, '34898707': 1, '24403624': 2} | {'3883399-1': 2} |
165,108 | 3883400-1 | 24,403,625 | noncomm/PMC003xxxxxx/PMC3883400.xml | Ultrasound guided percutaneous electro-coagulation of ilioinguinal and iliohypogastric nerves for treatment of chronic groin pain | A 60-year-old male patient was referred from general surgery department for assessment and management of right chronic groin pain. This patient had undergone right inguinal open meshplasty with preservation of ilioinguinal (II), iliohypogastric (IH) and genital nerves 6 months back for reducible right inguinal hernia of 10 months duration. The patient gave no history of right chronic groin pain or any other chronic pain before surgery. He developed chronic groin pain around the incision site, at the root of penis, upper medial part of right thigh and part of right scrotum for past 2½ months for which he was taking analgesics without satisfactory relief. Pain was of burning and pricking character, which was aggravated by walking and sitting. It was interfering with his daily and employment activities. There was no pain at the time of sexual intercourse and ejaculation. Tapping the skin medial to the right anterior superior iliac spine (ASIS) and over areas of local tenderness produced pain (Tinel's test positive).\nHistory and examination were suggestive of neuropathic pain (VAS 7/10) in the territory of II and IH nerves. The plan was to do a diagnostic nerve block with lignocaine followed by chemical neurolysis under ultrasound guidance. The II and IH nerves were identified by ultrasound (Micro MAXX®, Sonosite) with high frequency linear array probe (HFL38/13-6 MHz) in resolution mode, medial and above right ASIS. With the help of colour Doppler, ascending branch of deep circumflex iliac artery was identified to avoid mistaking it for nerve. The diagnostic block was carried out with 2 mL lignocaine 1% for each nerve. Patient had complete pain relief at rest and with activities, confirming it to be neuropathic. Following day chemical neurolysis of both nerves was carried out with ethyl alcohol (1 mL for each nerve) in two target areas (one just medial to ASIS and other one above ASIS) to ensure success of the intervention.\nPatient was reviewed after 4 weeks. Patient had absolute pain relief for one week and only 25% pain thereafter. Pain was still affecting patient's daily activities so diagnostic nerve block was performed again which completely relieved the pain. After discussion with surgeons, patient was told about next option of surgical neurectomy. Another less invasive, percutaneous electrocoagulation of both nerves with the possibility of recurrence of pain was also explained for which patient agreed and gave informed consent. Electrocoagulation of both nerves was planned with 3 Fr Bugbee flexible monopolar cautery electrode (Karl Storz, []). Before performing the procedure on this patient, simulation was done on animal model to study appearance of the electrode tip under ultrasound and energy to be used for electrocoagulation. It was possible to place electrode tip over targets after passing it through epidural needle. The energy of different intensities was applied (10, 15 and 20 W) for one second in fulguration mode to three different targets and coagulation zones were studied. 15 W was found suitable for this procedure. Ultrasound guided electrocoagulation of both nerves was done under spinal anaesthesia []. This procedure was carried out for both nerves in similar two targets to ensure success of the intervention. Patient was given analgesics for 3 days and tablet carbamazepine 100 mg 8th hourly for one week. Four months after procedure, patient had loss of sensation to touch, cold and pain in areas supplied by II and IH nerves. There was effective relief of pain and improvement in quality-of-life. | [[60.0, 'year']] | M | {'19747055': 1, '21765970': 1, '1113147': 1, '12457217': 1, '22622908': 1, '16878278': 1, '33606079': 1, '12945086': 1, '21365287': 1, '21502450': 1, '24403625': 2} | {} |
165,109 | 3883402-1 | 24,403,627 | noncomm/PMC003xxxxxx/PMC3883402.xml | Unusual occurrence of massive subcutaneous emphysema during ERCP under general anaesthesia | A 62-year-old woman with obstructive jaundice and deranged liver function tests was referred for an elective ERCP and stenting/sphincterotomy. Her past medical history and examination were unremarkable. She was given a general anaesthetic with endotracheal intubation. During ERCP which was indeed difficult, a peripapillary mass and a narrowed common bile duct without stones was noted. A sphincterotomy was tried repeatedly without success. There were no adverse anaesthetic events till late into the procedure when there was sudden drop in oxygen saturation from 99% to 88%. This prompted the anaesthesiologist to auscultate the chest who noted the presence of crepitus over the anterior as well as posterior aspect of chest. As the patient was in the semi-prone position and draped, further closer examination of the patient's face and neck revealed emphysematous swelling predominant on the right side, previously non-existent []. Suspecting retro-peritoneal perforation, the patient was turned supine and then she was noted to have slightly distended abdomen too. Oxygen saturation improved immediately after change of position and ventilation with 100% oxygen. A gastrointestinal surgery consultation was obtained and it was decided to undertake surgical exploration immediately. However, laparotomy did not reveal any definitive perforation even though there was minimal retro-duodenal staining with bile. Roux-en-Y hepaticojejunostomy and gastrojejunostomy was performed as a palliative measure. After surgery, the patient was shifted to intensive care unit (ICU) for elective ventilation where a chest X-ray revealed diffuse subcutaneous emphysema without any evidence of pneumothorax and pneumomediastinum []. She was weaned off the ventilator next morning, had a steady resolution of subcutaneous emphysema, and was discharged on day 12 without any jaundice and emphysema. | [[62.0, 'year']] | F | {'20800134': 1, '17509029': 1, '11932784': 1, '19152899': 1, '10903596': 1, '25331827': 1, '9619316': 1, '3234686': 1, '25331971': 1, '22341094': 1, '20970792': 1, '28791071': 2, '24403627': 2} | {'5525507-1': 1} |
165,110 | 3883403-1 | 24,403,628 | noncomm/PMC003xxxxxx/PMC3883403.xml | Airway management for tracheal stent insertion in a patient with difficult airway | A 56 year old, 45 kg male patient developed progressively worsening dyspnea after having undergone chemotherapy for non-small cell carcinoma of trachea. On examination, the patient was in respiratory distress with inspiratory stridor and marked bilateral wheezing. Upper airway was assessed to be normal with a Mallampati Class II. Bronchoscopic examination revealed a tight stricture getting intermittently blocked by inflammation and secretions. Patient was scheduled for interventional bronchoscopy with insertion of Dumon Y – stent across the stenotic trachea. The limbs of the Y would be inserted into left and right main bronchus while the long stem would remain in the trachea across the stricture.\nAfter premedication with glycopyrrolate 0.2 mg IM, anaesthesia was induced with fentanyl 2 μg/kg and sevoflurane 5-8% in oxygen (O2). However, direct laryngoscopy revealed a difficult airway (Cormack Lehane Grade III) and it was not possible to introduce the rigid bronchoscope. As visualization of glottic opening was not possible by direct laryngoscopy the fiberoptic bronchoscope (FOB) (nasal route) was used to guide the ventilating bronchoscope tip into laryngeal inlet []. After passage of the rigid bronchoscope anaesthesia was maintained with sevoflurane in O2 with spontaneous respiration by attaching a Mapleson D Circuit to the side arm of the ventilating bronchoscope [].\nThe FOB was then inserted via the rigid bronchoscope to examine the trachea-bronchial tree and adjust the tip of the rigid bronchoscope above the stricture. However, ventilation became inadequate and ETCO2 increased from 35 mmHg to 50 mmHg and injection suxamethonium 75 mg intravenous (IV) was administered and high-frequency jet ventilation (HFJV) commenced by placing the O2 driven jet through open end of the rigid bronchoscope. Propofol and fentanyl infusions at 50 μg/kg/min IV and 1 μg/kg/h respectively were administered to deepen the anaesthetic depth and diminish the pressure response. FOB examination revealed a crescent shaped tracheal stenosis 5.5 cm away from the vocal cords, causing 75% luminal obstruction. The FOB was then removed and under fluoroscopy guidance, a Y silicon Dumon stent was deployed across the tracheal stricture using the special introducer. After removal of the introducer, the FOB was reintroduced and with the bronchoscopic forceps the stent was adjusted to fit in proper place. During this procedure anaesthesia was maintained with jet ventilation via the ventilating port as there was insufficient space at inlet of rigid bronchoscope for placing the jet needle []. Jet ventilation remained suspended for about 3 min during the stent insertion maneuver. After stent insertion anaesthesia was discontinued. The O2 saturation was maintained above 95% before, during and after the stent insertion. Following resumption of adequate spontaneous breathing, the rigid bronchoscope was removed. | [[56.0, 'year']] | M | {'10349923': 1, '10443620': 1, '29159029': 1, '3942309': 1, '31772404': 1, '7521067': 1, '30429747': 2, '7512286': 1, '7425338': 1, '24403628': 2} | {'6180676-1': 1} |
165,111 | 3883584-1 | 24,403,839 | noncomm/PMC003xxxxxx/PMC3883584.xml | The long-term survival of a thymic carcinoma patient treated with S-1: a case report and literature review | A 73-year-old Japanese man was referred to our hospital in October 2008 because of an abnormal shadow in the mediastinum on chest X-ray. His medical history was unremarkable, and he had taken no medications. He had smoked 50 pack-years. Physical examination revealed no significant abnormalities except for his clubbed fingers. Laboratory findings were within normal ranges, except the serum cytokeratin 19 fragment (CYFRA) level of 16.6 ng/mL (normal range, 0–3.5 ng/mL). A subsequent computed tomography (CT) scan revealed a mass lesion in the anterior mediastinum involving the superior vena cava, with multiple pleural dissemination. Whole body 18F-fluorodeoxy glucose positron emission tomography (FDG-PET) showed strong activity in the anterior mediastinal lesions (7.1 maximum standardized uptake values) and the multiple pleural ones. A CT-guided needle biopsy of the main lesion in the anterior mediastinum was performed. The pathological diagnosis was squamous cell thymic carcinoma. Immunohistochemically, the tumor cells showed positive staining for CD117 (c-kit) and CD5, and negative staining for terminal deoxynucleotidyl transferase (TdT) in the lymphocytes in the tumor. The Ki-67 (MIB-1) index was more than 30%. The results of immunohistochemical analysis supported a diagnosis of thymic carcinoma. Immunohistochemical staining for the enzymes related to 5-FU metabolism was also performed: thymidylate synthase (TS) was strongly positive, dihydropyrimidine dehydrogenase (DPD) was weakly positive, and orotate phosphoribosyltransferase (OPRT) was not stained ().\nSince he had advanced stage disease (Masaoka–Koga staging system, IVa), he was treated with three cycles of first-line chemotherapy, including cisplatin (50 mg/m2) on day 1, doxorubicin (40 mg/m2) on day 1, vincristine (0.6 mg/m2) on day 3, and cyclophosphamide (700 mg/m2) on day 4, every 4 weeks from December 2008 to February 2009. After three cycles of chemotherapy, stable disease (SD) was observed. Additional radiotherapy (50 Gy) was given as multimodality treatment for the main anterior mediastinal lesion from March to April 2009. He had SD after the radiotherapy, and the serum CYFRA level was 14.0 ng/mL. In July 2009, the serum CYFRA level increased suddenly (34.7 ng/mL). Therefore, second-line chemotherapy with S-1 (50 mg/m2) twice a day was given. The administration of S-1 was conducted as follows: 2 weeks of administration and 1 week of withdrawal of S-1. The S-1 therapy was effective, since the main tumor and the pleural dissemination showed remarkable reduction in their sizes, and the serum CYFRA level became 1.7 ng/mL after two cycles of chemotherapy in September 2009 ( and ). The hematologic adverse events (AEs) were as follows: leukopenia (grade 3), neutropenia (grade 2), anemia (grade 2), and thrombocytopenia (grade 1), with no febrile neutropenia. There were no non-hematologic grade 3 or 4 AEs. The patient has been continued on S-1 without disease progression or impairment of his quality of life (QOL) for more than 4 years. | [[73.0, 'year']] | M | {'18758317': 1, '19011356': 1, '23313005': 1, '12942558': 1, '34581013': 1, '21273614': 1, '26291007': 1, '34771601': 1, '21550686': 1, '21502559': 1, '12712448': 1, '21665316': 1, '21892103': 1, '20951466': 1, '24403839': 2} | {} |
165,112 | 3883597-1 | 24,403,845 | noncomm/PMC003xxxxxx/PMC3883597.xml | Sudden and rapid progression of lung affectation but stability in kidney function: a case report of anti-neutrophil cytoplasmic antibody-associated vasculitis | A 69-year-old male patient presented as an outpatient with increased creatinine levels (281 μmol/L) associated with proteinuria (2.5 g/24 h) and hematuria (110 g/L). His history was not unusual and he denied any abuse of alcohol, drugs, or over-the-counter stimulants. Review of his history was negative for any recent fever, rash, contact with sick persons, or acute illness involving the respiratory or gastrointestinal tract. The examination of ANCA and a chest radiograph were unusual. He was diagnosed with ANCA-associated vasculitis after findings of a perinuclear ANCA (p-ANCA) positive reaction (495 U/mL), urine examination, renal insufficiency by creatinine assay, and lung infiltration by CT scan. After starting treatment with cyclophosphamide pulse therapy, sequential therapy with low-dose prednisone (10 mg qid [every other day]), he underwent a 4-year follow-up. During this time, the level of serum creatinine was stable (), and both proteinuria and hematuria improved (). A chest CT scan was performed, which showed that the lung fibrosis remained unchanged ().\nHowever, after the 4-year follow-up, the patient experienced a sudden and rapid deterioration in lung infiltration and exhibited pulmonary hemorrhage (PH) along with a progressively lower hemoglobin (). Interestingly, there was not only no increase in creatinine levels (), but also a negative conversion of p-ANCA. The galactomannan assay and Mycobacterium tuberculosis antigen-specific interferon (IFN)-gamma release assays (T-SPOT®-TB test; Oxford Immunotec Ltd, Oxford, UK) were both negative. After immunosuppressive therapy (methylprednisolone and cyclophosphamide pulse therapy for 1 week), and empirical widened coverage of anti-infective therapy, progressive lung fibrosis occurred, the PH worsened, and he died from respiratory failure. | [[69.0, 'year']] | M | {'21873269': 1, '17591523': 1, '23374071': 1, '17898039': 1, '23816724': 1, '21674416': 1, '16377381': 1, '21974704': 1, '22135282': 1, '16399845': 1, '19359982': 1, '18158235': 1, '20472100': 1, '22382223': 1, '23810690': 1, '17945475': 1, '16139567': 1, '21860222': 1, '24403845': 2} | {} |
165,113 | 3884113-1 | 24,416,056 | noncomm/PMC003xxxxxx/PMC3884113.xml | Adjuvant Treatment of Proper Endobronchial Management in Leiomyosarcoma | A 49-year-old female visited our hospital complaining of a painful mass in the right buttock and dyspnea. One month earlier, she had been diagnosed with leiomyosarcoma after excision of a mass in the right suprapubic area. Positron emission tomography-computed tomography (PET-CT) revealed metastases to the gluteal muscles and lungs.\nPalliative chemotherapy was recommended after the surgical excision because of the severe hip pain. Meanwhile a chest radiograph revealed haziness of the left hemithorax suggesting atelectasis of the left lung (). Bronchoscopy disclosed a huge encapsulated mass involving the left main bronchus ().\nThe tumor was removed bronchoscopically using a snare (Force FX TM-8C; Valley Lab, Boulder, CO, USA) and cryoextraction. A 5-cm encapsulated mass was extracted with a flexible cryoprobe (Erbokryo CA; ERBE Elektromedizin GmbH, Tübingen, Germany). After removing this huge mass, remnant tumor was seen in the left lower lobe. A chest radiograph after the bronchoscopic intervention showed improvement of the atelectasis (). Pathologic findings of endobronchial tumor showed a spindle cell neoplasm, which was consistent with metastatic leiomyosarcoma ().\nAfter recanalization of the left main bronchus via interventional bronchoscopy, the dyspnea disappeared and the patient received additional chemotherapy. Nevertheless, the endobronchial metastasis progressed and endobronchial obstruction recurred at the same site 4 months later. Interventional bronchoscopy for debulking was repeated, which relieved her symptoms, except mild nonspecific chest discomfort. The patient did not want further chemotherapy because of progression of the underlying leiomyosarcoma. She discharged to palliative care. | [[49.0, 'year']] | F | {'23424951': 1, '430724': 1, '22609980': 1, '2914299': 1, '15116003': 1, '23875060': 1, '19716140': 1, '2379579': 1, '22628181': 1, '12229189': 1, '19540646': 1, '18812690': 1, '22003155': 1, '24416056': 2} | {'3884113-2': 2, '3884113-3': 2} |
165,114 | 3884113-2 | 24,416,056 | noncomm/PMC003xxxxxx/PMC3884113.xml | Adjuvant Treatment of Proper Endobronchial Management in Leiomyosarcoma | A 40-year-old male was admitted the orthopedics department complaining of a mass on the right thigh. He underwent wide excision of this and pathology confirmed the diagnosis of leiomyosarcoma. Postoperative radiotherapy followed. Since multiple lung metastases were observed on PET-CT 1 month after radiotherapy, palliative chemotherapy followed. A craniotomy with tumor removal was performed for brain metastasis 1 year later.\nOne month after the craniotomy, worsening dyspnea developed. Total collapse of the left lung was observed on chest radiography (). Bronchoscopy was performed and an endobronchial tumor was observed in the proximal left main bronchus (). The tumor had a smooth mucosal surface and protruded into the left upper lobe bronchus. The tumor was removed using a snare and cryoextraction (). This relieved the dyspnea and a chest radiograph showed improved aeration (). The pathologic specimen of bronchial tumor revealed metastatic leiomyosarcoma (). Subsequently, palliative chemotherapy with pazopanib was administered.\nFive months after administering the pazopanib, the patient developed dyspnea and chest discomfort during expiration or position change. His cough worsened and produced blood-tinged sputum. Chest CT revealed on increased mass obstructing the left main bronchus again. Bronchoscopic examination showed endobronchial tumors obstructing the distal left main bronchus and openings of the left upper and lower lobes. Bronchoscopic tumor removal was repeated using cryoextraction. After debulking therapy, the symptoms were relieved and the patient was given additional palliative pazopanib chemotherapy. | [[40.0, 'year']] | M | {'23424951': 1, '430724': 1, '22609980': 1, '2914299': 1, '15116003': 1, '23875060': 1, '19716140': 1, '2379579': 1, '22628181': 1, '12229189': 1, '19540646': 1, '18812690': 1, '22003155': 1, '24416056': 2} | {'3884113-1': 2, '3884113-3': 2} |
165,115 | 3884113-3 | 24,416,056 | noncomm/PMC003xxxxxx/PMC3884113.xml | Adjuvant Treatment of Proper Endobronchial Management in Leiomyosarcoma | A 38-year-old female visited our emergency room complaining of worsening dyspnea and blood-tinged sputum. She had undergone a total hysterectomy for a uterine mass 5 years earlier. Chest radiography revealed multiple masses of various sizes in both lungs, suggesting pulmonary metastases. Chest CT revealed an elongated bronchial lesion in the left main bronchus, almost obstructing it. Abdominal CT revealed an 8.8-cm enhancing soft tissue mass with adjacent bone destruction at the sacrum, suggesting bone metastasis. Further evaluation including a biopsy was performed, and leiomyosarcoma with metastasis to lung, bone, heart, and brain was diagnosed.\nThe dyspnea worsened despite a first cycle of chemotherapy. Chest radiography revealed increased opacity of the left hemithorax, suggesting atelectasis (). Bronchoscopy revealed an encapsulated pedunculated mobile mass in the proximal left main bronchus (). Recanalization of the left main bronchus was performed using a flexible bronchoscope with cryoextraction (). A 6-cm mass was extracted from the left main bronchus (). The dyspnea was relieved and improvement of the atelectasis was observed on chest radiography (). Subsequently, the planned chemotherapy could be performed. | [[38.0, 'year']] | F | {'23424951': 1, '430724': 1, '22609980': 1, '2914299': 1, '15116003': 1, '23875060': 1, '19716140': 1, '2379579': 1, '22628181': 1, '12229189': 1, '19540646': 1, '18812690': 1, '22003155': 1, '24416056': 2} | {'3884113-1': 2, '3884113-2': 2} |
165,116 | 3884114-1 | 24,416,057 | noncomm/PMC003xxxxxx/PMC3884114.xml | Successful Rechallenge with Imatinib in a Patient with Chronic Myeloid Leukemia Who Previously Experienced Imatinib Mesylate Induced Pneumonitis | A 43-year-old man was admitted to the hospital for 2 weeks of dyspnea. He was diagnosed with CML 3 months ago and took 400 mg of imatinib/day for 2 months. His vital signs were blood pressure, 110/80 mm Hg; pulse rate, 72/min; respiratory rate, 20/min; and body temperature, 36.4℃. He presented with an acute ill-looking appearance but no specific findings were noted on neck and abdominal examinations. Heart beat was regular and breathing sounds were clear. The laboratory findings were as follows: white blood cells, 3,630/µL (segmented neutrophils, 54%; lymphocytes, 25%; eosinophils, 12%); hemoglobin, 12.4 g/dL; platelets, 136,000/µL, C-reactive protein, 0.31 mg/dL (normal range, 0.01-0.47 mg/dL); total protein, 5.7 g/dL; albumin, 3.4 g/dL, aspartate aminotransferase/alanine aminotransferase, 27/28 U/L; lactate dehydrogenase, 853 IU/L; and blood urea nitrogen/creatinine 13.4/0.99 mg/dL. His arterial blood gas analysis on room air revealed pH, 7.447; PaO2, 31.2 mm Hg; PaCO2, 79.0 mm Hg; and HCO3, 21.2 mEq/L with oxygen saturation of 96.6%. A chest X-ray showed diffuse ground glass opacities and reticular nodules on both lung fields (). A chest computed tomography (CT) scan revealed patchy ground glass opacity and septal thickening in the middle and lower zones of both lungs (). A pulmonary function test showed a forced vital capacity (FVC) of 3.85 L (83.6% of predicted value), forced expiratory volume in 1 second (FEV1) of 2.81 L (79.4% of predicted value), FEV1/FVC of 78.36%, and a carbon monoxide diffusing capacity of 50.2%. He underwent a bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBLB). The differential counts in the BAL fluid revealed macrophages, 15%; lymphocytes, 75%; neutrophils, 5%; and eosinophils, 2%. A microbiologic study including the culture of bacteria, fungus with Pneumocystis jirovecii, and Mycobacterium in bronchial specimens was all negative. The polymerase chain reaction assays of mycoplasma pneumonia, nine respiratory viruses such as influenza virus A and B, respiratory syncytial virus, metapneumo virus, rhino virus, parainfluenza virus, adeno virus, corona virus, bocavirus, and cytomegalovirus were all negative. Histological findings for the TBLB at the lingular segment demonstrated fibroblastic plug formations in the alveoli with infiltrations of chronic inflammatory cells and type II pneumocyte proliferation, mild fibrous thickening in the interstitium, which suggested organizing pattern of interstitial pneumonia (). No evidence suggested infectious pneumonia; thus, we strongly suspected drug-induced interstitial pneumonitis caused by imatinib. We stopped the imatinib and started 1 mg/kg prednisolone daily. After 2 weeks of treatment, the dyspnea and abnormal X-ray findings improved, and he was discharged with 30 mg prednisolone (). After 1 month of treatment, he restarted 300 mg imatinib to control the underlying disease concomitant with 20 mg prednisolone. No recurrence of respiratory symptoms was observed on a chest CT after 12 weeks of imatinib rechallenge (). | [[43.0, 'year']] | M | {'7504929': 1, '18057772': 1, '16598305': 1, '14724651': 1, '27121688': 1, '27534751': 1, '30813141': 1, '1864975': 1, '21823828': 1, '11071626': 1, '10992015': 1, '22076388': 1, '24416057': 2} | {} |
165,117 | 3884115-1 | 24,416,058 | noncomm/PMC003xxxxxx/PMC3884115.xml | Invasive Pulmonary Aspergillosis after Influenza A Infection in an Immunocompetent Patient | A 60-year-old man was admitted chiefly with shortness of breath, pleural chest pain. The patient presented with fever, cough, and whitish sputum 10 days prior to consultation. Treatment was received from a local hospital for upper respiratory tract infection, but no improvements were observed. The patient was transferred to our hospital presenting with shortness of breath and pleural chest pain.\nUnderlying disease includes hypertension, and the patient has been taking medication for 20 years.\nHe was a non-smoker and did not have alcohol abuse history.\nAt the time of consultation, the blood pressure was 120/70 mm Hg; heart rate, 102 beats/min; respiratory rate, 24 breaths/min; and body temperature, 38.1℃. The patient was conscious but showed acute ill-looking appearance. Physical examination showed normal conjunctiva with no palpable lymph nodes around the neck. On auscultation of the chest, coarse breathing sounds without wheezing in both lower lung fields were heard. The abdomen was soft, and bowel sounds were normal; no hepatomegaly and splenomegaly were noted. Skin lesions were not observed.\nIn the peripheral blood test, leukocytes were 7,650/mm3 (neutrophils, 79.3%; lymphocytes, 12.3%; and eosinophils, 0.8%); hemoglobin, 14.9 g/dL; platelets, 124,000/mm3; and erythrocyte sedimentation rate, 20 mm/hr (reference range, 0-20 mm/hr). C-reactive protein was 175 mg/dL (reference range, 0-3 mg/dL). In serum biochemistry, blood urea nitrogen/creatinine was 11.9/1.1 mg/dL; total protein/albumin, 6.3/3.6 g/dL; Ca/P, 7.7/2.7 mg/dL; aspartate aminotransferase/alanine aminotransferase, 43/36 IU/L; and total bilirubin, 0.83 mg/dL. For the hepatitis test in autoimmune serology screening, surface antigen of the hepatitis B virus and anti-hepatitis C virus were negative; anti-hepatitis B surface antigen was positive; in the thyroid function test results, T3 was 70.8 ng/dL (reference range, 58-159 ng/dL), whereas thyroid stimulating hormone was 0.701 µIU/mL (reference range, 0.35-4.94 IU/mL) and free T4 was 1.25 ng/dL (reference range, 0.7-1.48 ng/dL); and human immunodeficiency virus and venereal disease research laboratory results were negative. Serum level of IgA, IgG and IgM were within normal range. CD4 count was within normal. Although urinalysis did not show pyuria, microscopic hematuria and proteinuria were observed.\nChest radiography revealed focal consolidation in the right lower lung field, but no cardiomegaly.\nMultifocal ground glass opacities and peribronchial infiltration in both lungs were observed ().\nThe patient presented shortness of breath and pleural chest pain. Chest radiographic findings showed focal consolidation in the lung. Therefore, ceftriaxone+azithromycin were administered since community-acquired pneumonia was suspected. However, fever persisted and hypoxia worsened, and thus, the medication was changed to piperacillin/tazobactam+levofloxacin. However, fever, worsening hypoxia (SpO2, 82.3%; PO2, 43.7 mm Hg), and chest radiographic findings worsened in addition to worsening of the leukocytosis (13,890/mm3). Since community-acquired methicillin-resistant Staphylococcus aureus infection was suspected, vancomycin was administered, and bronchoscopy was performed.\nDiffuse mucosal hyperemia and wide, raised, and cream-colored plaques (pseudomembrane formation) were observed throughout the trachea and both main bronchi, without significant airway obstruction (). Polymerase chain reaction test for influenza A from bronchoalveolar lavage was positive.\nNumerous branching septate hyphae with acute angles less than 45° were observed ().\nThe characteristic septate hyphae of Aspergillus were observed in biopsy. Voriconazole was administered, and the patient was discharged as improvements were observed in the patients fever, hypoxia (SpO2, 97.7%; PO2, 91.0 mm Hg), and leukocytosis (6,270/mm3). However, we were not able to further test for patient's financial reasons. | [[60.0, 'year']] | M | {'23375859': 1, '29858759': 2, '22895826': 1, '15691249': 1, '23406508': 1, '12588484': 1, '25309610': 2, '23236320': 2, '30450275': 2, '17646966': 1, '27704024': 1, '18177225': 1, '19529909': 1, '29454654': 1, '20507748': 1, '33537328': 1, '33084566': 1, '29018774': 1, '32009092': 1, '17205443': 1, '16367817': 1, '24416058': 2} | {'4192313-1': 1, '6096900-1': 1, '3517947-1': 1, '6226581-1': 1} |
165,118 | 3884116-1 | 24,416,059 | noncomm/PMC003xxxxxx/PMC3884116.xml | An Elderly Man with Fatal Respiratory Failure after Eating a Poisonous Mushroom Podostroma cornu-damae | A 73-year-old man presented with nausea, vomiting, diarrhea, dry mouth, and hot flushes 3 hours after eating boiled wild mushrooms. He visited a primary clinic, where his blood pressure was 70/50 mmHg. Laboratory findings showed azotemia and leukocytosis, and he was referred to the emergency department of our hospital. His wife also experienced nausea and vomiting that resolved spontaneously. He had a history of cigarette smoking (35 packs/yr). His medical history was insignificant, and he had not been on any medication (including over-the-counter medication) and was denied contact with any sick persons, use of illicit drugs, exposure to animals, and recent travel outside Seoul.\nOn admission, his blood pressure was 106/51 mm Hg, heart rate was 115 beats/min, respiratory rate was 24/min, and body temperature was 37.1℃. He looked acutely ill but was mentally alert. Laboratory tests revealed the following: white blood cell (WBC) count, 34,670/µL (absolute neutrophil count [ANC], 32,243/µL); hemoglobin (Hb), 17.0 g/dL; platelet count, 322,000/µL; blood urea nitrogen, 47.9 mg/dL; serum creatinine, 1.6 mg/dL (reference, 0.6-1.1 mg/dL); total protein, 6.2 g/dL; albumin, 3.7 g/dL; aspartate aminotransferase (AST), 34 IU/L (reference, 5-40 IU/L); alanine aminotransferase (ALT), 19 IU/L (reference, 5-40 IU/L); lactic dehydrogenase (LDH), 313 IU/L (reference, 145-250 IU/L); creatine kinase (CK), 213 IU/L (reference, 38-160 IU/L); serum Na/K/Cl, 140/4.9/108 mEq/L; total CO2, 17.1 mmol/L; serum glucose, 180 mg/dL; HbA1c, 7.3%; C-reactive protein (CRP), 159 mg/dL (reference, 0.0-8.0 mg/dL); and prothrombin time (PT) international normalized ratio (INR), 1.24. After receiving 2 L/min of oxygen via nasal prongs, blood gas analysis revealed the following: pH, 7.351; PCO2, 30.1 mmHg; PO2, 65.4 mmHg; HCO3, 18.2 mmol/L; and SaO2, 91.8%. Chest radiography showed slight blunting of the left costophrenic angle (). Urinalysis revealed no proteinuria or hematuria. After admission, he received a large amount of fluid (9 L over 24 hours), and his clinical condition improved as vomiting and diarrhea had subsided until day 3 of hospitalization.\nOn day 4, he developed sudden shortness of breath, coughing, and severe respiratory distress. His laboratory examinations were more aggravated as follows: WBC count, 2,840/µL (ANC 2,720/µL); platelet count, 57,000/L; PT INR, 1.16; activated partial thromboplastin time (aPTT), 38.0 seconds; fibrin degradation product >20 µg/mL (reference, 0.0-5.0 µg/mL); plasma D-dimer, 6.56 µg/mL (reference, 0.0-0.5 µg/mL); CRP, 185 mg/L; AST, 82 IU/L; ALT, 50 IU/L; total bilirubin, 0.6 mg/dL; and serum creatinine, 1.6 mg/dL. Chest radiography revealed new widespread consolidations in the whole left lung (). A subsequent computed tomographic (CT)-scan showed extensive consolidations in the left lung and multiple consolidations from the sublobular to subsegmental areas in the right lung, as well as a small amount of pleural effusion in both lungs (). However, the CT scan did not show any intra-abdominal infection foci or abnormal findings to explain the severe pneumonia sepsis. Bronchoscopy showed no evidence of endobronchial lesions. He received mechanical ventilation and continuous renal replacement therapy (CRRT) owing to severe hypoxemic respiratory failure, a PaO2/FiO2<200 mm Hg, and acute oliguric kidney failure. However, he developed refractory shock and his condition rapidly deteriorated. We suspected a possible fulminant course of necrotizing pneumonia and considered surgical intervention and thus consulted the thoracic department. He underwent left pneumonectomy because the gross appearance of the lung inflammation suggested impending pulmonary gangrene. Skin lesions such as miliaria, bullae, and erythroderma were found on the chest wall, face, and upper extremities (not shown). Lung histology revealed varying stages of inflammation with areas of extensive hemorrhagic necrosis (), massive inflammatory cell infiltration and edema in the alveolar space and interstitium (), type II pneumocyte hyperplasia and prominent proliferation of young fibroblasts (), and the formation of a hyaline membrane along the alveolar wall ().\nHe subsequently suffered from rhabdomyolysis, kidney failure, hepatic failure, pancytopenia, disseminated intravascular coagulopathy, and prolonged shock. On day 7, his body temperature was 39.4℃, and his laboratory examinations were more aggravated as follows: WBC count, 120/µL (ANC, 80/µL); platelet count, 32,000/µL; PT INR, 1.50; aPTT, 41.7 seconds; fibrin degradation product>20 µg/mL (reference, 0.0-5.0 µg/mL); plasma D-dimer, 4.95 µg/mL (reference, 0.0-0.5 µg/mL); CRP, 250 mg/L; AST, 122 IU/L; ALT, 54 IU/L; total bilirubin, 5.0 mg/dL; serum creatinine, 2.0 mg/dL; LDH, 500 IU/L; CK, 4,997 IU/L; and serum myoglobin>1,000 ng/mL (reference, 10-92 ng/mL). A peripheral blood smear showed severe pancytopenia mimicking aplastic anemia. Treatment comprising fluid resuscitation with crystalloid and inotropics was performed. Although causative infectious pathogens were not identified from bronchial washing or blood and lung tissues, broad-spectrum antibiotics such as carbapenem, vancomycin, and respiratory quinolone were administrated. Despite the administration of granulocyte colony stimulating factor (GCSF), transfusion of platelet concentrates and packed red blood cells, high doses of inotropics (including norepinephrine, dopamine, and dobutamine), as well as lung protective ventilation strategy and CRRT, he did not recover from multiorgan failure and died on day 13 of hospitalization.\nThe wild mushroom specimen in question was sent to a team of expert mycologists, at the National Academy of Agricultural Science in Korea, were it was determined to be P. cornu-damae (). | [[73.0, 'year']] | M | {'27752639': 2, '6107465': 1, '20535367': 1, '12935559': 1, '23225831': 2, '19882971': 1, '12108023': 1, '9354511': 1, '24416059': 2} | {'5065333-1': 1, '5065333-2': 1, '3521283-1': 1, '3521283-2': 1} |
165,119 | 3884166-1 | 24,403,880 | noncomm/PMC003xxxxxx/PMC3884166.xml | A Retroperitoneal Bronchogenic Cyst Mimicking a Pancreatic or Adrenal Mass | A 42-year-old woman was admitted to the hospital with sharp continuous epigastric pain of 2 months duration with postprandial augmentation. She denied any other gastrointestinal symptoms or weight loss. Her medical and surgical history was insignificant beside psoriasis and smoking. She had neither a history of previous pancreatitis nor hypertension or clinical signs for hypercortisolism, but irregular vaginal bleedings. Physical examination was normal, with a BMI of 25.6 kg/m2. Serum laboratory tests showed signs of inflammation and a CT scan of the abdomen (fig. ) showed a cystic mass of 5 × 3.6 × 4 cm located adjacent to the pancreatic tail and left adrenal gland. She was treated with antibiotics. Endoscopic ultrasonography with fine needle aspiration was performed and showed a polycystic structure with anechoic and hyperechoic portions. Microbiological analysis and analysis for lipase were negative. There were no malignant cells, but carcinoembryonic antigen (CEA) was elevated with 3,777 µg/l (normal <5) in the cyst, but normal in the serum with 4.3 µg/l. Because of the association to the adrenal gland and the history of vaginal bleeding, endocrine evaluation for adrenal hypersecretion was performed. All tests were normal, including renin and aldosterone, plasma and urine catecholamines, metanephrines, estradiol, luteinizing hormone, follicle-stimulating hormone and cortisol.\nWe suspected a cystic adrenal tumor, therefore we performed a diagnostic laparoscopy. The cystic and encapsulated lesion with smooth and clear boundaries was not discriminable from the left adrenal gland. The pancreas, stomach, diaphragm and aorta were adjacent to the cyst, but not involved. The mass was resected laparoscopically with the left adrenal gland. Postoperative recovery was uneventful.\nIn the pathological workup the cyst had a smooth inner lining and contained a grey viscous mass. Microscopically, it consisted of ciliated, respiratory epithelium, seromucous glands and fully developed cartilage, without any malignant or dysplastic cells (fig. ). The adrenal gland was histologically inconspicuous and had no direct contact or connection to the cyst. | [[42.0, 'year']] | F | {'34848933': 1, '15453524': 1, '2297641': 1, '27011575': 1, '22664911': 1, '26137131': 1, '10776016': 1, '34544401': 2, '32964892': 2, '15481326': 1, '16447475': 1, '18468665': 1, '10365854': 1, '25031770': 1, '3933174': 1, '32337317': 1, '10895088': 1, '24403880': 2} | {'8454181-1': 1, '7945650-1': 1} |
165,120 | 3884168-1 | 24,403,898 | noncomm/PMC003xxxxxx/PMC3884168.xml | Intratarsal Keratinous Cyst – An Emerging Entity | A 76-year-old male with a history of a car accident in December 2008, with the subsequent repair of a ZMC fracture, a left paralytic ectropion repair using ear cartilage in March 2009, a left upper eyelid wedge resection for floppy eyelid syndrome in December 2009, left ptosis repair, blepharoplasty and the removal of a glass foreign body from the medial canthus in February 2010 presented with a complaint of left eye pain that had not changed since the car accident. Examination revealed a visual acuity of 20/25 +1 on the right, and pinhole visual acuity of 20/20 on the left. Additionally, he had Meibomian gland dysfunction and a tarsal cyst on the left upper eyelid at the site of the previous wedge resection. A wedge resection of the tarsal cyst was performed. Histologic analysis revealed a keratinous cyst in the tarsus, lined by stratified squamous epithelium featuring crenulations; the superficial layers of the epithelium were eosinophilic. There was keratinous material within the cyst as well as scarring and a chalazion surrounding the cyst (fig. ). There was not sufficient tissue to perform immunohistochemical analysis. | [[76.0, 'year']] | M | {'845755': 1, '22391742': 1, '19875094': 1, '23250332': 1, '18806654': 1, '31686960': 1, '24403898': 2} | {'3884168-2': 2} |
165,121 | 3884168-2 | 24,403,898 | noncomm/PMC003xxxxxx/PMC3884168.xml | Intratarsal Keratinous Cyst – An Emerging Entity | A 57-year-old Caucasian female with a history of bilateral upper and lower eyelid blepharoplasty presented to the clinic with a complaint of a recurrent left upper eyelid lesion that had been excised two years prior. Initially, the lesion had been present for about a year, had developed surrounding erythema, and had burst a few times, producing a white discharge. At that time the excised lesion had been identified as a benign epithelial cyst in the tarsus by pathology. After excision, the patient had relief until three months prior to presentation, when the cyst recurred, causing foreign body sensation in the left eye with blinking. Examination revealed pinhole visual acuity of 20/20 bilaterally. External examination was remarkable for acne rosacea of the upper and lower eyelids and mid face bilaterally as well as bilateral Meibomian gland dysfunction. On eversion of the left upper eyelid, there was a small, white, well-circumscribed, nodular lesion in the tarsus with inflammation of the surrounding conjunctiva (fig. ). The lesion was removed using a full-thickness excision of the conjunctival tarsus, and the defect was covered with an amniotic membrane patch graft.\nHistopathologic examination revealed an intratarsal keratinous cyst lined by stratified squamous epithelium with a smooth luminal aspect, containing cord-like keratin lamellae. The epithelium lacked goblet cells and sebaceous cells; however, there were few clear cells present (fig. ). Immunohistochemistry revealed expression of pancytokeratin (AE1/AE3) in the cyst cuticle and lining (fig. ). The expression of AE1/AE3 was nonspecific and also seen in adjacent Meibomian gland ducts (data not shown). In addition, there was expression of cytokeratin (CK) 903 in the epithelium, cuticle, and contents, and expression of CK 5/6 in the cyst lining and contents, but not cuticle (fig. ; table ). The sloughed keratinous material within the cyst strongly expressed CEA (fig. ), but we did not identify staining with this antibody in the normal Meibomian gland. The cyst was immunonegative for p16, CK 7, CK 18, CK 20, and EMA (table ). The Ki67 expression index was very low. | [[57.0, 'year']] | F | {'845755': 1, '22391742': 1, '19875094': 1, '23250332': 1, '18806654': 1, '31686960': 1, '24403898': 2} | {'3884168-1': 2} |
165,122 | 3884169-1 | 24,403,891 | noncomm/PMC003xxxxxx/PMC3884169.xml | Disseminated Superficial Actinic Porokeratosis on the Face Treated with Imiquimod 5% Cream | A 19-year-old, single, unemployed, Caucasian female (skin type III) presented with multiple facial lesions. She had been suffering from multiple pigmented annular papules and plaques on her face for 7 years. The lesions were slowly progressive with no signs of self-healing. She had been a normal, healthy, full-term baby and had normal physical milestones of development as a child. She appeared to be immunocompetent with no history of recurrent infections or any other skin lesions. Her parents and siblings (3 brothers and 2 sisters) were all normal and healthy. She had no family history of a similar condition or other skin problems. Her medical history was unremarkable with no other obvious complaints, and she was not overexposed to sunlight as she spent most of her time indoors. Upon examination, the central part of the face, i.e. the malar area and nose including the ala nasi, showed scattered annular papules and plaques of different sizes ranging from 0.3 to 2 cm. The lesions were asymptomatic and symmetrically distributed on both sides of the face (figs. , ). Routine laboratory tests were done and the results were within normal ranges. Multiple biopsies were taken from different lesions. The diagnosis was confirmed as DSAP, with a classic and very illustrative pathological picture (figs. –). Treatment options were multiple, though none was considered an obvious first choice. All physical modalities were excluded to avoid the possibility of side effects like scarring and disfigurement, as the lesions were located in the central part of the face and involved the skin overlying the nasal cartilage. We first prescribed a topical retinoic acid 0.1% cream that has keratolytic and antineoplastic effects, and it was assumed that this might rectify the faulty clonal epidermopoiesis. The patient applied the cream twice daily for 3 months without a significant response. Six months later, the patient presented again and the picture was almost unchanged except for a few small new lesions in the malar area and on the nose. We decided to give her imiquimod 5% cream to be used once a day (3 times per week) for 24 weeks. She came for a follow-up every 2 weeks. Her skin started to respond after 1 month. Side effects like erythema, crustations and pruritus were experienced and were controlled by topical emollient cream; we avoided corticosteroids and calcineurin inhibitors because they can counter the effects of imiquimod. On completion of the therapy, the response was very satisfactory for both the patient and the therapist. There were some lesions with only a partial response. Central scarring and the scars across the nose improved in their color, thickness and texture, constituting an added benefit of imiquimod in this indication. The patient maintained a once-weekly application of imiquimod. There has been no relapse after follow-up for 2 years now. | [[19.0, 'year']] | F | {'10844547': 1, '15459975': 1, '23834120': 1, '9854155': 1, '22571584': 1, '21914157': 1, '19059828': 1, '19664847': 1, '20451293': 1, '18025379': 1, '22148029': 2, '20101350': 1, '15928614': 1, '21507038': 1, '4864722': 1, '21182641': 1, '22984659': 1, '24403891': 2} | {'3229955-1': 1} |
165,123 | 3884172-1 | 24,403,902 | noncomm/PMC003xxxxxx/PMC3884172.xml | Restoration of Severely Impaired Eyesight in an Adolescent with Down Syndrome and Bilateral Cataract in South Asia | A 16-year-old South Asian male with DS from Saharsa, India, grew up as the beloved son of a landless laborer and a housewife. Only 3 months before presentation, his parents realized that there might be an impairment of vision contributing to the general cognitive difficulties of the adolescent. Despite the relatively modest road distance of about 130 km, the Sagarmatha Choudhary Eye Hospital in Lahan, Nepal, is hard to reach for the villagers of the region; nevertheless, its reputation in the area is remarkable. The patient's parents knew about the facility and managed to overcome major difficulties (the journey took 16 h by train and bus and stressed the economic means of the parents to their very limits) to bring their son to Lahan for treatment. The adolescent was diagnosed with cataract in both eyes (fulfilling all clinical criteria [] in the absence of the opportunity for genetic testing) and underwent surgery (phacoemulsification and implantation of a posterior chamber lens) in both eyes on 2 consecutive days (February 20 and 21, 2013, respectively). Surgery was successful from a macroscopic point of view (fig. , fig. ), and the patient responded to visual stimuli (e.g. movement of hands) that had eluded him prior to surgery. The adolescent had not been able to contribute to domestic chores at all previous to surgery, but might be able to do so in the future due to the apparent improvement in eyesight. | [[16.0, 'year']] | M | {'17491999': 1, '8694092': 1, '142592': 1, '19343968': 1, '15078680': 1, '16869362': 1, '17910079': 1, '22473965': 1, '23640505': 1, '23764677': 1, '24403902': 2} | {} |
165,124 | 3884175-1 | 24,403,887 | noncomm/PMC003xxxxxx/PMC3884175.xml | Balloon against Jackhammer Disorder | A 37-year-old woman was referred to the hospital in November 2012 for dysphagia. The patient had been experiencing thoracic pain and dysphagia for solids for 6 months and lost 20 kg. She had no prior medical history, was not taking any medication and did not smoke.\nEsogastroduodenal endoscopy with biopsy was normal, excluding eosinophilic esophagitis. Echoendoscopy and thoracic CT scan were also normal, with no muscular thickening and no mediastinal tumor. Esogastroduodenal transit did not show any esophageal dilatation. HRM (Sierra Given) was performed. The catheter was placed transnasally and positioned to obtain recordings from the hypopharynx to the stomach with 36 circumferentially sensitive sensors. The catheter was attached to the nose. The transducers were calibrated. The HRM protocol included at least one 30 s assessment of basal sphincter pressure and ten 5 ml water swallows in a supine position. Esogastric junction pressure was 32 mm Hg (normal <43) and integrated relaxation pressure (IRP) 20 mm Hg (normal <15), showing impaired relaxation after swallowing. The mean distal contractile integral (DCI) was 8,063 mm Hg cm s and the contractile front velocity was 74.3 cm/s (normal <6.3). Intrabolus pressure was high at 10 mm Hg (normal <8.4), suggesting esophageal stasis.\nBased on these results, pneumatic dilatation was performed with a Rigiflex balloon (Boston Scientific) positioned at the esogastric junction. The balloon was inflated to 30 mm 5 PSI for 1 min and 8 PSI for 1 min. Three months after endoscopy, the patient described clinical improvement with only two episodes of dysphagia, no chest pain, no regurgitation and weigh gain. HRM showed an esogastric junction pressure of 25 mm Hg (normal <43), IRP 12 mm Hg (normal <15), mean DCI 2,514 mm Hg cm s and normal peristalsis. The patient had no symptoms at 6 months. | [[37.0, 'year']] | F | {'22248109': 1, '22609465': 1, '23105996': 1, '10435697': 1, '22248105': 1, '23277105': 1, '29290915': 2, '22529685': 1, '21561346': 1, '23426591': 1, '21931377': 1, '24403887': 2} | {'5740105-1': 1} |
165,125 | 3884177-1 | 24,403,893 | noncomm/PMC003xxxxxx/PMC3884177.xml | Facial Pyoderma Gangrenosum in Senescence | A 90-year-old woman was referred to our department with a 3-week history of an ulcer on the lateral corner of the left eye. The painless wound appeared spontaneously, without any apparent trauma, and it had gradually enlarged. Concerning comorbidities, the patient suffered from hypertension and rheumatoid arthritis. The latter was treated with methotrexate 10 mg/week and prednisolone 2 mg/day. Initial physical examination in our outpatient department showed a deep ulcer on the lateral corner of the left eye, 1.5 × 1.5 cm in size, with a necrotic and fibrinoid surface (fig. ). The left eyelids were erythematously swollen and showed accompanying conjunctivitis. Histology from a skin biopsy taken from the edge of the ulcer showed a dense, mixed-cell, dermal infiltrate with numerous neutrophilic granulocytes and some macrophages (fig. ). Periodic acid-Schiff and Giemsa stains failed to indicate any infectious disease with bacteria or fungi. The laboratory investigation of the peripheral blood revealed normocytic anemia, lymphocytopenia and neutrophilia. C-reactive protein was increased with 21.08 mg/l (normal value < 5 mg/l) and antinuclear antibodies were negative. Bacteriological examination of an ulcer smear revealed growth of physiologic skin flora only. To sum up, the lesion was interpreted as PG. We started systemic therapy with high-dose prednisolone (75 mg/day), gradually tapered after 7 days. For topical treatment, rinsing with a physiological salt solution and ofloxacine eyedrops (5 times/day) were recommended after the ophthalmologic consultation. Within 2 weeks, the ulcer was reduced in size, showed a clean base without necrotic debris, and the border was less inflamed (fig. ). | [[90.0, 'year']] | F | {'20227565': 1, '34522619': 1, '31320974': 1, '12399665': 1, '31312710': 1, '15373292': 1, '23627664': 1, '8286258': 1, '15533059': 1, '18325095': 1, '21824126': 1, '14531875': 1, '21242400': 1, '11306846': 1, '23308303': 1, '24403893': 2} | {'3884177-2': 2} |
165,126 | 3884177-2 | 24,403,893 | noncomm/PMC003xxxxxx/PMC3884177.xml | Facial Pyoderma Gangrenosum in Senescence | An 89-year-old man was admitted to our department with a post-surgery wound dehiscence on the left part of the lower lip after squamous cell carcinoma surgery 13 days ago (tumor thickness 6.5 mm, depth of invasion level V). A wound revision was performed with suturing of the lesion by a three-layer wound closure and an antibiotic therapy with cefuroxim 500 mg twice daily for 8 days. In the following 6 weeks, the patient was re-admitted to our department several times because he suffered from recurrent wound dehiscence. The patient reported that the dehiscence was triggered by mechanical irritation, such as food intake or sneezing. He described a progressive pain in the suture area. Physical examination showed an ulcer with violaceous undermined borders on the left part of the lower lip (fig. ). After each admittance, he was treated with an extensive excision of fibrinoid debris and necrotic tissue, following an antiseptic lavage with betaisodona®. A synthetic wound dressing (Syspur Derm®, Paul Hartmann AG, Germany) was inserted. Bacterial swab examinations were sterile. Furthermore, incisional biopsy from the edge of the wound was performed. Histopathological examination showed a dense neutrophilic dermatitis without any evidence of residual neoplastic disease. Based on clinical and histopathological correlation, PG was suspected. To exclude the possibility of an allergic reaction to suture material, we performed an intracutaneous insertion of 7 different suture stitches (Vicryl rapide, PDS, Ethilon, Vicryl, Monocryl, Mersilene and Prolene, Johnson & Johnson MEDICAL GmbH, Germany) on the patient's upper right arm. After 48 h at all 7 testing sites, erythematous papules up to 3 mm in diameter were visible (fig. ). Histopathological examination of a biopsy specimen from the upper right arm showed a superficial mixed cell infiltrate of neutrophilic, but also some eosinophilic granulocytes. These findings were consistent with the pathergy phenomenon of PG. The patient's past medical history revealed no PG-associated comorbidities. Detailed examination failed to detect any systemic complications including inflammatory bowel diseases, hematological disorders or rheumatoid arthritis. Laboratory examination of peripheral blood was unsuspicious, including a negative serology for syphilis. Topical treatment with a triamcinolone acetonide-containing ointment significantly improved healing, and the patient suffered no relapses afterwards (fig. ). | [[89.0, 'year']] | M | {'20227565': 1, '34522619': 1, '31320974': 1, '12399665': 1, '31312710': 1, '15373292': 1, '23627664': 1, '8286258': 1, '15533059': 1, '18325095': 1, '21824126': 1, '14531875': 1, '21242400': 1, '11306846': 1, '23308303': 1, '24403893': 2} | {'3884177-1': 2} |
165,127 | 3884178-1 | 24,403,882 | noncomm/PMC003xxxxxx/PMC3884178.xml | Ameboma: A Colon Carcinoma-Like Lesion in a Colonoscopy Finding | The 45-year-old male patient denied any prior systemic disease. He had been buying and selling merchandise in Indonesia for 18 years. According to the patient's statement, he had suffered from intermittent bloody stool for nearly 1 month. He also denied any history of hemorrhoids and had not suffered from the same episode before. The increased bloody stool amount was first noted 4 days previously (on February 14) and subsequently about 5–6 times per day with a volume of 20–30 ml/time. He had also been suffering from abdominal pain over the periumbilical area, especially on the right site, for about 1 month. Intermittent fever was also during that week. He had eaten raw food with sashimi and uncooked eggs about 1 month previously. According to his statement, the locals clean the dishes with groundwater.\nDue to the above problem, he was taken to our emergency room. Colonoscopy was also done at the emergency room and showed multiple small polyps over the terminal ileum and multiple indurated ulcerations over the rectum and cecum (fig. ). A mild elevation in C-reactive protein and erythrocyte sedimentation rate were noted. Normocytic anemia was also noted and suspected to be related to the bloody stool. Bedside abdominal echography showed no obvious liver abscess. Pathologic examination was also performed and showed chronic inflammatory infiltrate of lymphoplasma cells, eosinophils and neutrophils in the edematous fibrous lamina propria. Focal aggregations of amoeba trophozoites were demonstrated by periodic acid-Schiff stain (fig. ). Ameboma was diagnosed and metronidazole was prescribed for invasive dysentery for 14 days. Additionally, iodoquinol was also prescribed for luminal infection and to prevent colonization. After 3 days of treatment the symptoms, including bloody stool and fever, had resolved. Follow-up colonoscopy showed much improvement 4 months post treatment and complete remission after 8 months (fig. ). | [[45.0, 'year']] | M | {'27688666': 2, '27882478': 1, '2332466': 1, '29552535': 2, '12397207': 1, '26666636': 2, '8863036': 1, '1520807': 1, '2891197': 1, '340132': 1, '590600': 1, '2871619': 1, '12700377': 1, '26841049': 1, '30333533': 1, '24403882': 2} | {'4678475-1': 1, '4678475-2': 1, '4678475-3': 1, '4678475-4': 1, '4678475-5': 1, '4678475-6': 1, '4678475-7': 1, '4678475-8': 1, '4678475-9': 1, '5846219-1': 1, '5037093-1': 1} |
165,128 | 3884180-1 | 24,403,890 | noncomm/PMC003xxxxxx/PMC3884180.xml | Fatal Metastatic Cutaneous Squamous Cell Carcinoma Evolving from a Localized Verrucous Epidermal Nevus | A 50-year-old Asian male presented with a partially pigmented 2-year-old lesion on the upper left side of his back. The lesion measured 3 cm in diameter, was round and had an irregular contour and surface. It had a verrucous pigmented rim on its medial side that measured 6–8 mm, while the prominent exophytic erythematous part measured 2.1 × 2.2 cm (fig. ). The exophytic part was almost round with an irregular surface. It was friable, bled easily, was though but not indurated and it was not attached to the underlying structures. The trunk also showed few post-pubertal lentigines, but no other nevi or seborrheic keratosis. The patient had no other co-morbidities and he was not immune-compromised. He had no personal or family history of skin or any other cancer. The lymph nodes were not enlarged at that time. An immediate total excision biopsy was done. The sutures were removed after 1 week with minimal scarring.\nThe pathological examination confirmed an epidermal nevus at the periphery of the lesion (fig. ). The epidermis showed bowenoid features with irregularly sized nuclei, a loss of polarity, dyskeratosis and mitotic figures (fig. ). Some sections from the periphery of the specimen showed multiple intraepidermal eddies of atypical cells or the Borst-Jadassohn phenomenon (fig. ). Sheets of poorly differentiated, atypical, pleomorphic keratinocytes migrated from the epidermis into the whole dermis, hair follicles (fig. ) and eccrine glands, including a perivascular and perineural invasion (fig. ). The tumour exhibited a maximal depth of 7 mm (fig. ).\nAtypical cells showed positive staining with cytokeratin markers and other squamous cell carcinoma markers, but they were negative for MART1 and S100. Focal, mild and sparse, subepidermal, lymphocytic infiltrates were found on some sections but were absent in many areas of the lesion. Thorough laboratory and radiological investigations were carried out. The results were: only mildly increased liver enzymes, an elevated C-reactive protein level and a mild impairment in the pulmonary function tests. The diagnosis was CSCC, stage T4N0M0.\nThe patient was immediately referred to the surgical department for the removal of sentinel lymph nodes. However, the surgeon decided not to excise any lymph nodes as long as the tumour could be totally removed in the absence of enlarged adjacent lymph nodes, demonstrated by clinical and ultrasound examination. The initial bone scan was negative for metastasis.\nSix weeks later (8 weeks after his first visit), the patient had a follow-up. Unfortunately, the regional lymph nodes were palpable, while the scar of the primary lesion and the surrounding area remained clear. The swelling was so extensive that it also infiltrated the surrounding subcutaneous tissue, measuring 12 × 17 cm (fig. ). An excision biopsy was taken from the lymph nodes and the surrounding subcutaneous tissues confirming the diagnosis of metastatic CSCC. The patient was again operated on for a wide removal of the axillary lymph nodes and adjacent tissues. After this surgical excision, the patient's general condition started to deteriorate. He was transferred to the Al Amal Cancer Hospital for chemotherapeutic and/or radiotherapeutic options; however; he was not eligible for either option and plans for intervention were postponed. The patient's general condition continued to decline with additional refractory anaemia, lymphocytopenia and hypoalbuminaemia.\nAfter another 4 weeks, a bone scan, CT and MRI images showed metastasis in both lungs (fig. ) and then 3 weeks later in the spinal cord (fig. ) and brain (fig. ), while the liver was spared. The patient received only palliative care during his entire stay, mainly to treat his anaemia and to control the pain. Eventually, he went into coma and then passed away. | [[50.0, 'year']] | M | {'5416794': 1, '21771334': 1, '20549904': 1, '7174995': 1, '649251': 1, '659700': 1, '28300931': 2, '6703257': 1, '15186052': 1, '23488626': 1, '22312497': 1, '22342554': 1, '18514604': 1, '8772026': 1, '23375456': 1, '15953076': 1, '17998704': 1, '16028780': 1, '8884151': 1, '25848348': 2, '23259077': 2, '22481931': 1, '16503894': 1, '22838090': 1, '7212744': 1, '25657916': 2, '19076196': 1, '24403890': 2} | {'4314886-1': 1, '4357678-1': 1, '3504205-1': 1, '5325030-1': 1} |
165,129 | 3884181-1 | 24,403,892 | noncomm/PMC003xxxxxx/PMC3884181.xml | Nodal Melanoma Metastasis under Infliximab Therapy in a Patient with Nevoid Melanoma First Misdiagnosed as Benign Nevus: A Potentially Dangerous Diagnostic Pitfall in the Era of Biologic Therapies | A 53-year-old Caucasian woman presented with a 40-year history of severe plaque psoriasis. The patient had recalcitrant psoriasis that was unresponsive to multiple conventional therapies such as methotrexate and acitretin. The patient also had a 20-year history (>800 treatments) of psoralen plus ultraviolet A (PUVA) therapy. Her past medical history was notable for a benign compound nevus on the left anterior thigh, which was completely excised in January 2011. In January 2012, the patient was screened (including physical examination, baseline laboratory studies, and screening for latent tuberculosis and chronic viral infections) and began etanercept therapy (50 mg subcutaneously twice weekly). After 3 months, the patient was responding insufficiently to etanercept and was switched to infliximab (5 mg/kg at weeks 0, 2 and 6, and then every 8 weeks thereafter). This treatment resulted in the complete disappearance of her psoriasis. Twelve months after beginning the infliximab treatment, the patient developed palpable lymphadenopathy in the left inguinal region. Given the suspect nature of the palpable mass, the patient underwent an inguinal lymph node biopsy. The histology of the biopsy specimen showed a metastatic melanoma. Positron emission and computed tomography scans were negative for distant metastatic disease. Infliximab was discontinued and a complete inguinal lymph node dissection was performed, which revealed no additional nodal involvement. A full skin evaluation did not reveal any suspicious lesions, but 4 cm below the metastatic lymph node, the scar from the previously removed nevus could be seen (fig. ). The histopathologic slides of the excised nevus were reviewed along with immunohistochemical studies, which led to the definite diagnosis of nevoid melanoma (Breslow thickness 1.44 mm; fig. ). A BRAF V600E mutation was detected and the patient was enrolled in a clinical trial of combined adjuvant therapy with dabrafenib and trametinib. | [[53.0, 'year']] | F | {'18512713': 1, '22928479': 1, '17434043': 1, '21549238': 1, '25857817': 1, '10843728': 1, '9091799': 1, '19344980': 1, '23568792': 1, '20055908': 1, '32791572': 1, '16176296': 1, '24403892': 2} | {} |
165,130 | 3884183-1 | 24,403,901 | noncomm/PMC003xxxxxx/PMC3884183.xml | Negative g-Force Ocular Trauma Caused by a Rapidly Spinning Carousel | A healthy, 10-year-old boy was examined in our institution due to bilateral diffuse subconjunctival hemorrhage. He had mounted a carousel that was attached to an electrical scooter, and had spun round on it very rapidly. The boy had no control of the carousel, but fortunately an adult managed to disconnect the scooter. The rapid spinning lasted for about 1 min, during which the boy suffered an intense headache, but did not lose consciousness. After dismounting the carousel, the boy complained of a headache, his face was swollen, both eyes were red and he was brought to the emergency room in our institution.\nOn examination, visual acuity was 20/20 OU. Bilateral subconjunctival hemorrhages were noted in both eyes (fig. ). The remainder of the examination was normal, with clear corneas and lenses, no intraocular hemorrhage of any kind and normal dilated fundus examination. Intraocular pressure was 12 mm Hg in both eyes, and pupillary responses were normal. There was no limitation in ocular motility. Additional findings of the physical examination included a mild swelling and periocular petechiae (fig. ) as well as petechiae in both ear canals and on the soft palate.\nHead CT was normal, with no evidence for intracranial or orbital hemorrhage. Optical coherence tomography showed a normal retinal structure (fig. ). The subconjunctival hemorrhages enlarged initially, but were then gradually resorbed and had disappeared completely within 1 month (fig. ). Visual acuity remained 20/20 throughout the follow-up. | [[10.0, 'year']] | M | {'16693583': 1, '20584025': 1, '11024431': 1, '19443716': 1, '20435592': 1, '7819181': 1, '24403901': 2} | {} |
165,131 | 3884184-1 | 24,403,885 | noncomm/PMC003xxxxxx/PMC3884184.xml | Cauda Equina Syndrome Secondary to Complicated Diverticulitis | A 58-year-old woman with no past medical history presented to the emergency department with progressive lumbar back pain radiating to her left leg. The pain radiated throughout the entire left leg. She also experienced a sensation of numbness in her lower left leg and foot. These symptoms had been progressing for the past 3 weeks. She had developed a gait disorder 5 days prior to presentation, which she assigned to a lack of coordination in her left foot. She also complained of urine and bowel incontinence. There was no abdominal pain. She only mentioned having had a fever 1 day prior to these complaints. During the preceding months she had lost 11 kg of body weight.\nOn physical examination the patient appeared ill. Vital signs showed a septic profile with a body temperature of 38.8°C. Neurological examination of the lower limbs revealed decreased strength in her left leg, with a strength of Medical Research Council (MRC) 3/5 in the iliopsoas, MRC 4/5 in the tibialis anterior, and MRC 4+/5 in the gastrocnemius. Strength in the right leg was entirely normal. There was normal sensation in the saddle area and the perianal area, as well as a normal anal sphincter tone. The straight leg test showed a positive Lasègue sign on the left side. Abdominal examination showed a soft, non-tender abdomen.\nLaboratory tests showed leukocytosis and an elevated C-reactive protein level (table ). Initially, urinalysis could not be performed because the patient was unable to urinate. On catheterization, however, she had a urine retention of 1,300 ml. A chest and lumbar spine X-ray were taken, which both showed no abnormalities.\nThe patient's septic condition worsened in the emergency department with a high-grade fever (40.9°C). Antibiotic therapy was started with Amoxicillin/Clavulanate and the patient was transferred to our center's intensive care unit.\nMRI of the spinal canal showed a fistula from the sigmoid colon to a presacrally localized abscess, most likely secondary to diverticulitis (fig. ). The abscess had expanded into the spinal canal, the paravertebral soft tissue on the left side, and the erector spinae muscles on both sides. Also, there were signs of sacral osteomyelitis and diffuse meningeal staining of the conus medullaris and the cauda equina. The presence of an epidural abscess could not be confirmed (fig. ). An additional chest/abdominal CT scan showed pleural fluid on both sides. There were multiple fluid and air collections located presacrally and retroperitoneally as well as along the psoas muscle. There was air in the spinal canal and the back muscles. There were no signs of spondylodiscitis (fig. ).\nConsequently, Hartmann's procedure was performed as treatment for the diverticulitis. Multiple intra-abdominal abscesses were drained peroperatively. Two intra-abdominal drains were placed to maintain postoperative drainage of the abscess pockets. Postoperative management consisted of broad-spectrum antibiotics and therapeutic heparin for a thrombus in the left iliac vein. On postoperative day one the patient was extubated, and on postoperative day four she was transferred to the gastrointestinal surgery ward. She remained hemodynamically normal and her stoma production started. The neurological symptoms did not resolve completely during her stay. Eventually, the patient was returned to her primary care center 2 weeks after admission to our hospital. There she was enrolled in an intensive rehabilitation program for her persisting neurological symptoms. | [[58.0, 'year']] | F | {'15685694': 1, '14987890': 1, '19212172': 1, '3402284': 1, '1085269': 1, '18003962': 1, '18479497': 1, '15784040': 1, '17024321': 1, '16741596': 1, '24403885': 2} | {} |
165,132 | 3884185-1 | 24,403,897 | noncomm/PMC003xxxxxx/PMC3884185.xml | A Case of Severe Ganciclovir-Induced Encephalopathy | A 55-year-old man started to receive hemodialysis because of diabetic renal failure 2 years previously. He underwent renal transplantation 1.5 years previously, and had been receiving immunosuppressants since then. Eight months after transplantation, the serum creatinine level increased to 4.4 mg/dl. He had CMV enteritis with occult blood in the stool and an elevated CMV pp65 (C7-HRP) antigen level in blood mononuclear cells. Intravenous ganciclovir (150 mg/day) was administered for 11 days, followed by valganciclovir (450 mg/day). Because the enteritis was very severe, ganciclovir and valganciclovir were not reduced to maintenance doses, which are generally half of starting doses. Two days after starting valganciclovir, he had unsteady gait, but could walk unaided. On the next day, the patient needed assistance with walking. His consciousness was mildly disturbed (E3, V5, and M6 on the Glasgow Coma Scale). One day later, he became delirious intermittently. Two days later, he was found on the floor after falling, without major injuries. He was suspected to be irritated and exhausted because of severe enteritis. Nine days after starting valganciclovir, his level of consciousness worsened (E3, V3, and M5), and he could not receive oral drugs, including valganciclovir. Neurologists were consulted. Encephalitis was unlikely, since no meningeal signs or fever was noted; the cell count was normal (0.33 cells/μl) in the CSF, and the protein concentration marginally elevated (54 mg/dl). CMV, herpes simplex virus, varicella-zoster virus, and Epstein-Barr virus DNA was later found to be negative in the CSF. Ganciclovir-induced encephalopathy was suspected, and the drug was withdrawn. Because of the risk of further falls, hemodialysis using a VPS-15HA membrane, a vitamin E-coated polysulfone membrane (Asahi Kasei Kuraray Medical, Japan) was performed twice in 2 days. His consciousness improved considerably after the first session of dialysis (E3, V4, and M6) and was completely restored on the next morning after the second session (E4, V5, and M6). The trough levels of ganciclovir in the serum and CSF were retrospectively measured and are shown in table . | [[55.0, 'year']] | M | {'2156162': 1, '3017630': 1, '32093125': 1, '28579869': 1, '26008808': 1, '29955472': 1, '25888518': 2, '7834927': 1, '30602515': 1, '16368917': 1, '24403897': 2} | {'4389576-1': 1} |
165,133 | 3884186-1 | 24,403,879 | noncomm/PMC003xxxxxx/PMC3884186.xml | A Rare Adrenal Incidentaloma: Adrenal Schwannoma | A 32-year-old female presented with new onset of intermittent right flank pain. During examination, a left adrenal mass was found incidentally on abdominal ultrasonography and she was referred to our institution for additional evaluation. Her medical and family histories were unremarkable. On physical examination the abdomen was soft, blood pressure was 130/85 mm Hg and pulse rate was 72 beats/min; sinus rhythm was normal. Blood count and biochemistry analysis were within normal limits.\nA heterogeneously enhancing soft tissue mass on the medial aspect of the left adrenal gland measuring 65 × 95 mm was visualized and no additional mesenteric or retroperitoneal lymphadenopathy was identified on the abdominal CT scans. As the mass was approximately 10 cm in size, [18F] fluorodeoxyglucose (18FDG) PET-CT was also performed. It showed intense uptake within the left adrenal nodule with a standardized uptake value (SUVmax) of 9.5, indicative of malignancy (fig. ). Endocrinological examinations including serum electrolytes, cortisol, urinary metanephrine, normetanephrine, adrenalin, noradrenalin and vanillyl mandelic acid, as well as plasma renin and aldosterone were within normal range. Also, a dexamethasone suppression test showed values within the normal range at onset and at 30 min. Due to the large size of the tumor, standard transperitoneal adrenalectomy was performed without complications. Postoperative pathological evaluation revealed schwannoma in the adrenal gland, a hard, well-circumscribed, encapsulated mass 4 × 8 × 10 cm in overall dimensions that was grossly compressing the adrenal parenchyma. Histologically, the tumor consisted of spindle cells with alternating areas of compact hypercellularity with irregular streams and without atypia or mitosis. Immunohistochemical analysis demonstrated rare Ki67-positive cells (<5%) while SMA, desmin, CD34 and CD117 staining was negative in tumor cells. In contrast to these results, S-100 and vimentin staining were diffusely positive across the tumor. Thus, the evidences corresponded to a benign adrenal schwannoma type Antoni A (fig. , fig. , fig. , fig. ). The patient was discharged uneventfully on the sixth day after surgery. | [[32.0, 'year']] | F | {'10871185': 1, '17950341': 1, '949140': 1, '21471169': 1, '16699318': 1, '19275863': 1, '9129444': 1, '15191348': 1, '25918605': 2, '10397094': 1, '32844073': 1, '7997539': 1, '11752075': 1, '12890971': 1, '17651570': 1, '12709623': 1, '19366556': 1, '5796779': 1, '32871401': 1, '7887865': 1, '19251536': 1, '12215852': 1, '32082650': 2, '31297325': 1, '25925356': 1, '17634884': 1, '13655396': 1, '12189753': 1, '15487453': 1, '12614096': 1, '30391738': 2, '24403879': 2} | {'4387351-1': 1, '6216048-1': 1, '7019206-1': 1} |
165,134 | 3884189-1 | 24,403,900 | noncomm/PMC003xxxxxx/PMC3884189.xml | A Case of Acute Posterior Multifocal Placoid Pigment Epitheliopathy Demonstrating Vogt-Koyanagi-Harada Disease-Like Optical Coherence Tomography Findings in the Acute Stage | A 33-year-old man presented with loss of vision in his right eye, which had developed the day before. Best-corrected visual acuity (BCVA) measured using the Landolt ring chart was 0.03 and 1.5 in the right and left eye, respectively (fig. , day −3; 3 days prior to steroid pulse therapy). No remarkable findings or inflammation were observed in the anterior segment of either eye. Fundoscopy revealed a yellow-white inflammatory subretinal lesion in the posterior pole of the right eye. Spectral-domain optical coherence tomography (SD-OCT) showed the presence of subretinal fluid with a subretinal septum. No fundoscopy or SD-OCT was performed in the left eye at the first hospital visit. The next day, he presented with rapid loss of vision in his left eye, and BCVA in his left eye had decreased from 1.5 to 0.1 (fig. , day −2). Fundoscopy revealed a yellow-white lesion in the posterior pole of his left eye, which was similar to the lesion in his right eye (fig. ). SD-OCT images of both eyes showed serous retinal detachment with a subretinal septum and an intraretinal compartmentalized cystic space (fig. ). In his left eye, pigment epithelial detachment (PED) was observed on OCT (fig. ). The lesions in both eyes were limited to the macular area. Blood examination showed no remarkable abnormalities. Antibodies for Toxoplasma gondii were negative. Furthermore, the rapid plasma reagin test and fluorescent treponemal antibody absorption test were negative. The patient did not complain of abnormal skin sensations, headaches, hearing problems, or prodromal symptoms suggestive of viral infection. Apparent hypofluorescence was unremarkable in the early phase of FA (fig. ). Fluorescein dye in both eyes became gradually enhanced in the late phase of FA (not shown).\nSteroid pulse therapy comprising methylprednisolone (1,000 mg/day) was intravenously administered for 3 days, followed by oral prednisolone for 17 weeks (tapering from 50 mg/day for 4 days, 40 mg/day for 4 days, 30 mg/day for 4 days, 20 mg/day for 20 days, 15 mg/day for 28 days, 10 mg/day for 48 days, and 5 mg/day for 14 days). After initiation of systemic steroid therapy, SD-OCT revealed that the amount of subretinal fluid decreased immediately, but the inner segment/outer segment (IS/OS) line remained disorganized (fig. ). Recovery of visual acuity was delayed compared to the rapid decrease in subretinal fluid. Goldmann perimetry performed 1 week after initiation of systemic steroid therapy revealed that a central relative scotoma persisted in both eyes (data not shown). Fifteen days after initiation of therapy, BCVA was 0.09 in both eyes (fig. ). In addition, OCT showed that the IS/OS line remained disorganized, the retina appeared to be thin, and the outer nuclear layer seemed to be poorly organized (fig. ). Until day 33 of therapy, BCVA was <0.1 in both eyes, and it changed to 0.1 in both eyes on day 34 after therapy initiation (fig. ). The response to therapy was slow and very different from that typically observed in VKH disease. We made the diagnosis of APMPPE on the basis of the rapid loss of vision bilaterally and the classic manifestation of yellow-white lesions in the fundus []. Eventually, vision began to recover and BCVA improved to 0.4 in the right eye and 0.5 in the left eye (fig. , day 48). At this time point, OCT revealed the partial recovery of the IS/OS line in the macular area of both eyes (fig. ). On day 111 of therapy initiation, BCVA improved to 0.7 in the right eye and 0.6 in the left eye (fig. ), and OCT revealed clear reorganization of the IS/OS line (fig. ). One year after therapy initiation, BCVA remained 0.7 in the right eye and 1.0 in the left eye. However, unfortunately, we were unable to perform the OCT and examine the fundus. | [[33.0, 'year']] | M | {'31097949': 2, '27373117': 1, '24843310': 1, '27652046': 2, '16340549': 1, '27160929': 1, '19491729': 1, '17073914': 1, '27020453': 1, '19996817': 1, '20457992': 1, '31380133': 2, '15300099': 1, '21034311': 1, '5661882': 1, '21250924': 1, '24403900': 2} | {'6489078-1': 1, '6652076-1': 1, '5009056-1': 1} |
165,135 | 3884191-1 | 24,403,886 | noncomm/PMC003xxxxxx/PMC3884191.xml | Anastomotic Recurrence of Sigmoid Colon Cancer over Five Years after Surgery | A 65-year-old female had a history of several cancers, including left breast cancer at 36 years of age, cecum cancer in her forties and right breast cancer at 64 years of age. Her mother, younger sister and daughter also each had a history of breast cancer. At 60 years of age, the patient underwent sigmoidectomy and right colectomy with D3 lymph node dissection for both sigmoid colon cancer (fig. ) and transverse colon cancer. During that operation, lavage of the remnant colon was performed before anastomosis, and automatic anastomotic devices were used. The pathological diagnoses revealed both sigmoid colon cancer and transverse colon cancer of T3N0M0, stage IIA. Both the oral and anal margins of the resected colon had a sufficient length: the transverse margin measured 135 mm (oral wedge) and 50 mm (anal wedge), while the sigmoid margin measured 40 mm (oral wedge) and 50 mm (anal wedge) (fig. ). At 3-year follow-up, colonoscopy and computed tomography revealed no signs of recurrence (fig. ). At 5-year follow-up, colonoscopy demonstrated surrounding flaring and swelling in the anastomotic area of the sigmoid colon (fig. ). A biopsy revealed an adenocarcinoma. The levels of the tumor markers CEA and CA19-9 were both within the normal limits at that time. There were no signs of recurrence in other locations, such as the liver and lungs, or other areas of the remnant colon. Therefore, under a diagnosis of anastomotic recurrence over 5 years after surgery, we performed lower anterior resection. The tumor measured 45 × 35 mm and was located at the previous anastomotic site (fig. ). Pathology revealed histological features that resembled the pattern of the previous tumor, and there were masses of cancerous cells in the subserosal and muscular layers near the fissured muscular layer, which comprised the anastomotic area (fig. ). In addition, in the mucous membranes surrounding the anastomotic area, there was hyperplasia of cancerous cells that pushed up layers from deeper mucous membranes. Furthermore, there were no cancerous cells in the mucous membranes, which indicated that the cells were derived from the primary lesion (fig. ). Based on these observations, the final diagnosis was anastomotic recurrence of sigmoid colon cancer after surgery. No lymph node metastasis was observed among the 18 dissected lymph nodes. There have been no signs of recurrence for the 2 years since the last surgery. | [[65.0, 'year']] | F | {'11934773': 1, '21152938': 1, '7774474': 1, '30187147': 2, '9749496': 1, '33076984': 2, '27577701': 1, '14529575': 1, '9171758': 1, '7575117': 1, '9428212': 1, '12099653': 1, '24403886': 2} | {'6125262-1': 1, '7574449-1': 1} |
165,136 | 3884193-1 | 24,403,899 | noncomm/PMC003xxxxxx/PMC3884193.xml | Retinal Pigment Epithelium Tear after Vitrectomy for Vitreomacular Traction Syndrome in an Eye with Retinal Angiomatous Proliferation | An 87-year-old Japanese man complained of a gradually progressive vision decrease in his right eye. He reported that he was being treated for mild hypertension. His ocular history revealed that he had untreated neovascular AMD in his left eye and open-angle glaucoma in both eyes. His best-corrected visual acuity was 0.15 in both eyes, and the intraocular pressure was 14 mm Hg in both eyes with latanoprost eye drops. Slit-lamp examination showed no abnormalities except for moderate cataracts in both eyes.\nOphthalmoscopy showed a shallow RPE detachment and macular edema accompanied by multiple drusen in his right eye (fig. ). His left eye had advanced AMD with a subretinal scar and severe macular edema. Optical coherence tomography (OCT) showed an RPE detachment, intraretinal hyperreflectivity and edema (fig. ). Vitreoretinal traction was also evident (fig. ). Fluorescein angiography showed a pooling of dye corresponding to the RPE detachment (fig. ). A hyperfluorescent spot suggesting a retinal choroidal anastomosis was observed by indocyanine green angiography (fig. ). He was diagnosed with RAP in the right eye.\nAfter 3 monthly injections of intravitreal ranibizumab, the RPE detachment was resolved (fig. ), and his visual acuity slightly improved to 0.2. Macular traction still remained, and we believed that vitreomacular traction limited the visual recovery.\nAfter discussing the rationale for vitreous surgery to remove vitreous traction and possible complications, the patient agreed to undergo surgery. Pars plana vitrectomy was performed using a 23-gauge system with cataract extraction and implantation of an intraocular lens. The posterior vitreous was separated from the retina using triamcinolone acetonide to make the vitreous more visible. We noted that the posterior hyaloid was firmly attached to the macular area of the retina, and it was carefully separated by aspiration with the vitreous cutter. The internal limiting membrane was not removed during surgery. No intraoperative complications were noted.\nOne month after the surgery, his visual acuity was 0.1 with normal foveal depression and no intraretinal fluid. Two months after the surgery, a large tear of the RPE was found over the entire macula (fig. ). The detached RPE contracted toward the angiomatous lesion. Although the patient did not notice any decrease in his vision, there was significant recurrence of the sub- and intraretinal fluid. Visual acuity in the right eye decreased slightly to 0.06 and then remained unchanged for more than 1 year. | [[87.0, 'year']] | M | {'18537930': 1, '2470124': 1, '16929069': 1, '20678805': 1, '16854830': 1, '18301024': 1, '21270433': 1, '18538742': 1, '22894573': 1, '21095010': 1, '10990052': 1, '11642370': 1, '24403899': 2} | {} |
165,137 | 3884194-1 | 24,403,905 | noncomm/PMC003xxxxxx/PMC3884194.xml | Postpartum Four-Vessel Cervical Artery Dissection | A 39-year-old woman had been followed by her obstetrician during her first pregnancy without any complication until the 34th week of gestation when she complained of unusual headaches and epigastric pain. Her blood pressure was found to be 190/110 mm Hg with positive proteinuria findings consistent with a preeclampsia syndrome. Blood samples showed thrombopenia (80,000/mm3) and mild elevation of liver function tests (serum glutamic oxaloacetic transaminase and serum glutamic pyruvic transaminase were 100 IU/mm3). These abnormalities were suggestive of the HELLP syndrome. An emergency caesarean section was performed. The infant's initial Apgar score was 2 and the newborn baby was immediately transferred to the Paediatric Reanimation Department with good achievement a few days later.\nThe mother's blood pressure was controlled with intravenous polytherapy (nicardipine and labetalol). She was transferred to the Reanimation Department due to thrombopenia; at the same time, acute renal failure was observed. On day 10, she presented with spontaneous abnormal movements of her right body side. No focal signs were detected. Brain imaging using fluid-attenuated inversion recovery magnetic resonance (FLAIR-MR) sequences revealed multiple bilateral high signal intensity in the frontoparietal lobes. T1-weighted, fat-suppressed axial cranial MR images (fig. ) showed the presence of a four-vessel CAD with no stroke seen on the diffusion-weighted images. Anticoagulation therapy was started and after a few days, while biological abnormalities improved progressively, the patient was transferred from the Reanimation to the Neurology Department.\nSeveral controls of the cervical arteries using the cervical echo Doppler and transcranial ultrasound system did not show artery modifications, but a second brain MRI revealed the complete withdrawal of the initial FLAIR lesions compatible with the reversible posterior leucoencephalopathy syndrome. The patient was discharged from the hospital at postpartum day 26. An MRA was performed at month 3 coupled with a cervical Doppler examination. Dissection signs had completely resolved at that time. The patient was asymptomatic and the baby was developing normally. After 1 year of follow-up, the patient did not complain of any neurologic symptom. | [[39.0, 'year']] | F | {'22341668': 1, '18268188': 1, '23107053': 1, '18977815': 1, '34964745': 2, '22067136': 1, '18535274': 1, '21148437': 1, '23498556': 1, '24403905': 2} | {'8615341-1': 1} |
165,138 | 3884199-1 | 24,403,883 | noncomm/PMC003xxxxxx/PMC3884199.xml | Repeated Pancreatectomy for Metachronous Duodenal and Pancreatic Metastases of Renal Cell Carcinoma | A 50-year-old woman was referred to our hospital for treatment of a duodenal tumor. She had a 13-year history of left nephrectomy for RCC. The histologic diagnosis was clear cell carcinoma of grade 2, pT2N0M0, and pStage II (according to the current TNM staging of the Union for International Cancer Control, 7th edition). The postoperative course was uneventful, and no adjuvant therapy was administered.\nThree years after nephrectomy, the patient underwent external beam radiation therapy (a total of 46 Gy) for a solitary metastatic bone tumor in the seventh thoracic vertebra. A further 7 years later, a solitary metastatic tumor was detected in the tail of the pancreas, and resection of the pancreatic tail and spleen was performed. Since the histologic findings of the pancreatic tumor were similar to those of the kidney, metachronous metastasis of RCC to the tail of the pancreas was assumed. A further 3 years after resection of the pancreatic tail, severe anemia was detected during a regular examination. The patient mentioned the presence of persistent tarry stools for 3–4 weeks. Esophagogastroduodenoscopy (EGD) was performed and revealed a mass in the descending portion of the duodenum.\nThe patient was admitted to our hospital and underwent detailed examinations. Her hemoglobin level was 7.5 g/dl. A subsequent EGD revealed an ulcerated polypoid mass in the descending portion of the duodenum; the mass was adjacent to the oral side, but did not involve the papilla of Vater (fig. ). Endoscopic ultrasonography showed the tumor to be partially invading the head of the pancreas (fig. ). Duodenography revealed a protruding lesion at the wall of the pancreatic side of the descending portion of the duodenum (fig. ). Abdominal enhanced computed tomography depicted a well-contrasted, hypervascular mass (fig. ). Magnifying endoscopy showed a diminished mucosal surface pattern of the gastrointestinal epithelium across the surface of the tumor (fig. ). Furthermore, combined with narrow-band imaging (NBI), it also showed a highly dense assembly of microvessels on the surface of the tumor that did not form a capillary network pattern (fig. ). A duodenal biopsy was performed, and the histopathologic diagnosis was inflammatory granulation with no malignancy. An additional duodenal biopsy for histologic confirmation was not performed to avoid uncontrollable duodenal bleeding from the biopsy site. Although partial resection of the pancreas had already been performed for a metastatic tumor of the pancreatic tail, the remnant pancreas was of rich volume (fig. ). There was no evidence of metastasis to the lung, liver, lymph nodes or other organs. Enterography showed no abnormal findings suggestive of metastasis to the small intestine. Fluorodeoxyglucose (FDG)-positron emission tomography showed specific FDG uptake in the duodenal tumor (standardized uptake value [SUVmax] 6.1). No abnormally enhanced lesions were identified in the body. The levels of both carcinoembryonic antigen and carbohydrate antigen 19-9 were within normal ranges. Resultantly, our diagnosis was metachronous, solitary duodenal metastasis derived from RCC, and we therefore performed a pancreaticoduodenectomy.\nThere were no findings of malignant ascites, carcinomatosis, omental implants or distant metastases to other abdominal organs; we therefore subsequently performed a subtotal, stomach-preserving pancreaticoduodenectomy, choledochojejunostomy, pancreaticojejunostomy, and gastrojejunostomy with preservation of the left gastric artery and vein to maintain the supply and drainage blood flow to the stomach after splenectomy. The pancreas was dissected just above the portal vein. Regional lymph node dissection was minimized.\nMacroscopic findings of the resected specimen indicated that the polypoid mass at the descending portion of the duodenum was ulcerative and friable (fig. ). Histologic findings revealed that the surface of the tumor was coated by granulation tissue consisting of inflammatory cells, fibrosis and edematous stroma (fig. ). Furthermore, the cancerous lesion comprised clear cells containing glycogen that were arranged in an alveolar pattern (fig. ). The tumor had invaded the duodenal muscle and partially invaded the pancreas at a depth of 2 mm. Although the tumor was located very close to the lower common bile duct and main pancreatic duct, histologic direct invasion was not observed in either duct. Immunohistology showed that the tumor cells were positive for vimentin and CD10 and negative for CK7 (fig. ). The histologic diagnosis was metastatic RCC.\nThe patient experienced postoperative leakage from the gastrojejunostomy. After percutaneous drainage of the intra-abdominal abscess, she recovered and was discharged on postoperative day 59. One year after surgery, the patient maintained a good glucose tolerance and received only oral hypoglycemic agents and no insulin therapy. Furthermore, she had no evidence of re-bleeding or recurrence of RCC. | [[50.0, 'year']] | F | {'33809634': 1, '883058': 1, '8731713': 1, '23009644': 1, '19555584': 1, '15029404': 1, '3498049': 1, '11291718': 1, '29948649': 1, '1195458': 1, '7253072': 1, '23024577': 1, '29435287': 1, '31533220': 1, '17210603': 1, '20249373': 1, '32547940': 1, '23343970': 1, '21577373': 1, '7272969': 1, '24403883': 2} | {} |
165,139 | 3884205-1 | 24,403,884 | noncomm/PMC003xxxxxx/PMC3884205.xml | Small-Sized, Flat-Type Invasive Branch Duct Intraductal Papillary Mucinous Neoplasm: A Case Report | A 70-year-old man was referred to our hospital for evaluation of a small cystic lesion in the pancreas head detected by health screening ultrasonography. He had neither clinical symptoms nor abnormal physical findings. Routine laboratory data also showed no abnormalities. Serum levels of carcinoembryonic antigen and carbohydrate antigen 19-9 were within normal limits. Enhanced computed tomography (CT) revealed a 17-mm cystic lesion without a solid component in the pancreas head. The cystic lesion was connected to the MPD (fig. ). Magnetic resonance cholangiopancreatography revealed that the cystic lesion was multilocular and connected to the MPD, which was not dilated (fig. ). On endoscopic ultrasonography (EUS), neither mural nodules nor wall thickness within the cystic lesion were detected (fig. ). ERCP revealed a 15-mm cystic dilation of the branch ducts without dilation of the MPD (fig. ). The cystic lesion had no features suggesting malignancy such as a cyst size of >3 cm, dilation of the MPD or an enhancing solid component within the cyst. However, cytological examination of the pancreatic juice collected during ERCP showed atypical columnar cells with loss of polarity, enlarged and hyperchromatic irregular nuclei and partially intracytoplasmic mucin (fig. ). These results suggested atypical cells with high-grade dysplasia, and the cystic lesion was diagnosed as a malignant BD-IPMN.\nPylorus-preserving pancreatoduodenectomy was performed. Intraoperative lavage cytology of the MPD of the remnant pancreas revealed no atypical cells. Pathological examination of the resected specimen showed a multilocular cystic lesion (fig. ), 16 × 9 × 15 mm in size, composed of dilated pancreatic branch ducts lined by columnar epithelium with mostly low-grade to focally high-grade dysplasia showing micropapillary growth (fig. ). In addition, small nests of irregular glands with enlarged nuclei invading the pancreatic parenchyma were noted over a distance of 11 mm, surrounded by myxoid stroma (fig. ). These features were consistent with the histological diagnosis of IPMN with an associated invasive carcinoma. Immunohistochemically, the neoplastic cells were positive for MUC5AC and MUC6, but negative for MUC1, MUC2 and CDX2, suggesting gastric-type IPMN. | [[70.0, 'year']] | M | {'17631133': 1, '22145009': 1, '19283746': 1, '25037612': 1, '22687371': 1, '17893501': 1, '29485131': 1, '26697446': 1, '16327281': 1, '18027021': 1, '15166958': 1, '22041510': 1, '21289527': 1, '19752768': 1, '18477671': 1, '17909912': 1, '24403884': 2} | {} |
165,140 | 3884206-1 | 24,403,894 | noncomm/PMC003xxxxxx/PMC3884206.xml | Incidental Finding of Lamellar Calcification of the Falx Cerebri Leading to the Diagnosis of Gorlin-Goltz Syndrome | A 53-year-old woman was referred to a radiologist for performance of a routine computed tomography scan of the head to exclude intracranial hemorrhage after accidental head trauma. The computed tomography imaging detected lamellar calcification of the falx cerebri, which is a pathognomonic feature of GGS (fig. ) []. Meeting the criteria for GGS and considering the pathognomonic feature of the lamellar calcification (major criterion for GGS), the patient was referred to the Department of Dermatology.\nAn examination of the patient's skin showed multiple small lesions clinically resembling basal cell carcinomas (BCCs) on the back, for which shave excisions were carried out. One suspected nodular BCC on the nose was removed by Mohs micrographic surgery. Pathologic examination of the lesions revealed 9 superficial BCCs on the back and 1 nodular BCC on the nose. The patient reported that the first BCC had occurred at the age of 27 years, and subsequently 4 other superficial BCCs had been removed by shave excision until the diagnosis of GGS was established. Furthermore, the patient presented with multiple palmar pits and marked syndactyly of the toes. Therefore, in our case, 2 major criteria [(I) lamellar calcification of falx cerebri, and (II) more than 2 BCCs] and 1 minor criterion (marked syndactyly of digits) were detected []. Additionally, the diagnosis of GGS was confirmed by positive testing for mutations in the tumor suppressor gene PTCH. Taking into account that GGS is an autosomal dominant genetic disease with nearly full penetrance and variable expressivity [], the daughters of our patient were screened for PTCH gene mutations as well. Although they both had no BCCs upon clinical inspection and none recorded in their medical history, one of the daughters tested positive for a PTCH gene mutation. | [[53.0, 'year']] | F | {'9096761': 1, '15717176': 1, '15545745': 1, '21887009': 2, '19032739': 1, '21152126': 1, '12925203': 1, '27448602': 1, '15696312': 1, '23633824': 2, '24403894': 2} | {'3162868-1': 1, '3636843-1': 1} |
165,141 | 3884207-1 | 24,403,881 | noncomm/PMC003xxxxxx/PMC3884207.xml | A Case of Adenocarcinoma of the Duodenum Arising from Brunner's Gland | A 70-year-old man was referred to our hospital for investigation of his abdominal discomfort. He had been followed with a diagnosis of diabetes mellitus for 45 years. On admission, physical examination showed no abnormalities, and all laboratory data were normal, except for an elevation in CEA and CA19-9. His CEA level was 128 ng/ml (normal 0–5 ng/ml) and his CA19-9 was 326 U/ml (normal 0–37 U/ml). Upper gastrointestinal endoscopy revealed an irregular elevated mucosa (fig. ) and a submucosal tumor with delle in the duodenal bulb (fig. ). Biopsy specimens revealed adenocarcinoma (the former) and hyperplasia (the latter). Abdominal computed tomography confirmed the presence of a mass originating in the submucosal layer of the duodenal bulb.\nUnder the diagnosis of adenocarcinoma of the duodenum, we proposed to perform pancreaticoduodenectomy, but the patient did not consent. Thus, partial resection of the bulbus and the antrum was performed instead. At laparotomy, we saw that the tumor had not invaded the serosa and that lymph node swelling was limited, so we dissected the lymph nodes, including No. 6, 8a, 8p, 12a and 16b1. The resected specimen showed two tumor types, in accordance with the preoperative findings (fig. ). Pathological examination of the oral side of the tumor showed that it was consistent with normal Brunner's glands (fig. , black arrows), Brunner's gland hyperplasia (fig. , white arrow) and adenocarcinoma supposed to arise from Brunner's glands (fig. , black arrowheads). The anal side of the tumor also revealed Brunner's gland hyperplasia and adenoma supposed to arise from Brunner's glands (fig. ). Immunohistochemical staining revealed that the site of normal and hyperplasia gland was positive for MUC5AC (data not shown) and MUC6 (fig. ), but the site of adenoma and adenocarcinoma was negative. Lymph node metastasis was positive for No. 6 and 8a. The patient's postoperative course was good, but adjuvant chemotherapy was not done because the he refused it. Fifteen months after the operation, laboratory data showed high levels of CEA and CA19-9 (45 ng/ml and 237 U/ml). Positron emission tomography and computed tomography revealed a tumor located near the head of the pancreas. Under the diagnosis of lymph node metastasis of duodenal cancer, we did pancreaticoduodenectomy. Pathological examination confirmed our preoperative diagnosis. The patient received adjuvant chemotherapy with S-1 and remained well after the surgery for 2 years. | [[70.0, 'year']] | M | {'28512587': 2, '10737576': 1, '31728658': 2, '31040631': 1, '5686390': 1, '53095': 1, '1964548': 1, '9779978': 1, '30568969': 1, '32637056': 2, '464691': 1, '29605945': 1, '28878614': 2, '17444863': 1, '17680313': 1, '29281782': 2, '25723745': 1, '24403881': 2} | {'5420418-1': 1, '5964285-1': 1, '5567001-1': 1, '7322633-1': 1, '6856237-1': 1} |
165,142 | 3884209-1 | 24,403,895 | noncomm/PMC003xxxxxx/PMC3884209.xml | PET-Negative Gastrointestinal Stromal Tumors | A 66-year-old woman presented with epigastric and right-sided chest pain. Endoscopy discovered a submucosal mass in the antrum. Percutaneous biopsy revealed a GIST. A subsequent PET/CT (fig. ) showed a 4-cm paragastric tumor, with a locoregional suspicious lymph node of 6 mm in diameter. There was no evidence of other metastasis. The tumor was FDG negative, except for a central area of the tumor with a maximum SUV of 4.2.\nThe patient initially refused the intended surgery because of fear of perioperative risks due to other medical conditions. She received palliative therapy with imatinib. After 3 months, PET/CT showed a slightly larger tumor with a lower FDG uptake with a SUV of maximum 3.0. Consequently, the patient agreed to be operated on. A distal gastrectomy was performed. Histology revealed a typical GIST of 7 cm in diameter with a mitotic count of <5/50 HPF. Because of the low-risk profile (low mitotic count, location of the tumor), the patient received no adjuvant therapy with imatinib. So far, there has been no evidence of tumor recurrence. | [[66.0, 'year']] | F | {'15547201': 1, '12528772': 1, '22076223': 1, '16222452': 1, '14734662': 1, '17193820': 1, '22572545': 1, '20457867': 1, '25276287': 1, '20599059': 1, '12957455': 1, '25566054': 2, '24403895': 2} | {'4280457-1': 1} |
165,143 | 3884210-1 | 24,403,896 | noncomm/PMC003xxxxxx/PMC3884210.xml | Male Breast Adenoid Cystic Carcinoma | The patient was a 41-year-old male who presented at the Department of Rehabilitation with symptoms of cervical back pain that had been occurring for 2 months. He was healthy, with an unremarkable medical history and a 20-pack per year smoking history. A cervical spine CT scan showed osteolytic changes with bony fragmentations in the C3, C5 and C6 transverse processes and vertebral bodies. These findings are suggestive of pathological fracture related to the presence of a tumor, such as metastatic carcinoma or multiple myeloma, rather than trauma (fig. ). To differentiate between metastatic bone lesions and the presence of a solid tumor, we examined the bone scan labeled with 99mTc, and the results showed only osteolytic bone lesions, with the exception of a small number of fractures. Therefore, we strongly suspected the presence of multiple myeloma (fig. ). We did a serum and urine protein electrophoresis evaluation and immunofixation, but there was no evidence of monoclonal gammopathy. In the bone marrow examination for excluding hematologic malignancies such as multiple myeloma and lymphoma, the findings showed that the hematopoietic cells were almost replaced by neoplastic cells with a glandular pattern. Neoplastic cells were stained with CK PAN (AE1/AE3) and were positive (fig. ).\nMetastatic carcinoma was suspected, but there were no abnormal findings in the esophagogastroduodenoscopy or colonoscopy. A PET-CT scan showed a disseminated hypermetabolic lesion (maxSUV = 7.4) in the axial skeleton, multiple, small-sized lung nodules and a multifocal hypermetabolic lung lesion (maxSUV = 2.6) as well as focal hypermetabolic lesions in the left axillary lymph nodes (maxSUV = 2.3) (fig. ). An ultrasound-guided core biopsy of the two 1.7-cm, low-echoic, pathological lymph nodes in the left level I axilla area was performed. The result of core biopsy was ACC (fig. ). Otorhinolaryngology and head and neck screening were performed to attempt to ascertain the location of the primary tumor in the salivary gland, as most ACC tumors in men are of salivary gland origin. However, this screening failed to identify specific lesions.\nTo identify the primary origin of the cancer, we reviewed the images and performed a whole-body physical examination. This revealed palpable and movable small nodular lesions in the periareolar area of the left breast, which had escaped interpretation on the PET-CT scan because the lesion was obscured (fig. ). There were clustered small nodules at the 1 o'clock and 2 o'clock locations around the left nipple upon physical examination. In a breast ultrasound, an irregularly-shaped solid lesion, 1.7 cm in size, was observed in the left subareolar area. The margin of the lesion was irregular and the internal area was relatively homogeneous and hypoechoic and was suggestive of a BI-RADS category 4C lesion. A core biopsy was performed and the pathology result was ACC, the same as that of the auxiliary lymph node biopsy (fig. ). Immunohistochemical staining was performed and the sample was negative for estrogen receptor (ER), progesterone receptor (PR) and c-erbB2, and positive for CK5/6 and P53. We finally diagnosed ACC of the breast with multiple lung and bone metastases and bone marrow involvement.\nThe authors submitted a waiver from their institutional review board (IRB) stating that this case report does not require IRB approval or oversight. | [[41.0, 'year']] | M | {'8387001': 1, '21896177': 2, '20653964': 1, '31393393': 2, '25885366': 2, '3011635': 1, '22546110': 1, '547120': 1, '16967686': 1, '28382475': 1, '1664513': 1, '193456': 1, '4309694': 1, '19474385': 1, '12001107': 1, '34970375': 2, '15221988': 1, '25516849': 1, '24403896': 2} | {'6708915-1': 1, '3184633-1': 1, '4329652-1': 1, '8683108-1': 1} |
165,144 | 3884889-1 | 24,470,721 | noncomm/PMC003xxxxxx/PMC3884889.xml | Successful Treatment of Tattoo-Induced Pseudolymphoma with Sequential Ablative Fractional Resurfacing Followed by Q-Switched Nd: YAG 532 nm Laser | A 45-year-old Chinese female developed pruritic nodules confined to the red areas of her tattoo over her left ankle [] 4 months after placement by a professional tattoo artist. The tattoo was in the form of a red heart containing two black eyes. Physical examination revealed erythematous nodules over the red-coloured portion of the tattoo, some coalescing into plaques.\nInitial differentials included an allergic contact dermatitis to the red dye, granulomatous reactions secondary to a foreign body, sarcoidosis or infections. Histology revealed a top-heavy lymphocytic infiltrate with numerous eosinophils within. Dark red, non-polarisable exogenous pigment was scattered throughout the dermis []. Immunohistochemical stains showed an infiltrate of mainly T cells, with a cluster of differentiation (CD) 4:CD8 ratio of 4:1. There were a few aggregates of CD20 positive B cells admixed within the infiltrate. Fungal and mycobacteria cultures were negative.\nBased on clinicopathologic findings, a diagnosis of tattoo-induced pseudolymphoma was made. The reaction did not respond to clobetasol proprionate United States Pharmacopoeia (USP) ointment 0.05% and intralesional triamcinolone acetonide 10 mg/ml. She received AFR laser treatment (SmartXide2, DEKA) monthly for three sessions, with good improvements and reduction in swelling and pigmentation. Parameters used included power: 30 watts, dwell time: 1500 μs, smart stack 1, spacing: 500 μm.\nShe was then treated with three sessions of Q-switched (QS) Nd:YAG laser (Revlite®, ConBio ™, USA) monthly at the following parameters: Red tattoo-532 nm, 3 mm spot size, fluences between 2 J/cm2 and 3 J/cm2. The black tattoo was treated with the 1064 nm, 4 mm spot size, fluences between 8 J/cm2 and 10 J/cm2.\nThe patient preferred to excise the residual black pigments and an elliptical excision specimen of both the “eyes” was done, which also incorporated adjacent skin that had been treated for the red tattoo pigmentation. Histology revealed epidermal spongiosis and reactive lymphoid hyperplasia with eosinophils, scar tissue as well as residual exogenous black tattoo pigment. However, no residual red tattoo pigment was present in the sites that had been treated for the red tattoo pigmentation []. The Grocott's Methenamine Silver (GMS) stain was used as the lymphocytes and eosinophils adopt the colour of the special stain (green in GMS) and are thus not visualised, while the red tattoo pigment retains its red colour, thereby, allowing the red tattoo to be recognised as exogenous pigment.\nshowed the end result after 7 months cessation of laser treatments. There was residual hypopigmentation but the overall lesion remained flattened with no recurrence of swelling and inflammation up to 12 months post-treatment. | [[45.0, 'year']] | F | {'16487133': 1, '21571169': 1, '18412226': 1, '30022936': 1, '8352605': 1, '21073602': 1, '7728477': 1, '26865796': 1, '19496897': 1, '24470721': 2} | {} |
165,145 | 3884890-1 | 24,470,722 | noncomm/PMC003xxxxxx/PMC3884890.xml | Follicular Unit Extraction as a Therapeutic Option for Vitiligo | A 12-year-old female patient presented with depigmented macule on the right eyebrow of 8 years duration []. She took various topical and oral medicines for the same without any relief. No new lesions were seen elsewhere in the body and size of the lesions remained stable for the past 2 years. On examination, she had single depigmented macule of the size 2 cm Χ 1.5 cm. Leukotrichia was found to be present. A diagnosis of focal type of vitiligo was made based on the clinical findings. Since the patient did not respond to the medical management, surgical correction with follicular unit transplant using the follicular unit extraction (FUE) technique was suggested. Written informed consent was taken. Donor hairs were harvested from the post auricular region using 1 mm skin biopsy punches. Donor area dressing was done using paraffin gauge dressing. The follicular units thus obtained were transplanted using an 18-G needle in the depigmented macules with 3 mm gap between the follicles. Paraffin gauze dressing was done for the recipient area and the dressing was removed after 5 days. Patient was followed-up every month. Patient was started on topical tacrolimus 2 weeks after the procedure. No post-operative complication was encountered. Repigmentation of the vitiligo patch was seen at the end of 6 weeks and complete pigmentation was seen at 12 weeks, with resolution of leukotrichia at the end of 6 months []. Colour match was excellent. There was no recurrence after 6 months of follow-up. | [[12.0, 'year']] | F | {'7351492': 1, '1714927': 1, '7633817': 1, '21508568': 1, '26865785': 1, '9555797': 1, '13833821': 1, '21572681': 2, '10208634': 1, '26909811': 1, '34211903': 1, '33911776': 1, '7928031': 1, '24470722': 2} | {'3081484-1': 1} |
165,146 | 3884891-1 | 24,470,723 | noncomm/PMC003xxxxxx/PMC3884891.xml | Epithelioid Hemangioendothelioma in a Child | A 9-year-old boy presented with an asymptomatic gradually enlarging nodular lesion since 5 months. Patient's past medical and family history were insignificant. On palpation, the lesion was non-tender, freely mobile and firm in consistency. It measured 3 cm × 2 cm and was situated in left iliac region []. The overlying skin was slightly thickened, reddish blue and attached to the lesion. There were no palpable lymph nodes. Based on the clinical examination, dermatofibroma, hemangioendothelioma and arteriovenous malformation were considered as differentials. The routine investigations were within the normal limits. The lesion was completely excised and sent for histopathological examination.\nMicroscopic examination revealed well-circumscribed tumour localised in the dermis, composed of mass of plump epithelioid shaped endothelial cells arranged in “nest-like” pattern with minimal vascular differentiation. The epithelioid cells showed intracytoplasmic vacuole (Primitive Lumina) and erythrocytes were seen in some of the lumina. Few vascular channels containing erythrocytes were seen on peripheral portions of the tumour. There was no cellular atypia and necrosis [Figures and ]. On Immunohistochemistry, CD 31 was positive, other endothelial markers were not done due to lack of facilities and our patient was not affordable. Other investigations such as chest X-ray, ultrasound abdomen and a whole-body bone scan were performed to exclude multicentric location and distant metastasis, but were found to be normal. Patient is on regular follow-up for the last 10 months with no signs of local recurrence or metastasis. | [[9.0, 'year']] | M | {'8517499': 1, '9130982': 1, '2644323': 1, '15505552': 1, '10601609': 1, '19492891': 1, '1569252': 1, '10767700': 1, '11342784': 1, '9855348': 1, '1689691': 1, '15218955': 1, '33133812': 2, '9714979': 1, '22470212': 2, '24470723': 2} | {'3312660-1': 1, '7586378-1': 1} |
165,147 | 3884932-1 | 24,470,664 | noncomm/PMC003xxxxxx/PMC3884932.xml | Infantile Perianal Pyramidal Protrusion with Coexisting Perineal and Perianal Hemangiomas: A Fortuitous Association or Incomplete PELVIS Syndrome? | A 3-month-old female child was seen by one of us (SV) in a multi-specialty hospital upon the request of the pediatrician. She was born after a normal full-term vaginal delivery and had no history of consanguinity in her parents. The child was thought to have hemorrhoids with a large hemangioma in the perineal area present since birth. Upon examination the child had a skin-colored perianal protrusion that was clearly located posterior to the anus []. There was in addition a large midline hemangioma that started from the gluteal cleft all the way to the medial root of the thigh skirting around the vulva []. The presence of inflammation around the pyramidal protrusion could not be assessed because of the presence of the large hemangioma. The parents said the child was born with a ‘red patch’ in the area of the hemangioma which evolved over the past 3 months into a hemangiomatous red plaque. The child also had a large hemangioma on the contralateral side of the face with involvement of the lower lip []. There were no neurological complaints. There were also small hemangiomas found on the ventral aspect of the left forearm and dorsum of the left foot. The hemangiomas were explained in detail to the parents and they decided not to give any oral corticosteroid for the perineal hemangiomas and rather wait for a year. A head and neck as well as spine X-ray was performed that did not show any abnormality. CT scan/MRI was refused by patient's parents due to cost factor. A referral was made to the pediatric surgeon for biopsy of the pyramidal lesion which they refused. A provisional diagnosis of large perineal hemangioma with a perianal skin tag was made. The large facial segmental hemangioma was thought to be Sturge-Weber syndrome with no clinical evidence of internal involvement. The patient was prescribed topical clobetasol cream under monthly supervision after which the lesion had flattened a little and showed a whitish area with pronounced flattening. | [[3.0, 'month']] | F | {'11568914': 1, '9762025': 1, '9415474': 1, '22099696': 1, '32317831': 1, '11146341': 1, '20598318': 1, '9572703': 1, '2743179': 1, '8961878': 1, '34244133': 1, '17191046': 1, '11860563': 1, '16847205': 1, '24470664': 2} | {'3884932-2': 2} |
165,148 | 3884932-2 | 24,470,664 | noncomm/PMC003xxxxxx/PMC3884932.xml | Infantile Perianal Pyramidal Protrusion with Coexisting Perineal and Perianal Hemangiomas: A Fortuitous Association or Incomplete PELVIS Syndrome? | A 2-month-old female child was brought to the same clinic where she was examined by both authors (i.e., SV and UW). The child had a flattened erythematous leaf-like midline protrusion located anterior to the anus which had grown gradually []. She too was born after a normal full-term vaginal delivery and had no history of consanguinity in her parents. The parents had noticed the lesion only after 2 weeks of her birth. A hemangiomatous plaque to the left of the anus had been noticed by the parents as a pink-colored macule which had gradually deepened in color but had not grown in size. There was no surrounding inflammation. There were no other skin findings or any other structural or functional abnormalities detected. The parents refused a pediatric surgical reference for any further intervention. They called after 2 months saying that the hemangioma was flattening rapidly but the leaf-like protrusion remained unchanged. A diagnosis of IPPP was made for both the cases with the diagnosis of the first case done retrospectively. This opens up the strong possibility of the IPPP being a part of PELVIS syndrome in which a ‘skin tag’ is described with perineal hemangioma and other features. | [[2.0, 'month']] | F | {'11568914': 1, '9762025': 1, '9415474': 1, '22099696': 1, '32317831': 1, '11146341': 1, '20598318': 1, '9572703': 1, '2743179': 1, '8961878': 1, '34244133': 1, '17191046': 1, '11860563': 1, '16847205': 1, '24470664': 2} | {'3884932-1': 2} |
165,149 | 3884933-1 | 24,470,665 | noncomm/PMC003xxxxxx/PMC3884933.xml | Trigeminal Trophic Syndrome | A 48-year-old woman complained of nonhealing painless progressive ulcer over the left nostril and skin over the upper lip of 6-months duration. She had herpes zoster affecting the left side of face 1-year back. For the facial ulcer, she was treated with both topical and systemic steroid and multiple antibiotics without any improvement. The ulcers were painless located on ala nasi, extending medially into the nasal septum, superiorly over the skin of the nose and inferiorly involving the skin over the upper lip on the left side. Their margins were clearly delineated [].\nThere were no vesicles around the ulcer or anywhere over the face. There were no hypopigmented patch or peripheral nerve thickening. Neurological examination revealed left-sided lower motor neuron type of facial palsy, decreased level of pain, and temperature sensation over the trigeminal area. Corneal and mastication reflexes were diminished. Her vitals and other systemic examination revealed no abnormality. Tzanck smear from the ulcer showed neutrophils and there were no acantholytic or multinucleate giant cells. Touch smear for bacteria and slit skin smear for acid fast bacilli were negative. Antinuclear, antineutrophil cytoplasmic; hepatitis B and C, and antibodies were negative. Tests for HIV and syphilis were nonreactive. Her blood glucose, renal, and liver function tests were within normal range. Electrocardiography (ECG) and chest radiography, computed tomography (CT) scan, and magnetic resonance imaging (MRI) of the brain were normal.\nTTS was diagnosed and the ulcer was treated with saline soaks and topical antibiotics. Patient was counseled and advised to avoid scratching or rubbing the site. There was remarkable improvement and the ulcer healed in 2 weeks time []. Patient is being followed-up and there is no recurrence since 1 year. | [[48.0, 'year']] | F | {'6811452': 1, '29678820': 1, '7085956': 1, '32226043': 1, '1890371': 1, '10583140': 1, '18711069': 1, '15099331': 1, '22016290': 1, '27559529': 1, '17567297': 1, '33380563': 1, '28954129': 1, '3705951': 1, '24470665': 2} | {} |
165,150 | 3884934-1 | 24,470,666 | noncomm/PMC003xxxxxx/PMC3884934.xml | Co-existence of Lichen Sclerosus et Atrophicus and Morphoea Along Lines of Blaschko | A 40-year-old female presented with slightly pruritic eruption that initially started over the left shoulder 2 years back and then gradually enlarged to involve the left arm, left side of her abdomen, and the left lower limb. The lesions reached the present size 1 year back and remained stable since then. An examination revealed discrete and confluent, porcelain white, sharply circumscribed, slightly atrophic, papuloplaques over the left arm and left leg and left side of abdomen with a sharp demarcation at the midline [Figures and ]. Several areas showed cigarette paper wrinkling suggestive of atrophy of epidermis. The genital, perianal, and the mucosal areas were normal. The skin over the posterolateral aspect of abdomen on the left side and over the lower part of thigh was slightly hyperpigmented and indurated. Over this affected area the epidermal changes were missing and the overlying skin was smooth.\nRoutine hemogram and urine examination were within normal limits. Biopsy done from the area with porcelain-white discoloration of epidermis showed follicular plugging overlying a thinned out epidermis devoid of rete ridges. Vacuolar alteration of basal cell layer was present. Moderate papillary dermal edema along with focal homogenization of the collagen was present. Periadnexal lymphohistiocytic infiltrate was evident in the dermis. The findings were consistent with LS []. Histopathology done from the smooth, hyperpigmented, and indurated lesion on the left flank showed a relatively normal epidermis without follicular plugging. The basal cell layer was intact without vacuolation. The dermis appeared homogenous and little oedematous []. There was patchy periappendageal inflammation and subcutaneous fat layer showed patchy septal inflammation with lymphocytic infiltrates. The histopathology was consistent with morphoea.\nThe patient was started on twice daily topical Tacrolimus 0.1% ointment. The lesions were less obvious after 1 month of starting the therapy though there was no change in the cutaneous atrophy component. | [[40.0, 'year']] | F | {'165510': 1, '26288432': 2, '9591796': 1, '7451694': 1, '8040398': 1, '7868709': 1, '24470666': 2} | {'4533562-1': 1} |
165,151 | 3884935-1 | 24,470,667 | noncomm/PMC003xxxxxx/PMC3884935.xml | Tuberculosis Verrucosa Cutis Presenting as Diffuse Plantar Keratoderma: An Unusual Sight | A 52-year-old male farmer, presented with a 9-month long history of thickening of left plantar skin, along with diffuse pedal edema of the same foot for last 2 months. He reported a history of a thorn prick injury 1 month preceding the onset of symptoms. At the onset, patient experienced only roughness of plantar skin which progressively developed a verrucous surface involving almost 80% of plantar skin of left foot. However, it remained asymptomatic until recent times, when he felt a chronic dull pain which was aggravated on walking. Patient had no past history of tuberculosis or any other systemic illness. He denied any history suggestive of immunodeficiency or constitutional symptoms of tuberculosis. He was a nonalcoholic and nonsmoker with a negative family history of tuberculosis.\nOn examination; he had left-sided, pitting, pedal edema. Almost 80% of plantar skin of left foot had developed diffuse keratoderma. The surface of the lesion was verrucous with variable degree of thickening becoming plaque-like at places, sparing the toes and instep, with ill-defined borders []. Posteriorly, the verrucous lesion had a well defined 2 cm broad linear extension up to the ankle. There was no evidence of scarring or any signs of healing. The other foot was uninvolved. He had no other cutaneous, mucosal lesions, or nail abnormalities. He was well-built and nourished, without any other abnormal systemic findings. There was no active focus of infection as evidenced by absence of fever, lymphadenopathy, leukocytosis, and a normal chest radiograph. There were two prominent BCG scar marks on his left arm. Routine investigations showed hemoglobin to be 12 g/dL; total leukocyte count of 11,000/mm3; differential count was 59 neutrophils (N), 2 eosinophils (E), 39 lymphocytes (L); and erythrocyte sedimentation rate was 45 mm in the first hour. Chest X-ray revealed no foci of old or recent tuberculosis or evidence of any other abnormalities. Mantoux reaction reading after 48 h was 20 mm. With these details, a provisional diagnosis of TVC was made and a punch biopsy was taken from the heel area. Histopathology of the plantar skin sample showed striking pseudoepitheliomatous hyperplasia with diffuse tuberculoid granulomatous inflammation in dermis []. There was no underlying bony involvement on X-ray of the foot. Patient was started on standard antitubercular regimen. | [[52.0, 'year']] | M | {'33425164': 1, '25972972': 1, '2196232': 1, '2498216': 1, '2670785': 1, '15725242': 1, '29851791': 2, '22121276': 1, '25071267': 1, '9797751': 1, '5665267': 1, '18888597': 1, '24470667': 2} | {'6392906-1': 1} |
165,152 | 3884936-1 | 24,470,668 | noncomm/PMC003xxxxxx/PMC3884936.xml | A Case of Ichthyosis Hystrix: Unusual Manifestation of This Rare Disease | A 46-year-old male patient presented with generalized severely thick skin since early childhood. According to his mother he was born normal and healthy. He gradually developed thickening of skin with adherent scales starting from face and progressively involving other parts of the body. None of the family members were affected. On examination, he had hyperkeratotic, thick, dry, adherent, mud-colored scales covering most of the body especially the face, scalp, and extremities including the flexures. Few areas had porcupine like spikes. Facial skin was so thickened and rugose that it became expressionless giving him a masked appearance []. His trunk was least involved with islands of normal skin []. Both, follicular prominence and atrophy were seen over the flexures of the forearm. He had associated nongradient palmoplantar keratoderma. Hairs were sparse and less over occipital area as it was covered with thick scale. He also had teeth and nail abnormality for the same duration. Most of the teeth were absent and canine was peg-shaped []. Subungual hyperkeratosis and prominent onychogryphosis were found in most of the nails []. There were no apparent ophthalmological or skeletal abnormalities, neither any hearing loss. His complete hemogram and chest X-ray were normal. On histopathology, we found orthokeratotic hyperkeratosis with sparse parakeratosis, irregular acanthosis, follicular plugging, and multiple binucleated keratinocytes in the epidermis along with upper dermal lymphocytic infiltrate, suggestive of ichthyosis [Figure and ]. On the basis of clinical features and histopathology, we arrived at the diagnosis of ichthyosis hystrix. | [[46.0, 'year']] | M | {'16417221': 1, '1234876': 1, '30410908': 1, '7671408': 1, '28405559': 1, '17355236': 1, '1225655': 1, '6206802': 1, '12603866': 1, '20994790': 1, '21844476': 1, '16679758': 1, '24470668': 2} | {} |
165,153 | 3884937-1 | 24,470,669 | noncomm/PMC003xxxxxx/PMC3884937.xml | Angiokeratoma Circumscriptum in a Young Male | A 20-year-old male presented to our department with the history of presence of multiple eruptions on his right leg since birth. These started as reddish-brown patches and became static after having initially increased in size proportional to the patient's growth. He complained of local pain and bleeding on trivial trauma. There was neither a personal history suggestive of any bleeding disorder nor occurrence of similar lesions in the family.\nSkin examination revealed multple, linear,[] dark red to brown, hyperkeratotic, warty plaques ranging from 2 × 3 cm to 7 × 5 cm with irregular borders on the shin of the right leg. On palpation, these were non-pulsatile, tender, slightly indurated, and non-compressible. Length and circumference of the legs were equal. No bruit was heard on auscultation.\nSystemic examination revealed no abnormal findings. Routine lab investigations were normal. No bony defect was detected in X-ray of the legs. Ultrasonography revealed a linear hypodense circumscribed soft tissue mass without any relationship to the underlying bone. Doppler study did not reveal any altered blood flow.\nMicroscopic evaluation of the deep incisional biopsy specimen showed hyperkeratosis, acanthosis, and extensive papillary dermal vascular proliferation, thereby, confirming our clinical impression of AKC [Figure and ]. He was referred to and managed by reconstructive surgeon via surgical excision, followed by skin grafting [Figures and ; ]. | [[20.0, 'year']] | M | {'7894939': 1, '16409270': 1, '6018992': 1, '12585833': 1, '3281548': 1, '15762221': 1, '15347502': 1, '3652499': 1, '225899': 1, '8993949': 1, '14276645': 1, '11903167': 1, '22121293': 1, '9675347': 1, '26155544': 1, '24470669': 2} | {} |
165,154 | 3884938-1 | 24,470,670 | noncomm/PMC003xxxxxx/PMC3884938.xml | Eccrine Poroma on the Face: An Atypical Presentation | A fifty-year-old woman presented with a slow-growing nodule over the left cheek for one and a half years. The lesion started as a tiny, 2 mm asymptomatic papule on the left cheek and gradually increased to 2 cm in size. There was no history of trauma, pre-existing skin lesion, or topical application at the site of the lesion. There was no family history of similar lesions.\nExamination revealed an ulcerated nodule with a peripheral hyperpigmented rim just lateral to the left nasolabial fold []. The lesion was about 1.5 cm in diameter, firm, mobile, non-tender, and did not bleed on touch. The local lymph nodes were not enlarged. Differential diagnoses considered were basal cell carcinoma, squamous cell carcinoma, amelanotic melanoma, and keratoacanthoma.\nExcision biopsy was performed, which surprisingly revealed broad anastomosing bands of uniform small cuboidal cells [], few ductal lumina, and narrow cystic spaces [] within the epidermis. Further, the cuboidal cells showed uneven cytoplasmic clearing []. The histological features clinched the diagnosis of an eccrine poroma. A few occasional mitoses were seen which suggested the requirement of a close follow-up in this patient. | [[50.0, 'year']] | F | {'30651691': 2, '27990401': 1, '11801771': 1, '19326001': 1, '11395548': 1, '29606822': 2, '19915263': 1, '13361538': 1, '27512190': 2, '16112378': 1, '24470670': 2} | {'5839896-1': 1, '6306575-1': 1, '4966403-1': 1} |
165,155 | 3885790-1 | 24,416,050 | noncomm/PMC003xxxxxx/PMC3885790.xml | Asymptomatic moyamoya syndrome, atlantoaxial subluxation and basal ganglia calcification in a child with Down syndrome | A 10-year-old girl with Down syndrome was referred to the pediatric department from the ophthalmologic department of Ulsan University Hospital because both basal ganglia calcification was incidentally detected on a brain computed tomography (). She had been managed at the ophthalmologic clinic because of strabismus. Brain computed tomography (CT) was performed to identify whether her ophthalmologic manifestations, including esotropia of both eyes, amblyopia in the left eye, cyclotorsion of both eyes, and horizontal nystagmus, would result from brain parenchymal lesion.\nShe was born at Ulsan University Hospital. At that time, she showed typical features of Down syndrome, and was diagnosed with Down syndrome by the identification of 47,XX,+21 on the cytogenetic study. Echocardiography after birth, which was performed due to cardiac murmur, revealed a 6.3 mm sized perimembranous ventricular septal defect and two small sized atrial septal defects. She had been examined by regular follow-up echocardiography evaluation without surgery, and was presented with no symptoms associated with cardiac anomalies until the time of admission. She had moderate mental retardation with full scale intelligence quotient score of 44. Her parents stated that they had never noticed apparent neurologic symptoms of the patient, including transient motor weakness before the admission.\nOn physical examination at the time of admission, she showed typical features of Down syndrome, including hypertelorism, depressed nasal bridge, low-set ears, epicanthal folds, protruded tongue, small chin, and simian creases on both hands. On cardiac auscultation, systolic murmur was heard. On neurologic examination, she was fully alert and conscious. She showed esotropia of both eyes, but, the limitation of both eye movements was absent. She exhibited horizontal nystagmus, without vertical nystagmus. No obvious other abnormalities were revealed on the cranial nerve examination. Motor weakness of four extremities was absent, although muscle tone was slightly hypotonic. The deep tendon reflexes on both knees were slightly exaggerated, and Babinski sign was absent in both. On laboratory findings, we observed total calcium, 9.8 mg/dL; ionized calcium, 4.60 mg/dL; parathyroid hormone, 44.94 pg/mL; ceruloplasmin, 22 mg/dL; serum copper, 96 µg/dL; thyroid-stimulating hormone, 1.20 mIU/L; and free thyroxine, 1.49 ng/dL. Investigations for autoimmune (antinuclear antibody, antidouble strand DNA antibody, anticardiolipin antibodies, antiphospholipid antibodies, and antineutrophilic cytoplasmic antibody), prothrombotic (protein C, S, Factor V Leiden mutation, and antithrombin III) and metabolic (serum amino acid and urine organic acid) disorders were within normal limits. Echocardiography performed, during the admission, revealed 3-mm sized perimembranous ventricular septal defect with small to moderate left to right shunt, but good ventricular function. Brain magnetic resonance imaging and magnetic resonance angiography, which were performed during the admission, showed as follows; multiple tiny ischemic lesions in both frontal white matters, compression of the cervical cord due to displaced odontoid process at the level of C1, and severe stenosis of the bilateral internal carotid arteries with collaterals to the bilateral cerebral hemispheres (, ). Four vessels angiography revealed occlusion of the proximal portion of the right internal carotid artery, occlusion of the left internal carotid artery bifurcation and the left middle cerebral artery, transdural collaterals from the middle meningeal, the superficial temporal, and the occipital arteries, and hypertrophic right vertebral artery with prominent leptomeningeal collaterals to both cerebral hemispheres. Three dimensional cervical spine CT showed subluxation and rotation of C2 on C1 with spinal canal narrowing, and os odontoideum ().\nHer family moved to another province far from our hospital, several months after the evaluation. According to the telephone interview with the patient's mother, the patient had been doing well with no neurologic symptom and no special management during the 2 years since the admission to our hospital. | [[10.0, 'year']] | F | {'6231537': 1, '17692191': 1, '150421': 1, '1909221': 1, '11063086': 1, '8115409': 1, '18277263': 1, '12427521': 1, '20173525': 1, '1532715': 1, '6185033': 1, '1385957': 1, '9550527': 1, '3156213': 1, '2958770': 1, '2937636': 1, '24416050': 2} | {} |
165,156 | 3885791-1 | 24,416,051 | noncomm/PMC003xxxxxx/PMC3885791.xml | Moyamoya syndrome occurred in a girl with an inactive systemic lupus erythematosus | A 17-year-old Korean female was brought to the pediatric department because of new-onset, right-sided weakness that was first noted 3 weeks ago when it affected her writing ability. Her speech was slurred and she complained tongue numbness. Her symptoms seemed to improve for a while at first, but afterward she did not completely recover for 1 month. There was no evidence of trauma, flu-like symptoms, or no report of nausea, vomiting, headache, or convulsions. Evaluating the patient's medical history, there were no complications at birth, and her development had been age appropriate. There was no family history of stroke in young individuals.\nIn September 2008, at 15 years of age, she was diagnosed with lupus nephritis. Initial clinical course showed severe manifestations with microangiopathic hemolytic anemia, thrombocytopenia, and hypertension. The renal biopsy finding indicated the lupus nephritis ISN/RPS Class IV-G(A). After the initial period of 6 months, her clinical symptoms were well controlled with treatment of azathioprine, prednisolone and antihypertensive drugs. However, after 22 months, new-onset right-sided weakness developed.\nOn admission, vital signs were stable. Neurological examination revealed asymmetric motor weakness with the grade of 4/5 in the right arm and leg. She was mentally alert, but had a subtle dysarthric speech despite of normal cranial nerves. Reflexes for both knees were symmetric and normal. Otherwise, her systemic examination was unremarkable.\nThe laboratory tests revealed the followings: leukocyte, 5,100/mm3; hemoglobin, 14.8 g/dL; platelet, 266,000/mm3; erythrocyte sedimentation rate, 2 mm/hr (0-20); C-reactive protein level, 0.01 mg/dL (0-0.5); C3, 64 mg/dL (79-152); C4, 12.4 mg/dL (10-40); and CH50, 37.6 U/mL (23-46). Electrolytes, renal and hepatic profiles, and lipid profile were normal. Anti-thrombin III, prothrombin time, activated partial thromboplastin time, protein C (120%), protein S (70%), and lipid profiles were all within normal limits. Urine analysis showed no proteinuria or microscopic hematuria. Immunologic evaluation revealed antinuclear antibody (ANA) of 1:80, antids-DNA immunoglobulin G (IgG)/IgM 7 (0.25)/14 (0.20) IU/mL, anticardiolipin IgG/IgM (-/-), lupus anticoagulant (-), antiphospholipid antibody (-), anti-Ro/anti-La (-/-), and venereal disease research laboratory test (VDRL) (-). Chest X-ray, electrocardiogram, and echocardiogram findings were all unremarkable. There was no evidence of aggravation of SLE activity.\nBrain magnetic resonance imaging (MRI) revealed no evidence of cerebral infarction or ischemic change (). Magnetic resonance angiography of the brain revealed stenosis of the supraclinoid portion of the left ICA and the right anterior cerebral artery (ACA). Conventional angiography showed severe narrowing of the distal portion of left ICA, the proximal portion of left middle cerebral artery (MCA), ACA and the right ACA (). These findings were compatible with MMD.\nThe patient was started aspirin, however, her right-sided weakness and dysarthria continued unabated. One week later, left encephalo-duro-arterio-synangiosis bypass surgery was performed. Postoperative MRI showed no interval change of right MCA branch and no further progression with new lesion. She has since recovered without new ischemic symptoms. No further neurological deficits occurred during a 14-month follow-up. Right-side surgery will be scheduled depending on follow-up neuroimaging results and the patient's neurological symptoms. | [[17.0, 'year']] | F | {'19335127': 1, '11971089': 1, '11035439': 1, '12136916': 1, '16581493': 1, '27843469': 2, '21752869': 1, '9566671': 1, '8145903': 1, '5775283': 1, '14717235': 2, '11486106': 1, '18625642': 1, '16028759': 1, '9385355': 1, '24416051': 2} | {'4531634-1': 1, '5100040-1': 1} |
165,157 | 3885951-1 | 24,432,233 | noncomm/PMC003xxxxxx/PMC3885951.xml | Phaeohyphomycotic dermatitis in a giant panda (Ailuropoda melanoleuca) caused by Cladosporium cladosporioides | The giant panda was an 18-year-old male, who lived in a semi-captive and semi-stocking environment at the Giant Panda Protection Research Center. The center is located in Ya'an, a humid, mountainous area in southwest China. A small amount of hair loss was observed at the dorsum nasi of the giant panda and the skin of the dorsum nasi was scratched by its front paws. The dermatic lesion on its dorsum nasi was measured as 12 cm×8 cm (). The skin lesion, which was initially hard, erythematous and displayed edema, later became pruritic. Skin scrapings were examined using a microscope and fungal spores were identified. The identification of these symptoms was defined as day 0.\nFive days after the symptoms were identified, the giant panda was first treated with antimycotic ketoconazole (600 mg/day orally), and the treatment regimen continued for 2 months. The skin redness disappeared and hair loss was recovered.\nHowever, the same symptoms recurred 3 months after the ketoconazole treatment stopped. The same treatment was reinitiated but the giant panda was not responsive to the second treatment with ketoconazole. Samples of the secretion, furfurescence, thrix and hair follicles of the dorsum nasi were collected. The samples were cultured on Sabouraud's dextrose agar (SDA) at 25 °C for 2 weeks. Black colonies were observed on the agar and the colony discs were subsequently cultured on potato dextrose agar (PDA) at 25 °C. After 48 h, yellowish-green colonies had formed and were approximately 1.0 cm in diameter. After 5 days, the colonies gradually turned black and were about 5 cm in diameter. The fungus grew well on SDA at 25–28 °C, grew poorly at 30 °C, and did not grow at 35 °C. Microscopic observation revealed that the fungus had long conidiophores with ellipsoidal conidia. The conidiophores were non-nodose and had both terminal and lateral ramifications ().\nPCR was performed to further identify the fungus. Genomic DNA was extracted and amplified using primers targeting internal transcribed spacer 1 (ITS1) and internal transcribed spacer 4 (ITS4). The resulting sequence of 509 nucleotides was subjected to a homology search using the BLAST alignment program of the GenBank database. The sequence was 99% identical to the C. cladosporioides gene, with a highest maximum score of 922 (GenBank accession number: JQ727688.1). The expected E value for the identity was 0.0, indicating that this result was non-random and statistically significant. The fungus was thus identified as C. cladosporioides. Oral itraconazole (700 mg daily) was administered. After 3 months of treatment, the edema disappeared and the infection was completely healed (). No reoccurrence was found one year after the recovery. | [[18.0, 'year']] | M | {'33932997': 1, '1815127': 1, '20020251': 1, '26179305': 1, '34291099': 1, '9711436': 1, '1291876': 1, '29157237': 1, '2170477': 1, '21707784': 1, '12472729': 1, '8544087': 1, '10838855': 1, '11270401': 1, '24947171': 1, '1239189': 1, '33441700': 1, '1108737': 1, '24432233': 2} | {} |
165,158 | 3887160-1 | 24,421,851 | noncomm/PMC003xxxxxx/PMC3887160.xml | Development of Six Tumors in a Sebaceus Nevus of Jadassohn: Report of a Case | A 70-year-old woman presented to our outpatient clinic with a verrucous growth on her scalp measuring 2.5×1.8 cm. The lesion presented at birth. While she was unaware of any specific changes in the lesion for decades, she noticed a rapid progression in the last two years. She was generally healthy and had no accompanying symptoms. The patient had no previous history of cutaneous or internal malignancies and a complete systems review was negative. There was no family history of malignancy.\nThe lesion of NS was completely excised with a free margin of 0.2 cm and submitted for histopathological evaluation. Macroscopic examination of the lesion revealed a 2.5×1.8 cm, yellowish, verrucous plaque devoid of hair. One large, vegetative plaque with a hemorrhagic crust lay within the yellowish plaque.\nHistopathological findings of the lesion by hematoxylin and eosin staining showed acanthosis, papillomatosis, sebaceus gland hyperplasia, and immature hair follicles in the dermis in the peripheral parts of the lesion. These findings were consistent with a diagnosis of NS. In addition, there were 6 adnexal tumors in the lesion including SCAP, PT, TAA, sebaceoma, TFI, and superficial epithelioma with sebaceus differentiation (SESD). The majority of the lesion was composed of SCAP, PT, and TAA. The tumors were separated from each other by loose fibrillary connective tissue stroma. These benign tumors were partly intertwined (, ).\nCytomorphologically, the SCAP was composed of irregular papillary projections covered by double layers of epithelium and stratified squamous epithelium with stroma containing lymphocytes and large numbers of plasma cells (). The deeper portion of the SCAP consisted of tubular structures with two layers of epithelial cells. While the luminal cells of the tubules were columnar and showed decapitation secretion, the outer layer was composed of flattened cells. These tubules were surrounded by a fibrous stroma. TAA was diagnosed from these findings ().\nThe PT was characterized by a well-circumscribed large basaloid dermal tumor without an epidermal connection. There were dispersedly brown pigmentation fields in the center of large tumor islands ().\nIn our case, small foci of follicular infundibulum, sebaceoma and SESD were observed. The TFI was composed of a plate like growth of basaloid keratinocytes with several points of attachment to the epidermis (). A collection of benign basaloid sebaceus cells suggestive of a sebaceoma was present and the sebaceoma existed as two types: superficial and deep in the dermis. There were cysts containing debris from holocrine degeneration (). The SESD was characterized by a sharply defined and well-circumscribed plate-like epidermal growth. Merging with the superficial keratinocytes was a cytologically bland basaloid cell population in which admixed mature sebocytes were present in clusters (). | [[70.0, 'year']] | F | {'14295518': 1, '16164741': 1, '9310725': 1, '16625081': 1, '28855922': 1, '17343592': 1, '34430463': 2, '32477993': 1, '10213487': 1, '20709645': 1, '15606837': 1, '25298254': 1, '17890915': 1, '19652586': 1, '28892945': 1, '16503928': 1, '10770429': 1, '5435080': 1, '27127368': 1, '28560218': 1, '24421851': 2} | {'8354425-1': 1} |
165,159 | 3887161-1 | 24,421,852 | noncomm/PMC003xxxxxx/PMC3887161.xml | Human Cutaneous Protothecosis: Report of a Case and Literature Review | A 68-year-old man presented with an ill-defined erythematous lesion with crust at the dorsal aspect of his left wrist (). The skin lesion developed approximately a month ago without any traumatic history, and the patient complained of poor oral intake and diarrhea for a week. He had been suffering from osteoarthritis and had taken medications for twenty years. He was surgically treated for small bowel perforation, adhesion, and panperitonitis 3 years ago. His occupation was gathering trash for recycling.\nA punch biopsy was performed to reveal the histologic features of granulomatous inflammation with confluent necrosis at the upper dermis containing many Prototheca organisms (, ). On periodic acid-Schiff after diastase digestion special staining, non-budding spherical organisms with multiple sporangia containing endospores were observed with a morula- or cartwheel-like appearance, which is the characteristic feature of the Prototheca species (). The organisms were approximately 20 µm in diameter and had a thick cell wall. Upon crystal violet staining, the organisms exhibited multiple small bluish dots ().\nFor specification of the species, small subunit ribosomal DNA (SSU rDNA) was amplified by polymerase chain reaction using the universal primers NS3 (5'-GCAAGTCTGGCCAGCAGCC-3') and NS8 (5'-TCCGCAGGTTCACCTACGGA-3'). The sequences were submitted to a BLASTn search by using the National Center for Biotechnology database (NCBI; ). The sequences of the SSU rDNA regions of the isolate were 99% identical with Prototheca zopfii.\nThe patient was given antifungal agent itraconazole, and the symptoms were relieved. | [[68.0, 'year']] | M | {'15472379': 1, '21732787': 1, '3816130': 1, '12901639': 1, '33912392': 1, '32117805': 1, '22348570': 1, '9700377': 1, '17428884': 1, '14206703': 1, '1008567': 1, '15078433': 1, '31959834': 1, '20117953': 1, '1659090': 1, '27081288': 1, '34943685': 1, '11966707': 1, '31649969': 1, '33366289': 1, '29376895': 2, '21964624': 1, '22837317': 1, '24421852': 2} | {'5770008-1': 1} |
165,160 | 3887162-1 | 24,421,853 | noncomm/PMC003xxxxxx/PMC3887162.xml | Diffuse Large B-Cell Lymphoma Arising in Warthin's Tumor: Case Study and Review of the Literature | A 60-year-old male patient was referred to an otorhinolaryngology clinic due to a lump on the left side of his jaw, which had grown in 2 months. Ultrasound sonography test examination revealed a cystic mass that was 24×17 mm in size with smooth contours. Multiple echogenic and reactive lymph nodes with partially visible hila were visualized in the neighboring upper jugular chain, with the largest being 16×10 mm in size. Following a neck magnetic resonance imaging and a preliminary diagnosis of WT, a left superficial parotidectomy was conducted. The patient had a history of smoking, hypertension, and coronary artery disease.\nLeft superficial parotidectomy materials were sent for pathologic examination in two pieces, which were 5×3.2×2 cm and 4.5×3×1.2 cm in size. Cross section analysis showed an off white-yellowish, well-contoured nodular tumor with a bleeding center of 4×2.5×2.2 cm. Microscopic examination indicated that the tumor had epithelial components with basaloid and oncocytic columns of cells neighboring lymphoid components (). In addition to the lymphoid follicles with distinct germinal centers, infiltration of large neoplastic cells with bizarre and extremely atypical morphology was seen in the lymphoid component (, ). The large neoplastic lymphoid cells constituted more than 50% percent of the lymphoid component.\nImmunohistochemical examination showed that neoplastic cells expressed strong positivity for CD20 (), CD79a, CD30 (), leukocyte common antigen, IgG, CD138, MUM1, and focal positivity for kappa. Staining for lambda, IgM, IgA, CD3, CD5, CD10, CD15, CD56, epithelial membrane antigen, Bcl2, Bcl6, cyclinD1, S100, pancytokeratin, cytokeratin 20, human melanoma black 45, actin, and desmin were negative. Latent Epstein-Barr virus (EBV) was shown to be negative in tumor cells by using EBV-encoded RNA chromogenic in situ hybridization. Due to these findings, the patient was diagnosed with "WT and CD30 positive diffuse large B-cell lymphoma in the parotid gland."\nFollowing the lymphoma diagnosis, a full body screen was performed. Results indicated lymphadenopathies of a pathologic size in the inguinal and iliac regions. In addition to these findings, the left suprarenal gland showed two nodular mass lesions, which were assessed as likely adenomas; however, this preliminary diagnosis was not confirmed by histopathology. Bone marrow biopsy revealed a normocellular bone marrow with no lymphoma involvement.\nThe patient was stage 3A and received six courses of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) therapy. During 6-month follow-up, the patient was free of disease. | [[60.0, 'year']] | M | {'10631864': 1, '17978770': 1, '16045788': 1, '20346432': 1, '3786253': 1, '31799320': 2, '19294485': 1, '31209701': 1, '15735173': 1, '32328910': 1, '31933816': 1, '17548964': 1, '8495960': 1, '33041588': 2, '19956447': 1, '24421853': 2} | {'6887602-1': 1, '7518491-1': 1} |
165,161 | 3887163-1 | 24,421,854 | noncomm/PMC003xxxxxx/PMC3887163.xml | Cystic Benign Phyllodes Tumor in the Inguinal Region | A 51-year-old female was admitted to our hospital for the evaluation of a mass in the right groin area that had been present for two years. She had no specific clinical history. A mass lesion was identified with positron emission tomography-comcomputed tomography. The mass was 27 mm in the lmgest diameter with F-18 fluoro-deoxyglucose uptake (SUVmax 1.4), and it was located on the superolateral side of the inguinal area (). The mass was excised under local anesthesia. A differential diagnosis of lymphoma is the major concern in this case.\nThe resected specimen was 4×3 cm in size and the tumor was well circumscribed. The mass showed numerous papillary nodular protrusions into a central elongated cystic cavity (3.5×2.5 cm). No necrosis or hemorrhage was observed within the mass (). Eccentrically, a whitish gray, solid soft tissue area (1.2 cm in diameter) was present.\nThe microscopic findings showed a central elongated cyst with intraluminal projections (), which were composed of hyperplastic epithelium-lined stroma (). Stromal cells had no mitosis, low-to-moderate cellularity, and mild cytologic atypia (). The lining epithelial cells were strongly positive for estrogen and progesterone receptors (). In the periphery of the mass, normal breast tissue was identified (). The final pathological results were consistent with a benign PT with prominent intracystic growth, derived from an ectopic breast. Three months after the procedure, the patient was doing well without any recurrence. | [[51.0, 'year']] | F | {'21169735': 1, '17394506': 1, '8181811': 1, '21393917': 1, '2161206': 1, '12525768': 1, '20514681': 1, '19125265': 1, '9861239': 1, '8394655': 1, '11469353': 1, '9553815': 1, '20551574': 1, '24421854': 2} | {} |
165,162 | 3887164-1 | 24,421,855 | noncomm/PMC003xxxxxx/PMC3887164.xml | Peritoneal and Nodal Gliomatosis with Endometriosis, Accompanied with Ovarian Immature Teratoma: A Case Study and Literature Review | A 34-year-old female (G2P2A0L2) visited our hospital with an eight-month history of a palpable abdominal mass. She had a history of two cesarean sections. Normal beta-human chorionic gonadotropin with increased alpha-fetoprotein (22.7 ng/mL), cancer antigen (CA) 125 (61.7 U/mL), and CA19-9 (553.7 U/mL) were observed. Contrast-enhanced computed tomography showed a large solid and cystic mass in the left lower abdomen and pelvis. A huge left ovarian tumor filling the peritoneal cavity was found during laparotomy, and yellow-colored ascites (200 mL) was expelled. The external surface of the tumor was pinkish-tan, transparent, smooth, and intact. No pelvic adhesions were observed. Multiple firm, gray-white nodules measuring 0.1 to 0.3 cm were observed on the omentum, uterine serosa and posterior cul-de-sac peritoneum. One surface hemorrhagic nodule that measured 0.3 cm was found on the right ovary. Left salpingo-oophorectomy, right wedge resection, partial omentectomy, and biopsy of the uterine serosal nodules were performed. Under the impression of ovarian malignant tumor, intraoperative frozen biopsies were taken from the omental nodules, left ovary and salpinx. For staging, pelvic lymph node sampling, partial omentectomy and posterior cul-de-sac peritoneal resection were performed.\nGrossly, the excised left ovarian mass measured 21.0 cm in diameter and weighed 2,350 g. A predominantly solid mass with multicystic changes, which were filled with clear watery fluid, was observed on the cut surface (). Thirty sections were taken from the ovarian tumor, and various mature tissues of tridermal lineage were demonstrated (); the mass was comprised predominantly of mature glial tissue (90%) with focal immature neuroepithelial cells forming rosette-like structures with few mitosis (). Some mature neurons were also observed within the glial tissue. Choroid plexuses were also found. These immature neuroectodermal foci, including primitive mesenchyma, led to the diagnosis of an immature teratoma, grade 1, according to the classification of Robboy and Scully. There were no other germ cell tumor components, including a yolk sac tumor. The peritoneum, cul-de-sac and uterine serosa, as well as the opposite ovarian surface, showed involvement of glial peritonei (grade 0). Nodules of the right ovary, peritoneum and omentum were composed of glial tissue (grade 0) (). Out of 32 dissected lymph nodes, one left hypogastric lymph node showed one mature glial implant, i.e., nodal gliomatosis (grade 0) (, inset). Endometrial glands with stromata were found within the peritoneal nodules (). Some scattered psammoma bodies were observed in peritoneal and nodal gliomatosis, as well as brain tissue in the ovarian immature teratoma. Immunohistochemically, glial fibrillary acidic protein (prediluted, polyclonal, Dako, Glostrup, Denmark) (, left) immunostaining confirmed the glial nature of the tissue. The endometrial stroma of endometriosis was stained with CD10 (56C6, prediluted, Dako) (, right).\nThree cycles of chemotherapy of bleomycin, etoposide and cisplatin (BEP) were performed. After the surgery, alpha-fetoprotein, CA125, and CA19-9 levels reached the normal range. During the nine-month post-operative follow-up period, the patient had an uneventful course. | [[34.0, 'year']] | F | {'10233836': 1, '2429641': 1, '3338678': 1, '21293285': 1, '2726609': 1, '12556543': 1, '13798885': 1, '18541896': 1, '7928061': 1, '8878019': 1, '4447001': 1, '5521737': 1, '6482446': 1, '34586943': 2, '11750968': 1, '9876685': 1, '27473411': 1, '15963555': 1, '581738': 1, '7167411': 1, '24421855': 2} | {'8485300-1': 1} |
165,163 | 3887165-1 | 24,421,856 | noncomm/PMC003xxxxxx/PMC3887165.xml | Cytomorphological Findings and Histological Correlation of Low-Grade Cribriform Cystadenocarcinoma of Salivary Gland in Fine-Needle Aspiration: A Case Study | A 57-year-old man had a barely palpable, nontender mass in the right cheek area, followed up over a nine-month period. Contrast-enhanced computed tomography revealed a mildly enhancing mass in an anterior superficial lobe of the right parotid gland (). On ultrasonography, the lesion appeared as an approximately 1.2 cm large, multiseptated, cystic, solid nodule (). FNA was performed and the cytologic examination led to a diagnosis of ductal epithelial proliferative lesion, favoring neoplasm, of uncertain malignant potential. Histologic confirmation was required.\nExcision of the lesion from the right parotid gland was performed. The surgical specimen revealed a tiny multicystic nodule (0.7 cm), which appeared to be surrounded by normal salivary gland tissue. The cut surface of the tumor was partly solid and partly cystic (). Microscopically, it was a salivary ductal epithelial neoplasm showing multiple cysts lined by relatively bland ductal cells and a partly solid proliferation of ductal cells (). Many of the superficial lining cells contained cytoplasmic apocrine-type microvacuoles that were periodic acid-Schiff-positive/diastase-resistant () and mucicarmine-positive (). The tumor cells were strongly and diffusely S-100 protein positive (). Myoepithelial markers (including actin and p63) highlighted the presence of cells surrounding the cystic spaces, confirming the intraductal nature of the lesion (). No invasive foci were noted. On the basis of these findings, we diagnosed the mass as a low grade cribriform cystadenocarcinoma, marginally excised.\nThe cellular smear showed many large sheets or clusters of ductal epithelial cells in an irregular overlapping arrangement (). These cytologic components could be aspirated from the solid area of this tumor (). Architecturally, the tumor cells had tight intercellular connections forming a vague cribriform (), solid, or pseudopapillary () arrangement. These were matched with histologic findings. The solid areas revealed a cribriform pattern, resembling ADH and low-grade ductal carcinoma in situ of the breast (). The pseudopapillary projections were found in the epithelial lining the cystic areas (). Cytologically, almost all tumor cells were bland looking, ductal epithelial cells with low nuclear/cytoplasmic (N/C) ratio. The nucleoli were found to be small, single, or entirely absent. Nuclear chromatin was fine or slightly coarse (). Squamoid or metaplastic changes of the tumor cells, mentioned by Nakazawa et al., were also identified in this case. Nuclear atypia was inconspicuous; however, one focus showed mild atypia with minimal nuclear size variation, prominent nucleoli, and vacuolated cytoplasm (, ). The background was relatively clear in the cystic space. Scattered inflammatory cells with hemosiderin-laden macrophages, neutrophils, and eosinophils were seen. No evidence of necrosis or mucin was found.\nWhole-body positron emission tomography-computed tomography scanning was conducted after surgery. A residual, enhancing nodular lesion in the anteriosuperior aspect of the right parotid gland showed mild, increased fluorodeoxyglucose uptake. Overall, residual malignancy was suggested. Postoperative chemotherapy or radiation therapy was not recommended. A follow-up was conducted two months later. At that time, the patient was healthy, with no evidence of recurrence or distant metastasis. | [[57.0, 'year']] | M | {'19477092': 1, '21068068': 1, '18983607': 1, '22151879': 2, '15252310': 1, '8780532': 1, '21319326': 1, '30555776': 1, '21442195': 1, '24421856': 2} | {'3307471-1': 1} |
165,164 | 3887166-1 | 24,421,857 | noncomm/PMC003xxxxxx/PMC3887166.xml | Mediastinal Thymolipoma with Striated Myoid Cells: Report of a Peculiar Case | A 54-year-old man had a large asymptomatic anterior mediastinal mass incidentally discovered by chest radiograph during a regular health check-up. He had no history of autoimmune diseases including myasthenia gravis, aplastic anemia, hypogammaglobulinemia, lichen planus, or Graves' disease. A computed tomography scan of the chest demonstrated a large mediastinal mass consisting of a mixture of fat and soft tissue contents, radiologically suggesting thymolipoma ().\nThe tumor was surgically removed. Grossly, it was fairly wellcircumscribed by a thin fibrous capsule, and measured 12.0×10.0×3.5 cm. The outer surface was soft, yellow fatty tumor with focal solid areas (). Histologically, the mediastinal mass was characterized by the presence of abundant mature adipose tissue admixed with areas containing remnants of thymic tissue (). The thymic tissue component varied from strands of atrophic thymic epithelium to large areas containing thymic parenchyma with medullary epithelial hyperplasia (). In areas, the tumor showed a frequent occurrence of polygonal, striated myoid cells with abundant eosinophilic cytoplasm (). These cells showed positive immunoreaction for desmin (1:200, clone D33, Dako, Glostrup, Denmark) and myoglobin (1:800, RB-9068-R7, Thermo, Fremont, CA, USA) in immunohistochemical study (), but negative reaction for alpha smooth muscle actin (1:400, Dako). The myoid cells also lacked cytological atypia and mitotic figures. | [[54.0, 'year']] | M | {'45151': 1, '15504081': 1, '33489056': 2, '17557569': 1, '7015872': 1, '2321702': 1, '2069215': 1, '8539231': 1, '24421857': 2} | {'7768564-1': 1} |
165,165 | 3887167-1 | 24,421,858 | noncomm/PMC003xxxxxx/PMC3887167.xml | Inflammatory Pseudotumor-like Follicular Dendritic Cell Tumor of the Spleen with Extensive Histiocytic Granulomas and Necrosis: A Case Report and Literature Review | A 76-year-old man underwent abdominal-pelvic computed tomography for follow-up of previous gastric cancer (disease-free state for five years after radical subtotal gastrectomy). A newly developed splenic mass had been increasing in size for the previous six months (1.9 to 3.4 cm) (). There was no evidence of local recurrence or metastasis, even on positron emission tomography-computed tomography. A splenectomy was performed. A well-defined gray-whitish firm nodule (3.2 cm in greatest dimension) was detected (). Microscopically, the well-defined lesion was composed of an admixture of inflammatory cells and spindle cells in a background of numerous histiocytic granulomas and central coagulative necrosis (). The oval to spindle cells had pale or eosinophilic cytoplasms with indistinct cell borders. The nuclei were enlarged and twisted or irregularly folded. Small but distinct, centrally located nucleoli were often evident (). Mitotic figures were not found. The infiltrating reactive inflammatory cells were predominantly composed of lymphocytes, plasma cells, and a small number of eosinophils. The immunohistochemical results are as follows: CD35+ (), CD21-, smooth muscle actin (SMA)-, and anaplastic lymphoma kinase- in oval to spindle cells, mixed imfiltration of CD3+ and CD20+ lymphocytes, and CD68+ in histiocytes (). The Ki-67 labeling index was about 30% in spindle cells. EBV in situ hybridization (EBV-ISH) revealed diffuse and strong positivity in oval to spindle cells (). Special stains for bacteria, acid-fast bacilli and fungi showed no organisms. | [[76.0, 'year']] | M | {'11301353': 1, '25766434': 1, '14720144': 1, '16533803': 1, '11774173': 1, '11395549': 1, '15029264': 1, '12866373': 1, '31908727': 1, '11231487': 1, '24421858': 2} | {} |
165,166 | 3887168-1 | 24,421,859 | noncomm/PMC003xxxxxx/PMC3887168.xml | Micropapillary Mucinous Adenocarcinoma of the Lung: A Brief Case Report | A 69-year-old woman presented with persistent cough for one month previously. The patient had a 13 pack-year smoking history and hypertension. Chest computed tomography revealed predominant ground glass opacity with patchy consolidation occupying almost the entire left lower lobe of the lung (). It was histologically confirmed as adenocarcinoma of the lung by transbronchial lung and lymph node biopsy. Analysis of EGFR and K-ras mutation by polymerase chain reaction-sequencing was performed on tissue from the lymph node biopsy, which revealed missense mutation of K-ras in the 12th codon, c.34G>T (p.G12C), without EGFR mutation. After neoadjuvant concurrent chemoradiation, a left lower lobectomy was performed. The gross examination revealed a poorly defined, large, mass-like lesion with scattered dilated bronchus, which was similar in appearance to lobar pneumonia and well correlated with radiologic findings ().\nMicroscopically, the tumor demonstrated a predominantly micropapillary growth pattern in the alveolar cavity and a lepidic growth pattern along the septa with hobnail features (). Neoplastic cells with abundant clear or amphophilic cytoplasm intermingled with each other (). Special stains (periodic acid Schiff, mucicarmine, and Alcian blue pH 1.0 and 2.5) revealed positivity in the cytoplasm of a few tumor cells (, inset). Tumor cells had well-defined cell borders and large, pleomorphic nuclei (), with occasional horse-shoe shaped nuclei and perinuclear clearing. Their nuclei had a contorted prominent membrane, similar to the raisinoid appearance induced by human papillomavirus infection, and had frequent prominent macronucleoli ().\nNo complications were noted in the two-month follow-up period after discharge. | [[69.0, 'year']] | F | {'25813151': 1, '22393100': 1, '19369630': 1, '15464447': 1, '18571764': 1, '21387273': 1, '17591931': 1, '20073607': 1, '22020674': 1, '21252716': 1, '24421859': 2} | {} |
165,167 | 3887169-1 | 24,421,860 | noncomm/PMC003xxxxxx/PMC3887169.xml | Primary Gastric Melanoma with Rhabdoid Features: A Case Report | A 59-year-old male presented with a gastric mass that was found incidentally on gastroesophagoscopy. The only relevant history was a recent cerebral hemorrhage that occurred three months prior. Endoscopically, the mass was polypoid in appearance at the posterior wall of the fundus (). Stomach computed tomography showed an enhancing mass measuring 4.0 cm in diameter with central necrotic change at the cardia, and one enlarged regional lymph node, measuring 1.0 cm in diameter, posterior to the cardia. Under the impression of gastric carcinoma, total gastrectomy was performed. Preoperative endoscopic biopsy was not undertaken. A fungating grayish-tan colored mass was found at the posterior wall of the cardia, involving the esophagogastric junction. The cut surface of the polypoid mass revealed a homogeneous grayish-tan in appearance with focal hemorrhagic changes and focal mucosal ulcer. The mass invaded mucosa, submucosa, and inner proper muscle. It extended to the esophagogastric junction. Histologically, a solid growth pattern and pseudoalveolar clefts were found (). The mass was infiltrated by round, small-to-large-sized cells with plump eosinophilic cytoplasm and peripherally compressed nuclei, creating an occasional rhabdoid appearance. The tumor cells had an irregular shape with angulated nuclei, irregular nuclear contours, and fine chromatin. Occasional marked pleomorphic cells were found. Mitotic figures were counted up to 15/10 high power fields. Metastatic foci were demonstrated in one of 20 regional lymph nodes. The observed normal-appearing gastric and esophageal mucosa showed no abnormalities. Under the impression of malignant melanoma, gastrointestinal stromal tumor, poorly differentiated rhabdomyosarcoma, myeloid sarcoma, lymphoma, or plasmacytoma, immunohistochemistry and electron microscopic examination was performed. The tumor cells, both rhabdoid and nonrhabdoid, were diffusely positive for vimentin (prediluted, V9, Dako, Glostrup, Denmark) and S-100 protein (prediluted, polyclonal, Dako) (). They showed focal nuclear positivity for Sox 10 (prediluted, goat polyclonal, Santa Cruz Biotechnology Inc., Santa Cruz, CA, USA) (). They were negative for human melanoma black-45 antigen (HMB-45 antigen, prediluted, Dako), Melan-A (prediluted, A103, Dako), CD117 (prediluted, c-kit, Dako), CD34 (prediluted, QBEnd10, Dako), calretinin (1:100, calret 1, Dako), pancytokeratin (prediluted, AE1/AE3, Dako), epithelial membrane antigen (prediluted, E29, Dako), myoD-1 (1:50, 5.8A, Dako), myogenin (1:50, MyG007, Biocare Medical, Concord, CA, USA), smooth muscle actin (prediluted, IA4, Dako), desmin (prediluted, D33, Dako), synaptophysin (prediluted, SY38, Dako), chromogranin (prediluted, DAK-A3, Dako), CD56 (1:100, 123C2, Dako), CD68 (1:50, PG-M1, Dako), leukocyte common antigen (prediluted, Dako), CD79a (1:50, JCB117, Dako), CD138 (prediluted, MI/5, Dako), and CD99 (prediluted, 12E7, Dako). Staining with Fontana-Masson technique highlighted dark, black-colored cytoplasmic granular pigments within the tumor cells just beneath the ulcer (), although some of those melanin pigments were identified on hematoxylin and eosin staining. The cells showed negative results for Prussian blue, periodic acid-Schiff, myeloperoxidase, toluidine blue, lysozyme, and terminal deoxynucleotidyl transferase tests. Ultrastructurally, closely-apposed oval-shaped tumor cells displayed a moderate amount of cytoplasm containing mitochondria and secondary lysosomes. Extensive repeated ultrastructural evaluation revealed a few stage-3 melanosomes (). Some tumor cells had focal paranuclear accumulation of intermediate filaments with entrapped organelles (). No demonstrable cell junctions were observed. All of these findings were consistent with malignant melanoma with rhabdoid features. The patient was re-evaluated. Upon careful physical examination of his entire body surface, including oral and anal mucosa, no lesions were found, and fundoscopic examination of the eye was also normal. He was diagnosed as a presumptive primary malignant melanoma with a rhabdoid phenotype, although no demonstrable intraepithelial melanocytosis or in situ lesion was observed. Three months later, the patient developed low back pain; spine magnetic resonance imaging with enhancement study showed a compression fracture at L3 and a small bone marrow lesion at S5, suggesting a metastatic spinal tumor at L3 and S5, which was confirmed to be meta static melanoma by percutaneous needle biopsy. Vertebroplasty was performed, and during the four months following the operation, palliative chemoradiotherapy with dacarbazine was administered. | [[59.0, 'year']] | M | {'22148039': 2, '8092393': 1, '25918612': 2, '22713795': 1, '15163228': 1, '9557778': 1, '25400765': 1, '16446716': 1, '19318811': 1, '7588356': 1, '6142031': 1, '7785665': 1, '33318781': 1, '24421860': 2} | {'3229054-1': 1, '4387358-1': 1} |
165,168 | 3887233-1 | 24,454,425 | noncomm/PMC003xxxxxx/PMC3887233.xml | Palatal-Myoclonus as a Presentation of Hashimoto Encephalopathy: an interesting case report | A previously healthy 32 years old; veterinarian male, referred to the emergency department complaining about 2 episodes of generalized tonic clonic seizure within the past 24 hours.\nAccording to his wife, he had depressed mood and psychomotor slowing during the past 3 weeks. He also had episodes of confusion, agitation, visual hallucinations, decrement of interpersonal relationship, generalized purities and flushing.\nThree days before admission, he had abnormal movements of the palate and tongue.\nOn physical examination, the patient was ill and lethargic. There was a low grade fever (Temp: 37.4 Auxiliary), but other vital signs were normal. His neck was supple. On general examination, there were itching excoriations on his skin, but other systemic examinations were normal. Neurologic examination was normal, apart from mild confusion and myorrhythmic movements of tongue and soft palate. The patients MRI showed abnormal symmetric hyper signality in both caudate nuclei and putamens on T2 and FLAIR. () There was no abnormal signal in medulla (inf.olive nucleus). ()\nThe CSF was clear with normal level of protein and glucose. There were 30 red blood cells, 20 lymphocytes and no polymorphonuclear in CSF. PCR for herpes simplex virus and mycoplasma were negative. The EEG showed intermittent delta and theta activity. Routine blood counts, renal function tests and glucose were within normal limits. Erythrocyte sedimentation rate (ESR) and C-reactive protein were normal. Liver function tests were elevated. Antinuclear antibodies, anti-ds-DNA, anti SS-A, anti SS-B, lupus anticoagulant, anti phospholipid Ab and RF were normal. The human immunodeficiency virus antibody was negative. Chest radiograph and ultrasonographic scan of the abdomen did not reveal any tumor. Thyroid function tests were within normal limits, but the thyroid thyroglobulin antibody (TGHA) titer was 120 IU/mL (normal < 22 IU/ml) and the thyroid peroxidase antibody (Anti-TPO) titer was 356U/mL (normal < 60 IU/ml).\nA diagnosis of HE was made, and the patient was treated with oral prednisolone 1 mg/kg/d, levothyroxin and anticonvulsants. Before starting treatment with prednisolone, the patient had 3-4 episodes of simple focal seizures per day which were not controlled with antiepileptic drugs. Less than 24 hours after the initiation of prednisolone, the palatal myorrhythmic movements, itching, and flushing were subsided and focal seizures never repeated. We did not do needle biopsy of thyroid gland. On the follow-up, at days 10 and 40, the patient had no seizures and the palatal myorrhythmic movements disappeared; and EEG was normal at 40th. The Anti- TPO level was decreased to 73 IU/ml after 6 months. | [[32.0, 'year']] | M | {'33101766': 1, '17878654': 1, '16525066': 1, '16476807': 1, '26629227': 1, '31388971': 1, '4161638': 1, '32775033': 2, '22207137': 1, '27957468': 1, '12580699': 1, '10023109': 1, '11839870': 1, '16786216': 1, '22754574': 2, '24454425': 2} | {'7394228-1': 1, '3382317-1': 1} |
165,169 | 3887994-1 | 24,968,440 | noncomm/PMC003xxxxxx/PMC3887994.xml | Duodenal web associated with malrotation and review of literature | A 1-day-old baby girl, born by normal spontaneous vaginal delivery, was evaluated for abdominal distension and history of having not passed meconium. An abdominal X-ray was suspicious for malrotation (Fig. ). The patient did not have any evidence of sepsis. An echocardiogram ruled out any congenital cardiac anomalies.\nThe patient underwent an exploratory laparotomy; findings demonstrated a distended duodenum with cecum and appendix at the left upper quadrant fixed by Ladd's bands. The contents of the stomach could not be milked down distally. A duodenotomy incision identified a large duodenal web with a blind pouch (Fig. ). Distal to the web, the duodenum had normal caliber lumen. The duodenal web was resected and the duodenotomy was closed primarily. The cecum was positioned in the left upper quadrant. An appendectomy was performed and the abdomen closed primarily.\nPostoperative care included monitoring in neonatal intensive care unit. Upper GI series (Figs and ) demonstrated no evidence of leakage. The patient tolerated a trial of pedialyte feedings and was subsequently discharged home. | [[1.0, 'day']] | F | {'27732773': 2, '16677901': 1, '17705733': 1, '8429481': 1, '16518597': 1, '11727020': 1, '17292689': 1, '19241073': 1, '17594104': 1, '26951229': 1, '19274797': 1, '25926924': 1, '17517978': 1, '24968440': 2} | {'5398358-1': 1, '5398358-2': 1, '5398358-3': 1, '5398358-4': 1} |
165,170 | 3887995-1 | 24,968,431 | noncomm/PMC003xxxxxx/PMC3887995.xml | A transmesenteric congenital internal hernia presenting in an adult | A 32-year-old man undergoing treatment for a depression with serotonin–norepinephrine reuptake inhibitors (SNRIs) and with one prior admission to the hospital due to rightsided abdominal pain was admitted to our department after 4 h with severe abdominal pain. The pain was acute in onset, stabbing and located in the whole right side of the abdomen, radiating to the back and to the right groin. The pain was associated with nausea and restlessness, and could not be reduced with either NSAID or high doses of morphine. On presentation, he was ill looking, sweating, restless and in agony. He was ABC stable, his abdomen distended with generalized tenderness and in his upper right quadrant he had a 10 × 10-cm hard very sore mass, with positive rebound tenderness. There were normal bowel sounds.\nParaclinical tests showed leucocytosis (10,1 × 109/l) and normal serum lactate. A computed tomography (CT) scan showing gastric retention, encapsulated dilated small intestines in the right upper quadrant with pneumatosis intestinalis and collapsed small intestines distally from this area interpreted as intestinal obstruction (Figs and ).\nAn emergency laparotomy was performed 12 h after the first appearance of the symptoms and 7 h after admission to the hospital. This revealed severe small bowel obstruction. Approximately 1 m of small bowel was found distended, herniated and strangulated through a 2-cm defect in the mesentery of the transverse colon. Furthermore, it was capsuled in the mesenteric peritoneum, resembling a balloon ready to burst. The intestines were greyish in colour, but vital without signs of necrosis or ischaemia (Fig. ). The peritoneal capsule was then removed and the defect widened, before the herniated small bowel could be loosened and retracted back through the hernia. The defect was sutured. The postoperative course was smooth, and the patient was discharged 4 days later. The patient has not been readmitted to the hospital at 18 months follow-up, but did complain of mild pain in the upper abdomen the first few weeks after discharge. | [[32.0, 'year']] | M | {'27999703': 2, '28685016': 1, '28700968': 1, '20152371': 1, '18618070': 1, '11687686': 1, '13727914': 1, '11996230': 1, '30487968': 2, '20336475': 1, '19727554': 1, '20091075': 1, '17458600': 1, '24968431': 2} | {'6250910-1': 1, '5141326-1': 1} |
165,171 | 3887996-1 | 24,968,438 | noncomm/PMC003xxxxxx/PMC3887996.xml | Congenital duodenal obstruction associated with Down's syndrome presenting with hematemesis | A 2-day-old girl was referred to our service with sudden onset of severe hematemesis. Prenatal history was significant for intrauterine growth retardation. She was born at the gestational age of 37/40 with a birth weight of 2.1 kg. On examination she was found to have dysmorphic features suggestive of Down's syndrome, along with hypotonia, intercostal muscle retraction and a mildly distended epigastric region. She was suspected of meconium aspiration and sepsis. Her initial investigations revealed WBC of 20 100 μl, Hb of 16.8 g/dl, platelets of 114 000/μl and CRP of 10 md/l. After intravenous antibiotics, she remained stable but on Day 2 of life, she was noted to have high levels of orogastric aspirates mixed with blood. A plain X-ray revealed a dilated stomach and duodenum, with air in the distal small bowel (Fig. ). An APT test was negative. Repeat laboratory evaluation revealed WBC of 23 200 μl, Hb of 10.1 g/dl and platelets of 140 000/μl. She was started on ranitidine 10 mg daily but she continued to have fresh blood in her orogastric tube and her hemoglobin level dropped to 8.0 g/dl with INR 10, PT 120′ and APTT 180′. She was managed accordingly and after resuscitation, an upper GI contrast study was performed to rule out malrotation or mid-gut volvulus. Her upper GI contrast suggested an incomplete duodenal obstruction (Fig. ). To look for a source of bleeding, an upper GI endoscopy was not performed due to duodenal obstruction and a risk of perforation. The baby was taken to the operating theater for exploration to determine the cause of her ongoing hematemesis and to correct her duodenal obstruction. On exploration, her stomach and proximal duodenum were dilated with a narrowing noted in the second part of the duodenum. There was no malrotation of her gut and the rest of the bowel and solid viscera were normal. A duodenotomy of her proximal dilated duodenum revealed a complete diaphragm with a hole allowing passage of a small probe down into the distal duodenum. The mucosa of the proximal dilated duodenum was edematous and friable with multiple erosions. She underwent diamond-shaped duodenoduodenostomy for her CIDO. Her postoperative course was uneventful and hematemesis resolved. She was started on gradual nasogastric to oral feeding, which she tolerated very well. Further investigations revealed a right ectopic kidney and minor congenital cardiac defects. Her chromosomal analysis showed a karyotype of 47, XX, + 21 consistent with Down's syndrome. | [[2.0, 'day']] | F | {'21760545': 1, '32015925': 2, '2943789': 1, '19021635': 1, '5331056': 1, '4271490': 1, '1532826': 1, '26951229': 1, '15185215': 1, '125724': 1, '24968438': 2} | {'6988673-1': 1} |
165,172 | 3887997-1 | 24,968,446 | noncomm/PMC003xxxxxx/PMC3887997.xml | Surgical emphysema following therapeutic colonoscopy | A 60-year-old female presented to our endoscopy suite after being referred by her general practitioner for investigation of bleeding and passage of mucus per rectum. She also complained of tenesmus and reduced appetite but no weight loss. Her past medical history included being diagnosed with ulcerative colitis 26 years ago during her first pregnancy for which she was treated with steroids and has been relatively healthy since then. She had a previous colonoscopy in 2008, following a positive bowel screening. This colonoscopy showed quiescent colitis confirmed by biopsy. During the present colonoscopy, she had biopsies taken from her rectum, sigmoid, descending and ascending colon given the findings of pseudopolyps.\nAfter her procedure she complained of swelling in her neck. This was not accompanied by shortness of breath, chest pain or abdominal pain. On examination, her oxygen saturation was 99% on room air, with normal respiratory and heart rate. Her respiratory examination was normal and her abdomen was soft and non-tender. She had marked crepitus over her clavicles, supraclavicular fossa and most of her neck. Her erect chest X-ray revealed air under both hemidiaphragms and significant pneumomediastinum (Fig. ). A computed tomography (CT) scan of her chest, abdomen and pelvis was requested. This CT-scan revealed very extensive spreading of surgical emphysema in the retroperitoneum, extending into the mediastinum with small volumes of free intraperitoneal gas (Figs –).\nThe patient was admitted and managed conservatively. This consisted of keeping her nil by mouth for 24 h, intravenous fluids and antibiotics. Her cervical emphysema gradually resolved and she was discharged 72 h after presentation without any surgical intervention. | [[60.0, 'year']] | F | {'18938166': 1, '15481289': 1, '8918445': 1, '14520289': 1, '6375617': 1, '8751774': 1, '20101766': 1, '19251006': 1, '17688713': 1, '23819071': 2, '24968446': 2} | {'3683440-1': 1} |
165,173 | 3887998-1 | 24,968,439 | noncomm/PMC003xxxxxx/PMC3887998.xml | Metastatic malignant soft tissue myoepithelioma: a case report showing complete response after locoregional and systemic therapy | A 61-year-old male patient with no particular previous illnesses was diagnosed with a well-differentiated STMM of the plantar surface of the second toe of the right foot, with infiltrated margins after excisional biopsy. Microscopically, myoepithelioma consists of nests and cords of epithelioid, plasmacytoid or spindle cells, with inconspicuous nucleoli, mainly eosinophilic cytoplasm and, in some cases, clear cells. The cells are embedded in a chondromyxoid, collagenous or hyalinized stroma; no significant nuclear pleomorphism is usually observed and mitoses are scarce. The malignant variant is exclusively recognized by its histological features, showing a marked cytologic atypia, prominent nucleoli, necrosis and a high mitotic index (>7/10 HPF) [, ]. The patient underwent computerized tomography (CT) of the chest and abdomen for staging; the results were normal. Considering the residual disease in the previous excision of the nodule, the patient underwent a radical disarticulation of the toe 20 days after excisional biopsy. Three months later, the patient presented a lump on the plantar surface of the third toe of his right foot, a subcutaneous nodule on the right ankle and another nodule on the back of the foot; excisional biopsy of the latter showed de-differentiated STMM. Because of these findings, a positron emission tomography-CT (PET-CT) [] was performed, which showed the presence of in-transit metastases in the right lower limb, extending to the middle third of the thigh. Ultrasound-guided fine needle aspiration cytology (FNAC) was performed at the level of the most accessible of these lesions, which confirmed secondary STMM (Fig. ). The patient was then treated with hyperthermic isolated limb perfusion (HILP) using human recombinant tumor necrosis factor 1 mg and melphalan 90 mg. During the subsequent follow-up, a complete response was documented (Figs. and ).\nFourteen months after the removal of the primary tumor, a pelvic magnetic resonance imaging (RMI) identified the presence of lymph nodes highly suspicious for malignancy in the right groin region. FNAC confirmed the presence of metastases from STMM and a complete surgical dissection of the inguinal-iliac-obturator lymph nodes was performed. The histological report documented the presence of metastases from differentiated STMM at the inguinal and iliac lymph nodes. During the following months, the patient was followed up clinically and radiologically, and all examinations were negative.\nTwenty-two months after the removal of the primary tumor, pathologic non-lymphatic nodules at the inguinal and iliac areas were noticed and initially treated with cisplatin plus doxorubicin, but concomitant pelvic progression was ascertained. Afterwards, three cycles of ifosfamid in continuous infusion (14 g/mq in 14 days every 28 days) and radiotherapy (50.4 gray in 28 fractions to the inguinal-iliac-obturator region and one boost of 64.8 gray into the area where the recurrences were found) were administrated with complete response. Currently, 36 months from the original diagnosis, the patient is alive and in good health, and undergoes follow-up examinations every 4 months. | [[61.0, 'year']] | M | {'28390395': 2, '15998380': 1, '7571084': 1, '12960802': 1, '17498593': 1, '10389622': 1, '15120917': 1, '15722831': 1, '24968439': 2} | {'5385017-1': 1} |
165,174 | 3887999-1 | 24,968,442 | noncomm/PMC003xxxxxx/PMC3887999.xml | Management of a bilobed testicle in a 12-year-old boy | A 12-year-old boy was referred by his GP to the paediatric surgery clinic in a district general hospital with a 6-week history of a lump near the inferior pole of his left testicle. It was non-tender, non-erythematous and had not changed in size or consistency. He was otherwise fit and well, with no family history of urological disorders. On examination, his abdomen was soft and non-tender, with a normal right testis and right spermatic cord. A smooth well-defined lump was palpated on the inferior pole of the left testis. The rest of the testicle had a normal consistency with a normal left cord. Ultrasound demonstrated that both testicles were of a similar size, with normal vascularity and normal epididymi. The palpable lump corresponded to a well-defined 1-cm mass attached to the lower pole of the left testicle (Fig. ). It had a similar echo pattern to the testicle with normal vascularity (Fig. ), consistent with the finding of a bilobed testis.\nThe boy was reviewed 3 months later in outpatient clinic. The size of the lump was unchanged, although he now reported intermittent pain and swelling of the lump. He was referred to a consultant paediatric urologist and his testicle rescanned 6 months after his initial presentation. He was asymptomatic at this time. The ultrasound findings were unchanged and confirmed normal testicular architecture. He will be reviewed again in 12 months, with a view to discharging him if no further changes are seen. | [[12.0, 'year']] | M | {'23164013': 1, '21234118': 2, '20019962': 1, '23293690': 1, '27904607': 2, '34150520': 1, '1493653': 1, '15292564': 1, '19765760': 1, '3547281': 1, '24968442': 2} | {'2994354-1': 1, '5122000-1': 1} |
165,175 | 3888000-1 | 24,968,437 | noncomm/PMC003xxxxxx/PMC3888000.xml | Primary ectopic breast carcinoma in a supernumerary breast arising in the anterior chest wall: a case report and review of the literature | A 48-year-old female presented with a 6-week history of a tender mass inferior to the right inframammary fold following trauma.\nAntibiotics were initiated for presumed infection; however, the mass persisted, prompting surgical referral. Clinical examination demonstrated a 2 × 1 cm superficial mass, with adjacent accessory nipple. An ultrasound confirmed a 15-mm hypo-echoic mass consistent with a sebaceous cyst; however, a core biopsy confirmed a Grade 2 invasive ductal carcinoma (IDC), strongly positive for oestrogen and progesterone receptors (Allred scores of ER 7, PR7). Immunohistochemistry for Her 2 status was negative (0).\nMagnetic resonance imaging (MRI) confirmed a 22 × 12 mm spiculated (Fig. ) mass inferior to the right inframammary fold deep to the accessory nipple, compatible with a supernumerary breast. There was evidence of overlying skin infiltration, with a clear margin of normal tissue between the tumour and costal fascia. No masses were noted in either breast.\nShe proceeded to a wide local excision of the entire accessory breast, nipple-areolar complex and an additional ellipse of skin. Sentinel node biopsy was negative, with no further axillary dissection performed.\nHistopathology revealed a Grade 2, 19-mm IDC. There was no lymphovascular invasion and radial margins were clear by >10 mm. The deep margin was 2 mm, but the anterior subcutaneous margin was 0 mm. In view of the positive anterior margin the patient proceeded to adjuvant radiotherapy.\nThe patient underwent a CT planning scan to assess the depth of tissue to be treated. A 10 × 7 cm rectangular electron field was applied to the tumour bed incorporating the excision scar. The patient was then treated with 9 megavoltage (MEV) electrons to a dose of 40 Gy in 15 fractions, over 21 days receiving 2.67 Gy per fraction. The Dmax treatment depth was 12 mm, with the 90% isodose at 18 mm. The ipsilateral breast was not included in the treatment field as the tumour was in a separate accessory breast which had been fully excised. The patient subsequently commenced Tamoxifen. Twelve weeks post-radiotherapy, she was in remission and all acute radiotherapy reaction had subsided. | [[48.0, 'year']] | F | {'19808163': 1, '18705419': 1, '15098783': 1, '7889223': 1, '20934398': 1, '17461909': 1, '18355913': 1, '2269968': 1, '7609527': 1, '19115699': 1, '24968437': 2} | {} |
165,176 | 3888001-1 | 24,968,436 | noncomm/PMC003xxxxxx/PMC3888001.xml | Splenic marginal cell lymphoma (SMZL): report of its presentation with spontaneous rupture of the spleen | We report a case of a 71-year-old woman with a past medical history of inflammatory myelitis, osteoporosis, asthma, coeliac disease and chronic back pain who was admitted as an emergency with new onset and severe back and abdominal pain. She described a 5-day history of persistent bilateral back pain radiating to the shoulder tips and groin worse when lying flat. There was no reported history of trauma or falls.\nThe patient was haemodynamically stable with a slightly distended soft but generally tender abdomen. On admission the haemoglobin was 61 g/l and haematocrit 0.189 l/l. She attended the emergency department 3 days before with a similar complaint but discharged home the same day. The haemoglobin was noted to be 117 g/l during that admission. The inflammatory markers were following: CRP 88 and white cell count 12.3; biochemistry and clotting screen was normal.\nCT scan (Fig. ) was consistent with splenic rupture. Laparotomy was performed and a large splenic haematoma with ∼1 l of blood in the abdomen and a splenectomy was performed. The postoperative period was complicated by a lower respiratory tract infection and some difficulty with mobilization but the patient made good recovery otherwise. During the post-operative period she developed a hypoglossal nerve palsy, which was assessed by the neurologist. An MRI scan failed to reveal a cause and the palsy settled spontaneously. She was discharged after 21 days.\nThe excised spleen weighed 560 g with blood clots and was macroscopically normal. Microscopically (Figs –), the histology and immunohistochemistry was consistent with a diagnosis of SMZL. Subsequently, a bone marrow biopsy and staging CT were performed and no distant spread of disease was demonstrated. The patient is undergoing regular follow-up in the haematology clinic. | [[71.0, 'year']] | F | {'28250305': 1, '17129392': 2, '9464787': 1, '19074110': 1, '9498498': 1, '11371838': 1, '22577390': 2, '22887176': 1, '22489356': 1, '12723734': 1, '24968436': 2} | {'3347250-1': 1, '1712221-1': 1} |
165,177 | 3888002-1 | 24,968,441 | noncomm/PMC003xxxxxx/PMC3888002.xml | Pneumoperitoneum as a result of a ruptured splenic abscess | A 48-year-old female with the past medical history of diabetes, coronary artery disease and psoriasis, presented with acute onset of diffuse abdominal pain 2 days prior to admission to the hospital. Upon presentation, she was tachycardic with a heart rate of 130 and hypotensive with a blood pressure of 78/45. The patient was afebrile and alert and oriented to person, place and time. She was resuscitated in the emergency department with intravenous fluids and her blood pressure and heart rate responded appropriately.\nHer initial labs showed a normal WBC level of 8.3 thou/mcL; however, the patient was acidotic with a pH of 7.29 and a lactic acid level of 3.5 mmol/l. An acute abdominal series was obtained demonstrating free air below the right hemidiaphragm (Fig. ). Physical examination showed a distended abdomen with tenderness, guarding and signs of peritonitis. The patient was subsequently boarded for an exploratory laparotomy with repair of perforated viscus, as that is the usual cause of pneumoperitoneum, especially under the right hemidiaphragm. Because the patient's vital signs stabilized after being resuscitated, the decision was made to obtain a CT scan to better assess the location of the perforated viscus. Much to our surprise, the patient did not have a perforated viscus, but a splenic abscess that had ruptured causing the pneumoperitoneum (Fig. ).\nThe patient then became increasingly confused and her vital signs again deteriorated demonstrating worsening sepsis. The patient was taken to the operating room where a laparoscopic splenectomy was attempted but was quickly converted to laparotomy with splenectomy due to the gross contamination of the abdomen. The abdominal compartment was copiously irrigated and the abdomen was closed. The surgery was without any complications. The patient was continued on antibiotics and taken to the intensive care unit for post-operative care.\nThe patient did well post-operatively. The splenic abscess grew Prevotella intermedia, a bacterium commonly found in the oral flora. Her blood cultures were negative. The patient underwent a full work-up looking for the source of the splenic abscess. A transesophageal echocardiogram was performed but was negative for any masses, thrombus or vegetation. Furthermore, a panorex was performed and was negative as the patient had reported tooth pain 1 week prior to her admission. The patient was finally deemed stable for discharge on post-operative day 10. She returned to the clinic on post-operative day 14 to receive her splenectomy vaccinations. | [[48.0, 'year']] | F | {'25795751': 1, '15290509': 1, '15492661': 1, '19415449': 1, '21319348': 1, '32020930': 1, '16676856': 1, '24968441': 2} | {} |
165,178 | 3888003-1 | 24,968,435 | noncomm/PMC003xxxxxx/PMC3888003.xml | Appendiceal endometriosis in a pregnant woman presenting with acute perforated appendicitis | A 33-year-old female patient at 31 weeks of gestation presented to our emergency department complaining of sharp constant upper abdominal pain that had grown progressively worse over the preceding 24 h period. She denied fever, chills, changes in her appetite, nausea or vomiting. Her past medical history was significant for unspecified inflammatory bowel disease and chronic urinary tract infections. She was confirmed by ultrasound to have a 31-week intrauterine pregnancy that until presentation had been largely uneventful. She was admitted to the maternal–fetal medicine service and was found to have a white blood cell count of 18.700/mm3 with 87% neutrophils and frequent contractions. The obstetricians administered Mg2+ for tocolysis and steroids for fetal pulmonary maturation, and the patient underwent an amniocentesis that was negative for chorioamnionitis. Her urinalysis was unremarkable and urine culture was sent and her workup had been otherwise unrevealing including ultrasound imaging of the right upper quadrant, which did not show any pertinent biliary pathology.\nThe patient underwent an MRI, which demonstrated a single fetus in cephalic vertex position, but could not completely visualize the appendix and was negative for inflammatory changes in the right lower quadrant or surrounding the terminal ileum. Initially, the patient improved clinically, however over the following 2 days her severe upper abdominal pain returned. A repeat MRI was obtained that now showed no appendix, but a multilobulated cystic mass complicated with an abscess measuring up to 13 mm and free fluid tracking along the right paracolic gutter and a small amount of perihepatic free fluid (Fig. ). Interestingly, the study also showed small rounded hypodensities in the base of the cecum that were initially interpreted as fecalith or intraluminal gas, but in retrospect might have presented ectopic endometrial glands. These findings in conjunction with her rising white blood count (21 000/mm3) and worsening physical examination were concerning for an acute perforated appendicitis and the patient was brought to the operating room for an open appendectomy in the presence of the obstetric team for fetal monitoring. Upon entering the abdomen abundant free fluid and a grossly inflamed, necrotic and perforated appendix were noted. Frozen section of the tissue came back as grossly inflamed perforated appendicitis and transmural intestinal endometriosis with extensive dezidualization (Fig. ). Given the extensive nature of the perforation, likely secondary to endometriosis at the base of the appendix, an ileocecetomy was performed. The patient tolerated the patient well and fetal monitoring did not show any signs of distress at the conclusion of the case.\nApproximately 5 h postoperatively the patient experienced painful contractions and fetal heart monitoring showed late decelerations. Pelvic examination showed an 8-cm dilated cervix. Subsequently the patient's membranes ruptured and she delivered a viable male infant who required 36 h of mechanical ventilation and newborn special care unit (NBSCU) admission. The patient made a full recovery in a few days and the infant did well in the NBSCU. | [[33.0, 'year']] | F | {'10819817': 1, '10533783': 1, '27106612': 2, '17572425': 1, '8110213': 1, '34691388': 1, '16828481': 1, '32309013': 1, '7489161': 1, '3717642': 1, '7206869': 1, '19169700': 1, '24968435': 2} | {'4840569-1': 1} |
165,179 | 3888004-1 | 24,968,434 | noncomm/PMC003xxxxxx/PMC3888004.xml | Giant rectal gastrointestinal stromal tumours: a diagnostic and therapeutic challenge | An elderly man in his seventies presented to the Urology department with two episodes of acute urinary retention. There were no rectal symptoms and rectal examination revealed an enlarged prostate. He underwent a laser transurethral resection of prostate during which a large palpable pelvic mass was noted. The subsequent urgent MR pelvis revealed a large tumour in the mesorectum arising from the anterior rectum 10 × 8.5 cm (Figs and ). A transrectal ultrasound and biopsy were performed; histology revealed a spindle cell tumour, with positive immunostains for CD117 and DOG1 with 5 mitoses per 50 high power fields. This was confirmed to be a locally advanced high-risk GIST.\nThe multidisciplinary team review advised neoadjuvant treatment with imatinib mesylate and further reassessment. Repeat MR pelvis and staging PET CT abdomen, thorax and pelvis were then performed. These showed a large extra-luminal tumour with downsizing in tumour bulk (6 × 5.7 × 5.4 cm) (Figs –) but in close approximation with the right seminal vesicle.\nFurther examination under anaesthesia, flexible sigmoidoscopy and cystoscopy were performed. The tumour was a large right-sided mass with no prostatic involvement. An elective Hartmann's procedure plus possible pelvic clearance was organized. Intraoperatively a large tumour in close proximity to the posterior bladder and seminal vesicles was found. Although there was no evidence of invasion, dissection of the plane between the GIST and posterior bladder was not possible, thus cystectomy and prostatectomy were performed with an ileal conduit and urostomy. The sigmoid colon and rectum were resected leaving an end colostomy as planned. In the postoperative period the patient developed signs of sepsis. A subsequent CT revealed an anastomotic leak at the ileo-ileal site. The patient returned to theatre, the anastomosis was taken down and a double barrel ileostomy sited. He initially went on to recover well. Operative histology revealed reduced mitotic activity and downgrading of tumour activity with clear R0 resection margins. | [[75.0, 'year']] | M | {'19303137': 1, '10636102': 1, '26036460': 1, '19434705': 1, '11213830': 1, '18942073': 1, '24968434': 2} | {} |
165,180 | 3888006-1 | 24,968,444 | noncomm/PMC003xxxxxx/PMC3888006.xml | The missing foley catheter: an unusual finding in vesicouterine fistula | A 28-year-old G1P1 was referred to our fistula hospital with a 5-month history of leaking urine per vagina. The leakage was intermittent, and immediately followed caesarean delivery of a stillbirth after labouring 3 days. Additionally, she had 5 months of amenorrhoea but cyclic haematuria accompanied by cramping pains.\nThe genital examination was unremarkable, with no leakage of dye following retrograde filling of the bladder. Given her complaints, she was scheduled for an exploratory laparotomy for suspected vesicouterine fistula. A foley catheter passed immediately before surgery was noted to have no urine drainage, but the examination confirmed proper transurethral placement.\nOn surgical exploration, the bladder was identified, but the foley bulb was curiously absent. Dissecting the bladder completely free from the uterus revealed the problem. Surrounded by dense scar tissue was the missing foley catheter, traversing the fistulous tract (Fig. ). Palpation of the uterus confirmed the inflated balloon was in the endometrial cavity. | [[28.0, 'year']] | F | {'7424506': 1, '21529808': 1, '9849756': 1, '22592760': 1, '13411039': 1, '24968444': 2} | {} |
165,181 | 3888007-1 | 24,968,443 | noncomm/PMC003xxxxxx/PMC3888007.xml | Metastatic breast cancer presenting as a gallstone ileus | A 76-year-old Caucasian woman was admitted with a 2-week history of intermittent colicky lower abdominal pain associated with bilious vomiting and mild abdominal distension. She was previously diagnosed with left breast cancer 18 years ago for which she had undergone an oncological wide local excision, focused external beam radiotherapy and adjuvant treatment with tamoxifen for 5 years. Unfortunately, she developed regional recurrence with bony metastases 17 years following her original diagnosis. She was treated with palliative chemoradiotherapy and had an excellent response to treatment.\nPhysical examination revealed an elderly lady with stable vital signs. The abdomen was mildly distended with tenderness and guarding noted in the peri-umbilical region. Laboratory investigations, including haematological, biochemical, inflammatory and coagulation profiles, were all within normal limits.\nA plain abdominal radiograph was also unrevealing. She proceeded to have computerized tomography (CT) scan of her abdomen and pelvis, which showed dilated small bowel loops consistent with small bowel obstruction. There were two filling defects noted, one in her proximal ileum suspicious for a non-calcified gallstone and the second distally in her terminal ileum (Figs and ). Appearances were suggestive of gallstone ileus. She was adequately resuscitated and underwent a laparotomy. The small bowel was narrowed in two segments: proximally secondary to an impacted gallstone and an area of stricturing noted distally. Both segments were resected with end-to-end anastomosis performed in a two-layer standard hand-sewn fashion. The clinical impression was one of dual pathology including Crohn's disease and gallstone ileus. She did not undergo a cholecystectomy or repair of her cholecystoduodenal fistula. She made an uneventful post-operative recovery and was discharged home well on Day 7.\nHistopathological examination of the resected specimens showed a 3-cm intraluminal gallstone within the proximal ileum, with focal metastatic carcinoma consistent with breast primary present in the adjacent mesenteric adipose tissue (Fig. ), while the more distal, strictured ileal segment revealed mucosal and transmural involvement by metastatic breast carcinoma (Fig. ). The tumour cells were strongly positive for cytokeratin, oestrogen receptor and focal progesterone receptor (<5%), consistent with metastatic breast carcinoma. They also displayed a mixed ductal and lobular differentiation pattern. | [[76.0, 'year']] | F | {'23295322': 1, '22866167': 1, '17058318': 1, '8211676': 1, '16177864': 1, '23396396': 1, '628727': 1, '22987332': 1, '12658481': 1, '19708319': 1, '24968443': 2} | {} |
165,182 | 3888033-1 | 24,470,810 | noncomm/PMC003xxxxxx/PMC3888033.xml | Childhood adrenoleukodystrophy – Classic and variant – Review of clinical manifestations and magnetic resonance imaging | A 7-year-old male child presented with decreased hearing. Child had normal neurology on examination. Clinical examination revealed bilateral sensorineural deafness. The motor and sensory functions were normal. There were no other positive clinical findings. Motor examination revealed 5/5 power in all 4 limbs; tone was normal but the deep tendon reflexes were exaggerated. Bilateral plantar reflexes were down going. The sensory system and gait was normal and there were no cerebellar signs. The visual evoked potential (VEP) and nerve conduction studies were normal.\nMagnetic Resonance Imaging (MRI) performed on a 1.5 Tesla system (MagnetomAvanto, Siemens, Germany) revealed the following findings as displayed in . Bilateral symmetrical linear and patchy areas of altered signal intensities were noted in the splenium and bilateral parieto-occipital white matter. Similar signal intensities were seen involving the medial geniculate bodies, extending to involve the region of the lateral lemniscus and along the brainstem. These regions appear significantly hypointense on T1 weighted and hyperintense on T2 weighted and FLAIR (fluid attenuated inversion recovery sequence) images. There were no signal intensities involving the corticospinal tracts or bilateral internal capsules. Post-contrast study showed typical peripheral enhancement in the corresponding areas of the parieto-occipital white matter and rim like restricted diffusion on diffusion weighted imaging (DWI). Such imaging features were consistent with pathognomonic description of ALD. | [[7.0, 'year']] | M | {'27375759': 1, '8791909': 1, '1771108': 1, '10584804': 1, '8636252': 1, '7567229': 1, '9715229': 1, '16021451': 1, '15876760': 1, '9010525': 1, '9278636': 1, '12355012': 1, '22914231': 1, '11204280': 1, '34728974': 1, '15888613': 1, '26670322': 1, '29386416': 1, '29390592': 1, '24470810': 2} | {'3888033-2': 2} |
165,183 | 3888033-2 | 24,470,810 | noncomm/PMC003xxxxxx/PMC3888033.xml | Childhood adrenoleukodystrophy – Classic and variant – Review of clinical manifestations and magnetic resonance imaging | A 6-years-old male child, born out of non-consanguineous marriage presented with regression in milestones since one year. He also had 4-5 episodes of non-projectile vomiting in the past 6 months. On clinical examination, hyperpigmentation of skin, nails, lips, and oral cavity were noted, which had appeared 4 months ago []. On neurological examination the child displayed hyperactivity and was showing easy distractibility. Motor examination revealed 5/5 power in all four limbs; tone was normal but the deep tendon reflexes were exaggerated. Bilateral plantar reflexes were down going. The sensory system and gait was normal and there were no cerebellar signs. An awake-sleep electroencephalogram (EEG) showed slow wave discharges. The VEP and nerve conduction velocity (NCV) studies were normal.\nMRI performed on a 1.5 Tesla system (MagnetomAvanto, Siemens, Germany) revealed the following findings as displayed in . Bilateral symmetrical linear and patchy areas of altered signal intensities noted in the brainstem; in the pontine fibers extending into the mid-brain (colliculus, tegmentum, and crus cerebri) and bilateral internal capsules (involving anterior limb and genu and posterior limb) and sub-thalamic region in the region of the pyramidal tracts. Similar signal intensities were seen in the white matter of bilateral cerebellar hemisphere including inferior and middle cerebellar peduncles. These areas appeared significantly hypointense on T1 weighted and hyperintense on T2 weighted and FLAIR images. The post-contrast study showed significant linear and peripheral enhancement in the above areas.\nThere were no areas of restricted diffusion on DWI images. On Magnetic Resonance spectroscopy there was partial N-acetyl aspartate (NAA) loss and partially raised lactate within these hyperintensities.\nIn view of the clinical features of adrenal insufficiency and the MRI features, the diagnosis of ALD was provided.\nBiochemical investigations showed plasma renin to be 4.23 units (high); Plasma aldosterone: 8.7 units (normal). The Adrenocorticotrophic hormone (ACTH) units were more than 1250 units (high). The ACTH stimulation test did not reveal any increase in cortisol levels. The confirmatory test was elevated levels of VLCFAs in plasma. These elevated levels were confirmed in a reputed lab in the USA (United States of America) [].\nThe diagnosis of ALD was provided on the basis of hyperpigmentation of skin and biochemical findings; this being consistent with adrenal insufficiency and the MR findings. | [[6.0, 'year']] | M | {'27375759': 1, '8791909': 1, '1771108': 1, '10584804': 1, '8636252': 1, '7567229': 1, '9715229': 1, '16021451': 1, '15876760': 1, '9010525': 1, '9278636': 1, '12355012': 1, '22914231': 1, '11204280': 1, '34728974': 1, '15888613': 1, '26670322': 1, '29386416': 1, '29390592': 1, '24470810': 2} | {'3888033-1': 2} |
165,184 | 3888034-1 | 24,470,811 | noncomm/PMC003xxxxxx/PMC3888034.xml | Holocord abscess in association with congenital dermal sinus | An 18-month-old girl child presented with rapidly progressive bilateral lower limb weakness with fever for 2 months. The mother of the child also noticed that she was not feeling any sensation below the nipples and had bladder incontinence. The fever was intermittent and the child was on several antibiotics without any benefit. Neurological examination showed conscious and active child. She had flaccid paraplegia with grade “0” power with absent deep tendon reflexes. Sensory examination showed absent sensation to pain below D4 level on both sides. Power, tone, and sensation were normal in the upper limbs. There were no signs of meningeal irritation. Careful examination of the back showed a very small dermal sinus in the lower lumbar region, discharging pus on digital pressure. Magnetic resonance imaging (MRI) showed multiple coalescing ring enhancing lesions in the spinal cord extending from D4 to L5 with cord edema upto cervical level []. The differential diagnosis considered were holocord glioma and holocord abscess. Modified osteoplastic laminotomy, described by Raimondi et al.,[] was performed from D4 to L5 []. The dermal sinus tract was seen to extend from the skin at L5 and end intradurally at the same level. Dura was opened and there was dermoid material and pus in the lumbar region The spinal cord was found to be diffusely enlarged. Myelotomy was performed at different levels and pus was evacuated from the spinal cord []. There were extensive arachnoidal adhesions. In view of the extensive nature of the abscess, no attempt was made to excise the abscess capsule. The dura was closed with fascial graft. The laminotomy flap was replaced []. The child stood the surgery well and the postoperative period was uneventful. Escherichia coli and Pseudomonas aeruginosa were grown on culture. She was started on antibiotics as per the sensitivity report. She started showing some neurological improvement within a few days in the form of some movement in both the legs. She was on long-term antibiotics for 8 weeks and physiotherapy. At 2 months follow-up, she had grade 3 power in the right lower limb and grade 1-2 power in the left. Sensory level was at D12 both sides. Follow-up MRI shows good resolution of the intramedullary abscess []. | [[18.0, 'month']] | F | {'16619639': 1, '27857795': 2, '972339': 1, '29497549': 2, '7969844': 1, '10361972': 1, '17595507': 1, '29269365': 1, '10602531': 1, '12586985': 1, '34221606': 2, '24470811': 2} | {'8247672-1': 1, '5108129-1': 1, '5782478-1': 1} |
165,185 | 3888035-1 | 24,470,812 | noncomm/PMC003xxxxxx/PMC3888035.xml | Congenital dermal sinus in mid-dorsal spine with large intramedullary dermoid cyst in an 18-months-old child | An eighteen-months-old female child presented with history of mild fever and intermittent discharge from a sinus in the mid-dorsal area. On examination, she was febrile with normal power and sensation in all limbs except mild stiffness of left leg. Posterior aspect of mid-dorsal spine (D5) shows a skin dimple with no active discharge at presentation []. Magnetic resonance imaging (MRI) of whole spine revealed a large intramedullary cystic lesion in the dorsal segment of spinal cord extending from D2 to D7 vertebral level with single posterior sinus tract opening to skin at D5 vertebral level. The cystic lesion shows T1-weighted (T1W) hypointense, T2-weighted (T2W) and short tau inversion recovery (STIR) hyperintense signal, restricted diffusion in diffusion-weighted image (DWI) and apparent diffusion coefficient (ADC) image. Small T1W isointense and T2W hypointense content noted inside the cystic lesion. No fat signal was seen inside the mass. Post-contrast MRI image shows no enhancement of the lesion []. Intramedullary spinal epidermoid/dermoid with posterior sinus tract was given as possible radiological differential.\nDorsal D1 to D8 laminectomy was done. Mid-line myelotomy and total tumor excision under microscope was done. Injection methyl prednisolone was given as bolus dose (30 mg/kg body weight) during intra operative period and continued for 24 hour in post-operative period with a dose of 5.4 mg/kg body weight. Tuft of hair and cheesy material with a thin capsule were removed in piece meal; portion of the capsule could not be removed as it was densely adhered to the cord. Dermal sinus tract was ending within the dermoid capsule [] and was also excised. Myelotomy was left open and water tight dural closure was done. Post operatively she developed mild weakness of left lower limb (grade-IV power) which she recovered by 10th post-operative day. She was discharged free of neurological deficit.\nThe histopathological diagnosis of the lesion confirmed dermoid cyst []. | [[18.0, 'month']] | F | {'33247382': 1, '7847126': 1, '14565519': 1, '15739068': 1, '32215158': 1, '1476931': 1, '1392585': 1, '25535223': 1, '1528450': 1, '2601815': 1, '3287667': 1, '10715723': 1, '24470812': 2} | {} |
165,186 | 3888036-1 | 24,470,813 | noncomm/PMC003xxxxxx/PMC3888036.xml | Tethered cord with tandem lipomyelomeningoceles, split cord malformation and thick filum | A three month old male infant presented with a congenital skin covered soft tissue mass in midline lumbosacral area. There were multiple cutaneous hemangiomatous skin changes over the soft tissue mass. Neurologic examination was intact especially in lower extremities. Thorough urological evaluation confirmed detrusor sphincter dys-synergy with high pressure bladder according to urodynamic study. Spinal MRI confirmed SCM type II associated with low lying conus and lipomatous stalk coming from spinal canal defect to the spinal cord and attached to the cord dorsally [].\nSurgical procedure was done in prone position with an elliptical incision. There were fascial and bony defects at L4 to S1 levels through which lipomatous tissue was invaginating from surface to the dura mater through that defect. Laminotomy above the defect was performed and normal dura mater was exposed and incised. There were two dural defects separated from each other with normal dura mater. The dorsal type lipoma was separated carefully from the neural placode in the spinal canal and then pial edges approximated by 6-0 non-absorbable sutures. Another lipomatous tissue as a distal type lipoma was in continuity with a short right hemicord exiting through a different dural defect in a more caudal and lateral location of the spinal canal. After durotomy and sharp cut of the lipomatous tissue, the cord was released. The left hemicord that was separated from lipomyelomeningoceles and right hemicord had a thick fatty filum terminale that was divided from the attachment to distal dural pouch []. Closure was performed in layers. Postoperative period was uneventful. | [[3.0, 'month']] | M | {'22656254': 1, '1407429': 1, '28247108': 1, '11599854': 1, '23025444': 1, '33042248': 2, '3989062': 1, '1407428': 1, '3883218': 1, '17179703': 2, '24470813': 2} | {'2721945-1': 1, '7519753-1': 1} |
165,187 | 3888038-1 | 24,470,815 | noncomm/PMC003xxxxxx/PMC3888038.xml | Moebius syndrome with Dandy-Walker variant and agenesis of corpus callosum | A 15-month-old male child 4th born to second degree consanguineous parents by spontaneous vaginal delivery, presented with inability to close his right eye, drooling of saliva, difficulty in sucking, and deviation of the face to the left since birth. This was in the background of global developmental delay and at present he can sit with support, does not progress beyond cooing with normal vision and hearing deficit [].\nOn examination, he has Grade III protein energy malnutrition (PEM) with his weight falling below 3rd percentile according to World Health Organization growth chart, length was between the 3rd and 25th percentile and his head circumference was appropriate for his length according to Dines formula. On examination he is conscious, playful, oriented to person. Cranial nerve examination reveals bilateral abducen nerve palsy and right-sided facial nerve palsy, as he was unable to blink his right eye with loss of angle of mouth on the right side and deviation of the mouth to the left. He has bilateral hearing loss which was confirmed with brainstem evoked response audiometry. The metabolic screening test, serum lactate, and ammonium were within normal limit.\nWith the clinical features the diagnosis of Moebius syndrome was entertained and neuroimaging was ordered as to rule out other abnormalities. The magnetic resonance imaging MRI of the brain showed complete agenesis of the corpus callosum with inferior vermian hypoplasia with prominent fourth ventricle which is consistent with the Dandy-Walker variant []. | [[15.0, 'month']] | M | {'9637807': 1, '5304353': 1, '1690128': 1, '11059047': 1, '8872479': 1, '23679990': 1, '27407651': 1, '15829555': 1, '24470815': 2} | {} |
165,188 | 3888039-1 | 24,470,816 | noncomm/PMC003xxxxxx/PMC3888039.xml | Angelman syndrome in three biological siblings: Focusing on the neuropsychiatric domain | A 7-year-old female presented with complaints of delayed milestones and seizures. She is the 2nd order in the family, born of nonconsanguinity with normal birth and delayed milestone. She attained neck holding at the age of 2 years, sitting with support at the age of 3 years, standing without support at the age of 4 years, walking without support at the age of 5 years. She has only palmer grasp now, feeds herself without spilling, and spells only bisyllables and is not toilet trained. There was no regression of attained milestones. When she started walking independently, she walked with wide-based ataxic gait. She was always smiley with a history of inappropriate laughter and happy demeanor. She did not have history of limb incoordination or any extrapyramidal symptoms. Her intelligence quotient was found to be 24 on Seguin Form Board test, suggestive of severe MR. | [[7.0, 'year']] | F | {'11343340': 1, '12566516': 1, '8933339': 1, '20445456': 1, '8178814': 1, '9048672': 1, '7684188': 1, '7573182': 1, '10536901': 1, '3688021': 1, '3409926': 1, '24470816': 2} | {'3888039-2': 2, '3888039-3': 2} |
165,189 | 3888039-2 | 24,470,816 | noncomm/PMC003xxxxxx/PMC3888039.xml | Angelman syndrome in three biological siblings: Focusing on the neuropsychiatric domain | An 8-year-old girl, the elder sister of the above, also had similar history of delayed milestones (social, motor, language and cognition), wide-based ataxic gait, since she learned walking with support. Always she used to remain happy and cheerful with frequent excessive, inappropriate laughter. There was no history of seizure, vision impairment, hearing loss, motor incoordination, and any extrapyramidal symptoms. Her social quotient (SQ) was found to be 15 on Vineland Social Maturity Scale, suggestive of profound MR. | [[8.0, 'year']] | F | {'11343340': 1, '12566516': 1, '8933339': 1, '20445456': 1, '8178814': 1, '9048672': 1, '7684188': 1, '7573182': 1, '10536901': 1, '3688021': 1, '3409926': 1, '24470816': 2} | {'3888039-1': 2, '3888039-3': 2} |
165,190 | 3888039-3 | 24,470,816 | noncomm/PMC003xxxxxx/PMC3888039.xml | Angelman syndrome in three biological siblings: Focusing on the neuropsychiatric domain | A 5-year-old girl, younger sister of the above two also had complaints of difficulty in speech, poor comprehension, and communication ability, compromised ability to do the activities of daily living with history of global delayed developmental milestones and ataxic gait. There was no evidence of vision impairment, hearing, motor function, or history of seizure. She had microcephaly with an open mouth and protruded tongue. Her SQ was found to be 19 on Vineland Social Maturity Scale, suggestive of profound MR. | [[5.0, 'year']] | F | {'11343340': 1, '12566516': 1, '8933339': 1, '20445456': 1, '8178814': 1, '9048672': 1, '7684188': 1, '7573182': 1, '10536901': 1, '3688021': 1, '3409926': 1, '24470816': 2} | {'3888039-1': 2, '3888039-2': 2} |
165,191 | 3888040-1 | 24,470,817 | noncomm/PMC003xxxxxx/PMC3888040.xml | An interesting case of Rathke's cleft cyst presenting as bilateral cryptorchidism | A 4.5-year-old boy presented to the Pediatric Surgery Department with bilateral undescended testes for which surgery was planned. Upon evaluation for short stature at the department of endocrinology, he was found to be born to nonconsanguineous parents, at full term by normal vaginal delivery; there was no history of prolonged labor or birth asphyxia and his birth weight was 2.5 kg. Mother noticed bilateral undescended testes at the time of delivery, but was reassured by the doctor that testes would descend subsequently. There was no history of seizures or prolonged jaundice in the neonatal period, but his motor and mental milestones were delayed. He remained shorter than his peers and his sister who is 2 years younger is taller than him. There was no history of chronic systemic disease, hypothyroidism, or features suggestive of raised intracranial pressure. On examination, his height is 84 cm (<3rd percentile), upper to lower segment ratio is 0.9 [], standard deviation score was 4, weight was 13 kg, and head circumference was 51 cm. He had frontal bossing, doll-like facies, central obesity, micropenis with stretched penile length of 2 cm, and both gonads were palpable in suprascrotal area with a poorly developed empty scrotal sac. His vitals and systemic examination were normal.\nInvestigations revealed a hemoglobin of 13.6 g% (11-15), total lymphocyte count of 5,600/mm3 (4-11,000), erythrocyte sedimentation rate of 15 (5-20), random blood sugar of 94 mg% (90-130), creatinine 0.8 mg/dl (0.6-1.5), free thyroxine (T4)-0.9 ng/ml (0.93-1.7), thyroid stimulating hormone (TSH)-7.27 mIU/ml (0.3-5.5), bone age-1 year, basal cortisol-13.76 μg/dl (8-20), follicle stimulating hormone (FSH)-0.63 mIU/dl (1.5-10.2), luteinizing hormone (LH)-0.1 mIU/dl (1.9-12), testosterone basal 0.025 ng/ml (3-6.5), stimulated testosterone with human chorionic gonadotropin (hCG) was 0.68 ng/ml (suggestive of normally functioning testes), and insulin-like growth factor 1 (IGF-1) was undetectable. Radiological evaluation revealed bilateral suprascrotal testes in ultrasound abdomen, a 3.8 × 3.6 cm suprasellar cystic lesion with compressed pituitary gland in magnetic resonance imaging (MRI) brain [Figures and ], which was hypointense on T1 and hyperintense on T2 images suggestive of cystic craniopharyngioma or Rathke's cyst. Child did not cooperate for an accurate visual field examination, but grossly he had some degree of visual impairment in left eye. Urine output and fluid input both were approximately 1 l.\nChild was subsequently treated with levothyroxine replacement in view of secondary hypothyroidism. After 4 weeks (with the normalization of T4 levels), he underwent right frontotemporal orbitozygomatic craniotomy, marsupialization of suprasellar cyst along with biopsy. His intra- and postoperative period was uneventful. Biopsy revealed a single-cell layered, cuboidal epithelium, suggesting a Rathke's cleft cyst. So the final diagnosis was Rathke's cleft cyst with hypopituitarism. At 6 and 12 weeks after surgery, child was reevaluated for pituitary function, which revealed persistent secondary hypothyroidism and low IGF-1 levels suggestive of growth hormone deficiency. Levothyroxine was reinstituted followed by initiation of growth hormone replacement along with 5 week hCG therapy (intramuscular (IM) 1,000 IU twice a week). This enabled gonadal descent into the scrotum 3 months after the completion of therapy. | [[4.5, 'year']] | M | {'5059976': 1, '7054442': 1, '6128816': 1, '11683607': 1, '2907897': 1, '4010898': 1, '1584358': 1, '17028442': 1, '1509341': 1, '17172066': 1, '9561565': 1, '15221359': 1, '6123359': 1, '15094270': 1, '18436703': 1, '10440036': 1, '2002366': 1, '24470817': 2} | {} |
165,192 | 3888041-1 | 24,470,818 | noncomm/PMC003xxxxxx/PMC3888041.xml | Failure of peritoneal and gallbladder shunts in a child with craniopharyngioma | A 1-year-old girl presented with a 7-day history of irritability and acute visual deterioration. Imaging revealed a calcified cystic suprasellar mass []. She underwent emergent endoscopic fenestration of the cyst with insertion of an ommaya reservoir to decompress the visual apparatus and ventricular system. Endoscopic biopsy of the lesion was undertaken at the same time, and a grade I adamantinomatous craniopharyngioma was diagnosed.\nPostoperatively, she developed a persistent CSF leak through her wound requiring insertion of an external ventricular drain (EVD). It was necessary to drain 40-50 mL/h of CSF to prevent symptoms and allow wound healing. CSF was sterile after 48 h culture with a white cell count of 8 cells per microliter and a red cell count of 550 cells per microliter. Endoscopic third ventriculostomy was not feasible so she underwent ventriculoperitoneal (VP) shunt insertion and debulking of the craniopharyngioma [].\nAfter multidisciplinary team discussion she travelled to the USA for proton beam therapy to the residual tumour. She had been experiencing intermittent abdominal pain and distension and while in the USA she developed symptomatic CSF ascites with CSF demonstrated in all four quadrants of the abdomen on ultrasound scanning. The unit she attended in the USA preferred to use ventriculogallbladder shunts when the peritoneum could not be used,[] and the VP shunt was revised to a ventriculogallbladder shunt with resolution of her ascites.\nAt nearly 2 years old, she developed a new craniopharyngioma cyst and underwent repeat endoscopic fenestration with Ommaya reservoir insertion. Postoperatively, she again developed a persistent CSF wound leak. This was associated with a distended, tender abdomen. Abdominal computed tomography showed a markedly dilated gallbladder []. It was clear that the accumulation of CSF within the gallbladder was preventing the shunt from functioning at its maximum capacity. At this time, she had no ascites so the distal shunt catheter was revised to a peritoneal catheter. The VP shunt allowed wound healing but within a month the CSF ascites had recurred [], and the peritoneal catheter was revised to an atrial catheter. Six months after insertion of the ventriculoatrial shunt, she remains asymptomatic. | [[1.0, 'year']] | F | {'7451068': 1, '14703222': 1, '33481136': 1, '3612454': 1, '18377303': 1, '11818742': 1, '31350574': 1, '12111487': 1, '27489721': 2, '7097343': 1, '24470818': 2} | {'4968107-1': 1} |
165,193 | 3888042-1 | 24,470,819 | noncomm/PMC003xxxxxx/PMC3888042.xml | Intracranial hydatid cyst is a rare cause of midbrain herniation: A case report and literature review | A 13-year-old male patient was admitted to the emergency department with headache, nausea, and vomiting. Neurologic examination revealed bilateral papilledema. Before cranial computed tomography (CT) was taken, the patient had generalized epileptic seizures. He was disoriented, and had anisocoria with dilatation of the right pupilla. His Glasgow Coma Scale value was 7 (E1 M5 V1). CT showed a solitary cystic lesion with a diameter of 10 cm, which was hypodense, sharply demarcated, without perifocal edema, and caused a shift of the midline structures to the contralateral side. The cyst was located in the right temporoparietal region [].\nBecause of the midbrain herniation signs, urgent operative intervention via a large craniotomy was performed. Once the dura mater was opened, a linear corticotomy was made. After visualization of the cyst wall, 0.9% saline solution was used to dissect the hydatid cyst from the surrounding brain to facilitate cyst removal (Dowling method modified by Arana-Iniguez). Valsalva maneuver was also applied to facilitate the removal of the cyst. The patient's head was lowered to further ease the cyst removal by gravity. The hydatid cyst was removed without rupture. The dura was closed in a watertight fashion after filling the remaining cavity with 0.9% saline solution [].\nPostoperative thorax and abdominal CT revealed no other foci. Fifteen days after the operation, a left hemiparesis occurred and cranial CT scan revealed a brain compressing subdural hygroma []; therefore, we inserted a subdural-peritoneal shunt. Based on cranial CT performed during follow-up, the subdural-peritoneal shunt was effective and no complaints were reported []. The neurological examination was normal. In the same CT examination done for follow-up, we noted that the site from where the hydatid cyst was removed was flask-shaped, relaxed space, and filled with cerebrospinal fluid (CSF) []. However, in the follow-up at the fourth month after intracranial operation, we saw a distended cyst that had replaced the flask and relaxed CSF-filled space from where the hydatid cyst was removed; interestingly, we noted a jet-flow with flow void effect as an influx. This appearance suggested that a one-way valve mechanism had developed that filled the space [].\nIn , the jet-flow and flow void of the high-speed CSF can be easily observed. This setup demonstrates the one-way valve mechanism of CSF flow, which causes enlargement of the porencephalic cyst. The patient underwent a third surgical procedure, and fenestration of the cyst to the subdural space was performed. A postoperative follow-up cranial CT scan showed no signs of the porencephalic cyst []. Postoperative albendazole therapy was then applied for 4 months (10 mg/kg per day). The patient was discharged following normal neurological exams. Currently, the patient is healthy and attending school without any epileptic seizures. | [[13.0, 'year']] | M | {'1553045': 1, '7143070': 1, '1944831': 1, '19805978': 1, '17765392': 1, '9689630': 1, '2609435': 1, '10514582': 1, '28127351': 1, '18192651': 1, '12213372': 1, '11685527': 1, '11862441': 1, '9436832': 1, '25260205': 1, '10883898': 1, '3989600': 1, '18518745': 1, '19431138': 1, '18639016': 1, '12042915': 1, '24470819': 2} | {} |
165,194 | 3888043-1 | 24,470,820 | noncomm/PMC003xxxxxx/PMC3888043.xml | Disseminated tuberculosis with paradoxical miliary tuberculomas of brain in a child with rickets | A 5-year-old marasmic male child was referred to our institute for multiple lymphadenopathy. His mother in the same household had pulmonary tuberculosis. On admission, the child had no history of cough, dyspnea or night sweats. However, he was lethargic and unable to stand or sit without support. His general examination was quite remarkable for the evidence of severe protein energy malnutrition (PEM) and rickets. He had marked pallor, generalized xerosis, crusted lesions with boil on the lower back, and hyperpigmented friction dermatosis on buttocks. His crawling movement was further complicated by valgus deformity of legs due to nutritional rickets. Cervical [] and groin examination showed multiple matted lymph nodes forming a large cervical and inguinal lymphadenopathy. His ocular examination revealed nebular keratopathy in both eyes. His initial evaluation at the previous institute did not reveal any malignancy as a possible etiology for large cervical and inguinal lymphadenopathy. His detailed clinical neurological evaluation did not reveal any positive findings. Hematological investigation revealed severe anemia (Hemoglobin - 7.3 gm/dl), leukocytosis (Total Leukocytes Count - 15,700/mm3; 84% neutrophils, 12% lymphocytes, 3% monocytes, and 1% eosinophils), and mild thrombocytopenia (87,000/mm3). Biochemical investigation for rickets showed hypocalcemia (serum calcium - 6.31 mg/dl), normal phosphate (serum phosphate - 4 mg/dl), higher alkaline phosphate - 310, and low Serum 25 (OH) D - 8 ng/ml. He was seronegative for human immunodeficiency virus (HIV) on Enzyme-Linked Immunosorbent Assay (ELISA) test; Mantoux test was also negative. The chest X-ray revealed miliary Koch's infiltration with a widening of the costochondral junction of the ribs due to rickets in the bony thorax []. Abdominal and thoracic ultrasound revealed mild hepatomegaly, mild splenomegaly, and enlarged mesenteric, inguinal, peripancreatic, celiac axis, periportal, and bilateral iliac lymph nodes. Corroborative radiological evaluation for rickets (X-ray both knee and wrist joint) showed metaphyseal widening with splaying and fraying of epiphysis and changes related to osteoporosis, and pencil line thinning of the epiphyseal cortex, suggesting rickets []. Fine needle aspiration cytology of cervical lymph node showed features of tuberculous lymphadenitis with the presence of acid-fast bacilli.\nAntituberculosis therapy including Streptomycin injection (according to CAT-1 of the National Tuberculosis Control Program) and oral steroids were started. Cytology report confirmed the Koch's etiology. Severe PEM and vitamin deficiency were aggressively managed in the hospital with supervised high protein diet and oral therapeutic supplementation of Vitamin A and Vitamin D with calcium. During 2 weeks of observation in the pediatric ward, the child recovered gradually and was discharged home with oral medicines. Within 3 weeks of discharge, the patient presented again to the pediatric intensive care unit with generalized tonic-clonic convulsions. There was no focal neurological sign on examination. The computed tomography (CT) scan of brain revealed multiple small tuberculomas in the brain parenchyma scattered like millets in the cerebral hemispheres, bilateral cerebellum, and brain stem []. Few of them were conglomerate in the left cerebellum, right parietal region, and pons with periventricular hypodensity. Anticonvulsant therapy was started and Streptomycin and steroid were added to the chemotherapy. Afterward, the child was symptom free. | [[5.0, 'year']] | M | {'19535154': 1, '7426915': 1, '20031301': 1, '6146749': 1, '18776821': 1, '15519120': 1, '9143584': 1, '3749451': 1, '18400795': 1, '455781': 1, '16644409': 1, '5128206': 1, '8183466': 1, '9655548': 1, '7888539': 1, '7965125': 1, '24470820': 2} | {} |
165,195 | 3888044-1 | 24,470,821 | noncomm/PMC003xxxxxx/PMC3888044.xml | Tumefactive presentation of a supratentorial cavernous hemangioma: A report of two cases | A 14-year-old boy presented with a 7-month history of progressive left-sided hemiparesis and bifrontal, throbbing headache. There was rapid worsening of the weakness and associated headache and vomiting over 5 days preceding his first admission at a local hospital. On examination, he was drowsy, but obeyed commands infrequently. CT of the head revealed a large mass lesion occupying the right parieto-occipital region. As MRI facility was not available nearby and he was not in a position to be shifted for MRI, he was taken up for tumor excision. Intraoperatively, the mass lesion was found to be highly vascular and surgery was abandoned. On arrival at our center, the child was not obeying any commands, had left-sided hemiparesis, and had no vision. A fundoscopy revealed gross papilledema and a massive bulge at the craniotomy site.\nRadiological evaluation at our center included CT and MRI scans, which revealed a well-defined mass in the right parieto-occipital region of size 10 × 8 × 8 cm, extending into the atrium of the right lateral ventricle [Figure –]. On T1 scan, multiple low- to high-signal intensities were observed within the lesion, signaling blood of different ages. Upon administration of contrast, minimal enhancement of the lesion was observed. On T2 scans, multiple hypo- to hyperintense signals within the lesion were noted, with massive edema involving the surrounding brain parenchyma. The lesion had herniated out of the previously performed craniectomy defect, thereby easing ICP.\nWith a provisional diagnosis of malignant neoplasm, the child underwent re-exploration via previously performed craniectomy. The mass lesion was well defined, encapsulated, multilobulated, and extraparenchymal, and had a reddish brown color. The lesion was excised as a single specimen, as there was a good plane of dissection between the tumor and brain parenchyma. The tumor was firm, rubbery, and compressible, with multiple blood-containing cavities. Postoperatively, the patient gradually regained consciousness and the left-sided weakness improved to a near-normal state. The excised specimen showed a relatively circumscribed lesion composed of blood-filled vascular channels of varying sizes. There were thick-walled vascular channels with intervening stroma showing variable fibrosis and moderate lymphomononuclear cell infiltration. Many vessels showed fibrin thrombi. Elastic fibers within the vessel wall were highlighted as black fibers upon Elastic Van Gieson staining. The final histopathological diagnosis was cavernous hemangioma [Figure –]. | [[14.0, 'year']] | M | {'16988810': 1, '27585994': 1, '20665038': 1, '34247276': 1, '18066488': 1, '32724292': 1, '24470821': 2} | {} |
165,196 | 3888045-1 | 24,470,822 | noncomm/PMC003xxxxxx/PMC3888045.xml | “Rabbit Ear” scalp deformity caused by massive subdural effusion in infant following bilateral burr-hole drainage | This 10-month-old male infant, presented at our neurosurgical emergency services with a history of depressed sensorium for 2 days. This followed a road traffic accident 10 days ago in which he was sitting at the rear seat of car and was the sole survivor. On physical examination, his pulse rate was 158/min and blood pressure 68/40 mm Hg. On neurological assessment at admission, he was arousable, moving limbs spontaneously with good cry. His anterior fontanelle was tense. Pupils were mid dilated and non-reacting to light. Hematological and biochemistry profiles were within the normal limits. Computerized tomography (CT) scan of the head done immediately after accident revealed presence of thin subdural hygroma in both frontoparietal regions with compression of adjacent brain []. Ophthalmologist evaluation for vision showed doubtful presence of vision. He was being managed with cerebral decongestant with regular visual assessment and monitoring under ophthalmologist. Repeat CT scan and magnetic resonance imaging (MRI) done after 10 days, following depression of sensorium revealed enlargement of bilateral SDE [] and fundoscopy revealed presence of bilateral papilledema. Patient was shifted to operating room for emergency evacuation of SDE. Right parietal and left parietal burr-holes were placed with drainage of subdural hygroma; the fluid collection was xanthochromic and under raised pressure. The post-operative period was uneventful except rapid bulging of scalp wound at burr-hole site seen bilaterally. He was put on acetazolamide and furosemide with spironolactone for 2 weeks. Despite these, scalp swelling continued to increase rapidly on both burr-hole sites with impending rupture of wound at burr-hole site. The bulging of scalp mimicked bilateral ‘rabbit ear’ appearance []. CT scan showed the presence of massive SDE causing mass effect []. So he was again taken to operation theatre and under general anesthesia a right sided subduro-peritoneal shunt was carried out. Subdural fluid sample was sent for cytopathology, biochemistry and aerobic, anaerobic, fungal and mycobacterial tuberculosis culture. In the post-operative period, subduro-peritoneal shunt was working well with complete resolution of subdural fluid collection. He was discharged on fifth post-operative day following shunt surgery.\nAt the last follow-up 6 months after surgery, he regained significant visual recovery and MRI carried out at follow-up showed a mild hydrocephalus with no residual subdural collection [Figure –]. He was regularly followed-up in pediatrics and the observed developmental milestones were corresponding to the child's age. | [[10.0, 'month']] | M | {'19653019': 1, '16408582': 1, '25002786': 1, '19922275': 1, '12382175': 1, '25878758': 1, '16776372': 1, '19877806': 1, '11059660': 1, '30459888': 2, '11048634': 1, '12111491': 1, '22044363': 1, '20195617': 1, '24470822': 2} | {'6208233-1': 1} |
165,197 | 3888046-1 | 24,470,823 | noncomm/PMC003xxxxxx/PMC3888046.xml | Primary congenital sacrococcygeal neuroblastoma: A case report with immunohistochemical study and review of literature | A 9-month-old baby girl brought to pediatric surgery outpatient department, presented with swelling in the sacral region since birth. The swelling was initially small and later increased to present size. Local examination showed round and globular swelling measuring 6 × 5 cm. Overlying skin was unremarkable. Systemic examination findings were noncontributory. The female child was born full term by uneventful normal vaginal delivery. She achieved normal milestones for age and was immunized appropriately for age. Laboratory investigations such as liver function tests, renal function tests, plasma glucose levels, and chest X-ray were normal.\nMagnetic resonance imaging findings of the sacrococcygeal mass: T2W sagittal images showed a well-defined lobulated lesion in the pre and retro sacrococcygeal region, encasing the coccyx. The lesion was isointense to hyperintense in signal intensity and showed fine internal septations within []. On the basis of clinical and radiological findings, a provisional diagnosis of solid variant of sacrococcygeal teratoma (SCT) was made.\nCaudal and general anesthesia was given and the swelling was entirely excised with the part of the coccyx. Postoperative course was uneventful.\nOn gross examination, the tumor was well-circumscribed having smooth and lobulated external surface, firm in consistency, and measured 6 × 6× 5 cm. On cut section, the tumor appeared nodular, greyish white with focal hemorrhagic areas in the periphery [].\nOn microscopic examination, a well-circumscribed tumor composed of round to oval cells with prominent nucleoli and scanty to moderate cytoplasm. The cells were seen arranged predominantly in nests. Also seen were few sheets and lobules, separated by thick and thin fibrovascular septae. Many areas showed presence of Homer Wright pseudorosettes []. At few places, the tumor showed neuropil and schwannian stroma. Amid this, were noted large cells differentiating into ganglion cells [ inset]. There was paucity of abnormal mitotic figures and karyorrhectic activity. On the basis of these findings, diagnosis of neuroblastoma in the sacrococcygeal region was considered and representative sections were subjected to immunohistochemical studies.\nImmunohistochemistry (IHC) was performed with the following panel of antibodies utilized for differential diagnosis of small round cell childhood tumors which included neuroendocrine markers like neuronspecific enolase (NSE) (Dako) chromogranin-A (clone 5H7, Leica) and synaptophysin (clone 27G12, Leica). Other markers included were S-100 (Leica), CD99 (clone 12E7, Dako), leucocyte common antigen (LCA) (clone, 2B11 + PD 7126), pancytokeratin (PanCK) (clone AE1/AE3, Dako), epidermal growth factor receptor (EGFR) (clone EGFR.25, Leica), cyclin D1 (clone P2D11F11, Leica), P53 (cloneDO-7, dako) and Ki-67 (clone MIB-1, Dako). Strong cytoplasmic immunoreactivity was observed with NSE [], synaptophysin and it was patchy with chromogranin []. S-100 showed positive cytoplasmic immunoreactivity. CD99 [], LCA, PanCK, and EGFR were nonreactive. Cyclin D1 showed strong nuclear immunoreactivity []. P53 was negative and Ki67 labelling index was less than 1%.\nThe above histomorphological studies confirmed the diagnosis of low grade primary sacrococcygeal neuroblastoma (SCN). | [[9.0, 'month']] | F | {'16137166': 1, '14523568': 1, '15117991': 1, '10421273': 1, '4817867': 1, '17482563': 1, '3395959': 1, '23248428': 1, '20558371': 1, '28116200': 2, '6737018': 1, '18413728': 1, '19034443': 1, '24470823': 2} | {'5237461-1': 1} |
165,198 | 3888047-1 | 24,470,824 | noncomm/PMC003xxxxxx/PMC3888047.xml | Pilomyxoid astrocytoma with high proliferation index | A 12-years-old male presented with focal seizures, headache and vomiting of 10 days duration. On clinical examination there were no neurological deficits or papilledema. Computed tomogram (CT) scan of the brain showed a solid-cystic lesion in the left temporal lobe, which was hypo- to hyperdense with peripheral contrast enhancement []. Gross total resection of the tumor was done and sent for histopathological examination. Immediate post-operative period and further recovery was uneventful.\nMicroscopic examination showed a cellular tumor, composed of monomorphous, bipolar cells arranged in dyscohesive sheeting pattern [], interspersed with microcytic spaces and abundant myxoid matrix []. The tumor cells were small to medium-sized, with scant cytoplasm and bipolar, short cytoplasmic processes arranged in an angiocentric fashion, forming pseudorosettes []. Brisk mitotic activity and foci of necrosis [] was seen. However, no Rosenthal fibers or eosinophilic granular bodies were noted. Immunohistochemical studies showed strong glial fibrillary acid protein (GFAP) positivity [ inset], p53 positive in occasional tumor cell nuclei [] and Ki-67 labeling index varied from 30-40%% [] in different areas of the tumor. Tumor cells were immunonegative for epithelial membrane antigen (EMA) [ inset].\nThe patient had received 50Gy radiation with concurrent temozolamide (TMZ) over a period of 5 weeks at 75 mg/m2. The patient presented with recurrence after two years. Histological examination of the repeat gross total excision specimen showed radiation-induced geographic necrosis [Figure and ], ghost outlines of angiocentric tumor pattern [], with preserved tumor islands, retained angiocentricity and myxoid matrix [ inset], and Ki-67 labeling index of 40-50% []. The patient had received 50 Gy radiation with concurrent TMZ over a period of 5 weeks at 75 mg/m2, followed adjuvant TMZ at 150 mg/m2 from day 1 to day 5, cycles repeated every 4 weeks. | [[12.0, 'year']] | M | {'25521223': 1, '28503586': 1, '10515229': 1, '22217612': 1, '3984542': 1, '33958332': 1, '22826787': 1, '20461521': 1, '18344915': 1, '21286484': 1, '20619565': 1, '22157620': 1, '14683543': 1, '21107083': 1, '15775869': 1, '14561626': 1, '21534206': 1, '24470824': 2} | {} |
165,199 | 3888049-1 | 24,470,826 | noncomm/PMC003xxxxxx/PMC3888049.xml | Unusual cause of hyperammonemia in two cases with short-term and long-term valproate therapy successfully treated by single dose carglumic acid | A 15-year-old male with a 1-month history of epilepsy, headache, and hemiparesis was admitted to the pediatric intensive care unit with altered mental status. The child was managed conservatively along with intravenous antibiotics and was started on intravenous valproate at a dosage of 33 mg/kg and levetiracetam 44 mg/kg was added for breakthrough seizures. He was intubated because of impaired consciousness with increasing confusion and lethargy and increased seizure frequency. The alteration of consciousness was subsequent to the administration of valproate and not due to a progression of the disease. A metabolic encephalopathy was suspected. Laboratory investigations revealed hyperammonemia 283 μg/dl 6 days after initiating valproic acid (VPA) and no signs of hepatotoxicity. There was no family history of any genetic-metabolic disorder. The valproate was ceased and a single dose of carglumic acid 100 mg/kg per day was administered through a nasogastric tube, followed by a rapid improvement in the patient's mental state and a return of the ammonia level to the normal range (50 μg/dl). Blood ammonia remained normal thereafter. | [[15.0, 'year']] | M | {'21981101': 1, '22858088': 1, '19952878': 1, '20184937': 1, '19667003': 1, '22587482': 1, '30034506': 1, '22180549': 1, '21978982': 1, '22305367': 1, '23271612': 1, '21700501': 1, '28007335': 1, '23798907': 1, '32905024': 1, '22619383': 1, '24470826': 2} | {'3888049-2': 2} |
Subsets and Splits
No community queries yet
The top public SQL queries from the community will appear here once available.