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
|
|---|---|---|---|---|---|---|---|---|---|
164,300 | 3835511-1 | 24,265,532 | noncomm/PMC003xxxxxx/PMC3835511.xml | Striatal Dopaminergic Functioning in Patients with Sporadic and Hereditary Spastic Paraplegias with Parkinsonism | This 50-yr-old male was admitted because of a progressive gait disturbance that developed at age 40. Five years later, he became wheelchair-bound. Neurological examination showed severe spasticity in both legs, with marked enhanced tendon reflexes and extensor plantar responses. Muscle strength was decreased to grade 4 (Medical Research Council) in both lower extremities. The patient showed intermittent myoclonus in both legs that started 10 yr prior. He did not have cerebellar dysfunction. Eye movement examination revealed square wave jerk. He had a cystostomy because of urinary retention. Genetic studies for SCA types 1, 2, 3, 6, and 7 and SPG4 were all negative. His mother exhibited spastic gait in her 40s; however her gait disturbance were progressed minimally, and she could walk independently for 30 yr. Other family members were healthy and without any neurological problems. | [[50.0, 'year']] | M | {'16382784': 1, '8618704': 1, '17348041': 1, '19224311': 1, '19453405': 1, '10825354': 1, '14656074': 1, '15242610': 1, '21802993': 1, '16463348': 1, '13045451': 1, '19608203': 1, '19823194': 1, '25729637': 1, '31616253': 1, '11593289': 1, '17030474': 1, '15465400': 1, '9153623': 1, '11087796': 1, '21381113': 1, '31292381': 1, '19085270': 1, '19380697': 1, '6133167': 1, '15548552': 1, '11315592': 1, '19230631': 1, '24265532': 2} | {'3835511-2': 2, '3835511-3': 2, '3835511-4': 2, '3835511-5': 2, '3835511-6': 2, '3835511-7': 2} |
164,301 | 3835511-2 | 24,265,532 | noncomm/PMC003xxxxxx/PMC3835511.xml | Striatal Dopaminergic Functioning in Patients with Sporadic and Hereditary Spastic Paraplegias with Parkinsonism | A 51-yr-old male was diagnosed with SSP. When he was 33 yr old, he experienced right leg weakness and gait disturbance that progressed very slowly. On neurological examination, he presented with spasticity and muscular strength grade 4 in the lower limbs. The patient had increased DTRs in the lower limbs, with bilateral extensor plantar responses and ankle clonus. He is now 56 yr old, and his neurological signs are similar to the initial findings. | [[51.0, 'year']] | M | {'16382784': 1, '8618704': 1, '17348041': 1, '19224311': 1, '19453405': 1, '10825354': 1, '14656074': 1, '15242610': 1, '21802993': 1, '16463348': 1, '13045451': 1, '19608203': 1, '19823194': 1, '25729637': 1, '31616253': 1, '11593289': 1, '17030474': 1, '15465400': 1, '9153623': 1, '11087796': 1, '21381113': 1, '31292381': 1, '19085270': 1, '19380697': 1, '6133167': 1, '15548552': 1, '11315592': 1, '19230631': 1, '24265532': 2} | {'3835511-1': 2, '3835511-3': 2, '3835511-4': 2, '3835511-5': 2, '3835511-6': 2, '3835511-7': 2} |
164,302 | 3835511-3 | 24,265,532 | noncomm/PMC003xxxxxx/PMC3835511.xml | Striatal Dopaminergic Functioning in Patients with Sporadic and Hereditary Spastic Paraplegias with Parkinsonism | This 44-yr-old female presented with a 1-yr history of progressive weakness in the lower limbs. Her gait was spastic and ataxic in nature. To ascertain whether she had a genetic disease, she was evaluated for SCA types 1, 2, 3, 5, 7, and 8 and all were normal. She presented with normal cranial nerves, and spasticity muscular strength grade 4 in the lower limbs. Her gait became aggravated, and she is now at the age of 49 yr wheelchair-bound. | [[44.0, 'year']] | F | {'16382784': 1, '8618704': 1, '17348041': 1, '19224311': 1, '19453405': 1, '10825354': 1, '14656074': 1, '15242610': 1, '21802993': 1, '16463348': 1, '13045451': 1, '19608203': 1, '19823194': 1, '25729637': 1, '31616253': 1, '11593289': 1, '17030474': 1, '15465400': 1, '9153623': 1, '11087796': 1, '21381113': 1, '31292381': 1, '19085270': 1, '19380697': 1, '6133167': 1, '15548552': 1, '11315592': 1, '19230631': 1, '24265532': 2} | {'3835511-1': 2, '3835511-2': 2, '3835511-4': 2, '3835511-5': 2, '3835511-6': 2, '3835511-7': 2} |
164,303 | 3835511-4 | 24,265,532 | noncomm/PMC003xxxxxx/PMC3835511.xml | Striatal Dopaminergic Functioning in Patients with Sporadic and Hereditary Spastic Paraplegias with Parkinsonism | This 59-yr-old male was healthy until the age of 49 yr, when he experienced unsteadiness and gait imbalance. He complained of sensory paresthesia in his feet and orthostatic dizziness. Neurological examination revealed spastic ataxia and postural instability. The patient showed spasticity of the legs with motor weakness. He had autonomic dysfunction on breath test (expiration/inspiration ratio=1.04, normal range>1.08), and the other autonomic functions were normal. He was taking amantadine (200 mg per day) without effect. Orthostatic dizziness disappeared during the follow-up period. Genetic evaluation for SCA types 1, 2, 3, 6, 7, and 17, SPG 4, and Friedreich ataxia (frataxin) were all negative. The patient is now 63 yr old and shows spastic gait, leg weakness, and paresthesia. Orthostatic dizziness, and other dysautonomic symptoms were not present. | [[59.0, 'year']] | M | {'16382784': 1, '8618704': 1, '17348041': 1, '19224311': 1, '19453405': 1, '10825354': 1, '14656074': 1, '15242610': 1, '21802993': 1, '16463348': 1, '13045451': 1, '19608203': 1, '19823194': 1, '25729637': 1, '31616253': 1, '11593289': 1, '17030474': 1, '15465400': 1, '9153623': 1, '11087796': 1, '21381113': 1, '31292381': 1, '19085270': 1, '19380697': 1, '6133167': 1, '15548552': 1, '11315592': 1, '19230631': 1, '24265532': 2} | {'3835511-1': 2, '3835511-2': 2, '3835511-3': 2, '3835511-5': 2, '3835511-6': 2, '3835511-7': 2} |
164,304 | 3835511-5 | 24,265,532 | noncomm/PMC003xxxxxx/PMC3835511.xml | Striatal Dopaminergic Functioning in Patients with Sporadic and Hereditary Spastic Paraplegias with Parkinsonism | A 64-yr-old male presented with a progressive spastic gait and pain in both legs starting 4 yr prior. On neurological examination, he revealed spasticity with brisk DTRs and ankle clonus. He exhibited postural hand tremor and rigidity, primarily on the right side, as well as subtle bradykinesia on rapid alternating movement. On cranial nerve function test, the patient had sensory neural hearing loss and showed head-shaking nystagmus suggestive of left vestibulopathy. His arm motor function was normal, and the motor strength of his legs was mildly decreased (Rt hip 3, Lt hip 4+, Rt knee 4, Lt knee 4+). He complained of urinary hesitancy and difficulty on initial examination; however, approximately 2 months after the initial examination, voiding symptoms disappeared spontaneously. Other autonomic dysfunctions were not observed. He was taking anti-parkinsonian medications for 13 months (last medications were levodopa 600 mg, pramipexole 1.5 mg, amantadine 100 mg per day); however the medications were discontinued due to ineffectiveness and continuous aggravation. SPG4 was normal and SCA genetic tests were not performed. On his last visit (at the age 67 yr), neurological symptoms were worse. He was wheelchair-bound and showed severe dysarthria, dysphagia, and generalized myalgia. He died at the age of 68 yr. | [[64.0, 'year']] | M | {'16382784': 1, '8618704': 1, '17348041': 1, '19224311': 1, '19453405': 1, '10825354': 1, '14656074': 1, '15242610': 1, '21802993': 1, '16463348': 1, '13045451': 1, '19608203': 1, '19823194': 1, '25729637': 1, '31616253': 1, '11593289': 1, '17030474': 1, '15465400': 1, '9153623': 1, '11087796': 1, '21381113': 1, '31292381': 1, '19085270': 1, '19380697': 1, '6133167': 1, '15548552': 1, '11315592': 1, '19230631': 1, '24265532': 2} | {'3835511-1': 2, '3835511-2': 2, '3835511-3': 2, '3835511-4': 2, '3835511-6': 2, '3835511-7': 2} |
164,305 | 3835511-6 | 24,265,532 | noncomm/PMC003xxxxxx/PMC3835511.xml | Striatal Dopaminergic Functioning in Patients with Sporadic and Hereditary Spastic Paraplegias with Parkinsonism | This 34-yr-old female visited the hospital because of spastic gait and weakness in the right leg. She had the symptoms for 2 yr, and they slowly became worse. The DTRs were markedly exaggerated with positive Babinski signs. The motor strength of her legs was grade 4. She had right-beating nystagmus on virbration. The patient had the following parkinsonian features: dysarthria, hypomimia, hypophonia, postural tremor, rigidity, and bradykinesia (Unified Parkinson's Disease Rating Scale, 22). She complained of occasional orthostatic dizziness; however, orthostatic blood pressure, urinary function tests were normal. The genetic analysis excluded SPG3, 4, and SCA 2, 3, and 17. After taking anti-parkinsonian medications (levodopa/carbidopa 1200 mg, pramipexole 1.5 mg per day), tremor, bradykinesia, and rigidity were partially improved. During the 5-yr follow-up period, she experienced intermittent dizziness that was not troublesome, and neurological symptoms were slightly aggravated. | [[34.0, 'year']] | F | {'16382784': 1, '8618704': 1, '17348041': 1, '19224311': 1, '19453405': 1, '10825354': 1, '14656074': 1, '15242610': 1, '21802993': 1, '16463348': 1, '13045451': 1, '19608203': 1, '19823194': 1, '25729637': 1, '31616253': 1, '11593289': 1, '17030474': 1, '15465400': 1, '9153623': 1, '11087796': 1, '21381113': 1, '31292381': 1, '19085270': 1, '19380697': 1, '6133167': 1, '15548552': 1, '11315592': 1, '19230631': 1, '24265532': 2} | {'3835511-1': 2, '3835511-2': 2, '3835511-3': 2, '3835511-4': 2, '3835511-5': 2, '3835511-7': 2} |
164,306 | 3835511-7 | 24,265,532 | noncomm/PMC003xxxxxx/PMC3835511.xml | Striatal Dopaminergic Functioning in Patients with Sporadic and Hereditary Spastic Paraplegias with Parkinsonism | This 73-yr-old male showed gait disturbance at age 71. On walking, he showed a typical spastic gait with postural abnormality. The patient had spastic paraparesis with bilateral Babinski signs and brisk DTRs. After reviewing his sleep pattern, we concluded he had rapid eye movement (REM) sleep behavior disorder (RBD) and periodic limb movement disorder (PLMD). He was diagnosed approximately 8 yr prior with benign prostate hypertrophy, and was suffering from voiding symptoms including frequencyuria and urgency. The genetic tests, including Friedreich ataxia, SPG4, and SCA types 2 and 3, were normal. | [[73.0, 'year']] | M | {'16382784': 1, '8618704': 1, '17348041': 1, '19224311': 1, '19453405': 1, '10825354': 1, '14656074': 1, '15242610': 1, '21802993': 1, '16463348': 1, '13045451': 1, '19608203': 1, '19823194': 1, '25729637': 1, '31616253': 1, '11593289': 1, '17030474': 1, '15465400': 1, '9153623': 1, '11087796': 1, '21381113': 1, '31292381': 1, '19085270': 1, '19380697': 1, '6133167': 1, '15548552': 1, '11315592': 1, '19230631': 1, '24265532': 2} | {'3835511-1': 2, '3835511-2': 2, '3835511-3': 2, '3835511-4': 2, '3835511-5': 2, '3835511-6': 2} |
164,307 | 3835515-1 | 24,265,536 | noncomm/PMC003xxxxxx/PMC3835515.xml | An Unusual Presentation of Diabetic Ketoacidosis in Familial Hajdu-Cheney Syndrome: A Case Report | A 21-yr-old Korean man was referred to the Department of Endocrinolgy and Metabolism from his primary physician for proper management of hyperglycemia and metabolic impairment on September 21 of year 2012. During the 2 yr since the patient's discharge from military service he had lost approximately 10 kg in body weight. At admission, he complained of polyuria and polydipsia. He had no known medical past history or fracture history, but his mother and elder brother had been diagnosed with HCS along with osteoporosis and multiple compression fractures of the spine (, ). On admission the patient showed stable vital signs and alert mental status. Blood pressure was 107/63 mmHg and pulse rate was 59/min. His height was 175 cm (50 percentile) and weight was 55 kg (10 percentile). The physical examination revealed dysmorphic features, such as marked eyebrow, which was similar to that of his brother in whom HCS had been diagnosed at the age of 20. Short and clubbed fingers were found (). Laboratory investigations revealed hemoglobin of 11.8 g/L, total white blood cell count of 4,800/µL (73% neutrophils). Serum biochemistry, hormones, and bone markers were tested (). No antibody to glutamic acid decarboxylase was detected. Arterial blood gas analysis revealed metabolic acidosis (pH 7.318, base excess -15.2 mM/L, bicarbonate 8.6 mM/L) with incomplete respiratory compensation (pCO2 17.2 mmHg). Urine analysis revealed that specific gravity of 1.024, strong positive in glucose and ketone bodies. A chest X-ray was normal and electrocardiography showed normal sinus rhythm. After an initial treatment for DKA, evaluations for metabolic bone disease were performed. Laboratory findings showed follicle-stimulating hormone 0.7 IU/L, luteinizing hormone 1.2 IU/L, testosterone 2.25 ng/mL (7.8075 nM/L), osteocalcin 8.9 ng/mL (1.5219 nM/L), 25-hydroxy-Vitamin D 5.7 ng/mL (14.2272 nM/L), intact parathyroid hormone <8 ng/L, and urine N-telopeptide 151 nM/mM creatinine. A hand X-ray revealed brachydactyly of the 5th finger of both hands (). Thoracolumbar spine X-ray revealed a fracture of T12 and L3 (). Skull X-ray revealed wormian bone (). Bone mineral density revealed osteoporosis (L1-L2 0.826 g/cm2, T-score -2.6, Z-score -2.5, total femur 1.203 g/cm2, T-score 2.0, Z-score 1.8) (). To confirm the diagnosis of HCS, DNA sequencing was conducted. The sequence analysis revealed that the patient and his mother had a heterogenetic mutant allele in the NOTCH2 gene (c.6443T>G) () leading to an amino acid substitution from a leucine to a stop codon (p.Leu2148*) (). The patient was diagnosed with HCS and DKA. To manage DKA, fluid resuscitation and insulin treatment was initiated. His metabolic acidosis normalized. Since then the patients has been treated with 1,250 mg of calcium carbonate and 1,000 IU of cholecalciferol per day under the diagnosis of osteoporosis and compression fracture due to HCS, and with 5 units of basal insulin and 7 units of pre-prandial insulin injection for type 1 DM. Laboratory analyses conducted during the course of treatment showed improved HbA1c (7.5%) and fasting glucose (175.8 mg/dL or 9.768 mM/L). | [[21.0, 'year']] | M | {'22457355': 1, '21378985': 1, '22002679': 1, '22461559': 1, '18815778': 1, '16773578': 1, '23401378': 1, '22749662': 1, '20096396': 1, '21681853': 1, '10842072': 1, '22492351': 1, '23117206': 1, '33520214': 2, '22964416': 1, '11343321': 1, '18918373': 1, '14303950': 1, '24265536': 2} | {'7820303-1': 1} |
164,308 | 3835516-1 | 24,265,537 | noncomm/PMC003xxxxxx/PMC3835516.xml | Glufosinate Herbicide Intoxication Causing Unconsciousness, Convulsion, and 6th Cranial Nerve Palsy | A 34-yr-old man ingested 150 mL of glufosinate ammonium herbicide on November 06, 2012. Formulation of the herbicide is as follows: glufosinate ammonium, 18%; alcohol (C12-14), ethoxylated monoether with sulfuric acid, sodium salts (CAS No. 68909-66-0) as surfactants, 28.5%; antifoaming agent and coloring agent, 0.5%; and water, 53%. One hour after ingestion, gastric lavage was performed at the primary care clinic. At 5 hr after ingestion, the patient underwent hemodialysis for 2 hr (). Eight hours after ingestion, the patient complained of diplopia. Eleven hours after ingestion, the patient exhibited a stuporous mental state and was transferred to the toxicology center in our hospital. Physical examination showed a blood pressure of 100/70 mmHg and pulse rate of 76 beats/min. Chest radiography and electrocardiography showed no abnormalities. Initial laboratory findings, including complete blood cell count, liver function test, urea nitrogen, creatinine, and electrolytes, were within normal limits.\nAbout 1 hr after arrival at our emergency room, bronchial secretion increased and respiration become shallow with hypoxia (arterial oxygen tension [PaO2], <70 mmHg) and CO2 retention (arterial carbon dioxide tension [PaCO2]>50 mmHg). Tracheal intubation was performed and mechanical ventilation was initiated.\nAccording to the protocol for the management of large amounts of herbicide ingestion, a loading dose of an intravenous lipid emulsion (ILE) was administered, and subsequently, the patient was maintained on lipid emulsion infusion.\nOn the second day of the hospital stay, the patient exhibited drowsiness. In the intensive care unit, the patient was treated with hemoperfusion for extracorporeal elimination of toxic ingredients of the herbicide, and was put on mechanical ventilation for apnea. The patient developed tonic-type convulsions, for which he received lorazepam injection. On the fifth day of the hospital stay, the patient underwent successful weaning trials and was extubated, after which he complained of double vision. Ophthalmologic examination showed that he had esotropia (10 prism diopters), and the angle increased while gazing to the right and left. The Lancaster red-green test () showed incomitant uncrossed diplopia at 9 different positions of gaze, and the deviation increased for both the right and left gaze (). These results suggest that the patient had bilateral sixth cranial nerve palsy. A second test performed 1 day later showed an improvement in the esodeviation with symptomatic relief. On the seventh hospital day, brain magnetic resonance imaging (MRI) was performed to rule out organic disease. No structural abnormality was found, especially in the intracisternal, intracavernous, and intraorbital portions through which the sixth cranial nerve passes. In addition, diplopia and nystagmus intensity began to subside. On the ninth hospital day, diplopia disappeared and the nystagmus intensity decreased further. The patient was discharged on the eleventh day without any ophthalmologic abnormality or abnormal blood chemistry results. | [[34.0, 'year']] | M | {'2379893': 1, '16668090': 1, '31723872': 2, '11335070': 1, '17982243': 2, '32340163': 1, '21149144': 1, '13906716': 1, '16966570': 1, '23099315': 1, '11450748': 1, '9491336': 1, '10509445': 1, '22480254': 1, '21833717': 1, '22219606': 1, '22787363': 1, '22627464': 1, '9266815': 1, '6838420': 1, '9492096': 1, '9338028': 1, '24265537': 2} | {'6849054-1': 1, '2693861-1': 1} |
164,309 | 3835517-1 | 24,265,538 | noncomm/PMC003xxxxxx/PMC3835517.xml | ANCA-Negative Wegener's Granulomatosis with Multiple Lower Cranial Nerve Palsies | A 54-yr-old female was referred for neurologic consultation due to progressive dysarthria, dysphagia and left facial palsy for past 5 month in January 2011. She had no underlying medical illness such as diabetes mellitus, and had underwent first functional endoscopic sinus surgery (FESS) for bilateral maxillary sinusitis 4 yr ago. She had remained tolerable after the surgery for about 3. 5 yr then the symptoms of headache, hearing difficulty, dysarthria and dysphagia slowly emerged and progressed for about 6 months. Under the diagnosis of recurrent sinusitis involving frontal, ethmoid, sphenoid and maxillary sinuses, second FESS and broad-spectrum antibiotics were treated. Although these surgical and medical treatments were adequately performed, her symptoms worsened as aggravated hearing problem, severe bulbar symptoms and newly developed left facial palsy. The first brain MRI with using contrast enhancement was performed, only to reveal still existing bilateral otomastoiditis and extensive sinusitis (). Cerebrospinal fluid (CSF) had no cell counts and normal protein level. The empiric steroid therapy (methysol 2 mg/kg/day) without definite diagnosis has somewhat stabilized these series of aggravating symptoms. At then, she was referred to our clinic for more detailed evaluation.\nIn January 2011 when she was admitted in our clinic, all the vital signs including body temperature were stable. Accompanied by severe bulbar symptoms of dysarthria and dysphagia, she complained of constitutional symptoms such as general weakness, poor oral intake and weight loss of up to 12 kilograms during past 6 months. Neurologic examination revealed various lower cranial nerve palsies of both sides, which were overall more prominent on left side than right side. Demonstrated clinical manifestations and positive neurologic findings are; facial diplegia, progressive hearing difficulty resulting in near-deafness, bilateral tinnitus and hyperacusis, right deviation of uvula, bilateral decreased gag reflexes, flaccid dysarthria with breathy voice and hypernasality, dysphagia especially in liquid food, left vocal cord palsy, left sternocleidomastoid muscle weakness, impaired tongue protrusion and giggling, and fasciculation with slight atrophy on the left side tongue. In contrast to these various lower cranial nerve involvements, there was no afferent pupillary defect, and no limitation in extraocular eye movements. Otolaryngology report was given as extensive sinusitis involving bilateral frontal, sphenoid, and maxillary sinuses with nasal cartilage erosion and bilateral chronic otitis media.\nLaboratory studies revealed increased C-reactive protein (CRP) of 29.90 mg/dL (normal range 0-0.3 mg/dL), increased erythrocyte sedimentation rate (ESR) of 67 mm/hr, mild anemia (hemoglobin 10.8 g/dL), but no eosinophila and mild leukocytosis (12,800/µL). Renal function (BUN 15 mg/dL, creatinine 0.6 mg/dL, glomerular filtration rate 111 mL/min) and microscopic urine analysis were normal. Serology test for anti-Ro/SSA, anti-La/SSB, antinuclear, anticardioplin antibody, and ANCA were negative. Neither immunofluorescence method nor direct enzyme-linked immunosorbent assay method for proteinase 3 and myeloperoxidase antigens could detect ANCA. FANA screening and VDRL were also negative, and angiotensin converting enzyme was within normal as 28 U/L (normal range 18-55 U/L). Rheumatoid factor was minimally elevated up to 20.4 IU/mL (normal range 10-18 IU/mL). Serum folate, vitamin B1 and vitamin B12 were measured as normal level. Tumor markers including AFP, CEA, CA19-9, and CA125 were normal.\nChest CT revealed no hilar enlargement or parenchymal lung lesion. Neck CT showed high density lesions in the nasopharynx with enhancement, penetrating the skull base and extensive sinusitis expanding into orbit and retromaxillary fat-pad (). Whole body positron emission tomography (PET) showed hypermetabolic lesion in bilateral tonsil area with multiple lymph nodes in level II/III of neck and also in bilateral frontal, ethmoid, sphenoid, and maxillary sinuses ().\nIn the facial nerve conduction study, the compound muscle action potential (CMAP) amplitude of left facial nerve was decreased to 60% that of right side. Blink reflex test showed delayed ipsilateral R1 and R2, but normal contralateral R2 responses on both supraorbital nerve stimulations. These electrophysilogical findings were indicative of bilateral facial neuropathies, more severe in the left side.\nEndoscopic biopsy was performed twice due to failure to obtain pathologic confirmation at first biopsy. The first biopsy specimen was obtained from nasopharyeal wall layer through a punch biopsy under local anesthesia, but the second endoscopic biopsy was performed far deeper layer of retropharyngeal area under general anesthesia. Tissues obtained from the second biopsy showed granulomatous inflammation and vasculitis involving small sized blood vessels with ischemic necrosis and destruction of cartilage (). Period acid-Schiff, Gomori methenamine silver and acid-fast bacilli stains showed no histological evidence of fungal organism or mycobacterium. Immunostains for CD3 and CD56 had no abnormal infiltration of natural killer T cells. Ebstein-Barr virus-encoded RNA in situ hybridization resulted in negative findings.\nAll the clinical manifestations, laboratory and imaging studies indicated ANCA negative WG involving otolaryngeal space, upper respiratory tract and multiple lower cranial nerves. She was transferred to rhematology department and began therapy with steroid (methysol 1 mg/kg/day) and cyclophosphamide (2 mg/kg/day). Within the ten days of these treatments, the patient stated that her sufferings of headache and constitutional symptoms were prominently diminished. The hearing difficulty, which compelled her to wear a hearing aid, improved dramatically leaving no more need of the device. After three months of combination therapy of tapering steroid (prednisolone 0.6 mg/kg/day) and steady cyclophosphamide (2 mg/kg/day), the patient restored to almost her normal bulbar function and removed nasogastric tube for oral intake. Further repeated ANCA study was negative. Brain MRI was re-evaluated with a time interval of five months from the first MRI study, and showed persistent sinusitis of both frontal, ethmoid, sphenoid, and maxillary sinuses, in despite of marked improvement of clinical symptoms. Moreover, focal thickening with enhancement of the meninges around the area of tentorium cerebelli, inferior aspect of frontotemporal lobe adjacent to sinuses and nasopharynx were newly observed (). Clinical significance of these imaging findings was somewhat debatable because previous neurologic defects such as dysarthria, dysphagia, and hearing impairment had improved remarkably. However, re-evaluated CRP was still high as 5.75 mg/dL, which implied for further immunotherapy. Ultimately, potent immunotherapy consisting of high dose steroid (methysol 2 mg/kg/day) and cyclophosphamide (3 mg/kg/day) was performed. Three weeks later, the follow up CRP fall to 0.39 mg/dl and ESR normalized as 3 mm/hr. Taking only cyclophosphamide (0.25 mg/kg/day) after tapering steroid, she is still in a remission state for more than a year. | [[54.0, 'year']] | F | {'29525845': 1, '30680194': 1, '21629538': 1, '2001873': 1, '10616016': 1, '21861078': 1, '12480669': 1, '16322901': 1, '20647199': 1, '8388187': 1, '22389798': 1, '23024954': 1, '17658554': 1, '22953096': 2, '30774363': 1, '21125151': 1, '17299018': 1, '12823849': 1, '22754821': 1, '34580346': 2, '22430874': 1, '19508947': 1, '11052460': 1, '16728414': 1, '19253059': 1, '21447129': 1, '10968148': 1, '8774975': 1, '17143671': 1, '10580955': 1, '9408075': 1, '16611871': 1, '24265538': 2} | {'8476614-1': 1, '8476614-2': 1, '8476614-3': 1, '3420613-1': 1} |
164,310 | 3835632-1 | 24,265,560 | noncomm/PMC003xxxxxx/PMC3835632.xml | A Suspicious Breast Lesion Detected by Dynamic Contrast-Enhanced MRI and Pathologically Confirmed as Capillary Hemangioma: a Case Report and Literature Review | We present an asymptomatic 30-year-old female Asian patient who gave birth 2 years ago. There is no history of hormone intake or family history of breast cancer. Ten months ago, the patient underwent breast palpation, mammography, and ultrasound (for the first time). The results present a nodule (1.6 × 1.2 × 0.9 cm3) locating on the right breast at 7 o'clock position with an oval shape, clear boundary, internal hypoechoic, and calcifications. Radiologists classify the lesion as BIRADS category 3. The patient requested the lesion to be surgically removed. This lesion was pathologically confirmed as breast fibroadenoma with calcification.\nBefore the surgery, MR scanning (GE Signa HDxt 3.0T) was performed according to the recommendation of a clinician. DCE-MRI did not detect enhancement of the mass (breast fibroadenoma with calcification). Surprisingly, a new small nodule (0.7 × 0.5 × 0.4 cm3) was found at the upper outer quadrant of the right breast. MRI presented an oval mass located on the mammary gland, anterior to the superficial pectoralis fascia with a well-defined margin. An unenhanced T1-weighted image did not display the mass well (), while a T2-weighted image showed the mass area of high signal (). DCE-MRI presented a strong homogeneous enhancement of the mass (). The region of interest was obtained from the enhancing area of the lesion to generate a time-signal intensity curve. The curve was classified as washout type (). Radiologists classify this mass as BI-RADS category 4B. Ultrasound was then carefully performed on the patient (for the second time), showing an oval nodule with a well-defined margin and internal hypoechoic (). Color Doppler flow imaging presented rich blood flow signals ().\nThis suspicious breast lesion was strongly advised to be surgically removed. Before surgery, two experienced radiologists made a surface marker for the lesion based on the DCE-MRI and ultrasound presentation. In the operating room, the surgeon confirmed the lesion again by ultrasound (for the third time) before the surgery. The lesion was pathologically confirmed as breast capillary hemangioma, which is a benign vascular tumor and rarely seen in the breast (). Immunohistochemical staining showed strong positive CD34 expression and negative CK expression of the lesion, which supported our diagnosis (). | [[30.0, 'year']] | F | {'22434703': 1, '21228938': 1, '15615997': 1, '23323026': 1, '22778559': 1, '8372222': 1, '4091183': 1, '11906894': 1, '18253071': 1, '3966983': 1, '24265560': 2} | {} |
164,311 | 3835633-1 | 24,265,561 | noncomm/PMC003xxxxxx/PMC3835633.xml | Isolated Left Ventricular Apical Hypoplasia with Infundibular Pulmonary and Aortic Stenosis: a Rare Combination | A 33-year-old male patient was referred to our hospital for evaluation of a cardiac murmur. The cardiac murmur was found incidentally during a preoperative evaluation for hemorrhoids. He had no history of congenital heart disease, and there was no family history of premature coronary artery disease, cardiomyopathy or sudden cardiac death. A continuous cardiac murmur was heard during the entire heartbeat. A chest radiograph showed no abnormalities. An electrocardiogram (ECG) indicated a normal sinus rhythm, right axis deviation, incomplete right bundle branch block, right atrial enlargement and right ventricular (RV) hypertrophy. Transesophageal and transthoracic echocardiography showed a spherically shaped LV and dilation of all cardiac chambers with good global LV systolic function (). They also revealed heavily calcified aortic valve with moderate to severe stenosis (), mild mitral regurgitation and RV outflow tract acceleration due to infundibular hypertrophy (). Grade II diastolic dysfunction was also noted (E/A ratio, 1.9; isovolumic relaxation, 90 ms; deceleration time, 210 ms).\nCardiac magnetic resonance imaging (MRI) was performed using a 1.5-T scanner with standard ECG-based referencing (Siemens Symphony, Erlangen, Germany), and the findings supported and better defined the echocardiographic results. The MRI sequences were as follows: Anatomical evaluation was performed with transverse dark-blood HASTE (repetition time [TR]/echo time [TE], 700/27; matrix size, 256 × 123; slice thickness, 8 mm) with or without fat saturation. Dynamic evaluation was done with an echo-planar cine true fast imaging with steady-state precession (TR/TE, 66.2/1.3; matrix size, 192 × 113; slice thickness, 5 mm) in two-, three-, four-chamber and short axis views. Phase-sensitive inversion recovery turbo FLASH pulse sequence was also performed for the detection of LV myocardial scar (TR/TE, 826.4/3.3; matrix, 256 × 156; slice thickness, 6 mm) 10 minutes after intravenous injection of gadobutrol (0.2 mmol/kg). The LV exhibited a truncated and spherical configuration with rightward bulging of the interventricular septum and elongation of the RV. The papillary muscles originated from the flattened anterior apex (). Quantitative RV ejection fraction was normal (52%). Transverse HASTE images outlined fat within the myocardium at the LV apex (). There was no delayed enhancement of the myocardium to indicate myocardial fibrosis. Further, there was also infundibular pulmonary stenosis and aortic stenosis () without an atretic mitral valve. These echocardiographic and cardiac MRI anatomic features were consistent with isolated LV apical hypoplasia combined with infundibular pulmonary stenosis and aortic stenosis. | [[33.0, 'year']] | M | {'22093515': 1, '32128492': 2, '27114737': 2, '20591617': 1, '21443580': 1, '22576770': 1, '22105200': 1, '20738942': 1, '18574051': 1, '19070501': 1, '26417869': 1, '26798219': 2, '17503030': 1, '21911343': 1, '21131656': 1, '15084556': 1, '22694050': 1, '24265561': 2} | {'4834181-1': 1, '7047060-1': 1, '4720816-1': 1} |
164,312 | 3835638-1 | 24,265,566 | noncomm/PMC003xxxxxx/PMC3835638.xml | Biliary Peritonitis after Radiofrequency Ablation Diagnosed by Gadoxetic Acid-Enhanced MR Imaging | An 80-year-old female with liver cirrhosis from hepatitis C was presented to our hospital with elevated serum alpha-fetoprotein. Liver dynamic CT consisting of two phases was performed with a four-channel multi-detector row CT (LightSpeed QX/i; General Electric Medical Systems, Milwaukee, WI, USA). Two-phasic scanning was started with a 35-delay for the arterial phase, and a 90-second delay for the portal venous phase, after the beginning of contrast medium injection. The patient had a hepatic mass in segment VIII, which showed marked enhancement in the arterial phase and low attenuation compared to liver parenchyma in the portal venous phase, which was diagnosed as typical hepatocellular carcinoma (). RFA was the most appropriate treatment as opposed to surgery, and was performed using three bipolar RF electrodes (CelonPOWER system; OLYMPUS Winter & Ibe GmbH, OLYMPUS, Japan) with a 30-mm exposed tip. Contrast-enhanced CT in the portal venous phase obtained seven days after RFA revealed an ablated tumor with sufficient margin (). The patient had a good course and was discharged two weeks after RFA therapy.\nTwo months later, the patient had sudden upper abdominal pain, and underwent a dynamic contrast-enhanced CT scan. The scan demonstrated marked enhancement on the surface of the liver, fluid collection around the liver, and dirty omentum. Biliary peritonitis was suspected. The next day, MR imaging with Gd-EOB-DTPA (Primovist, Bayer-Schering, Berlin, Germany) was performed to evaluate biliary leakage from the liver.\nMRI examination was performed with a 1.5-T clinical unit (Signa EXCITE EchoSpeed Plus; General Electric Medical Systems, Milwaukee, WI, USA) using a TORSO coil. Liver Acquisition with Volume Acceleration sequence was used with the following parameters: TR/TE/FA: 5.3 ms/2.3 ms/12°, 4.6-mm thickness without gap, and 320 × 192 matrix. A bolus of 0.025 mmol/kg body weight Gd-EOB-DTPA was injected intravenously by a power injector at a rate of 1 mL/s. Enhanced T1-weighted images were acquired 20, 60, and 300 minutes after the administration of Gd-EOB-DTPA (). Enhanced T1-weighted MR imaging acquired at 300 minutes showed increased signal intensity in the fluid collection around the liver than in the image acquired at 20 minutes. Linear high signal areas to match the RFA puncture route were also demonstrated, suggesting a biliary leakage route from the bile duct to the fluid collection around the liver. Biliary peritonitis was diagnosed. Percutaneous drainage for fluid collection around the liver was performed, and the collected fluid was confirmed as bile. | [[80.0, 'year']] | F | {'1395339': 1, '19959517': 1, '15688109': 1, '20592937': 2, '10470888': 1, '9000414': 1, '18369632': 1, '15690326': 1, '16419172': 1, '20966318': 1, '24265566': 2} | {'2893324-1': 1} |
164,313 | 3835639-1 | 24,265,567 | noncomm/PMC003xxxxxx/PMC3835639.xml | MR Imaging Findings of Extraovarian Endocervical Mucinous Borderline Tumors Arising from Pelvic Endometriosis | A 35-year-old woman (gravida 0, para 0, abortus 0) presented with dysmenorrhea and vague lower abdominal pain. Her past history was unremarkable. At the time of presentation, she was having regular menstrual cycles. She was afebrile and her abdomen was soft and flat without any tender points. Laboratory data were normal except for the elevated level of CA 125 (44 U/mL; normal 0-35 U/mL) and CA 19-9 (271 U/mL; normal 0-37 U/mL).\nMRI showed an elongated tubular cystic lesion with high signal intensity on both T1- and T2-weighted images in the left adnexa, which was diagnosed as hematosalpinx. An axial T2-weighted image showed a stellate low signal intensity lesion with obliteration of the cul-de-sac, suggestive of deep infiltrating endometriosis. The uterus was diffusely enlarged with focal adenomyosis and an intraumral myoma in the posterior wall. Two small cystic lesions with mural nodules were present in the cul-de-sac along the posterior margin of the uterus. These cystic components showed high signal intensity similar to that of urine on T2-weighted images as well as high signal intensity that was slightly higher than that of myometrium on fat-suppressed T1-weighted images. Peripheral nodular enhancement was seen in the cystic lesions on gadolinium-enhanced T1-weighted images, raising the possibility of malignancy (). Mural nodules were not definitely seen in the left fallopian tube.\nSurgery revealed severe pelvic endometriosis involving bilateral ovaries, the cul-de-sac, rectosigmoid colon and left hydrosalpinx. On histopathologic examination, cul-de-sac masses and the left fallopian tube showed mucin-filled cysts and branching papillary projections. The papillae were lined by endocervical type mucinous cells. Microscopic examination revealed a gradual transition from the epitheliums of endometeriosis to mucinous tumor cells. The pathologic diagnosis was confirmed as endocervical mucinous boderline tumor involving the cul-de-sac and left fallopian tube arising from extensive pelvic endometriosis. | [[35.0, 'year']] | F | {'18575828': 1, '20955384': 1, '3334969': 1, '12459620': 1, '12218816': 1, '16431901': 1, '13087921': 1, '16289714': 1, '20093596': 1, '11093056': 1, '338141': 1, '11293158': 1, '22582363': 1, '24265567': 2} | {} |
164,314 | 3835641-1 | 24,265,569 | noncomm/PMC003xxxxxx/PMC3835641.xml | Endovascular Treatment of Aneurysm of Splenic Artery Arising from Splenomesentric Trunk Using Stent Graft | A 35-year-old man presented with recurrent upper abdominal pain. Physical examination and laboratory investigations did not reveal any abnormality. Abdominal ultrasound showed an aneurysm arising from the splenic artery. CT angiography revealed a 2.2 cm saccular aneurysm arising from splenic artery close to its anomalous origin from the SMA. The aneurysm was located posterior to the body of pancreas (). The CT angiography showed that the splenic artery was the first branch of SMA and there was only a 5 mm distance between the ostium of splenic artery and the aneurysm. The next branch from the SMA was the first jejunal branch, 1 cm distal to the splenic artery ostium. The proximal SMA measured 7 mm and the distal splenic artery 6 mm in diameter. An endovascular therapy was planned. It was decided to 'trap' the aneurysm by occluding the splenic artery distal to the aneurysm with coils and placing a stent graft in the SMA to block the inflow into the aneurysm. Under conscious sedation and local anesthesia the left axillary artery was punctured and a 45 cm long 8 Fr sheath (Flexor, Cook, Bloomington, IN, USA) was placed into the upper abdominal aorta. In addition, a pigtail catheter was introduced from a right femoral route and placed at the level of SMA ostium to enable angiography prior to stent deployment. A 4 Fr vertebral Glide catheter and 0.35" hydrophilic wire (Terumo, Tokyo, Japan) combination was used to engage the splenic ostium and the catheter was negotiated through the aneurysm into the normal segment just distal to aneurysm. Four 0.38" steel coils (Tornado, Cook, Bloomington, IN, USA) with diameters ranging from 7 mm to 4 mm were delivered through the catheter to occlude the splenic artery (). The catheter was then withdrawn into the SMA and exchanged for a stiff Amplatz wire. An 8 mm × 4 cm Fluency Plus stent graft (Bard, Tempe, Arizona, USA) was negotiated over the wire and deployed with its proximal end at the SMA ostium and distal end just short of the first jejunal branch. Angiography showed complete exclusion of aneurysm and good filling of all SMA branches (). Distal splenic artery was seen reforming through left gastric and right gastroepiploic collaterals. The patient made an uneventful recovery and was discharged on the third day after procedure. There were no splenic infarcts. Follow-up CT scans at one month and three years showed patent SMA and complete thrombosis of the aneurysm. There were no splenic infarcts. | [[35.0, 'year']] | M | {'16132382': 1, '16967221': 1, '14709694': 1, '11357975': 1, '15944609': 1, '20382495': 1, '5639690': 1, '7457704': 1, '25053916': 1, '8911418': 1, '12089631': 1, '27134524': 1, '32952628': 1, '17765650': 1, '21315545': 1, '24265569': 2} | {} |
164,315 | 3835643-1 | 24,265,571 | noncomm/PMC003xxxxxx/PMC3835643.xml | A Case of an Idiopathic Massive Osteolysis with Skip Lesions | A 38-year-old man is initially presented with pain and decreased strength in the left arm. Laboratory results were normal including renal function tests (urea, creatinine), thyroid and parathyroid hormone (PTH) tests. There was no history of any trauma and family history of similar diseases.\nRadiography of upper left extremity showed no abnormalities during this time. The patient was treated with non-steroidal anti-inflammatory drugs but the pain was not relieved, the patient was referred for radiography 2 years later. During this time, radiography demonstrated almost complete non-contiguous osteolysis in the left elbow, proximal and distal radius, ulna, wrist, carpal bones, proximal and distal metacarpals and phalanges (). Static images from bone scintigraphy with 99 technecium methylene diphosphate (99mTc-MDP) showed increased activities in the osteolysed regions (). To define the extent of the osteolysis, CT examination was performed. CT confirmed the osteolysis and accompanying soft tissue masses in the affected regions. MRI demonstrated that the soft tissue masses and the affected bones were hypointense to isointense with muscle on T1-weighted imaging (TR/TE, 732/12) and were of heterogeneous signal intensity on T2-weighted imaging (TR/TE, 3986/99) (). The lesions showed contrasting enhancements on T1-weighted sequences after intravenous administration of gadopentate dimeglumine.\nMulti-site fine needle aspiration biopsy and tru-cut biopsy were undertaken; bone specimens were taken from proximal, distal radius and proximal ulna for pathological examinations. This revealed fibrous tissue containing proliferated vascular structures replaced with bone trabeculae and bone marrow (). The pathological specimen obtained at open biopsy of a lesion in the left proximal radius also confirmed such findings. The patient was referred for radiation therapy. Radiation therapy was utilized to control the tumors locally and to alleviate associated pains. Because of its solely palliative role, full disease resolution was never accomplished. After one year follow-up there was no radiologic change. | [[38.0, 'year']] | M | {'3968152': 1, '25276311': 1, '30024550': 2, '5578225': 1, '32090147': 1, '25405099': 1, '11981608': 1, '9352051': 1, '3968151': 1, '2796865': 1, '13263344': 1, '28384557': 1, '807583': 1, '1447260': 1, '13568048': 1, '9230956': 1, '24265571': 2} | {'6086544-1': 1} |
164,316 | 3835645-1 | 24,265,573 | noncomm/PMC003xxxxxx/PMC3835645.xml | Brain Stem Hypoplasia Associated with Cri-du-Chat Syndrome | A girl aged 1 year and 4 months was admitted for evaluation of psychomotor retardation and an operation for exotropia. She was born at term (gestational age: 40 weeks) by cesarean delivery after an uneventful pregnancy, and was the first child of healthy, unrelated parents. The family history was unremarkable. On admission, she was presented with a characteristic high-pitched cry and microcephaly. She showed delayed motor functions and crawling. She was able to sit unassisted but unable to stand; her developmental characteristics lagged behind for the normal chronologic age. On neurologic examination, she had left leg hypotonia, with normal tendon reflexes and normal cranial nerve signs.\nWhen she was 6 months old, the ventricular septal defect was detected by echocardiography. Chromosome analysis revealed 46,XX,del () (p15.2) one month after delivery, resulting in a diagnosis of 5p-syndrome. When the patient was 1-year-old, MRI revealed brain stem hypoplasia (most prominently in the pons), with normal cerebellum, diffuse mild atrophy of the deep white matter of both cerebral hemispheres, as well as mild thinning of the corpus callosum and mega cisterna magna. The anterior limb of the internal capsules on both sides showed reduced myelination, which decreased as compared to normal myelination patterns for 1-year-old infants (). | [[1.0, 'year'], [4.0, 'month']] | F | {'9916835': 1, '7791950': 1, '8328329': 1, '15849798': 1, '28676976': 1, '19651480': 1, '17924529': 1, '8215223': 1, '24265573': 2} | {} |
164,317 | 3835646-1 | 24,265,574 | noncomm/PMC003xxxxxx/PMC3835646.xml | Desmoplastic Fibroma of Bone in a Toe: Radiographic and MRI Findings | A 14-year-old male was presented with a several month history of intermittent painful swelling in the first toe. A clinical examination showed that the first toe was swollen, painful and tender to palpate. There was no specific medical or family history. Forefoot radiographs revealed a well-defined, thin sclerotic margined, oval, osteolytic lesion in the distal phalanx of the first toe. The lesion was eccentrically located within the medullary cavity. There revealed the cortical thinning and cortical breakthrough in dorsal aspect of the distal phalanx. The associated pathologic fracture through the distal portion of the osteolytic lesion was also demonstrated. There were no demonstrable matrix mineralization or distinctive periosteal reaction (). On MRI, the well demarcated, ovalshaped, osteolytic lesion was composed of the central and peripheral parts showing different signal intensity and gadolinum contrast enhancement from each other. The central part of the mass showed low signal intensity on both T1-weighted image (T1WI) and T2-weighted image (T2WI). This central area was little contrast-enhanced on fat-suppressed T1WI after gadolinium administration. The surrounding peripheral part showed isointensity or intermediate signal intensity on T1WI, intermediate to high signal intensity on T2WI and short tau inversion recovery (STIR) image, and heterogeneous gadolinium with areas of intense enhancement, and no to minor enhancement in other areas. MRI also clearly demonstrated the cortical thinning and breakthrough in the dorsal aspect of the distal phalanx, with extension of the small soft tissue mass into the subungal area of the first toe (). The patient underwent total excision of the lesion. Histologically, the tumor was not encapsulated, but was vaguely delineated from the non-neoplastic tissue. At low power, the tumor displayed a central hypocellular area surrounded by peripheral cellular area. At high power view, the central hypocellular area showed sclerosis with dense collagen deposition. The peripheral cellular area was composed of spindle cells forming fascicles. The tumor cells exhibited minimal cytologic atypia and no mitotic figures. On microscopic examination, no osteoid or chondroid materials were identified, essentially excluding the possibilities of osteogenic or chondrogenic bone tumors. These histopathological features were consistent with a benign fibrogenic tumor, suggesting a diagnosis of desmoplastic fibroma of bone (). | [[14.0, 'year']] | M | {'10794556': 1, '1913545': 1, '21783062': 1, '26379329': 1, '9265665': 1, '3034013': 1, '15908527': 1, '17126252': 1, '24265574': 2} | {} |
164,318 | 3835648-1 | 24,265,576 | noncomm/PMC003xxxxxx/PMC3835648.xml | Placental Transmogrification of the Lung | A 31-year-old female patient presented with cough and mild dyspnea to the outpatient department. She had a history of being diagnosed with pulmonary tuberculosis 7 years ago, which she had completely recovered after 6 months of antituberculosis drug regimen. There was no other notable history. Dullness and decreased breathing sound at the left side was noted after auscultation. Complete blood count revealed no abnormal finding, and no acid-fast bacilli was discovered on the sputum culture test. Plain chest radiography showed giant bulla in the left lung and subsegmental consolidation in the left lower lung field. Chest computed tomography (CT) scan revealed the left upper lobe of the lung being replaced by bullae of various sizes, and subsegmental consolidation had broad contacted with bullae at the lingular segment of the left upper lobe (). The right lung showed no abnormality. The patient's pulmonary function test signified moderate obstructive respiratory failure. Wedge resection of the left lung using video-assisted thoracoscopy was performed under the preoperative diagnoses of giant bullae and pulmonary emphysema. During operation, the left lower lobe was found to be severely compressed by the bullae. Gross examination of the wedge resected lung was 15 × 9 × 4 cm in size with dilated giant bullae (). Spongiform emphysema was observed at the cut surface, and there were partial solid changes and fibrotic progression. Examination of the solidified sites by light microscope indicated general emphysematous changes with destruction of the alveolar wall in addition to numerous tissues replaced by structures similar to the chorionic villi. The villous structure was adjacent to, or in some cases adhered to, the destroyed alveolar wall and interlobular septa. Immunohistochemical staining of the epithelium lining the villous structure was partly positive for thyroid transcription factor-1, and the interstitial cells at the core was positive for CD-10, D2-40 and vimentin (). There was no human chorionic gonadotropin positive cell.\nThe chest tube was removed 8 days after surgery. Pathologic examination of the surgical specimen reported placental transmogrification of the lung. Chest CT scan was taken to rule out the possibility of remnant bullae or transmogrified tissue. The giant bullae at the left upper lobe were removed, but some fibrotic tissue still remained. Additional left upper lobectomy was planned to remove the remaining tissue in order to minimize recurrence. However, the patient did not complain of any symptoms and refused further operation. She was explained about the possibility of recurrence and scheduled visits to the outpatient clinic on a regular basis for follow-ups. | [[31.0, 'year']] | F | {'33199330': 1, '15208118': 1, '28858088': 2, '7726366': 1, '26210476': 1, '19161794': 1, '15116336': 1, '9236366': 1, '29043210': 2, '11958661': 1, '9333786': 1, '24265576': 2} | {'5634434-1': 1, '5585482-1': 1, '5585482-2': 1} |
164,319 | 3835649-1 | 24,265,577 | noncomm/PMC003xxxxxx/PMC3835649.xml | Solitary Fibrous Tumor of the Pleura Manifesting as an Air-Containing Cystic Mass: Radiologic and Histopathologic Correlation | A 58-year-old woman was transferred to our hospital due to an incidentally identified lung mass at an outside hospital. She was a never smoker and was asymptomatic. Chest radiography showed an ovoid, thick-walled, air-containing cystic mass in the right upper lung zone where the mass was located eccentrically and surrounded by crescent-shaped air on the medial aspect (). Chest CT demonstrated a 4.5 cm-sized, spindle-shaped cystic mass containing air within the superior portion of the right major fissure (). This mass had an irregular thin wall with a thickness of several millimeters. A well-defined soft tissue nodule was eccentrically located within the lesion and measured approximately 3 cm. The nodule had a homogeneous density of 25 Hounsfield units (HU) on pre-contrast CT and was relatively homogenously enhanced after intravenous contrast administration, with a density of 50 HU (). Her previous chest CT images, performed at the outside institution approximately 2 years prior, revealed a same-sized cystic mass containing a much smaller internal soft tissue nodule compared to the lesion on the present CT images (). F18-fluorodeoxyglucose (FDG) PET/CT demonstrated mild FDG uptake with a maximum standard uptake value (SUVmax) of 2.3 ().\nVideo-assisted thoracoscopic surgery was performed to resect the tumor. An encapsulated ovoid cystic mass was shown in the pleural space, with a broad short pedicle and prominent blood vessels over the thin serosal lining of the tumor (). The stalk arose from the posterior portion of the visceral pleura enveloping the right upper lobe within the upper portion of the right major fissure. Cut sections of a gross specimen revealed an air-containing cystic mass with an eccentrically yellow-gray-colored solid nodule (). Histopathologic examination with hematoxylin-eosin (HE) staining revealed spindle-shaped tumor cells mixed with collagen fibers in the tumor and its cystic wall without evidence of mitosis or nuclear pleomorphism, representing a benign SFT (). There was cleft-like cystic air space surrounding the solid nodule, which was partially lined by cuboidal cells (). Immunohistochemical studies of the tumor showed diffuse staining for CD34 and vimentin, and the cells lining the cystic portion of the tumor stained positively for TTF1 and CK7, suggesting that they were pneumocytes originating from alveoli or terminal respiratory epithelium (). Antibodies for actin, desmin, S100, and CD117 (c-kit) were nonreactive in the tumor cells. | [[58.0, 'year']] | F | {'18525234': 2, '2665534': 1, '32774859': 2, '21566534': 1, '11156076': 1, '1913308': 1, '12016362': 1, '12740474': 1, '29245313': 1, '1566702': 1, '208101': 1, '11422800': 1, '24265577': 2} | {'2627258-1': 1, '7399368-1': 1} |
164,320 | 3835975-1 | 24,311,897 | noncomm/PMC003xxxxxx/PMC3835975.xml | Robot assisted laparoscopic retroperitoneal lymph node dissection in testicular tumor | A 23 year old male presented to us with right testicular swelling for 4 months. There was no significant past history or family history of testicular tumors. On examination, the right testis was enlarged and hard while the rest of the clinical examination was unremarkable. Tumor markers were found to be marginally raised. The levels of α-foetoprotein and β-HCG were 370 ng/ml and 650 U/L respectively. A computed tomography (CT) scan abdomen, however, revealed only subcentrimetric lymhnodes (clinically insignificant) in the retroperitoneum []. A right high inguinal orchidectomy was performed and the histopathology showed mixed NSGCT with predominnent embryonal component with vascular and lymphatic invasion. The tumor markers after 6 weeks were normalized. The patient was staged as clinical stage Ib American Joint Committee on -T2N0M0. He was given the option of RPLND or chemotherapy, and after explaining about the implications of both forms of treatment, he opted for RPLND. The patient was subsequently taken up for right modified template robot assisted laparoscopic transperitoneal RPLND.\nThe patient was placed in right lateral oblique position (60°) and pneumoperitoneum was created using Verres’ needle. A total of 6 ports were used []. The camera port (12 mm) was placed just lateral to umbilicus on the left. Two 8 mm robotic metallic ports were placed just lateral to umbilicus on the right at a distance of 8 cm from the camera port. A third robotic port was placed for the 4th robotic arm just medial to the anterior superior iliac spine. One 5 mm port was placed for liver retraction at the subxiphoid area. An assistant port (12 mm) for retraction, suture transfer and specimen retrieval was placed below the camera port towards the pelvis. The robot was docked with the cart being kept behind the back of the patient. On the right robotic arm, a monopolar curved scissors and needle holder were used. On the left, a bipolar Maryland forceps, needle holder and prograsp forceps were used. The prograsp forceps was very useful in holding and retracting tissues. However, the third robotic arm was not helpful because of crossing over and collision of the robotic instruments.\nThe dissection was started with mobilisation of hepatic flexure, ascending colon and caecum followed by kocherisation of the duodenum. The right gonadal vessels were identified and dissected from the inferior vena cava (IVC) till the right deep inguinal ring. The silk suture, which was used to ligate the vessels and cord during orchidectomy was identified and the right gonadal vessels along with a part of spermatic cord were excised. The right ureter, which was the right lateral limit of dissection, was identified, and carefully dissected from the paracaval tissue. Paracaval lymphatic tissue was dissected from the right renal hilum (proximal limit) to the right common iliac artery bifurcation [distal limit, ]. Excised tissues were retrieved through the 12 mm assistant port using home made plastic bag at the end of the procedure. The retrocaval tissue behind the IVC and the interaortocaval tissue between aorta and IVC were carefully dissected, and divided between clips (liga, weck). We didn’t use any hemostatic agent. During this dissection, the aorta and IVC were gently retracted by the assistant and the lumbar vessels were identified and preserved. With the help of 3D view and magnification, postganglionic sympathetic fibres and sympathetic ganglia were also identified and preserved. Pre-aortic dissection was done from the left renal vein (proximal limit) to the inferior mesenteric artery (distal limit). While dissecting, the wall of the aorta was injured, which was easily repaired with the 5-0 prolene suture. At the end of the dissection, we could see the skeletonised IVC, exposed aorta, right ureter and right and left renal hilum []. Hemostasis was ensured at the end of the procedure and the mobilised colon was repositioned. | [[23.0, 'year']] | M | {'21420231': 1, '24669137': 1, '32420211': 1, '18722704': 1, '16413370': 1, '30875289': 1, '29353314': 1, '27503429': 1, '29021655': 1, '25598598': 1, '20840317': 1, '33850353': 1, '24311897': 2} | {} |
164,321 | 3835982-1 | 24,311,904 | noncomm/PMC003xxxxxx/PMC3835982.xml | Rectal impalement with bladder perforation: A review from a single institution | A 23-year-old man was admitted to the hospital 4 h after falling on a wooden stake. He was complaining of an unbearable anal pain with rectal bleeding. He had neither hematuria nor abdominal pain. Physical examination showed a soft non-tender abdomen. Perineal examination could not be done as the patient refused any investigation without anesthesia. Nevertheless, we had been able to place a urine catheter which showed unclear urine without hematuria. RU was performed to exclude any bladder involvement. It showed passage of contrast into the extraperitoneal anterior wall of the rectum []. The patient was taken to the operative room. Examination under anesthesia showed a small laceration at the left anal margin extending into the anus. In the rectal exam, sphincter tonus was normal. At 7-8 cm from the anal verge, we found about 1-2 cm hole on the anterior wall of the rectum. Injection of the saline through the Foley catheter showed a leak through the posterior bladder wall into the rectum which confirmed the CT finding. There was no free air or contrast leak within the peritoneal cavity. Elective loop sigmoidostomy was performed. Rectum was sutured through the anal canal with 3/0 vicryl, followed by rectal washout. A large presacral drainage was also performed. The patient had an eventless postoperative course. The Foley catheter was removed on postoperative day 21. One month later, a control cystogram was performed showing no leak into the rectum. The colostomy was closed 2 months after the first surgery. | [[23.0, 'year']] | M | {'5090764': 1, '8483173': 1, '20453778': 1, '6712124': 1, '959978': 1, '16952579': 1, '10900842': 1, '1841470': 1, '14047852': 1, '3746959': 1, '11754851': 1, '11903691': 1, '6507755': 1, '33224733': 1, '3398098': 1, '9783600': 1, '33732622': 1, '6632094': 1, '26188926': 1, '9061736': 1, '7760417': 1, '7373677': 1, '7860673': 1, '3579027': 1, '1854066': 1, '2705824': 1, '17297321': 1, '24311904': 2} | {'3835982-2': 2} |
164,322 | 3835982-2 | 24,311,904 | noncomm/PMC003xxxxxx/PMC3835982.xml | Rectal impalement with bladder perforation: A review from a single institution | A previously healthy 15-year-old boy presented to University Hospital Hassan II of Fez. Three hours ago, he had fallen onto the handlebar of the bicycle and sustained a penetrating injury of the rectum. The patient and his parents reported copious watery drainage expelled per rectum. No sign of hematuria was reported as he had not voided since the trauma. Upon arrival at our emergency room, the patient complained only of pelvic pain and inability to void. No abdominal pain was reported by the patient. On physical examination, the abdomen was free of tenderness. Rectal exam revealed about 2-3 cm defect in the anterior rectal wall at 6-7 cm of the anal verge. RU demonstrated a communication between extraperitoneal posterior bladder wall and the anterior rectal wall. Foley catheter was inserted for bladder decompression. We temporarily decided to avoid diverting colostomy by the close surveillance of patient. During this period, no signs of hyperthermia, abdominal pain, or abnormal changes in the urine were observed. Two weeks after the injury, a control cystogram was performed, which showed no communication between the rectum and urinary bladder. Foley catheter was removed 1 week later. | [[15.0, 'year']] | M | {'5090764': 1, '8483173': 1, '20453778': 1, '6712124': 1, '959978': 1, '16952579': 1, '10900842': 1, '1841470': 1, '14047852': 1, '3746959': 1, '11754851': 1, '11903691': 1, '6507755': 1, '33224733': 1, '3398098': 1, '9783600': 1, '33732622': 1, '6632094': 1, '26188926': 1, '9061736': 1, '7760417': 1, '7373677': 1, '7860673': 1, '3579027': 1, '1854066': 1, '2705824': 1, '17297321': 1, '24311904': 2} | {'3835982-1': 2} |
164,323 | 3835990-1 | 24,311,912 | noncomm/PMC003xxxxxx/PMC3835990.xml | Sarcomatoid carcinoma of prostate involving the whole lower urinary tract and rectum | A 64-year-old man presented to his urologist with complaints of voiding symptoms and urinary retention. The initial prostate specific antigen (PSA) level was 9 ng/ml. He was treated with transurethral resection of prostate (TURP) outside our institute which showed high grade prostatic adeno-carcinoma (Gleason score 4 + 5 = 9). The staging workup subsequently showed a locally advanced prostate cancer. He was also started on hormonal therapy with leutinizing hormone releasing hormone (LHRH) analogue. Two months after initial surgery, he again went into painful urinary retention and per urethral catheterization failed, so a re-do TURP was done. The histopathology confirmed it to be adeno-carcinoma with sarcomatoid variant.\nIn the next six weeks, his voiding symptoms got worse again with gross hematuria culminating into urinary retention and obstructive uropathy, so a supra pubic catheter and bilateral percutaneous nephrostomy (PCN) tubes were placed and he was referred to us for further management. The PSA at this stage was 0.5 ng/ml. The patient also developed severe constipation with deep seated pelvic and perineal pain.\nOn physical examination, a stony hard mass was felt in suprapubic area through which suprapubic catheter was coming out. Another hard mass was felt in the perineum involving the bulb of penis. The digital rectal examination revealed a huge irregular stony hard mass with total occlusion of rectal lumen. An abdomino-pelvic magnetic resonance imaging (MRI) was done that showed an abnormal heterogeneous signal intensity mass lesion in the pelvis showing peripheral post contrast enhancement with central necrotic component measuring 13 × 11 × 14 cm in antero-posterior (AP), transverse and cranio-caudal dimensions and extending down into the perineum where it measures 8.9 × 3.8 × 5.2 cms involving the root of penis. Superiorly it was infiltrating into urinary bladder completely filling its lumen, anteriorly abutting the symphysis pubis, posteriorly involving rectum with loss of fat planes and laterally extending up to lateral pelvic wall []. No metastatic lesion was found on computerized tomography (CT) scan of chest and bone scans.\nBecause of extensive involvement of whole pelvis with severe urinary- and bowel-related symptoms, a total pelvic exenteration with pelvic lymphadenectomy along with urinary and fecal diversion with ileal conduit and an end colostomy formation was done. The pelvic tumor was completely resected in two pieces because of difficult dissection without any spillage and gross evidence of disease at the end of procedure. The residual preineal part of tumor was dealt with an elliptical perineal incision and it was removed from pelvic floor with a wide margin of normal tissue along with total penectomy. A margin of tissue was also resected form anterior abdominal wall along the cystotomy tract [].\nThe pelvic floor was repaired and re-enforced with a vicryl mesh. Hence, complete gross clearance was done with no palpable or visible disease.\nThe final histopathology revealed a mixture of carcinomatous and sarcomatous components comprising of diffuse sheets and aggregate of oval and spindle shaped cells arranged in short interlacing fascicles with areas of chondroid, osteogenic, and squamous differentiation. Cytokeratin AE1/AE3 and cytokeratin cam 5.2 immunohistochemical stains were performed on representative blocks, which showed focal positivity. PSA staining was negative. Two of recovered pelvic lymph nodes showed tumor involvement [].\nThe patient made a wonderful recovery and is still alive 4 months after surgery with no evidence of disease recurrence and metastasis on subsequent imaging (CT scan chest, abdomen, and pelvis). | [[64.0, 'year']] | M | {'18793296': 1, '14976541': 1, '33088717': 1, '18325477': 1, '9313326': 1, '15643196': 1, '17170745': 1, '12588610': 1, '16461109': 1, '15907718': 1, '8625205': 1, '7206104': 1, '20224731': 1, '19589566': 1, '8493872': 1, '8494104': 1, '24311912': 2} | {} |
164,324 | 3835991-1 | 24,311,913 | noncomm/PMC003xxxxxx/PMC3835991.xml | The unusual nested carcinoma of the renal pelvis | A 54-year-old postmenopausal lady presented with pain in the right arm and right upper thigh of three months duration. There was no history of trauma. General physical examination revealed bilateral axillary and inguinal lymphadenopathy. There was mild tenderness on palpation over the right arm and thigh; however, no definite swelling was noted. Rest of the systemic examination was normal. Complete hemogram, liver and renal function tests, and urinalysis were within normal limits.\nThe patient gave history of attending a primary health center where a provisional diagnosis of bone tuberculosis was made considering its high incidence in a developing country like india, and the patient was started on antitubercular therapy (ATT). The patient was referred to a higher center with no signs of improvement on ATT. Radiological investigations demonstrated a mass in the right radius and right iliac bone, and a fine needle aspiration cytology performed from these sites showed features of a metastatic carcinoma.\nFor further evaluation and investigation of the primary site, a whole body PET-CT and 99mTc-MDP bone scan were done. 99mTc-MDP bone scan exhibited abnormal increased tracer concentration in multiple hot spots in calvarium, ribs, right ilium, and right radius; suggestive of skeletal metastases. Whole body PET-CT scan showed metabolically active large lytic destructive lesions in multiple bones and a subtle enhancing soft tissue in midpolar region of left kidney. The patient was referred to our center for further management. Based on the above investigations, a clinical diagnosis of skeletal metastases with a possible primary in the kidney was considered. Three consecutive urine samples were sent for cytology, which was negative for malignant cells.\nWith the possibility of renal primary, the patient was taken up for laparotomy. Peroperatively, both kidneys appeared unremarkable, except that the left renal pelvis and upper one third of left ureter were indurated with adhesions present between them and the surrounding tissue. Rest of the visualized intra-abdominal structures were within normal limits. Hence, a left nephro-ureterectomy was performed.\nGross specimen consisted of left kidney with left adrenal gland and perinephric fat altogether measuring 9 × 6 × 5 cms, enclosed within the gerota's fascia with attached ureter of 11 cm length. On cutting open, a grey-white tumor was identified in the wall of the renal pelvis, which was extending into one of the calyceal walls and into the upper 7 cm of the ureter []. No papillary growth was identified. Rest of the kidney and adjacent adrenal gland appeared uninvolved. Regional lymph nodes were not included in the specimen.\nMicroscopically, a tumor was seen arising from the urothelium with massive infiltration of the lamina propria []. The tumor cells were arranged in the form of irregularly distributed variable-sized organized nests, few of which showed cribriform and microcystic pattern. The cells were medium-sized with mild to moderate anisocytosis, moderate amount of pale eosinophilic cytoplasm, rounded nuclei, granular chromatin with inconspicuous nucleoli in most of the cells. No significant nuclear polymorphism was noted in the majority of the tumor; however, few large tumor cells displaying some atypia were seen in the deeper areas. Mitoticfigure were rare with one mitoticfigure identified per 10 high power fields. Small irregular nests of tumor cells were seen invading in between the deep muscle bundles and also perineurally. Nests of tumor cells were seen invading into the pericalyceal areas of lower half of left kidney. Sections from pelvis and ureter showed tumor cells infiltrating through the wall of the pelvis and the ureter into the peripelvic fat. Renal vessels, resected margin of the ureter, and left adrenal gland were, however, free of tumor.\nThe differential diagnosis included the NVUC and neuroendocrine tumor, for which immunohistochemistry was performed. The tumor cells were diffusely immunoreactive for Pan CK, CK 7, CK 20 [] and were negative for neuroendocrine markers NSE and Chromogranin []. Based on these findings, final diagnosis of nested variant of urothelial carcinoma was made.\nIn view of the widespread metastases, postoperatively, the patient was put on systemic chemotherapy including cisplatin and gemcitabine. Till date, she has received three cycles of chemotherapy, but her clinical condition has not improved in these three months. | [[54.0, 'year']] | F | {'8902836': 1, '1495929': 1, '18434765': 1, '19171439': 1, '11229644': 1, '12589642': 1, '2712189': 1, '14681330': 1, '12684120': 1, '8976784': 1, '12960809': 1, '17445674': 1, '19800100': 1, '11411650': 1, '24311913': 2} | {} |
164,325 | 3835992-1 | 24,311,914 | noncomm/PMC003xxxxxx/PMC3835992.xml | Superior vena cava syndrome due to metastasis from urothelial cancer: A case report and literature review | A 59-year-old man presented with dyspnea, face and neck edema, strangled feeling, cough and fatigue in April 2008. Symptoms rapidly advanced over the course of 1 week. He was known case of UC for which he underwent transurethral resection for bladder tumor (TURBT) (the size was 7 cm, left wall, UC, G2 > G1, pTa) in April 2005. In addition, 2 weeks later left nephroureterectomy had been performed for the left ureter and renal-pelvis cancer. The entire mucosa of the ureter and renal-pelvis were replaced by papillary tumor [] and histological examination showed UC, G2>>G3, pT2, ly0, and v0. Because of high grade invasive cancer, two courses of adjuvant chemotherapy had been administered using methotrexate, vinblastine, doxorubicin hydrochloride, and cisplatin (MVAC therapy). In February 2006, multiple bladder tumors (9 papillary tumors, size was 5 to 15 mm) had recurred and TURBT was performed (UC, G2, pTa). No metastatic lesions were found until the second admission. After TURBT the patient came the hospital twice for the convenience of the patients, and he was not followed for 1 year and presented again with SVC symptoms. The face, neck, and right upper limb were edematous and varicosis of the right jugular vein was apparent. Computer tomography (CT) scan revealed multiple lung masses involving a 5 cm tumor in the right upper lung field and multiple lymph nodes in the mediastinum []. Massive nodes constricted the upper part of the SVC and merging section of bilateral brachiocephalic veins. Bone scintigraphy revealed a hot spot in the forehead bone. Glucocorticoid (bethamethasone 16 mg/day) was administered and radiotherapy was started on the second day of admission. Twenty days later CT-guided biopsy was performed and metastasis of UC was diagnosed histopathologically []. A total 40 Gy of radiation proved ineffective and edema of the face and upper limbs continued. A modified intravenous anti-cancer chemotherapy was initiated with paclitaxel at 60 mg/m2 on days 1 and 8, gemcitabine at 1000 mg/m2 on days 1 and 8, and cisplatin 70 mg/m2 on day 2.[] Facial edema and dyspnea temporarily worsened with fluid infusion, but symptoms improved and the size of tumors decreased after one course of this chemotherapy. After three courses of the chemotherapy, most lung metastases had vanished and the main lung tumor had decreased to a diameter of 2 cm. Lymph nodes became reduced in size, but compression of the SVC remained. However, collateral circulations around the compression of the SVC and the dorsal chest wall developed, improving SVC syndrome. About ten months later patient died due to cerebral bleeding around the forehead metastasis. | [[59.0, 'year']] | M | {'17089065': 1, '10443740': 1, '10754173': 1, '18566082': 1, '10591825': 1, '17324762': 1, '8404209': 1, '32178979': 1, '26634162': 2, '7299933': 1, '9817039': 1, '16604414': 1, '17929122': 1, '17476012': 1, '19015618': 1, '2791428': 1, '11813185': 1, '15673535': 1, '24311914': 2} | {'4655034-1': 1} |
164,326 | 3835995-1 | 24,311,917 | noncomm/PMC003xxxxxx/PMC3835995.xml | Renal cell carcinoma-associated adult dermatomyositis treated laparoscopic nephrectomy | A 77-years-old Caucasian lady, with known rheumatoid arthritis and hypothyroidism, presented with a 4 months history of facial rash and progressive muscle weakness affecting the legs more than the arms. Examination revealed Gottron papules (erythematous papules on the dorsal surface of the phalangeal joints), heliotrope rash (oedematous erythema of the eyelids) and proximal muscle weakness []. Blood biochemistry revealed a creatine kinase (CK) level of >6000 U/L (normal 0-70 U/L) and positive ANA (Antinuclear Antibody). The electromyography (EMG) studies and a muscle biopsy were subsequently performed. The EMG showed signs of severe myopathic features. The needle EMG examination showed profuse spontaneous activities affecting the upper and lower limb muscles indicating an active stage of myositis. The muscle biopsy showed infiltration of the muscle fibres by lymphocytes and macrophages associated with muscles fibres necrosis. In view of these findings, a diagnosis of dermatomyositis was made. The patient was admitted due to the severe muscle weakness and was commenced on high dose steroids and intravenous immunoglobulins (IV-IG) without any significant benefit. Cancer screening was undertaken and a staging computed tomography (CT) of the chest, abdomen and pelvis revealed a 4 cm upper pole solid mass on the left kidney, suggestive of renal cell carcinoma. She was referred to urology and underwent a laparoscopic nephrectomy four weeks after presentation. Histopathology confirmed conventional (clear cell) renal cell carcinoma with focal nuclear changes accounting to Fulham grade 2. The carcinoma was confined to the kidney with no evidence of lympho-vascular invasion (stage pT1 a). Following the nephrectomy the patient was continued on steroids and had further monthly IV IG. Within four weeks of the nephrectomy her CK had improved to less than 200 U/L and there was a significant improvement in muscle strength []. She remained in hospital for 3 months during which she made a remarkable recovery with the disappearance of the rash, normalisation of CK levels and consistently improving muscle power. She was discharged on a reducing dose of prednisolone and within 6 months was back to normal life. | [[77.0, 'year']] | F | {'9182452': 1, '29429069': 1, '19730376': 1, '20569766': 1, '14709696': 1, '11197446': 1, '10352646': 1, '8228798': 1, '20083689': 1, '17053509': 1, '2288311': 1, '6859800': 1, '26786527': 1, '34053989': 1, '21165524': 1, '20398365': 1, '24311917': 2} | {} |
164,327 | 3835996-1 | 24,311,918 | noncomm/PMC003xxxxxx/PMC3835996.xml | Adrenal rests associated with ectopic testis in an adult: Clinical significance | A 26-year-old man, married with two children, presented with a swelling in the right groin, which was present since early childhood. His past history was irrelevant. On examination, he was generally well and his vital signs were normal as well as his chest and abdomen. Local examination revealed a reducible inguinoscrotal hernia on the right side, and an undescended testis on the left, with underdeveloped hemiscrotum.\nHis routine laboratory investigations were unremarkable and computerized axial tomography (CT)/ultrasound (US) scans of the inguinal region showed normally located right testis with an inguinoscrotal hernia, while on the left side, the testis was lying at the level of the deep inguinal ring. At operation: A right indirect inguinal hernia was found for which mesh repair was performed. On the left side, the testis, expectedly, was found at the deep ring and orchidectomy was done.\nThe patient recovered well and was discharged in good condition for follow-up in the outpatient clinic.\nHistopathological examination of the excised testis showed atrophic changes, with adjacent adrenal rests in which the three cortical layers were represented [Figures -]. | [[26.0, 'year']] | M | {'11989564': 1, '17066272': 1, '21738545': 2, '19367442': 1, '18931550': 1, '33778389': 1, '27601037': 1, '11739428': 1, '7124773': 1, '18941436': 1, '16845852': 1, '34095993': 1, '16650300': 1, '15679804': 1, '9165472': 1, '24311918': 2} | {'3121001-1': 1} |
164,328 | 3835997-1 | 24,311,919 | noncomm/PMC003xxxxxx/PMC3835997.xml | Genital self-amputation or the Klingsor syndrome: Successful non-microsurgical penile replantation | A 29-year-old man, unmarried, with a history of schizoaffective personality disorder, but non-compliant with the antidepressant and antipsychotic medication, was admitted for self-cutting of penis 2 cm distal from the mons of the pubis.\nAt the admission, mental examination revealed apathetic and conscious patient but uncooperative. Eye contact was poor, speech was inaudible. There was no suicidal intent.\nAt the urological examination, we found a subtotal cut of the penis, interesting the anterior and lateral parts of the penile skin, and a clear cut trough cavernosal bodies, and urethra. The penis remained suspended only by a thin posterior part of the skin [Figures and ]. The patient received two intravenous antibiotics.\nImmediately the patient was addressed in the operating room. The microsurgery was not available and the only chance to attempt to save the functionality of the organ was a non-microsurgical replantation of the amputated penis. A suprapubic cystostomy was first performed. The operation started about 5 hours after the trauma.\nA 16-Fr silicon catheter was inserted through the urethra of the amputated part and the proximal urethral stump and passed on into the bladder. End-to-end urethral anostomosis was made with a two-layer closure over a using 5-0 polyglycolic acid [].\nAfterwards the cavernous bodies of the penis were connected with 3-0 polyglycolic acid continuous suture.\nWe did not perform anastomosis of the dorsal vein, artery or the nerve. Buck's and dartos fascia were reapproximated, and the skin was closed.\nThe patient was transferred to the inpatient psychiatric unit for further management.\nTwenty-four hours after the surgery, we did not observe any skin necrosis and the urethral mucosa showed a reassuring pink colour.\nOne week after the surgery, the patient in an agitation crisis pulled up his foley catheter.\nWe removed the cystostomy catheter 2 weeks later, after that the normal micturation was acquired.\nThe clinical exam 1 month after surgery showed a viable penis [Figures and ], with a pink urethral mucosa, and a normal sensation. The patient reported normal micturition and spontaneous morning erections 3 months after surgery. Those results were maintained 1 year after surgery.\nDuring the psychiatric interview patient reported that the act was preceded by religious delusions and hallucinations: He was hearing voices and feeling guilty, and thought that God would punish him.\nThe psychiatrist ordered lithium 900 mg/day, sertraline 50 mg/day and risperidone 3 mg/day. The patient was fully remitted at the first month. | [[29.0, 'year']] | M | {'12092662': 1, '33217659': 1, '25943096': 1, '6614331': 1, '25878930': 1, '426611': 1, '5696664': 1, '31956464': 2, '28540229': 1, '1822958': 1, '2400297': 1, '13971387': 1, '25844175': 2, '3968751': 1, '21407886': 1, '18574705': 1, '11775199': 1, '10671884': 1, '34285757': 1, '20490665': 1, '2073234': 1, '21270503': 1, '12058248': 1, '4210991': 1, '24311919': 2} | {'6949675-1': 1, '6949675-2': 1, '4380914-1': 1} |
164,329 | 3835998-1 | 24,311,920 | noncomm/PMC003xxxxxx/PMC3835998.xml | Primary localized amylodoisis of bladder: Is there a need for cystoscopic surveillance? | A 71-year-old gentleman was referred to hematuria clinic for his single episode of visible painless haematuria. There was no history of lower urinary tract symptoms and no previous urological problems. He had dipstick hematuria and urine cytology was reported as benign. His ultrasound scan of kidneys and bladder revealed thickened posterior bladder wall and small simple renal cysts bilaterally. At flexible cystoscopy a solid lesion on the posterior bladder wall resembling invasive bladder was seen []. Transurethral resection was performed and the specimen was submitted for histopathological examination. Final histology reported a primary amyloidosis of AL type. A thorough systematic investigation including rectal biopsy, bone marrow biopsy and SAP (serum amyloid P component) scintigraphy showed no evidence of systemic amyloidosis. Three years from his initial diagnosis a small urethral lesion was noted which was biopsied and histology confirmed it as primary amyloid of AL type []. Since then he has remained asymptomatic without progression of vesical amyloidosis. | [[71.0, 'year']] | M | {'1427808': 1, '11126834': 1, '11729210': 1, '30021741': 1, '10233590': 1, '29541594': 1, '28974964': 2, '16635518': 1, '24311920': 2} | {'3835998-2': 2, '5604108-1': 1} |
164,330 | 3835998-2 | 24,311,920 | noncomm/PMC003xxxxxx/PMC3835998.xml | Primary localized amylodoisis of bladder: Is there a need for cystoscopic surveillance? | A 78-year-old gentleman attended hematuria clinic with two weeks history of intermittent, macroscopic hematuria which was associated with irritative lower urinary tract symptoms. Ultrasound scan of kidney and bladder revealed normal appearing kidneys bilaterally with slight thickening of the bladder, which was confirmed by CT scan []. A flexible cystoscopy showed a craggy appearing solid lesion with some hemorrhagic areas situated over the trigone of bladder and was not involving ureteric, resembling invasive bladder tumour. Transurethral resection was performed and submitted for histological examination. Histology was reported as primary (AL) amyloid and there was no evidence of either in situ or invasive urothelial malignancy. Systematic investigations were performed which were negative for systemic amyloidosis. There was no recurrence of vesical amylodosis and he remains asymptomic 4.5 years after initial diagnosis. | [[78.0, 'year']] | M | {'1427808': 1, '11126834': 1, '11729210': 1, '30021741': 1, '10233590': 1, '29541594': 1, '28974964': 2, '16635518': 1, '24311920': 2} | {'3835998-1': 2, '5604108-1': 1} |
164,331 | 3836393-1 | 24,131,077 | noncomm/PMC003xxxxxx/PMC3836393.xml | Variegate porphyria complicated by systemic AA amyloidosis: a case report | A 35-year-old Japanese woman was admitted to our hospital for the evaluation of bilateral arthropathy affecting the joints of her hands, feet, knees, ankles, shoulders and hips. She complained of pain in multiple joints, but swelling was not apparent. C-reactive protein was 4.0 mg/dL, but rheumatoid factor was negative. Radiographs did not disclose any loss or narrowing of the joint spaces. She did not have a family history of such arthropathy or of autoimmune disease, including rheumatoid arthritis. Two years later, blister formation after exposure to the sun and reddish urine were noted, and these symptoms showed repeated remission and exacerbation. At 40 years old, evaluation of the urinary excretion of porphyrin metabolites showed that daily ∂ aminolevulinic acid (ALA) excretion was 1.68 mg/dL (normal range: <0.5 mg/dL), while the daily excretion of porphobilinogen (PBG) was 1.42 mg (normal range: <0.8 mg) and coproporphyrin was 0.274 mg (normal range: <0.10 mg). Accordingly, VP was diagnosed. In addition to avoidance of sun exposure, administration of prednisolone (20 mg daily) was effective for her skin lesions and arthropathy, but symptoms recurred after tapering. She also noted that a low-dose contraceptive improved her symptoms. At the age of 45 years, her renal function declined with a serum creatinine of 3.4 mg/dL. In addition, she had nausea, vomiting, abdominal pain without rebound tenderness and clonic convulsions. After administration of phenobarbital, reddish urine appeared and muscular weakness progressed to atonic quadraparesis. Acute porphyria was diagnosed because of elevated urinary excretion of PBG to 51.296 mg daily (normal range: <0.8 mg) and coproporphyrinogen to 0.271 mg daily (normal range: <0.16 mg). Her symptoms subsided after plasma exchange plus high-dose glucose therapy. In addition, hematin therapy was effective for her porphyria after four months. However, hypertension became refractory and renal dysfunction progressed. At the age of 47 years, hemodialysis was started when her serum creatinine was 11.0 mg/dL and congestive heart failure had developed. At the age of 49 years, her gastrointestinal symptoms and skin lesions became worse. Gastroduodenoscopy and colonoscopy did not reveal any ulcerated lesions. However, she died of refractory thrombocytopenia and anemia due to bleeding from the gastrointestinal tract. Throughout the clinical course, C-reactive protein (CRP) remained positive, rheumatoid factor was negative, serum AA protein was over 20 µg/mL (normal range: <8.0 µg/mL) and radiographs did not show any joint deformities consistent with rheumatoid arthritis. | [[35.0, 'year']] | F | {'10856069': 1, '19268004': 1, '7986228': 1, '2679059': 1, '20955962': 1, '20051751': 1, '7591586': 1, '11086643': 1, '21231814': 1, '24131077': 2} | {} |
164,332 | 3836932-1 | 24,278,654 | noncomm/PMC003xxxxxx/PMC3836932.xml | Rupture of De Novo Anterior Communicating Artery Aneurysm 8 Days after the Clipping of Ruptured Middle Cerebral Artery Aneurysm | A 47-year-old man with subarachnoid hemorrhage in Hunt & Hess grade III was admitted via emergency room. The initial brain CT revealed a thick subarachnoid blood clot around basal cistern and left sylvian fissure (). A 64-detector 3D-CT angiography showed a saccular aneurysm on the left M1 portion of middle cerebral artery and no additional aneurysms (). Considering the distribution of the hemorrhage on CT scans and 3D-CT angiography, we concluded that the cause of the hemorrhage was rupture of the left M1 aneurysm. Immediately, we underwent pterional approach and aneurysmal clipping. On the microsurgical view, M1 aneurysm was surrounded with clots and we confirmed the obliteration of the aneurysm. The patient's mentality was improved to Hunt & Hess grade II. On the next day, right side hemiparesis was developed and the diffusion weighted magnetic resonance images showed ischemic change on the left posterior limb of the internal capsule (). We performed digital subtraction angiography (DSA) which revealed mild vasospasm and performed intra-arterial nimodipine injections. There was no abnormal finding in the anterior communicating artery ().\nAt eight days after the clipping, sudden deterioration of mentality appeared and the follow-up brain CT showed an intraparenchymal hematoma on the right frontal lobe (). We performed the DSA again, which revealed severe vasospasm on the left M1 and A1 portion and the left anterior communicating artery aneurysm that was not found in the previous 3D CT angiography and DSA (). Simultaneously, coil-embolization was performed for the anterior communicating artery aneurysm with Target coils (4×10, 3×6, 2×4, and 2×2) (Boston scientific, Stryker) and it was obliterated completely on the final angiography. We injected intra-arterial nimodipine for the vasospasm (). After the coil embolization, the patient was kept in confused mentality status. However, one month-follow up CT scan showed left cerebral hemisphere ischemia due to vasospasm and hemorraghic transformation (). He was cooperative but was discharged with moderately severe disability due to right hemiplegia (mRS 4). | [[47.0, 'year']] | M | {'11154818': 1, '11157187': 1, '17009025': 1, '16002762': 1, '16000333': 1, '21772796': 1, '21990653': 1, '12296657': 1, '11203121': 1, '15814945': 1, '18300883': 1, '21822984': 1, '21643681': 1, '17210317': 1, '26664872': 2, '16099260': 1, '19086441': 1, '17495215': 1, '27808265': 1, '21471840': 1, '18688012': 1, '33551447': 1, '10969934': 1, '20948398': 1, '14167089': 1, '14663570': 1, '24278654': 2} | {'4653326-1': 1} |
164,333 | 3836933-1 | 24,278,655 | noncomm/PMC003xxxxxx/PMC3836933.xml | Bilateral Superior Cerebellar Artery Infarction after Stent-Angioplasty for Internal Carotid Artery Stenosis | A 75-year-old female patient visited our hospital with bilateral weakness (left>right) which had suddenly developed and persisted for 15 days. She also had hypertension and unstable angina which had been treated with antihypertensive and antiplatelet medications for 10 years. She had no history of diabetes mellitus, hyperlipidemia, and smoking, etc.\nOn neurological examination, bilateral hemiparesis (left : Grade IV, right : Grade IV+) was detected. The brain computed tomography scan showed a low-density area only in the right post-central gyrus, and diffusion weighted-magnetic resonance imaging (d-MRI) showed bilateral anterior cerebral artery-middle cerebral artery borderzone infarction (), and MR angiography showed high grade stenosis of both proximal ICA ().\nCatheter angiography was performed 4 days after admission. Common carotid angiography showed 85% stenosis of the left ICA origin () and 90% stenosis of right ICA origin (). Vertebral angiography showed total occlusion of the right VA and multifocal stenosis of the left VA with faint flow to basilar artery (BA) (). PComAs on both sides were well visualized and filling to both posterior cerebral arteries (PCAs) (). However, retrograde flow to BA was not seen. Meanwhile, right occipital artery (OA) from right ECA supplied right VA and then BA to basilar tip ().\nWe performed stent-angioplasty for left ICA stenosis first under local anesthesia immediate after the catheter angiography (). During the procedure no remarkable events took place, and the neurologic symptoms of the patient remained unchanged after the procedure. Two days after stent-angioplasty for left ICA stenosis, treatment for right ICA lesion was performed under local anesthesia (). During the procedure no remarkable events took place, and the neurologic symptoms of the patient remained unchanged immediate after the procedure. After the 2nd session of angioplasty, we control the patient's systolic blood pressure (BP) about 120 mm Hg because of the hyperperfusion syndrome prevention. However, the consciousness level of the patient was getting worse after the procedure from alert to stuporous state. Two days after the 2nd procedure, d-MRI was performed and it showed acute cerebellar infarction in the territory of both SCAs, but no other newly developed infarction on both cerebral hemispheres (). | [[75.0, 'year']] | F | {'1942654': 1, '14210007': 1, '11441200': 1, '12881609': 1, '16888253': 1, '21037845': 1, '19760056': 1, '14695593': 1, '16339466': 1, '23323169': 2, '17416813': 1, '19104793': 1, '8128527': 1, '8571426': 1, '24278655': 2} | {'3539083-1': 1, '3539083-2': 1} |
164,334 | 3836934-1 | 24,278,656 | noncomm/PMC003xxxxxx/PMC3836934.xml | Rotational Vertebral Artery Compression : Bow Hunter's Syndrome | A 50-year-old woman had been experiencing paroxysmal vertigo attacks for a 10 month period prior to visiting our institute. The patient's vertigo was aggravated when she turned her head to the left side. The patient underwent an inner ear exam, which was unremarkable. She was treated with conservative medication for 10 months but did not improve.\nCerebrovascular images were checked for further evaluation. From angiographic computerized tomography (CT) scans, we observed no definitive abnormal findings except for hypoplastic left VA in the neutral position (). In dynamic vertebral angiography, no steno-occlusive lesions were found in the neutral position (), but complete occlusion of right VA was found at atlanto-axial level when the patient turned her head to the left side ().\nWe decided on a surgical decompression of the left VA at the cross transverse foramen of the first cervix as the surgical approach. We placed an adhesive, fibrous bandage between the left VA and the transverse foramen. During the surgical procedure, the bandage was gently dissected and released with decompression of the surrounding bony structure. After surgery, the patient's symptoms improved, and her neck three-dimensional angiography CT scans demonstrated complete decompression of the right vertebral artery (, ). There were no follow-up complications. | [[50.0, 'year']] | F | {'12461405': 1, '9120624': 1, '12507149': 1, '3748345': 1, '3379466': 1, '10746615': 1, '19772401': 1, '28503618': 2, '7121817': 1, '20010391': 1, '15979646': 1, '29882351': 1, '27610119': 1, '26301025': 1, '34698085': 1, '3918425': 1, '732978': 1, '25328557': 1, '21789571': 1, '9257311': 1, '24278656': 2} | {'3836934-2': 2, '5417278-1': 1} |
164,335 | 3836934-2 | 24,278,656 | noncomm/PMC003xxxxxx/PMC3836934.xml | Rotational Vertebral Artery Compression : Bow Hunter's Syndrome | A 42-year old woman reported severe vertigo, dizziness, right upper extremity tingling sensations and aggravated syncope when she turned her head to the right. Upon otolaryngological examination, she presented right beating nystagmus when her head was rotated to the right in the sitting or supine position. There were no abnormal findings except for hypoplasia of the posteroinferior cerebellar artery termination of the left VA on magnetic resonance angiography. No significant changes were observed with neck rotational positions.\nHowever, dynamic cerebroangiography showed no steno-occlusive lesions of left VA in the neutral position (), but an occlusion of the left VA was observed at the C1-2 level from right head rotation (). We performed decompressive surgery based on a diagnosis of BHS. We selected a surgical approach and found an adhesion between the left VA and transverse foramen. We performed a decompressive transversarium foraminectomy.\nAfter the surgery, CT angiography showed a released left VA. The patient experienced a complete recovery. | [[42.0, 'year']] | F | {'12461405': 1, '9120624': 1, '12507149': 1, '3748345': 1, '3379466': 1, '10746615': 1, '19772401': 1, '28503618': 2, '7121817': 1, '20010391': 1, '15979646': 1, '29882351': 1, '27610119': 1, '26301025': 1, '34698085': 1, '3918425': 1, '732978': 1, '25328557': 1, '21789571': 1, '9257311': 1, '24278656': 2} | {'3836934-1': 2, '5417278-1': 1} |
164,336 | 3836935-1 | 24,278,657 | noncomm/PMC003xxxxxx/PMC3836935.xml | Malignant Solitary Fibrous Tumor of Tandem Lesions in the Skull and Spine | A 54-year-old female patient was referred to our spine center with a two-month history of lower back pain and radiating pain in both legs, which was persistent even at night. She had constipation with a symmetric leg muscle power grade of IV/V. Lumbo-sacral magnetic resonance imaging (MRI) showed a heterogeneously enhanced 6.7 cm sized lesion involving the sacrum, sacroiliac joint, and both illium with extraosseous mass formation containing a necrotic portion ().\nWe performed positron emission tomography/computed tomography (PET/CT) and bone scan to assess for metastatic lesion. PET/CT and bone scan revealed hot uptake in the sacro-pelvic area, T8, and occipital skull area. Thoracic MRI showed an epidural enhancing 1.7 cm sized lesion in the posterior element of T8 (). In addition, findings on brain imaging studies showed an enhancing mass measuring 3.8 cm with occipital bone destruction and mild brain compression (). Percutaneous biopsy of S1 was performed for diagnosis. Pathology of the biopsy indicated a type undetermined malignant neoplasm, suspected as a malignant spindle cell tumor.\nOn angiography, large feeding vessels were observed in sacral lesion, and tumor embolization using Gelfoam was performed prior to perform an open surgery. Partial removal of the tumor in sacroiliac area was done; then, gross total removal of the tumor at T8 was subsequently performed in a single stage. The tumor was extremely hypervascular and was not well demarcated. For the remnant tumor of the sacroiliac area, postoperative stereotactic radiosurgery using Novalis Tx (BrainLAB, Inc., Ammerthalstrabe, Germany) was administered at a dose of 4 fractionated 32 Gy, which encompassed 100% of the tumor volume. After open surgery and radiosurgery, her lower back pain and radiating pain showed improvement. Three weeks after first open surgery, gross total removal of the tumor in the occipital skull was performed after tumor embolization.\nTumors of the sacro-pelvic area, T8, and occipital area showed identical histologic features. The tumors consisted of fusiform or spindle cells and showed a hemangiopericytoma-like perivascular pattern or a so-called patternless pattern, with intervening irregular hyalinzed collagen bundles, typical for a solitary fibrous tumor. The tumors showed multifocal necrosis, high cellularity, and marked cellular atypia. Mitotic figures were frequently observed [up to 10/10 high-power fields (HPFs)]. By immunohistochemistry, the tumor cells were positive for CD34, CD99, Bcl-2 and EMA (focal-like +). As a result, the tumors were diagnosed as malignant solitary fibrous tumor ().\nAlthough there was a lack of clinical evidence about adjuvant chemotherapy or radiotherapy, adjuvant chemotherapy with adriamycin and cisplatin was administered. However, just 2 days after starting adjuvant chemotherapy, one month after the initial surgery, she complained of aggravated upper back pain and paraparesis. Findings on follow-up thoracic MRI showed a recurred expansive posterior epidural mass with cord compression at T8 (). For the recurred tumor at T8, stereotactic radiosurgery was administered at a dose of 4 fractionated 32 Gy. After radiosurgery, her upper back pain and paraparesis showed improvement.\nGradually, she complained of progressive whole body pain. Despite chemotherapy, follow-up whole spine MRI and chest radiography revealed metastasis in multiple vertebrae and both lungs. Although palliative cervical and thoracic spine radiotherapy (dose of 5 fractionated 15 Gy) was performed for relieving intractable pain, her symptoms did not show improvement and the patient finally died due to poor general condition six months after first open surgery. | [[54.0, 'year']] | F | {'29619522': 1, '20488732': 1, '9447390': 1, '17316417': 1, '16359538': 1, '9346183': 1, '19707501': 1, '28494796': 2, '10665915': 1, '1415270': 1, '11306751': 1, '30817590': 1, '33344498': 2, '11920476': 1, '25368773': 2, '25593784': 2, '26155787': 2, '22575866': 1, '9850176': 1, '30481195': 1, '7260861': 1, '16274816': 1, '32181204': 1, '12884217': 1, '15620963': 1, '633933': 1, '20566782': 1, '29390521': 1, '21872387': 1, '9160308': 1, '24278657': 2} | {'4287906-1': 1, '4287906-2': 1, '7744481-1': 1, '4217067-1': 1, '5426015-1': 1, '4495700-1': 1} |
164,337 | 3836936-1 | 24,278,658 | noncomm/PMC003xxxxxx/PMC3836936.xml | Incidental Superior Hypophygeal Artery Aneurysm Embedded within Pituitary Adenoma | A 70-year-old woman presented with visual disturbance for one year. The results of the neuro-ophalmologiclal examination revealed bitemporal hemianopsia. Magnetic resonance (MR) imaging showed persistence of a homogeneously enhancing suprasellar lesion, compressing the visual pathways. Endocrine tests confirmed normal pituitary function. However, the axial or coronal MR images presented a flow void in the medial portion of the right internal carotid artery (ICA) (). These MR images raised the suspicion of a coexisting intrasellar aneurysm before the planned transsphenoidal procedure. Consequently, 3-dementional computed tomography (CT) angiogram demonstrated the opthalmic segment of the right distal ICA (). Four vessels angiogram confirmed a right superior hypophygeal artery aneurysm and the saccular aneurysmal sac that had a 4.13 mm neck, 6.27 mm width, and 3.99 mm height ().\nFirst, we treated the aneurysm by coil embolization with the goal of maintaining the patency of the ICA (). Two months later, she underwent surgery via the transsphenoidal microsurgical approach for the removal of the tumor. We carefully dissected the tumoral portion surrounding the coil-treated aneurysm and a total resection of the tumor was successfully achieved. The patient's postoperative course was uneventful. Immunohistological microscopic findings confirmed the diagnosis of a non-functional PA. Postoperative endocrine tests confirmed normal pituitary function. MR at 8 month follow-up after surgery demonstrated complete obliteration of the aneurysm and no sign of regrowth of the adenoma (). | [[70.0, 'year']] | F | {'29527388': 2, '524269': 1, '1890460': 1, '22054212': 1, '26823732': 1, '20854061': 1, '28127130': 1, '18040122': 1, '951636': 1, '34603930': 1, '22304451': 1, '712374': 1, '15925789': 1, '12852886': 1, '3220712': 1, '3562822': 1, '9085282': 1, '15035295': 1, '21307138': 1, '19934939': 1, '13564252': 1, '22223518': 1, '29404193': 1, '18392428': 1, '24885333': 2, '26220796': 2, '24278658': 2} | {'4518590-1': 1, '4155771-1': 1, '5838828-1': 1} |
164,338 | 3836937-1 | 24,278,659 | noncomm/PMC003xxxxxx/PMC3836937.xml | Spinal Hemangiopericytoma Which Needed Intraoperative Embolization due to Unexpected Bleeding | A 21-year-old man visited complaining of neck pain and a tingling sensation in both hands. The intensity of neck pain had progressed over 1 year. The tingling sensation in the hands had developed 1 month prior and had worsened. Motor function and reflexes did not decrease. His magnetic resonance (MR) images revealed 2.5-cm well-circumscribed ventral IDEM mass at the level of C1-2 (). The mass showed iso-signal intensity on T1-weighted images and high signal intensity on T2-weighted images. The lesion was homogenous and vividly enhanced with intravenous administration of gadolinium. Small cystic components were seen on T2-weighted images. The tumor did not expand or erode spinal canal or soft tissue. The tumor did not reveal calcification, adjacent bone erosion, and multilobulated lesions. No additional lesions were identified including brain, abdomen, and pelvis. Our presumptive diagnosis at preoperative state was meningioma, but schwannoma, and neurofibroma were also considered.\nThe patient underwent left hemilaminectomy at C1, left partial hemilaminectomy at C2, and partial resection of the foramen magnum. When the dura was opened, an IDEM tumor was placed behind spinal accessary nerves. The plane between the spinal cord and the mass placed well defined, and the flesh-red tumor was observed (). The tumor was partially exposed only after some accessary nerves had been cut. Because of narrow surgical field, we planned internal debulking first. While performing internal debulking by ultrasonic aspirator to remove the ventral IDEM tumor, unexpected tumor bleeding developed and was difficult to stop. Tumor bleeding could not be controlled by bipolar coagulator, and was stopped after compressing the lesion using microfibrillar collagen (Avitene™) for some minutes. It is difficult to find feeding arteries because of narrow surgical field. We halted the surgery and checked the spinal angiography at the angiography suite, which revealed two major feeders of the deep cervical branch from the vertebral artery (). Two main feeders were embolized by titanium coil or particles. After endovascular embolization of the feeder vessels, the tumor resection was resumed. The tumor was completely removed with minimal bleeding. The patient had a fast postoperative course, and the tingling sensation in his hands and his neck pain disappeared. He was discharged without symptoms and has been recurrence-free for the past 1 year ().\nA gross specimen revealed a nodular mass of soft tissue measuring 2×2 cm. The cut surface of the mass was reddish yellow. Hematoxylin and eosin staining showed a highly cellular neoplasm growing in patternless sheets and with high mitotic activity. There was proliferation of the tumor cells with oval nuclei, and the tumor was surrounded by abundant blood vessels resulting in the "staghorn" appearance typical of hemangiopericytoma (). After immunostaining, the tumor cells were negative for CD34 and epithelial membrane antigen. On the basis of these findings, we made a final diagnosis of hemangiopericytoma. | [[21.0, 'year']] | M | {'21641873': 1, '2797389': 1, '21857403': 1, '34845431': 2, '3885414': 1, '11841738': 1, '15042684': 1, '14306325': 1, '1985083': 1, '18768724': 1, '6784553': 1, '25797775': 1, '11322435': 1, '3975803': 1, '25059986': 1, '13754624': 1, '18162866': 1, '24278659': 2} | {'8627340-1': 1} |
164,339 | 3836938-1 | 24,278,660 | noncomm/PMC003xxxxxx/PMC3836938.xml | Totally Ossified Metaplastic Spinal Meningioma | A 61-year-old woman had a 1-year history of numbness in right lower extremities that progress bilaterally and ascend to his half lower body below the umbilicus. She also noticed progressive urinary incontinence without weakness in lower extremities. Neurological examination revealed a hypoesthesia to touch and pain from T10 to S2 level. Also, she had positive sign in Romberg test. Both patella and ankle reflexes were hypoactive and the Barbinski signs were bilaterally negative. Computed tomographic images without enhancement show a large hyperdense intradural round mass occupying the whole spinal canal at T9-10 level (). Magnetic resonance imaging revealed a large T9-10 intradural extramedullary mass that was hypointense to spinal cord on T1- and T2-weighted sequences, partial enhancement was apparent after Gd administration (). The spinal cord was severely compressed and displaced toward the right.\nUnder general anesthesia, the patient was positioned prone after placement of transcranial muscle motor evoked potentials (Tc-MEPs) and somatosensory evoked potentials (SSEPs) monitoring devices. A T9-10 laminectomy was performed after midline splitting of T9-10 spinous process. The dura mater was exposed and the tumor location has been confirmed at the T9-10 level by ultrasonography. An operative microscope was used during opening the dura, the dura was incised at the midline, exposing a hard and totally ossified, round dural-based extramedullary mass compressing and displacing the spinal cord to the right. We performed tumor debulking with an ultrasonic surgical aspirators. Adhesions between the tumor and the spinal cord were meticulously separated after adequate internal decompression of the tumor (). Surgery proceeded slowly and en block resection of the tumor was performed without significant changes in MEP monitoring. Using the microscissors and microdissector, the tumor and its dural attachment at the left side were removed from the spinal cord. The dural attachment was thoroughly coagulated using bipolar cauterization. A gross-total resection could be achieved with en block manner. Finally, the dura was closed with a Gore-Tex membrane (W. L. Gore & Associates, Inc., Flagstaff, AZ, USA) as an intradural sheet to prevent arachnoiditis.\nHistopathological findings demonstrated delicate oval nuclei and inconspicuous nucleoli, lightly eosinophilic cytoplasm, proliferate forming cellular whorls. Within the tumor, marked heterotopic ossification occurs without psammoma bodies (). The findings were consistent with spinal metaplastic meningioma. Postoperative MR imaging revealed no evidence of residual tumor. Three days after surgery, the patient had regained full urinary continence, ambulated without an assistive device, and exhibited normal motor strength with residual numbness in her both feet. Prior to discharge from the hospital on postoperative day 31, she could ambulate without a cane and both leg numbness and sensation of pain as well as touch had improved. However, left foot numbness persisted and it caused her hard to go up the stairs for herself. She benefited from a short stay in the inpatient rehabilitation unit. | [[61.0, 'year']] | F | {'7143063': 1, '5993008': 1, '31445511': 2, '5070871': 1, '2705343': 1, '934461': 1, '25744347': 1, '34754555': 2, '3964853': 1, '10752106': 1, '31579830': 2, '11389409': 1, '10365544': 1, '7172649': 1, '5002424': 1, '11714573': 1, '8278859': 1, '6699695': 1, '18136836': 1, '8874546': 1, '31320828': 1, '10070460': 1, '4631926': 1, '10660020': 1, '7207766': 1, '4810300': 1, '8162148': 1, '24278660': 2} | {'6708243-1': 1, '8571252-1': 1, '6773579-1': 1} |
164,340 | 3836939-1 | 24,278,661 | noncomm/PMC003xxxxxx/PMC3836939.xml | A Long-Term Survival Case of a Primary Malignant Intracerebral Nerve Sheath Tumor | A 13-year-old boy was hospitalized due to the headache on the right side of his head, which began 2 weeks ago and the vomiting, which began a week ago. The patient was alert and showed no neurologic abnormalities. There was no evidence of neurofibromatosis such as neurofibroma, café au lait spot, Lisch nodule, optic glioma, distinctive bony lesion (dysplasia of the sphenoid bone or long bone cortex) and freckle in the groin or axilla with no family history of neurofibromatosis. Brain magnetic resonance imaging (MRI) revealed about 6.7 cm diameter, a well defined highly enhancing mass in the right frontal lobe (). The preoperative differential diagnoses were glioblastoma multiforme, anaplastic astrocytoma, and malignant meningioma. A surgical resection was carried out through right frontal craniotomy.\nMacroscopically, it was a well-circumscribed yellowish nodular round mass having gelatinous surface without dural attachment (). It was intra-parenchymal tumor and had a distinct cleavage plane in the superficial brain parenchyma, but not in the deeper portion. Microscopically, the tumor was highly cellular and mainly consisted of spindle cells in interlacing and interwoven fascicles. Perivascular whirling and condensation of tumor cells were observed. The tumor was well-demarcated from the adjacent brain tissues. Each tumor cell had a fusiform nucleus and aligned in a convoluted form with a moderate degree of mitotic activity and a high nuclear-to-cytoplasmic ratio. Myxoid parts were scattered in the tumor ().\nImmunohistochemistry showed that the tumor cell was positive for S100 (), but negative for glial fibrillary acidic protein (GFAP), CD34, synaptophysin and desmin. These histological and immunohistochemical findings supported the diagnosis of a primary MINST.\nThirty times of fractionated whole-brain radiation were performed with 5760 cGy over the period of 6 weeks after surgery. Brain MRI follow-up examination was performed every six months, but there was no evidence of tumor recurrence. Brain MRI at 50 months after his first surgery revealed a 1.0×1.0 cm of well-defined enhancing mass in the right frontal convexity and leptomeningeal enhancement in the right frontal area (). Whole spinal MRI showed no evidence of metastasis. No malignant cell was observed in his cerebrospinal fluid study. Second gross total resection (GTR) was performed. The histopathological examination showed a primary MINST, which was consistent with the histopathological results from the first surgery. The brain MRI at four months after second surgery revealed a tumor of 1.0×0.5 cm in size in the right anterior frontal convexity and pachymeningeal enhancement in the right frontal area (). As the patient's guardian wanted to treat the patient in another hospital, so the patient was transferred to another hospital. Third GTR was carried out. The histopathological examination also showed a primary MINST. As the tumor was repeatedly relapsed despite the GTR which was performed twice before, radiation therapy and chemotherapy were performed according to the Korean Society for Pediatric Neuro-Oncology (KSPNO)-S081 Protocol, which can be used for a high-grade brain tumor. Thirty times of fractionated radiation were carried out with 6000 cGy after his third surgery and 4 times of combination chemotherapy with vincristine (1.5 mg/m2 IV push, Day 0, 7), etoposide (75 mg/m2 in normal saline dose ×2.5 times IV over 2 hours, Day 0-4), carboplatin (300 mg/m2 in normal saline 125 mL/m2 IV over 1 hour, Day 0, 1), and ifosfamide (1500 mg/m2 in dextrose water 200 mL/m2 with Mesna (300 mg/m2 IV over 1 hour, Day 0-4) were applied. The irregular enhancing lesion (3.0×4.0 cm) was found in a brain MRI at six months after the third surgery (). As the size of this lesion was found to be smaller than the previous one in the brain MRI at thirteen months after the third surgery, this lesion is considered to be the result of radiation injury rather than tumor recurrence (). He is still alive at 77 months after his first surgery and complaining of mild headache and dizziness, but there are no other neurological abnormalities. | [[13.0, 'year']] | M | {'3082508': 1, '18496188': 1, '22493760': 1, '15527099': 1, '14744297': 1, '16293873': 1, '9452273': 1, '3799145': 1, '6319616': 1, '18795600': 1, '21327709': 1, '14624071': 1, '8351625': 1, '10659023': 1, '24278661': 2} | {} |
164,341 | 3836940-1 | 24,278,662 | noncomm/PMC003xxxxxx/PMC3836940.xml | Chronic Subdural Hematoma after Eccentric Exercise Using a Vibrating Belt Machine | A 75-year-old man presented with a headache and intermittent left side numbness from 10 days ago. He had suffered from hypertension, diabetes mellitus, renal insufficiency and gout. On admission, he was in a relatively normal status without any significant neurological deficit. We suspected transient ischemic stroke, so we performed magnetic resonance imaging (MRI). On MRI, the T1-weighted IR images revealed bilateral subdural isointense collections (). On T2-weighted SE and T2-FLAIR images, bilateral subdural collections showed hypointensity (). We also performed gradient-echo T2-weighted MR imaging. The image of the right side collection was more prominently hypointense than the left side (). Bilateral hematomas were appeared as homogeneous lesion without multilayered or lobulated structure. Besides, arachnoid cyst was appeared on left temporal pole ().\nWe also obtained computed tomographic (CT) scans. The CT scan revealed thick crescent homogeneous isodensity lesions in both fronto-temporo-parietal regions without midline shift ().\nBased on the CT and MR findings, we could diagnose as bilateral CSDH. We tried to obtain any trauma history in detail again. However, the patient did not have even a minor trauma, except for using a vibration belt massage to his own head for 20 days.\nHe was treated by burr-holes in the both parietal regions. We could identify the subdural neomembrane on both sides. Dark red fluid was evacuated from both sides. We kept silastic drainage tubes for 24 hours, which were removed on the next day. Postoperatively, CT scan revealed removal of CSDH with subdural air. We could find an arachnoid cyst (AC) on the left temporal pole which was unnoticed on the preoperative CT scan ().\nThe patient discharged to home after release of symptoms. Follow-up CT scans obtained 2 months after surgery, revealed an asymptomatic recurred CSDH on the left fronto-temporo-parietal region (). An AC was remained on the left temporal pole.\nOn 5 months after surgery, the recurred CSDH was completely resolved spontaneously (). However, the AC was still found on the left temporal pole. | [[75.0, 'year']] | M | {'16949843': 1, '9865409': 1, '12937485': 1, '4001427': 1, '18382976': 1, '19398915': 1, '23634275': 2, '21635806': 1, '6736357': 1, '11807186': 1, '7996597': 1, '3668635': 1, '19901817': 1, '12145124': 1, '6689790': 1, '31720270': 2, '12581336': 1, '10636168': 1, '33096872': 1, '9217648': 1, '24278662': 2} | {'3638278-1': 1, '6826096-1': 1} |
164,342 | 3836941-1 | 24,278,663 | noncomm/PMC003xxxxxx/PMC3836941.xml | Intractable Occipital Neuralgia Caused by an Entrapment in the Semispinalis Capitis | A 66-year-old, female developed episode of paroxysmal, severe lancinating pain in her left occipital region, correlating to the area of the GON. Mild pain had started 6 months before and had increased progressively in severity and frequency. The pain originated at the occipitocervical junction and radiated to the vertex. It was sharp, lancinating in nature and exacerbating factors such as neck flexion were not found. At times, it was associated with nausea and photosensitivity. Attacks of lancinating pain with a severity score of 9/10 on a visual analog scale (VAS) lasted for a few seconds. A residual dull aching pain remained between the attacks (score 5/10 on a VAS). The frequency of paroxysmal pain progressively increased and eventually became almost constant for 1 month until, she became bed-ridden without being able to do her daily activities. On examination, a painful, dysesthetic allodynia was present over the left GON distribution. During evaluation, she was placed on more than 7 medications that included non-steroidal anti-inflammatory drugs (NSAIDs), antiemetics, anticonvulsants including carbamazepine, lamotrigine, and gabapentin, β-blockers, anticholinergics, steroids, and triptans. Opioids including 40 mg of IRcodon and transdermal fentanyl patch were tried in vain. A block of the GON could provide transient relief of neuralgic pain for only about 1 hour. Radiofrequency ablation of the GON was tried with minimal effect. Intramuscular injection of diclofenac sodium could provide only 2-3 hours of partial relief. The flexion and extension views of the cervical spine showed no evidence of subluxation or arthritic change. The magnetic resonance imaging (MRI) of the brain and cervical spine was obtained and was negative for any mass lesion and upper cervical degenerative change. On the T2-weighted sequence, an anomalously low PICA loop was observed on the left. The distal loop extended to the level of the C1 lamina (). To evaluate the possibility of the low-lying PICA loop compressing the upper cervical roots, a computed tomographic myelography was performed and showed the same finding of the MRI () : the left PICA was low so that it extended to the level of C1. A superficial vascular ultrasound with a 17 MHz linear transducer showed no abnormal vascular contact along the course of the GON.\nConsidering the intractability of the occipital neuralgia and the possible vascular compression of the upper cervical root, the explorations of the GON, the dorsal rami of the C2 root, and intradural C2 root were planned. A linear midline incision was made and the dissection was carried down along the course of the GON. Upon entering the semispinalis perforation of the semispinalis caipits, tight constriction and entrapment of the GON was noted (). Careful dissection was performed to leave a generous amount of space surrounding the entrapment site to prevent postoperative constriction. There was no compression upon dissection of the proximal course of the GON around the inferior oblique muscle and the proximal dorsal rami of the C2 lateral to the foramen (). After left-sided hemilaminotomy of C1 and dural opening, the low-lying PICA loop was not found to be in contact with the left side of the C2 posterior root (). At the conclusion of the operation, the overlying wounds were closed avoiding any remnant constriction or compression along the course of the GON. Immediately upon awakening from the anesthesia, the patient was free of severe lancinating, neuralgic pain and experienced only the typical incisional pain of a surgical procedure. All medications including gabapentin, NSAIDs, and opioids were discontinued until postoperative three months. During 12 months' follow-up, she had no attack of lancinating pain of occipital neuralgia, although she experienced some paresthesia in her left occipital region. No hypesthesia and no allodynia were found at the follow-up. | [[66.0, 'year']] | F | {'2385345': 1, '7135065': 1, '6491740': 1, '756019': 1, '2047922': 1, '1582835': 1, '8666535': 1, '21457245': 1, '15087807': 1, '26136602': 1, '14758238': 1, '28331643': 2, '14979299': 1, '10332322': 1, '18021267': 1, '26504278': 1, '24278663': 2} | {'5346380-1': 1} |
164,343 | 3837087-1 | 24,278,860 | noncomm/PMC003xxxxxx/PMC3837087.xml | Giant Ascending Colonic Diverticulum Presenting With Intussusception | A 30-year-old female was transferred to Chosun University Hospital with a 1-month history of left lower quadrant pain. The laboratory examination at admission was unremarkable. Physical examination revealed left lower quadrant tenderness with a palpable abdominal mass. Plain abdomen X-ray presented a gas-filled cavity in the middle right abdomen. The computed tomography scan from an outside hospital showed a target sign suggesting intussusception in the mid to lower colon on the left side (). The intussusception was soon resolved spontaneously without any procedure. To evaluate the lesion, we performed a colonoscopic examination, which revealed a huge submucosal tumor (SMT)-like lesion in the ascending colon (). The abdominal computed tomographic scan was checked again for further assessment of the SMT-like lesion and showed 4.2-cm-sized encapsulated fluid of high density with a thick wall on the right side of abdomen ().\nA laparoscopic right hemicolectomy was performed because of uncertainty of the diagnosis and for a one-stage treatment. On the operation findings, the lesion which was 5 cm × 5 cm in size was located in the mid portion of the ascending colon and extended to the hepatic flexure. Also, multiple enlarged lymph nodes were found around the ileocolic and the middle colic vessel and were removed by using an en bloc resection. Gross finding of the removed specimens showed a large diverticulum on the ascending colon (). Histologic examination of the diverticulum showed the full thickness of the bowel wall from the mucosa to the serosa (). The patient made a quick recovery and was discharged 7 days after surgery without any complication. | [[30.0, 'year']] | F | {'14631129': 1, '19152443': 1, '12752356': 1, '9749503': 1, '15012168': 1, '30413459': 1, '5557762': 1, '18555044': 1, '28374053': 1, '8420256': 1, '20509001': 1, '3516602': 1, '26385691': 1, '25681218': 1, '10482713': 1, '29699586': 2, '9219776': 1, '25574112': 1, '24278860': 2} | {'5921542-1': 1} |
164,344 | 3837088-1 | 24,278,861 | noncomm/PMC003xxxxxx/PMC3837088.xml | Acute Cholecystitis After a Colonoscopy | A 35-year-old male underwent a screening colonoscopy. A 5-mm-sized sessile polyp was noted in the sigmoid colon. After informed consent had been obtained, an uneventful hot snare polypectomy was performed. About 48 hours after the procedure, the patient visited the emergency room with epigastric pain. Physical examination revealed right upper quadrant and epigastric tenderness. The patient had a blood pressure of 150/90 mmHg, a heart rate of 105 beats/min, a respiratory rate of 22 times/min, and a body temperature of 38.5℃. Peripheral blood tests showed white blood cell, 13,300 mm2; neutrophils, 75.0%; hemoglobin, 17.0 g/dL; and platelets, 309,000 mm2. Blood biochemistry showed total bilirubin 1.7 mg/dL; serum glutamic oxalacetic transaminase, 42 IU/L; serum glutamic pyruvic transaminase, 115 IU/L; alkaline phosphatase, 263 IU/L; blood urea nitrogen, 5.9 mg/dL; creatinine, 1.1 mg/dL; and C-reactive peptide, 29.3 mg/dL.\nOn a plain radiograph, no intraperitoneal air was seen. Abdominal computed tomography showed a 1-cm-sized gallstone in the gallbladder neck, which had a thickened wall and pericholecystic inflammation (). The patient was treated with intravenous antibiotics, and his symptoms and lab findings were improved within 3 days. He was discharged 7 days after admission and was given oral antibiotics; he planned to undergo a cholecystectomy. | [[35.0, 'year']] | M | {'18938166': 1, '12094896': 1, '11374738': 1, '30173321': 1, '20101766': 1, '20889070': 1, '17431770': 1, '33194458': 2, '21549374': 1, '30755891': 1, '24278861': 2} | {'7654559-1': 1, '7654559-2': 1} |
164,345 | 3837368-1 | 24,285,986 | noncomm/PMC003xxxxxx/PMC3837368.xml | A Keratocyst in the Buccal Mucosa with the Features of Keratocystic Odontogenic Tumor | A 74-year-old man consulted our clinic with a complaint of swelling in the right buccal region. The patient noticed the swelling 5 years ago and had received aspiration therapy repeatedly to reduce the swelling. He was generally healthy and had no medical history. On examination, an elastic firm, movable mass of 50mm was palpable in the right buccal region (Fig. ). The overlying mucous membrane was normal.\nThe mass was not adhesive to either the mucous membrane or the skin. The maxilla was totally edentulous and only bilateral canines and the left first premolar were present in the mandible. Hypoesthesia or palsy of the right face, trismus, swelling of cervical lymph nodes or the right parotid gland was not observed. CT examination revealed a well-circumscribed oval cystic lesion of 35 mm with the density slightly less than that of muscle in the anterior region of the masseter muscle (Fig. ). On MRI, the lesion showed a low signal on the T1-weighted image and a heterogeneous high signal on the T2-weighted image including intermediate signal in the under portion (Fig. ). Aspiration through the buccal skin revealed whitish liquid with squamous cells and shadow cells with a few neutrophils and lymphocytes in the cytological examination.\nThe clinical diagnosis of epidermoid cyst at the right buccal mucosa was made. The lesion was intraorally extirpated under general anesthesia through the incision along the anterior border of the mandibular ramus (Fig. ). Detachment of the lesion from the surrounding tissue was relatively easy. Stensen’s duct was not related to the lesion. The wound was closed after placing a silicon drain. Wound healing is good and no sign of recurrence has been observed for more than 4 years after the surgery.\nThe lesion was cystic, 30 × 25mm, weighed about 6g and contained brown serous liquid with a few cellular debris or keratin scale (Fig. ). Histologically, the lesion was lined with squamous epithelium with parakeratinization corrugated on the surface (Fig. ). The basal layer consisted of cuboidal cells showing palisading of the nuclei and a smooth border (Fig. ). No daughter cysts, epithelial islands, hair | [[74.0, 'year']] | M | {'25566331': 1, '15666156': 1, '34859636': 1, '12674852': 1, '3958272': 1, '265486': 1, '19778743': 1, '15798450': 1, '8977689': 1, '31205710': 2, '26770859': 2, '15599351': 1, '21270459': 1, '17697167': 1, '18212556': 1, '20955942': 1, '16820906': 1, '19837326': 1, '33967521': 1, '28706783': 1, '33154804': 2, '24285986': 2} | {'7600203-1': 1, '6537049-1': 1, '6537049-2': 1, '4684858-1': 1} |
164,346 | 3838205-1 | 24,277,990 | noncomm/PMC003xxxxxx/PMC3838205.xml | Overcoming technical challenges when treating atypical hemolytic uremic syndrome with therapeutic plasma exchange | A 21-year-old female with a known history of aHUS presented to our emergency department in September 2012. She complained of general aches and pains consistent with her previous episodes of aHUS. She had three relapses before when she was aged 8, 10, and 20. They all occurred in September. She presented with 3 days of fever and sore throat followed by dark urine. On examination, she was found to be afebrile, alert, oriented, and she was talking in full sentences. Her sitting blood pressure was 140/90 mmHg and her heart rate was 70 beats per minute with an oxygen saturation of 99% on room air.\nHematological investigations revealed a hemoglobin (Hb) count of 96 g/L, platelet count 16 × 109/L, red cell count 3.01 × 1012/L, and a hematocrit of 0.27 L/L. These results were consistent with normochromic normocytic anemia with mild polychromasia. There was also marked thrombocytopenia and microangiopathic anemia on the film, features suggestive of relapse of known HUS. Biochemistry results showed a lactate dehydrogenase (LDH) level of 1,201 U/L, haptoglobin <0.06 g/L, potassium 3.7 mmol/L, urea 20.7 mmol/L, creatinine 202 umol/L, estimated glomerular filtration rate 27 mL/minute, and C-reactive protein (CRP) of 27 mg/L. High levels of LDH and low haptoglobin levels pointed to severe hemolysis while the renal markers, such as creatinine, urea, and estimated glomerular filtration rate showed a deteriorating renal function. Earlier attempts to perform the biochemical tests had been futile due to severe hemolysis.\nAfter a multidisciplinary team collaboration that involved the Intensive Care Unit, Hematology, Nephrology, and the Emergency Department, a left femoral vascath was inserted with the view of commencing TPE using 3 L of fresh frozen plasma (FFP). The procedure was scheduled to be done in the emergency department using a membrane based TPE machine (mTPE). Plasma FLUX PSu 2S plasma exchange filters manufactured by Fresenius SE & Co (Bad Homberg, Germany) were used. These filters have a surface area of 0.6 m2, blood priming volume of 70 mL, and a plasma sulphone membrane. Filtration is primarily based on pressure gradients allowing filtration of molecules of up to 1,000 kDa including immunoglobulins, complement factors, and albumin. Soon after commencing TPE, the machine showed a blood leak alarm and it was evident that the membrane had ruptured gauging by the color of the effluent (). The filter was replaced with another one which also did not last long before the machine showed a blood leak alarm. The procedure was aborted after exchanging only three bags of FFP. An attempt was made to resume treatment with a centrifuge based TPE machine (cTPE). The centrifugal device we used was a Spectra Optia Apheresis System, a product of Terumo BCT (Lakewood, CO, USA). This machine operates by separating blood products according to their specific gravity using centrifugal force. The spill over alarm persisted on cTPE and “red blood cell detected” was shown on the machine. At this point, it was agreed to stop TPE due to the nature of the technical problems which were attributed to severe hemolysis.\nOn day 2, it was agreed that the patient could be treated with eculizumab (complement C5 blocker), but the drug was not available for compassionate access. We then revisited cTPE and the consultant overseeing our cTPE machine was contacted. We were advised to “disable” the red blood cell detector and cTPE was initiated successfully using a very low inlet flow rate ranging from 20–70 mL/minute with an anticoagulant infusion rate of 1.0 mL/minute. Heavily hemolyzed effluent was noted (). The patient continued to receive daily cTPE until day 16 when a decision was made that she had reached clinical remission, and she was discharged home. Her hematological () and biochemical () results continued to improve. A follow up of this patient after 6 weeks revealed that she was clinically well and had resumed her normal daily routines. However, plans had been made for her to have a permanent vascular access in the form of an arterio-venous fistula (AVF) in the setting of these recurrent aHUS episodes which seem to resolve after aggressive TPE treatment. | [[21.0, 'year']] | F | {'19846853': 1, '23251215': 1, '20556434': 1, '22160007': 1, '21882233': 1, '23026949': 1, '23364625': 1, '23542698': 1, '15168377': 1, '21902819': 1, '20865645': 1, '24277990': 2} | {} |
164,347 | 3838601-1 | 24,146,340 | noncomm/PMC003xxxxxx/PMC3838601.xml | Surgical Management of Retroperitoneal Leiomyosarcoma Arising from the Inferior Vena Cava | The patient is a 62-year-old female involved in a motor vehicle accident. Upon presentation, she complained of back pain and underwent magnetic resonance imaging (MRI) demonstrating an incidental finding of a 5-cm retroperitoneal soft tissue mass adjacent to the aorta and extending inferiorly from the right renal artery and vein. Further workup with contrast-enhanced computed tomography (CT) of the abdomen and pelvis was obtained demonstrating a 4.6 cm × 4.1 cm × 4.4 cm mass inferior to the right renal vein and posterior to the inferior vena cava with no other associated abnormalities (Fig. ). She then underwent image-guided core needle biopsy of this lesion with pathology demonstrating a spindle cell neoplasm consistent with leiomyosarcoma.\nDue to the proximity of the lesion to the aorta and renal vasculature, she underwent neoadjuvant radiation therapy to a dose of 50 Gy with a boost up to 55 Gy. An MRI of the abdomen and pelvis performed 4 weeks after radiotherapy was completed and redemonstrated the mass, now measuring 5.5 cm × 3.3 cm × 3.4 cm and extending from just below the left renal vein to 3 cm above the confluence of the common iliac veins without invasion of the aorta (Fig. ). She then underwent resection of the mass, which necessitated complete resection of the inferior vena cava (IVC), with reconstruction utilizing a Gore-Tex graft (W.L. Gore & Associates Inc., Flagstaff, AZ; Fig. ). Final pathology demonstrated >75 % of tumor cytoreduction and closest margin of 1 mm. Postoperatively, she was placed on a heparin drip and discharged on lifelong warfarin and aspirin per our vascular surgery protocol. At her 12-month follow-up appointment, she was doing well with a patent graft. Unfortunately, follow-up imaging obtained 14 months after surgery revealed evidence of extensive pulmonary metastases, not amenable to resection, and partial thrombosis of her graft. | [[62.0, 'year']] | F | {'21176932': 1, '8920790': 1, '29147340': 2, '15631921': 1, '26629783': 1, '26661949': 1, '27920691': 2, '14522326': 1, '16701120': 1, '20463568': 1, '10091791': 1, '16044929': 1, '22347712': 1, '31420622': 1, '34178689': 2, '19558690': 2, '21792507': 1, '17896156': 1, '34306875': 2, '25460472': 1, '16858193': 1, '8376012': 1, '22386140': 1, '18841423': 1, '10664496': 1, '34392834': 1, '16983035': 1, '26943403': 2, '21147299': 1, '20516760': 1, '20494550': 1, '24146340': 2} | {'8226245-1': 1, '8294025-1': 1, '2710329-1': 1, '2710329-2': 1, '5649677-1': 1, '4747934-1': 1, '5118828-1': 1} |
164,348 | 3839837-1 | 24,294,010 | noncomm/PMC003xxxxxx/PMC3839837.xml | Acute liver failure caused by mushroom poisoning: a case report and review of the literature | A 63-year-old male patient was admitted to the emergency room with weakness, nausea, vomiting, and diarrhea. After specific query, he reported ingesting several wild mushrooms about 36 hours earlier. Severe nausea, vomiting, and diarrhea had set in 7–8 hours after ingestion. He had a medical history of hypertension and colon carcinoma. He had undergone surgery and had received chemotherapy two months earlier. Also, he had no metastasis in the liver, he did not consume alcohol, and he did not use any medication.\nOn admission, he was awake and fully oriented, and all vital signs were normal. His physical examination was unremarkable except for dehydration. Arterial blood gas analysis was normal. Hepatitis B surface antigen, hepatitis B core antibody, immunoglobulin M, and antihepatitis C antibody were found to be nonreactive. A hepatitis B virus DNA analysis was performed using the polymerase chain reaction and was determined to be negative. The patient was then admitted to the internal medicine intensive care unit. After performing a gastric lavage via a nasogastric tube, activated charcoal was initiated and continued at a dose of 50 grams every 6 hours. The patient was rehydrated via intravenous (iv) administration with 0.9% sodium chloride and 5% dextrose to guard against the risk of hypoglycemia. Simultaneously, silibinin, at a bolus dose of 5 mg/kg iv, was initiated and followed by a continuous iv infusion of 20 mg/kg/day. Acetylcysteine was given by continuous iv infusion for 21 hours, with a total dose of 300 mg/kg (150 mg/kg over 1 hour, followed by 50 mg/kg over 4 hours, followed by 150 mg/kg over 16 hours). Infusion of penicillin G in doses of 1,000,000 U/kg/day, multivitamin, and alpha lipoic acid were started. Treatments are summarized in . Complete blood count, biochemistry measurements, and blood gas monitoring were performed every 6 hours ( and ).\nAt 6 hours after his admission, aspartate aminotransferase (AST) was 880 U/L; alanine aminotransferase (ALT) 665 U/L; lactate dehydrogenase (LDH) 1,028 U/L; total bilirubin 4.9 mg/dL; direct bilirubin 1.9 mg/dL; prothrombine time (PT) 36.5 seconds; and international normalized ratio (INR) 3.11. His lactate level was normal. Vitamin K (menadion 20 mg/day, iv) and metilprednisolone in doses of 1 mg/kg/day were started.\nAt 12 hours from admission, AST was 1,836 U/L; ALT 1,232 U/L; LDH 1,471 U/L; total bilirubin 7.2 mg/dL; direct bilirubin 3.1 mg/dL; PT 73.1 seconds; and INR 6.86. Upon arterial blood gas analysis, the patient had metabolic acidosis with a normal anion gap (AG) and respiratory alkalosis (pH 7.38; partial pressure of carbon dioxide [pCO2] 26.5 mmHg; partial pressure of oxygen [pO2] 49.5 mmHg; bicarbonate [HCO3] 17.8 mmol/L; base excess [BE] −8.8 mmol/L). Fresh frozen plasma were administrated at a dose of 15 mL/kg, and hemodialysis was performed for 3 hours.\nAt 30 hours the patient’s general condition began to worsen. He was somnolent and mildly tachypneic. His AST level was 1,900 U/L; ALT 1,473 U/L; LDH 2,582 U/L; PT 76.5 seconds; INR 7.22; hemoglobin (Hb) 12.5 g/dL; and platelets (PLTs) 123,000/mm3. We consulted the committee for liver transplantation, but because of the patient’s colon carcinoma, our application was not accepted. Current therapies were continued. Hemodialysis was performed again for 4 hours, and fresh frozen plasma was given again. After dialysis and fresh frozen plasma replacement, AST was 1,843 U/L; ALT 984 U/L; LDH 3,826 U/L; PT 42.6 seconds; and INR 3.71.\nAt 48 hours from the time of admission, AST was 1,207 U/L; ALT 1,797 U/L; LDH 4,318 U/L; total bilirubin 9.8 mg/dL; direct bilirubin 3.3 mg/dL; PT 47.7 seconds; INR 4.21; Hb 11.8 g/dL; and PLTs 33,000/mm3. Arterial blood gas analysis revealed high AG metabolic acidosis with respiratory alkalosis (pH 7.36; pCO2 20.3 mmHg; pO2 52.3 mmHg; HCO3 14.7 mmol/L; BE −13 mmol/L; and AG 25.3 mmol/L). His fibrinogen level was in the normal range (200–400 mg/dL) and D-dimer concentration was elevated (>2000 μg/L). No schistocytes were apparent in a peripheral blood smear.\nThe patient had developed a flapping tremor. His blood ammonia level was 281 μg/dL. Hepatic encephalopathy treatment, including 500 mL of branched chain amino acid solution (HepatAmine®; B. Braun Medical Inc, Irvine, CA, USA) over a 12-hour period and infusion of ornithine-aspartate at a dose of 20 g/day and lactulose at a dose of 45 g/day, was started.\nAt 54 hours AST was 3,570 U/L; ALT 3,282 U/L; LDH 4,379 U/L; total bilirubin 12.4 mg/dL; PT 111 seconds; INR 11.05; and PLTs 29,000/mm3. Hemodialysis was performed for 4 hours and fresh frozen plasma was given again. At 60 hours, PT was 34.6 seconds; INR 2.92; Hb 8.6 g/dL; and PLTs 12,000/mm3. One unit of erythrocyte suspension was given.\nAt 72 hours after admission, the patient lost consciousness. His body temperature was 38.2°C, pulse rate 112 beats per minute, respiratory rate 32 breaths per minute, and blood pressure 89/57 mmHg. Meropenem was started at a dose of 500 mg every 12 hours for sepsis.\nAt 78 hours AST was 1,096 U/L; ALT 2,004 U/L; LDH 1,615 U/L; PT 100.6 seconds; INR 9.87; and PLTs 7,000/mm3. Hemodialysis was performed for 4 hours, and fresh frozen plasma and platelet infusion at a dose of 1 U/10 kg were given. Arterial blood gas analysis showed pure respiratory alkalosis (pH 7.54; pCO2 23.8 mmHg; HCO3 24.1 mmol/L; BE −1.6 mmol/L).\nAt 84 hours the patient’s serum lactate was 16.59 mg/dL, ammonia 415 μg/dL, and serum creatinine was 2.6 mg/dL, so he was considered to have developed hepatorenal syndrome. His arterial blood gas values were pH 7.39; pCO2 8.4 mmHg; pO2 86.8 mmHg; HCO3 10.4 mmol/L; BE: −20.2 mmol/L; and respiratory rate was 38 breaths per minute. The patient was sedated and intubated.\nAt 90 hours from his admission, the patient went into cardiac arrest and was revived after resuscitation. On arterial blood gas analysis; pH 7.07, pCO2 32.6 mmHg, pO2 94.7 mmHg, HCO3 9.9 mmol/L, BE −18.9 mmol/L and then intravenous sodium bicarbonate, was given.\nAt 96 hours, the patient’s AST was 329 U/L; ALT 855 U/L; PT 77.3 seconds; INR 7.31; PLTs 13,000/mm3; Hb 8.3 g/dL; serum creatinine 3.3 mg/dL; serum lactate 112 mg/dL; pH 7.36; pCO2 22.3 mmHg; HCO3 15.3 mmol/L; and BE −12 mmol/L. At 98 hours after his admission, the patient went into cardiac arrest again and died. | [[63.0, 'year']] | M | {'1124787': 1, '18030554': 1, '11820073': 1, '27988828': 1, '17902123': 1, '34484676': 1, '3745203': 1, '3378810': 1, '20397738': 1, '8368976': 1, '12475187': 1, '31561486': 2, '34070525': 1, '1749051': 1, '33322477': 1, '16736443': 1, '1368802': 1, '29979397': 1, '14505933': 1, '20535367': 1, '6105483': 1, '8326897': 1, '17559970': 1, '19454504': 1, '26019894': 2, '12935559': 1, '26637206': 1, '23742066': 1, '26835473': 2, '11721773': 1, '24294010': 2} | {'4441771-1': 1, '6963215-1': 1, '6963215-2': 1, '4724762-1': 1, '4724762-2': 1} |
164,349 | 3839843-1 | 24,293,990 | noncomm/PMC003xxxxxx/PMC3839843.xml | Central retinal artery occlusion in association with fibromuscular dysplasia | A 14-year-old girl with a previous diagnosis of FMD was referred to the clinic of ophthalmology with the complaint of severe blurring in the right eye for 2 days. She had a diagnosis of FMD for 4 years. According to her medical history, obtained from the patient file belonging to the Department of Pediatrics, she had unusually high systemic blood pressure (180/110 mmHg), recorded at a regular visit. After realizing decreased flow in her left renal artery by Doppler ultrasonog-raphy, she received the angiography procedure. The final report of the procedure was that band-like narrowing in the left renal artery gives rise to thought fibromuscular dysplasia with no pathologic finding in vertebral arteries. Two months after establishing the diagnosis of FMD (which was four years ago), she received balloon angioplasty and started captopril to control her systemic blood pressure.\nOcular examination revealed afferent pupillary defect in the right eye. Best corrected visual acuity (BCVA) was 20/400 in the right eye and 20/20 in the left eye. The examination of anterior segment and ocular pressure were bilaterally within normal limits. Because of the cherry-red spot sign in the macula of the right eye, the patient immediately underwent stereoscopic color fundus photography and fluorescein fundus angiography. Fundus examination revealed that the left eye was normal () but that the right eye contained ischemic whitening with a cherry-red spot area (). Fluorescein fundus angiography demonstrated abnormal retinal arterial and choroidal dye filling in the early phase in the right eye. CRAO was diagnosed from the characteristic appearance ( and ).\nThere were no systemic risk factors such as diabetes mel-litus, hyperlipidemia, or smoking. Hematologic evaluation was also performed and all coagulation parameters were within normal limits. The patient then underwent carotid evaluation and echocardiographic study to determine the source of possible embolism. For carotid evaluation, carotid angiography was performed. Echocardiographic and carotid angiographic findings were within normal limits and there was no source of embolism such as valve or wall lesions. There was no tenderness in the face over the temporal artery. Erythrocyte sedimentation rate, C-reactive protein, and platelet count were all within normal limits.\nThe systemic blood pressure of the patient was 140/95 mmHg without antihypertensive drugs. She reported that she was taking her systemic antihypertensive drugs irregularly. At the latest visit (6 months ago), the patient’s right BCVA decreased to hand motion. | [[14.0, 'year']] | F | {'6214933': 1, '21088355': 1, '9098313': 1, '17170616': 1, '11798987': 1, '31528195': 1, '1821202': 1, '19896327': 1, '15187675': 1, '18441166': 1, '23715093': 1, '10647953': 1, '16138997': 1, '10768337': 1, '24293990': 2} | {} |
164,350 | 3840140-1 | 24,285,971 | noncomm/PMC003xxxxxx/PMC3840140.xml | A Case of GNE Myopathy Presenting a Rapid Deterioration during Pregnancy | A 27-year-old Korean woman complained of difficulty walking during her first pregnancy. She found it more comfortable to walk on her toes than on her heels. She subsequently experienced impaired foot dorsiflexion with frequent falling and rapid wasting of both anterior tibialis muscles during the third trimester and following delivery. A review of her history revealed normal mental and physical development, and she reported no difficulties with daily activities prior to pregnancy. Her family history was significant in having an older sister with a similar progressive limb weakness, appearing in the lower limbs at 19 years and in the upper limbs at. The neurologic examination of our case revealed marked weakness and atrophy of both anterior tibialis muscles with no weakness of the quadriceps and upper limbs. Motor strengths were decreased in ankle dorsiflexion [1/5 on the Medical Research Council (MRC) Scale] and plantar flexion (4/5 on the MRC Scale). She had normal deep tendon reflexes in all limbs without sensory deficit. She had no pathologic reflexes. The serum CK level was slightly elevated to 302 U/L (normal range 21-215 U/L). Electromyography revealed myopathic changes.\nA computed tomography scan of her skeletal muscles showed severe fatty infiltration of the gastrocnemius, soleus, and tibialis anterior muscles with sparing of the quadriceps muscles (). A muscle biopsy performed on the vastus lateralis muscle revealed mild myopathic changes without RV. After obtaining informed consent, 11 coding exons (exons 2-12) of the GNE gene were analyzed, which revealed compound heterozygous mutations changing the ATG (methionine) to ACG (threonine) at codon 29 (M29T) in exon 2 and the GAT (aspartic acid) to AAT (asparagine) at codon 208 (D208N) in exon 4 (). | [[27.0, 'year']] | F | {'10354198': 1, '32917266': 1, '12473753': 1, '14733963': 1, '16372135': 1, '30467490': 1, '20300792': 1, '14707127': 1, '19448634': 1, '11929971': 1, '29305133': 1, '18815882': 1, '7252518': 1, '16810679': 1, '24285971': 2} | {} |
164,351 | 3840180-1 | 24,286,046 | noncomm/PMC003xxxxxx/PMC3840180.xml | Two-Year Follow-up on the Use of Absorbable Mesh Plates in the Treatment of Medial Orbital Wall Fractures | A 31-year-old female (patient No. 1) had a left medial orbital wall fracture. The expanded orbital volume was calculated to be approximately 1.85 mL. Nine days after trauma, the swelling improved and the patient showed mild enophthalmos. Medial wall reconstruction with an absorbable mesh plate was performed. The 2-year follow-up CT scan showed an asymmetric orbit but the enophthalmos was corrected (). | [[31.0, 'year']] | F | {'11071611': 1, '10847282': 1, '9385951': 1, '30332891': 1, '28913237': 1, '10359243': 1, '9427925': 1, '11358097': 1, '11340383': 1, '25075357': 1, '8531006': 1, '18520394': 1, '16360856': 1, '24286046': 2} | {'3840180-2': 2} |
164,352 | 3840180-2 | 24,286,046 | noncomm/PMC003xxxxxx/PMC3840180.xml | Two-Year Follow-up on the Use of Absorbable Mesh Plates in the Treatment of Medial Orbital Wall Fractures | A 27-year-old male (patient No. 2) suffered blunt trauma and developed a right medial orbital wall fracture. The expanded orbital volume was approximately 3.5 mL. Eight days after trauma, the medial wall was reconstructed with an absorbable mesh plate. The 2-year follow-up CT scan showed orbital asymmetry and grade 1 enophthalmos was noted at the right side (). Hertel's exophthalmometry showed 2 mm of mild enophthalmos. | [[27.0, 'year']] | M | {'11071611': 1, '10847282': 1, '9385951': 1, '30332891': 1, '28913237': 1, '10359243': 1, '9427925': 1, '11358097': 1, '11340383': 1, '25075357': 1, '8531006': 1, '18520394': 1, '16360856': 1, '24286046': 2} | {'3840180-1': 2} |
164,353 | 3840184-1 | 24,286,050 | noncomm/PMC003xxxxxx/PMC3840184.xml | Reconstruction of Various Perinasal Defects Using Facial Artery Perforator-Based Nasolabial Island Flaps | A 48-year-old man underwent excision of a venous malformation in the right nasal sidewall. After the defect was detected, we decided to cover it with a facial artery perforator-based flap that was 3 cm in width and 6 cm in length. A perforator from the lateral nasal artery was identified. A FTSG was needed for an additional procedure on the caudal area of the defect because of the large size of the defect. Mild venous congestion was seen on postoperative day 1 but healed with conservative management (). | [[48.0, 'year']] | M | {'10845282': 1, '21285769': 1, '11884796': 1, '3421650': 1, '25473207': 1, '22286424': 1, '14504524': 1, '22711203': 1, '10738335': 1, '19010102': 1, '20207209': 1, '21285776': 1, '25931780': 1, '15897023': 1, '18945660': 1, '19505613': 1, '31725677': 1, '21119447': 1, '19698916': 1, '23067088': 1, '23233887': 2, '25473214': 1, '4579000': 1, '29786262': 1, '2605399': 1, '27942380': 1, '15793435': 1, '24286050': 2} | {'3840184-2': 2, '3840184-3': 2, '3840184-4': 2, '3518005-1': 1} |
164,354 | 3840184-2 | 24,286,050 | noncomm/PMC003xxxxxx/PMC3840184.xml | Reconstruction of Various Perinasal Defects Using Facial Artery Perforator-Based Nasolabial Island Flaps | A 72-year-old man underwent BCC excision in the right nasal sidewall. Following cancer resection, he had a large defect over the nasal sidewall. A perforator from the lateral nasal artery was used. A 180° rotation island flap that was 3 cm in width and 5 cm in length was designed, and a FTSG was needed for an additional procedure on the nasal dorsum. Mild flap congestion on postoperative day 1 was resolved without surgery (). | [[72.0, 'year']] | M | {'10845282': 1, '21285769': 1, '11884796': 1, '3421650': 1, '25473207': 1, '22286424': 1, '14504524': 1, '22711203': 1, '10738335': 1, '19010102': 1, '20207209': 1, '21285776': 1, '25931780': 1, '15897023': 1, '18945660': 1, '19505613': 1, '31725677': 1, '21119447': 1, '19698916': 1, '23067088': 1, '23233887': 2, '25473214': 1, '4579000': 1, '29786262': 1, '2605399': 1, '27942380': 1, '15793435': 1, '24286050': 2} | {'3840184-1': 2, '3840184-3': 2, '3840184-4': 2, '3518005-1': 1} |
164,355 | 3840184-3 | 24,286,050 | noncomm/PMC003xxxxxx/PMC3840184.xml | Reconstruction of Various Perinasal Defects Using Facial Artery Perforator-Based Nasolabial Island Flaps | A 35-year-old man had a SCC on the right alar base and nasal floor. Following cancer resection, a perforator of the superior labial artery was identified at the nasolabial fold using a handheld Doppler probe. Dissection of the pedicle was performed until tension-free rotation was achieved to cover the defect. A 120° rotation island flap that was 2 cm in width and 3 cm in length was elevated and pivoted on this perforator. A 3-dimensional reconstruction of the deep defect was achieved using the versatile perforator flap (). | [[35.0, 'year']] | M | {'10845282': 1, '21285769': 1, '11884796': 1, '3421650': 1, '25473207': 1, '22286424': 1, '14504524': 1, '22711203': 1, '10738335': 1, '19010102': 1, '20207209': 1, '21285776': 1, '25931780': 1, '15897023': 1, '18945660': 1, '19505613': 1, '31725677': 1, '21119447': 1, '19698916': 1, '23067088': 1, '23233887': 2, '25473214': 1, '4579000': 1, '29786262': 1, '2605399': 1, '27942380': 1, '15793435': 1, '24286050': 2} | {'3840184-1': 2, '3840184-2': 2, '3840184-4': 2, '3518005-1': 1} |
164,356 | 3840184-4 | 24,286,050 | noncomm/PMC003xxxxxx/PMC3840184.xml | Reconstruction of Various Perinasal Defects Using Facial Artery Perforator-Based Nasolabial Island Flaps | A 71-year-old man underwent excision of SCC on the columella and part of the septal cartilage. After the defect was localized, we decided to cover the deep nasal floor defect and reconstruct the columella with a bilateral facial artery perforator-based flap that was 3 cm in width and 4 cm in length. We customized the reconstruction using a facial artery perforator-based flap (). | [[71.0, 'year']] | M | {'10845282': 1, '21285769': 1, '11884796': 1, '3421650': 1, '25473207': 1, '22286424': 1, '14504524': 1, '22711203': 1, '10738335': 1, '19010102': 1, '20207209': 1, '21285776': 1, '25931780': 1, '15897023': 1, '18945660': 1, '19505613': 1, '31725677': 1, '21119447': 1, '19698916': 1, '23067088': 1, '23233887': 2, '25473214': 1, '4579000': 1, '29786262': 1, '2605399': 1, '27942380': 1, '15793435': 1, '24286050': 2} | {'3840184-1': 2, '3840184-2': 2, '3840184-3': 2, '3518005-1': 1} |
164,357 | 3840188-1 | 24,286,054 | noncomm/PMC003xxxxxx/PMC3840188.xml | Total Maxillary Reconstruction Using a Double-Barreled and Double Skin Paddle Fibular Flap after Total Maxillectomy | We present the case of a 12-year-old girl with a large mass in the maxilla that had grown for the previous six months, was diagnosed as chondroblastoma, and was treated with bilateral class IIIc maxillectomy [] (high maxillectomy) and reconstruction with a double-barreled and double perforator-based skin paddle fibula flap ().\nIn the supine position, starting with the scanning of the two perforator vessels from the peroneal artery, an incision was made along the edge of the fibula and dissection was performed from the flexor hallucis longus and brevis down to the periosteum, preserving the two skin paddles with a perforator vessel in each one.\nSimultaneously, a bilateral class III maxillectomy was carried out, removing the maxilla by performing a Lefort I type osteotomy. The tumor measured 10 cm×12 cm, covering the entire floor of the maxilla, from the soft palate at the back to the upper lip in the front, affecting the vomer and the nasal floor ().\nThe fibula was sectioned into two pieces with one skin paddle in each one, preserving the peroneal artery as the principal pedicle. Both segments were fixed "in situ" with screws, and subsequently the pedicle was sectioned at the proximal end. Each end of the bone was fixed to the ascending ramus of the maxilla with a 2.0-mm mini-plate titanium system (, ).\nThe superior skin paddle was deepithelized in the central zone and sutured to the mucosa of the nasal septum. The lateral edge of the superior skin paddle was sutured to the lateral wall of the nasal mucosa at the level of the piriform aperture to make a place for the nasal floor. The inferior skin paddle was sutured to the soft palate in the posterior edge and to the labial mucosa at the anterior end. The anastomosis of the artery and the vein were made using a saphenous vein graft from the facial to the peroneal vessels passing through the cheek, using simple stitches with 9-0 nylon.\nThe follow-up period was 6 months, and there was no evidence of tumor recurrence. The functional results were excellent with a well-tolerated liquid and soft diet. The patient's speech is satisfactory overall; however, her consonants are slurred. The histological study reported a solid tumor well defined by a pseudo-capsule, limited by the gingival mucosa. Small polyhedral tumor cells were found with scattered osteoclastic giant cells with no atypical mitosis. The diagnosis was chondroblastoma (-). | [[12.0, 'year']] | F | {'23096602': 1, '23524826': 1, '17061275': 1, '18090733': 1, '25931780': 1, '10585601': 1, '2734406': 1, '15861059': 1, '6934807': 1, '8253461': 1, '24286054': 2} | {} |
164,358 | 3840817-1 | 24,282,629 | noncomm/PMC003xxxxxx/PMC3840817.xml | Monoclonal gammopathy-associated pauci-immune extracapillary-proliferative glomerulonephritis successfully treated with bortezomib | A 60-year-old male was referred to our department in April 2011 by his nephrologist due to an increase in his serum creatinine to 140.8 µmol/L (1.6 mg/dL), haematuria of 290 erythrocytes/µL and spot urine protein–creatinine ratio (UPCR) of 1.9 g/g. He had also developed hypertension during the previous months. His medical history was uneventful, and physical examination did not show any pathologies. He subsequently underwent renal biopsy showing pauci-immune extracapillary-proliferative glomerulonephritis (4 of 14 crescents) with mild tubular atrophy and interstitial fibrosis (20%), as well as lymphohistiocytic infiltration (A). Polymorphonuclear leukocytes were present in the glomerulus and in the interstitium. Immunofluorescence revealed minimal mesangial deposits of C3 and complement complex C5b-9 as well as IgM. Immunofluorescence for IgA, IgG and fibrinogen was negative. No amyloid deposits or myeloma casts were identified. Serum C3 and C4 complement levels were normal. Serum ANCAs and cryoglobulines showed negative results. No anti-glomerular basement membrane antibodies were present. The patient received four monthly cycles of cyclophosphamide i.v. along with steroids. Informed consent was obtained from the patient prior to submission of this manuscript. During therapy, serum creatinine stabilized to 132.0 µmol/L (1.5 mg/dL), proteinuria (UPCR) decreased to 0.5 g/g and haematuria improved to 32 erythrocytes/µL, respectively. Maintenance treatment with azathioprine was started and the patient returned to the care of his nephrologist.\nAfter 10 months, the patient was referred to our service again with newly diagnosed macrohaematuria, increased UCPR of 5 g/g and elevated serum creatinine of 169.8 µmol/L (1.93 mg/dL). A thorough investigation revealed an increased serum β2-microglobulin of 3.5 mg/L and a pathological serum IgG kappa/lambda free light-chain ratio of 5.2 (normal value: 0.26–1.65) due to elevated serum IgG kappa light chains of 74.0 mg/L (normal range: 3.3–19.4 mg/L). A bone marrow biopsy showed an increase of monoclonal kappa-positive plasma cells to 10%. Re-evaluating the initial renal biopsy, no evidence of light-chain deposition disease, fibrillary glomerulonephritis or amyloidosis was present.\nA new renal biopsy was performed, again showing pauci-immune extracapillary-proliferating glomerulonephritis (4 of 10 crescents) with mild interstitial fibrosis. In this biopsy, there was also no evidence of classical myeloma-associated kidney disease. Due to the relapse of rapidly progressive glomerulonephritis after cyclophosphamide therapy and leucopenia during azathioprine treatment, we decided to administer two doses of 1 g of rituximab i.v. within 4 weeks and maintained the patient on a reduced dose of azathioprine in combination with cyclosporine A [, ]. Unfortunately, the patient showed rapid deterioration of his renal function within the following 8 weeks to a serum creatinine level of 303.6 µmol/L (3.45 mg/dL), an increase of proteinuria to 9 g/g and an increase of haematuria. The serum kappa/lambda free light-chain ratio also increased to 9.1. Because of the underlying plasma cell dyscrasia and rapidly worsening kidney function, we decided to start the patient on the proteasome inhibitor bortezomib (1.3 mg/m2 body surface i.v. on Days 1, 8, 15, 22) in combination with dexamethasone based on the treatment recommendations for multiple myeloma. After the first cycle of bortezomib/dexamethasone, serum creatinine decreased to 140.8 µmol/L (1.6 mg/dL), and urinalysis showed reduced proteinuria of 2 g/g. A control biopsy was performed revealing residual sclerosed crescents, completely sclerosed glomeruli, mild interstitial fibrosis and tubular atrophy (B). No signs of active extracapillary proliferations were detected. After the second cycle of bortezomib/dexamethasone, the patient showed clinical remission with serum creatinine levels of 103.8 µmol/L (1.18 mg/dL), minimal proteinuria of 0.48 g/g, no haematuria and well-controlled hypertension. Maintenance therapy of monthly bortezomib was initiated, and the patient showed stable serum creatinine values as well as stable proteinuria with 8 months of follow-up. | [[60.0, 'year']] | M | {'31043084': 2, '16299691': 1, '22920642': 1, '20042399': 1, '9366584': 1, '9132659': 1, '22470380': 1, '10344392': 1, '26120578': 2, '15102968': 1, '22313839': 1, '20647199': 1, '29043147': 1, '21447128': 1, '21233415': 1, '12631105': 1, '22897729': 1, '32884934': 2, '6436718': 1, '5437895': 1, '19858394': 1, '23047823': 1, '24282629': 2} | {'7443628-1': 1, '6498773-1': 1, '4478318-1': 1} |
164,359 | 3840819-1 | 24,282,444 | noncomm/PMC003xxxxxx/PMC3840819.xml | Pathologic improvement after high-dose melphalan and autologous stem cell transplantation for primary systemic amyloidosis | On 4 February 2004, a 56-year-old man was referred for admission to our hospital because of worsened nephritic syndrome and weight loss. Laboratory test results on admission were total protein, 5.4 g/dL; serum albumin, 2.5 g/dL; s-UN, 9 mg/dL; s-Cr, 0.6 mg/dL; alkaline phosphatase (ALP), 347 IU/L and urinary protein excretion, 5.36 g/day. Polyneuropathy and septal myocardial hypertrophy (13 mm) were also observed. To elucidate the cause of nephrotic syndrome, skin, fat and open renal biopsies were performed on 3 March 2004. The renal biopsy specimens showed amorphous periodic acid-Schiff (PAS)-positive, Congo red-positive and direct fast scarlet (DFS)-positive deposits in the mesangium, glomerular vascular pole, tubulo-interstitium and walls of interlobular arteries and arterioles (Figure A and B). These deposits were present in all 23 glomeruli (100%) and 29 of 38 arterial and arteriolar walls (76%), in which 22 walls had circumferential deposits. These deposits show characteristic apple-green birefringence in polarized light and potassium permanganate resistance. Immunohistologic staining was positive for amyloid P and negative for amyloid A. These deposits were also observed in skin, adipose tissue and gastric biopsy specimens. Bone marrow aspirates showed a slight excess of plasma cells without abnormal morphology. We diagnosed him with primary systemic AL amyloidosis of λ (lambda) type.\nBeginning in February 2004, the patient received two courses of VAD (vincristine, 0.4 mg/day, and doxorubicin, 9 mg/m2/day, on Days 1–4, and dexamethasone, 40 mg/day by infusion on Days 1–4, 9–12 and 17–20), with the second course initiated 4 weeks later. After the second course, the granulocyte colony-stimulating factor (G-CSF) was administered subcutaneously at 10 g/kg/day for 4 days to mobilize haematopoietic stem cells in the peripheral blood, and 3.35 × 106/kg CD34-positive cells were collected.\nOn 20 September 2004, melphalan (230 mg or 140 mg/m2) was administered intravenously; 7 days later, cryopreserved stem cells were infused just after thawing. After these therapies, proteinuria was gradually abating (Figure ): 5.91 g/day on 24 December 2004; 0.85 g/day on 7 March 2005 and 0.12 g/day on 11 January 2006. Serum albumin increased from 1.9 g/dL on 26 July 2004 to 2.2 g/dL on 7 March 2005 and 3.4 g/dL on 11 January 2006. Monoclonal protein in both serum and urine continued to be negative on immunofixation after therapies.\nOn 15 January 2007, the patient was readmitted to our hospital for follow-up evaluation. Laboratory findings on admission were total protein, 6.0 g/dL; serum albumin, 3.5 g/dL; s-UN, 21 mg/dL; s-Cr, 1.0 mg/dL; AST, 25 IU/L; ALT, 25 IU/L and ALP, 480 IU/L. Urinary protein excretion was 0.03 g/day. Echocardiography showed a decrease in septal myocardial hypertrophy from 13 mm to 9 mm. Nerve conduction velocity examination still showed polyneuropathy. Complete haematologic and clinical responses (cardiac, renal, gastrointestinal and factor X response) were evident according to the criteria described by Skinner et al. [] and Gertz et al. []. A second renal biopsy was performed about 2 years following the first. The specimen showed a mottled reduction in amyloid staining in interlobular arterial walls and arteriolar walls. Furthermore, decreased amounts and intensities of Congo red and DFS staining were observed in the mesangium and glomerular vascular pole (Figure C and D). We scored all glomeruli and vascular walls in each sample. Amyloid-positive glomeruli were decreased from 100% to 89% (16 of 18 glomeruli) and amyloid-positive vascular walls were decreased from 76% to 50% (6 of 12 walls). By electron microscopic examination, small electron-dense deposits were seen in the subepithelial region. However, randomly arranged fibrils, characteristic of amyloid, were slightly obscure (Figure F). A gastric biopsy specimen also disclosed the disappearance of amyloid deposition. Thus, these therapies resolved AL amyloidosis not only clinically but also pathologically in our patient. | [[56.0, 'year']] | M | {'9578896': 1, '14739213': 1, '7878478': 1, '21387097': 1, '14734330': 1, '16044444': 1, '24282444': 2} | {} |
164,360 | 3841278-1 | 24,294,459 | noncomm/PMC003xxxxxx/PMC3841278.xml | Supratentorial Intracerebral Schwannoma : Its Fate and Proper Management | A 25-year-old man presented with recent aggravation of focal seizure activities on the right arm was referred to our clinic. His first seizure attack took place 9 years ago which started with involuntary right arm tremors followed by a loss of consciousness and generalized tonic-clonic convulsions. When he first visited the neurology clinic at that time, a neurologic examination revealed no abnormalities. He had no relevant medical history, no stigmata of neurofibromatosis and no family history of this disorder. His magnetic resonance images (MRI) of the brain revealed a small mass measuring 1.5×1.0×1.1 cm at the left precentral gyrus. The mass was predominantly hypointense to gray matter on the T1 weighted images, heterogeneously hyperintense on the T2 weighted images and showed intense enhancement on gadolinium injection (). Peritumoral edema was observed, affecting mainly the left precentral gyrus. No cystic change was found. Initial radiologic differential diagnoses were a pleomorphic xanthoastrocytoma, ganglioglioma, and dysembryoplastic neuroepithelial tumor. Since he was free of symptoms between seizure attacks and well tolerated his condition after taking anti-epileptic medication, he refused to undergo surgical treatment. During the 9 years of follow-up, he had no trouble in carrying on with his daily life. He complained of only intermittent focal seizures presented as right arm tremors and transient weakness, and these seizure attacks mostly occurred under stress conditions like sleep deprivation and heavy alcohol consumption combined with poor drug compliance. A brain MRI which was taken two years after the initial image work-up, revealed no interval change. Because the patient's symptom was stable, no further imaging study was pursued.\nNine years later, he complained of sudden prolonged seizure duration with right arm weakness, which became progressively worse over the past month prior to his visit. A neurological examination revealed a grade IV weakness of the right elbow and hand. Immediate brain computed tomography and MRI were taken. The size of the enhancing portion had increased to 2.3×1.9×1.6 cm, and a large peritumoral cyst measuring about 5.5 cm in diameter had newly appeared (). No intratumoral calcification and cystic wall enhancement were observed.\nSince the mass was located at the precentral gyrus, awake craniotomy was performed to minimize postoperative motor and sensory deficit. A rectangular fronto-parietal craniotomy was done to expose the enhancing mass with the help of a neuronavigation system. Sonography was used to identify the mass located just beneath the cortical surface which appeared to have a bluish discoloration (). By direct cortical stimulation with a nerve stimulator, we confirmed that the motor cortex of the right wrist and hand were located just on the posteromedial side of the mass. Dissection started from the anterior part of the mass. It was well circumscribed from adjacent brain tissue and could be removed in an en bloc fashion. During removal, slight yellowish but cerebrospinal fluid like clear fluid gushed out from the cyst. The patient did not present any worsening of neurologic deficit during the surgery.\nThe patient's postoperative course was uneventful and his right forearm and hand weakness improved. One month after the operation, he had a postoperative MRI which revealed no residual enhancing mass, but the cyst remained although its size was somewhat decreased (). At one year and six months of follow-up, he has remained seizure free since the operation and his right hand and arm weakness has recovered to near normal status, with no difficulty in his daily living.\nThe specimen consisted of several fragments of soft tissues, measuring 2.0×1.0 cm in the largest dimension. Grossly, the cut surfaces showed a gray yellow myxoid appearance. Under light microscopy, the tumor was composed of alternating Antoni A and B areas, i.e., cellular and less cellular areas (). There was neither hemorrhage or necrosis or cystic change. The tumor had fascicular arranged spindle cells with elongated nuclei without nuclear pleomorphism. There was few mitoses (1/10 HPF) and the MIB-1 (Ki67) labeling index was low (1.03%). The tumor cells were robustly positive for S100 protein. Electron microscopic examination was consistent with Schwannoma, which showed elongated cells surrounded by a continuous external lamina and scattered Luse bodies (long space collagen). | [[25.0, 'year']] | M | {'22559850': 1, '21327709': 1, '9726470': 1, '19263360': 1, '1111146': 1, '5935382': 1, '18972118': 1, '30013644': 1, '8708655': 1, '6433498': 1, '28096224': 1, '28428901': 2, '8331403': 1, '17021734': 1, '15998381': 1, '29026664': 2, '30151105': 2, '7815115': 1, '31786630': 1, '9766275': 1, '32547830': 2, '29492148': 2, '8971843': 1, '24294459': 2} | {'5385903-1': 1, '7294175-1': 1, '5820873-1': 1, '5629864-1': 1, '6101529-1': 1} |
164,361 | 3841279-1 | 24,294,460 | noncomm/PMC003xxxxxx/PMC3841279.xml | Delayed Retroperitoneal Hemorrhage due to Lumbar Artery Pseudoaneurysm after Lumbar Posterolateral Fusion | A 55-year-old female patient with lower back pain and neurogenic intermittent claudication, who previously underwent L4-L5 PLF due to spinal stenosis, was presented to our hospital. She experienced small thalamic infarction 5 years ago, and since then, she started antiplatelet and antihypertensive medications. Lumbar spine magnetic resonance image revealed L3-L4 spinal stenosis due to adjacent segment degeneration. Hence, the patient underwent L3-L4 PLF. To prepare the bone fusion bed, the transverse process of L3 and L4 was decorticated with a drill. There was minimal bleeding during the surgery, and post-operative complaints only include mild back pain. However, on the 9th post-operative day, the patient complained of a sudden onset of severe abdominal pain and distension. Moreover, the patient was hemodynamically unstable (8.5 g/dL hemoglobin concentration; 90/60 mm Hg blood pressure; and 120/minute pulse rate). A late abdominal complication was suspected, and an emergency computed tomography (CT) scan of the abdomen with contrast enhancement was performed. This investigation revealed retroperitoneal hematoma in the right psoas muscle () and iatrogenic right L3 transverse process fracture (). The patient was resuscitated with blood transfusion, intravenous fluids for hypovolemic shock and an interventional radiologist was urgently consulted. Lumbar spinal angiography showed the delayed hematoma due to rupture of the 2nd lumbar artery pseudoaneurysm () and coil embolization was done at the ruptured lumbar artery (). After we confirmed the successful embolization through the final angiogram, the patient was sent to the ward. Since then, the patient's postoperative progress proceeded normally with recovery of the hemodynamic parameters. Two weeks after the embolization, follow-up abdominal CT showed the decreased retroperitoneal hematoma (). | [[55.0, 'year']] | F | {'3685349': 1, '13108907': 1, '18303450': 1, '12590222': 1, '12217278': 1, '20675335': 1, '32034509': 1, '31384657': 1, '10656911': 1, '19558292': 1, '20527650': 1, '12638116': 1, '24294460': 2} | {} |
164,362 | 3841280-1 | 24,294,461 | noncomm/PMC003xxxxxx/PMC3841280.xml | Cervical Compressive Myelopathy due to Anomalous Bilateral Vertebral Artery | A 70-year-old woman, recently diagnosed with hypertension, presented with symptoms of gradually progressive gait disturbance and weakness in lower extremities for about 8 months. When she was admitted, she could not go up a flight of stairs alone. Neurologic examination revealed grade III paraparesis of both upper and lower extremities. Sensory examination was intact to pain, touch, temperature and proprioception. The long tract signs were positive on both sides such as hyperreflexia, toe sign and the Hoffmann sign.\nMRI revealed large flow voids in the region of the craniovertebral junction that resulted in compression of the cord (). Computed tomography (CT) angiography revealed bilateral vertebral arteries looped medially, like having the appearance of kissing each other on the dorsal surface and deeply indented into the cord substance (). There were no other bone or soft tissue anomalies in the region.\nA midline suboccipital craniotomy with C1 laminotomy and C2 partial laminectomy for preserving paraspinal muscle of C2 was performed. Dura was incised from foramen magnum to C2 level. Both vertebral arteries were showed tortuous, looped medially each other on the posterolateral surface of the cord, and spinal cord was compressed by them. First, right side arachnoid membrane around VA was dissected and excised dentate ligament to release VA from spinal cord. And then anchoring suture from VA to the arachnoid membrane and dentate ligament was made using 6-0 nylon. And then Teflon sponge was inserted to the space between VA and the spinal cord for microvascular decompression. Same procedure was performed to left VA and enough decompression was achieved. At the end of the operation we repaired occipital bone flap & C1 lamina.\nA postoperative CT angiography was immediately obtained and it showed that the bilateral.\nVAs were separated from each other without interruption in blood flow at the C1 level (). She could walk and go up a flight of stairs at the one month follow-up and the symptoms of the patient gradually improved over 2 years of the postoperative period. Six months after the operation, the MRI showed the full recovery of the spinal cord contour and the laterally transposed VA (). | [[70.0, 'year']] | F | {'4058734': 1, '12823896': 1, '6879426': 1, '18670330': 1, '32547823': 2, '11458746': 1, '198692': 1, '8331414': 1, '31681148': 1, '8902976': 1, '11453419': 1, '1714048': 1, '19558301': 1, '24294461': 2} | {'7294169-1': 1} |
164,363 | 3841281-1 | 24,294,462 | noncomm/PMC003xxxxxx/PMC3841281.xml | Cystic Giant Sacral Schwannoma Mimicking Aneurysmal Bone Cyst : A Case Report and Review of Literatures | A 54-year-old man visited the outpatient clinic complaining tingling sensation and paresthesia of left lower limb for several years. His symptoms have been aggravated during the last several months and there has been no improvement after conservative cares. He had no history of previous trauma or constitutional symptoms. Neurologic examination revealed weakness of dorsiflexion and plantaflexion on the left foot with mild hypesthesia at the left S1 dermatome. Perineal and perianal sensations were also mildly decreased.\nPlain radiograph showed an osteolytic lesion limited to left sacral ala sparing lumbar spine (). Magnetic resonance (MR) imaging of lumbar spine revealed about 5.1×3.4×3.6 cm sized multilocular cystic mass with canal invasion and bone erosion of the left S1 body. The mass showed homogenous hypointense signal on T1-weighted image and hyperintense signal on T2-weighted image. The lesion showed multi-septated cystic rim enhancement after administration of gadolinium contrast, but no definite solid portion was found. The left S1 root was also obliterated, which seemed to be main cause of leg symptoms. No extraspinal component was identified (). Computer tomography (CT) myelography was performed and intraosseous expensile mass involving left sacrum was presented along with left S1 root compression and displacement of the left sacral nerves below. A subtle pathologic fracture involving upper endplate of S1 was also noted ().\nThe initial preoperative diagnosis was ABC, with several radiologic clues such as the location of mass, which was presenting in sacrum and compromising the neural arch, near totally cystic nature and balloon-like expanslile remodeling of bone. Even fluid-fluid level was not revealed in the imaging studies, ABC was strongly suspected.\nWe underwent a grossly-total tumor resection with lumbosacral reconstruction via posterior approach. A well-defined, encapsulated, cystic mass was found in epidural space adherent to nerve sheath displacing sacral nerve roots peripherally. The borders of tumor was carefully identified and dissected to preserve capsule of the cystic component under operating microscope. Once pericapsular dissection was done, en-bloc tumor resection was followed and sacral nerve roots were successfully preserved under spontaneous and evoked electromyography monitoring (). Since more than half of S1 body had been eroded by the tumor, an instrumented stabilization with fixation from L4 to the ilium (iliac screws) was performed ().\nAfter the operation, the patient was completely recovered from the preoperative symptoms without any neurologic deterioration (). Histological examinations confirmed the diagnosis of cystic schwannoma with both Antoni A and Antoni B growth patterns in a fibrillar background (). Verocay bodies were noted, and the tumor cells were reactive to S-100 protein antibody.\nThe patient was discharged at 1 week after the operation without any complications. Outpatient clinic follow-up for 6 month showed no symptom recurrence and the patient was tolerable. Serial follow-up X-rays at 3 and 6 month after surgery showed no implant-related complications. | [[54.0, 'year']] | M | {'6700817': 1, '11493858': 1, '20724268': 1, '9537134': 1, '22806341': 1, '17825763': 1, '9229103': 1, '10685494': 1, '23053752': 1, '22104695': 1, '11380354': 1, '17538274': 1, '34321263': 2, '1590138': 1, '19886817': 1, '23320224': 2, '26195080': 1, '17589680': 1, '1546375': 1, '18453671': 1, '11302622': 1, '17674301': 1, '18779869': 1, '18424359': 1, '16228684': 1, '1436363': 1, '8658260': 1, '3734878': 1, '17082954': 1, '20657016': 1, '17145411': 1, '15350043': 1, '24294462': 2} | {'8319961-1': 1, '3540697-1': 1} |
164,364 | 3841282-1 | 24,294,463 | noncomm/PMC003xxxxxx/PMC3841282.xml | Spinal Extradural Arachnoid Cyst | A 59-year-old man presented with both leg radiating pain and paresthesia for 4 years. Other physical findings were not remarkable. MRI showed a large, elongated posterior extradural cyst from T12 to L3 with surrounding bony erosion ().\nThinned overlying laminas were found on operative field and the cyst was ruptured during laminectomy. There was a hole-like dural defect near to right L1 root sleeve and we performed primary closure of the defect. Some part of cyst wall was also removed.\nThe patient's symptoms were gradually subsided and follow up image taken 1 month after the operation showed complete disappearance of the cyst (). | [[59.0, 'year']] | M | {'30937244': 2, '27857455': 1, '12811286': 1, '31772799': 2, '21830055': 1, '21562735': 1, '21430982': 1, '27857934': 2, '22573100': 1, '33163451': 2, '21256754': 1, '26543656': 2, '21430498': 1, '31000984': 1, '17038930': 1, '33880206': 2, '21842210': 1, '3343608': 1, '34877296': 2, '26512289': 2, '16505968': 1, '11458745': 1, '24294463': 2} | {'3841282-2': 2, '8610881-1': 1, '8053457-1': 1, '5110915-1': 1, '5110915-2': 1, '7607008-1': 1, '4620383-1': 1, '6433457-1': 1, '4623189-1': 1, '4623189-2': 1, '6854211-1': 1} |
164,365 | 3841282-2 | 24,294,463 | noncomm/PMC003xxxxxx/PMC3841282.xml | Spinal Extradural Arachnoid Cyst | A 51-year-old female patient visited our clinic with left buttock pain and paresthesia for 3 years. Her pain was aggravated on coughing and she also complained of gait disturbance with both sole numbness on walking.\nMRI showed a large extradural cyst located posterior to the cord from T11 to L2 (). CT myelogram was performed to verify any communication between the subarachnoid space and the cyst (). The contrast dye diffused within the subarachnoid space reaching to T11-12 level, and then leaked into the cyst. It scantly spreaded above the level and we assumed that there might be communication between the cyst and the subarchcnoid cyst at T11-12 level.\nAt first, total laminectomy of T10 and T11 was planned to find a dural defect. The laminae overlying the cyst were thinned. The cyst wall was ruptured during laminectomy and CSF-like fluid was gushed out. Under microscopic exploration, transparent cyst wall was found at the margin of bony exposure (). There was a hole-like, 0.5 cm sized dural defect at the right posterolateral side of the dural sac at T11 level. A rootlet got caught in the dural defect and formed a loop around the hole (). The root was repositioned into the intradural space and the dural defect was repaired with black silk 6-0. The cyst wall was severely adherent to the dural sac and there was moderate bleeding on dissecting the cyst. We only fenestrated the cyst. Some portion of the cyst wall was removed and sent to pathologic department. Pathologic findings were compatible to arachnoid cyst ().\nHer symptoms relieved gradually and follow up MRI taken at 2months after the operation showed much decreased cyst and there was no evidence of cord compression due to residual cyst (). | [[51.0, 'year']] | F | {'30937244': 2, '27857455': 1, '12811286': 1, '31772799': 2, '21830055': 1, '21562735': 1, '21430982': 1, '27857934': 2, '22573100': 1, '33163451': 2, '21256754': 1, '26543656': 2, '21430498': 1, '31000984': 1, '17038930': 1, '33880206': 2, '21842210': 1, '3343608': 1, '34877296': 2, '26512289': 2, '16505968': 1, '11458745': 1, '24294463': 2} | {'3841282-1': 2, '8610881-1': 1, '8053457-1': 1, '5110915-1': 1, '5110915-2': 1, '7607008-1': 1, '4620383-1': 1, '6433457-1': 1, '4623189-1': 1, '4623189-2': 1, '6854211-1': 1} |
164,366 | 3841283-1 | 24,294,464 | noncomm/PMC003xxxxxx/PMC3841283.xml | Phrenic Nerve Stimulation for Diaphragm Pacing in a Quadriplegic Patient | A 62-year-old female patient reported severe dyspnea and showed tachypnea. She was diagnosed as a pulmonary thromboembolism and immediate anticoagulation with warfarin was started. In the morning two days after anticoagulation, she felt heaviness in both legs and progressive paraparesis. A neurologist evaluated her and requested a lumbosacral magnetic resonance imaging (MRI) of the lumbar spine. While waiting for the MRI, her paraparesis progressively increased and ascended; eventually she became quadriplegic and respiratory distress developed. After checking whole spine MRI, an emergent endotracheal intubation was performed and neurosurgical consultation was taken. On examination, she was alert and no focal cranial nerve sign was observed. However, grade 0 quadriplegia was noticed and tendon reflex was not elicited. An acute epidural hematoma was noted on the high cervical cord (). After informed consent, an emergent decompression of the cervical cord was performed. Unfortunately, her quadriplegia did not improve after surgery. Moreover, self-respiration was not elicited. Thereafter, she was maintained with artificial ventilation during the following three months in the intensive care unit. During the follow-up, no spontaneous, voluntary respiration was observed and the MRI checked at postoperative 3 months showed a myelomalacia at the C2 level suggesting high cervical cord injury (). A standard apnea test did not elicit voluntary respiratory effort. After 3 months of artificial ventilation, we decided to perform a trial of diaphragm pacing, and informed consent was taken. An electromyographic examination of the diaphragm was performed for the verification of contraction of the diaphragm.\nA stimulation lead implantation was performed in right neck under general anesthesia with the head rotated to the left side. Muscle relaxant was not used to monitor intraoperative contraction of the diaphragm. A linear horizontal skin incision about 4 cm was made at 4 cm rostral to the right clavicle, crossing the posterior border of the sternocleidomastoideus muscle. After dissecting the subcutaneous tissue and retracting the sternocleidomastoideus medially, the anterior scalene muscle and the right phrenic nerve was identified over the scalene muscle with monopolar electrical stimulation (50 Hz, 1 msec, 1.5-2 volts, Eclipse Neurological Workstation, Axon Systems, USA) (). The nerve sheath of the phrenic nerve was carefully dissected about 3 cm in length, but we did not dissect the space between the nerve and the scalene muscle. A quadripolar, cylindrical electrode (Quatrode®, St. Jude Medical, Plano, TX, USA) was placed along the phrenic nerve and fixed with 3 sutures to the surrounding connective tissue (). A great care was taken to avoid injury and entrapment of the phrenic nerve. During intraoperative stimulation, a brisk downward deflection of the diaphragm was noticed under fluoroscopy. The distal end of the electrode was tunneled subcutaneously to the left subclavicular subcutaneous pocket and connected to the implantable pulse generator (IPG) (Eon-Mini Rechargeable®, St. Jude Medical, Plano, TX, USA).\nTwo days after surgery, the IPG was turned on. The second proximal contact of the quadripolar electrode was used as cathode and the third contact was used as positive. The other contacts were set to "off". After turning the IPG on, the carbon dioxide pressure of the exhalation and the arterial oxygen saturation in the fingertip were monitored until the values became stable. The carbon dioxide pressure in the end tidal breath was adjusted to about 400 mm Hg by changing the output strength of the IPG. The parameters of chronic stimulation were 1(-), 2(+), 300 µsec, 30 Hz, 2 seconds on with 4 seconds off in cyclic mode. After confirmation of stable end-tidal PCO2 and SaO2 levels for more than two hours in the patient's bed, the patient was allowed to ambulate in a wheelchair without mechanical ventilation. The ambulation time was gradually increased up to two hours. However, the weaning time of mechanical ventilation could not be increased more than three hours due to fatigue of breathing chest muscles. She was weaned and could ambulate in the wheelchair for two to three hours in daytime, spending time with her family, and she was dependent on the mechanical ventilator for the rest of the time. At an examination twelve months after the operation, she could ambulate with diaphragm pacing in the wheelchair for two to three hours in daytime. Mild neck discomfort around the electrode implantation site was noticed; however, there was no evidence of granulation or infection during the follow-up. She suffered pneumonia two times after diaphragmatic pacing which was easily controlled with antibiotics during the follow-up. | [[62.0, 'year']] | F | {'7420149': 1, '14518531': 1, '33355853': 1, '31001405': 1, '24500497': 1, '12648917': 1, '1275596': 1, '27083568': 1, '3908966': 1, '5694362': 1, '17691389': 1, '4294147': 1, '9619308': 1, '7251445': 1, '4921341': 1, '9601109': 1, '24294464': 2} | {} |
164,367 | 3841284-1 | 24,294,465 | noncomm/PMC003xxxxxx/PMC3841284.xml | Split Cord Malformation Combined with Tethered Cord Syndrome in an Adult | A 34-year-old woman was admitted with neck pain, back pain, and recurrent bilateral leg weakness of 2 months duration. On physical examination, there was a paresis with 4/5 muscle strength and deep tendon hyperreflexia in both legs. There were no pathologic reflexes. The initial neurologic evaluation revealed sensory numbness below the level of the C-7 dermatome. An urodynamic study, somatosensory evoked potentials, and electromyograhic evaluation showed no abnormalities.\nThe radiologic examination revealed mild scoliosis of the thoracic spine, hemi-vertebrae at T6 level, and butterfly vertebral bodies at T11 level. Spina bifida was also present. The admission cervicothoracic MRI showed that the spinal cord was divided into two segments from the level of C-7 to T-11, and there was development the subcutaneous fat within the bifid spine. No fibrous septum was demonstrated on MR imaging. On lumbosacral MRI, the conus was shown to be low-lying, ending at the L5 level (). CT myelography showed that dye was leaked incidentally into the epidural and intradural spaces. A fibrous septum divided the spinal cord and extended to the epidural space and attached to the ventral cortex of the lamina at T9 level ().\nThe patient underwent surgery to prevent progression of the neurologic damage resulting from tethering of the spinal cord. A laminectomy was performed from the level of T-8 to T-11. The T-9 and T-10 spines were bifid and abnormal fat tissue was infiltrated between the bisected spinous process. Surgery revealed a symmetric duplication of the spinal cord above the level of T-10, which was encompassed by a single dural sac, and the fibrous septum was recognized between the divided hemi-cord at the T-9 level, 10 mm above the cord reunion site (T-10). The fibrous septum splits and tethers spinal cord. The tethered spinal cord was released by resection of the fibrous septum ().\nAfterwards, a L5 and S1 laminectomy was performed to release the tethered cord syndrome by the thickened filum terminale. The nerve roots appeared slightly adherent to the dura at the L-5 level, and resection of the thickened filum was made because the cord was tethered at the level of the lesion (). The patient had an uneventful postsurgical recovery.\nAt the 7-year follow-up examination, the patient had a normal neurologic examination, with no complaints of episodic paraparesis or back pain. | [[34.0, 'year']] | F | {'11568396': 1, '16383252': 1, '12517623': 1, '14618409': 1, '25466279': 1, '15602364': 1, '19837020': 1, '7620358': 1, '19119938': 1, '31538247': 1, '31528428': 2, '18206241': 1, '10973414': 1, '1407429': 1, '15494114': 1, '17644919': 1, '3889701': 1, '15292638': 1, '1407428': 1, '15587620': 1, '20179945': 1, '15292632': 1, '10869139': 1, '20593997': 1, '14749200': 1, '24294465': 2} | {'6744800-1': 1} |
164,368 | 3841480-1 | 24,327,970 | noncomm/PMC003xxxxxx/PMC3841480.xml | Management of sizeable carotid body tumor: Case report and review of literature | A 40-year-old Yemini woman was admitted to King Fahd Hospital of University, Alkhobar, Saudi Arabia with a progressively growing mass in the left anterolateral side of her neck of 5 years duration. She presented with neck tenderness, dizziness, increasing number of fainting attacks, and shortness of breath. There was no history of hoarseness, dysphagia, or palpitation and no family history of a similar condition. Her left pupil was slightly more contracted than the right pupil (Horner's syndrome). The rest of her neurologic examination was unremarkable. Indirect laryngoscopy revealed slightly impaired movement of left vocal cord. Contrast enhanced computed tomography (CECT) scan of the neck with subsequent CT angiograms of the carotids showed a 6.9 × 5 × 5.8 cm hypervascular solid left carotid space mass encasing the common carotid artery (CCA) and both internal CA (ICA) and external CA (ECA) and splaying the carotid bifurcation with no luminal narrowing or thrombosis [Figures and ]. There was no contralateral tumor. The tumor was diagnosed as carotid body paraganglioma of Shamblin group III. Horner's syndrome was due to tumor encasement of the left sympathetic chain. No preoperative embolization was performed. The tumor was surgically removed under locoregional anesthesia and supplemented with intravenous propofol after reconstruction of the CA. The hypoglossal and vagus nerves were identified during surgery and preserved. Part of CCA, ICA, and ECA were removed with tumor and ICA was reconstructed with polytetrafluroethylene (PTFE) graft [Figures and ]. Monitoring of the neurological status was achieved by simple clinical evaluation of the state of consciousness under local anesthesia, and by continuous regional oxygen saturation (rSO2), measured with a cerebral oximeter (Somanetics Invos cerebral oximeter). It was not necessary to place a shunt. Blood loss was about 450 ml. Postoperatively, a transient swallowing difficulty and tongue deviation developed probably due to stretching of the hypoglossal nerve during the operation. On laryngoscopic examination, left-sided vocal cord paralysis was aggravated. There was no cerebrovascular event after surgery. Histopathology examination confirmed the preoperative diagnosis of paraganglioma []. Six months after surgery, the patient could speak and swallow normally and her tongue deviation disappeared. Graft surveillance at 9 months revealed a patent graft with normal flow. | [[40.0, 'year']] | F | {'10964291': 1, '17670685': 1, '19097531': 1, '23336858': 1, '20607078': 1, '12001071': 1, '19125710': 1, '9685134': 1, '18797418': 1, '11957547': 1, '5127724': 1, '29259765': 2, '23141675': 1, '14999807': 1, '17171183': 1, '17400487': 1, '21844248': 1, '22111087': 2, '29084935': 1, '24327970': 2} | {'5717474-1': 1, '3219857-1': 1} |
164,369 | 3841495-1 | 24,339,644 | noncomm/PMC003xxxxxx/PMC3841495.xml | Cerebral infarction leading to hemiplegia: A rare complication of acute pancreatitis | A 48-year-old male, heavy alcohol consumer for the prior 6 years, presented with complaints of severe abdominal pain, vomiting and constipation for 3 days. There was no history of diabetes mellitus, hypertension, smoking, and previous abdominal pain. On admission to our hospital (on day 4 of illness), he was hemodynamically stable, had abdominal distention with diffuse tenderness and absent bowel sounds. Laboratory work up on day 4 revealed serum amylase: 898 U/l, lipase: 968 U/l, glucose: 116 mg/dl, Na+: 132 mEq/l, K+: 3.6 mEq/l, creatinine: 1.9 mg%, calcium: 8.6 mg/dl, bilirubin: 1.4 mg/dl, aspartate transaminase/alanine transaminase: 74/28 IU/l, alkaline phosphatase: 181 IU/l, serum proteins/albumin: 7.1/2.9 g/dl, serum cholesterol: 124 mg/dl, triglycerides: 130 mg/dl, Hb: 8.4 g/dl, leucocytes: 15,400/μl, platelets: 98,000/μl, prothrombin time: 18 s, activated partial thromboplastin time: 40 s. Computed tomography (CT) of abdomen [] carried out with intravenous contrast on day 4 revealed enlarged, diffusely enhancing pancreas, multiple peripancreatic fluid collections and bilateral pleural effusions, confirming grade E AP (CT severity index = 4). He was kept nil per orally. Continuous nasogastric aspiration, intravenous fluids, stress ulcer prophylaxis and subcutaneous unfractionated heparin 5000 units twice/day for venous thromboprophylaxis were started. On day 5, he developed right sided hemiplegia. CT of brain revealed large left MCA territory infarct [Figures and ]. Electrocardiogram (ECG) and echocardiography of the heart were normal. Work up for hyperhomocysteinemia, protein C and S deficiency, factor V Leiden, antithrombin III deficiency and antiphospholipid antibody syndrome was negative. Patient developed high grade fever and progressive acute renal failure from day 6. He was started on piperacillin + tazobactam and metronidazole. He was intubated on day 8 for progressive respiratory distress and hypoxia and was shifted to intensive care unit for mechanical ventilation. On day 9, he developed shock and was discharged against medical advice upon insistence of his relatives. | [[48.0, 'year']] | M | {'18648144': 1, '15365199': 1, '9308332': 1, '32393730': 1, '29875900': 1, '7930445': 1, '15380848': 1, '3387968': 1, '22347150': 1, '12853683': 1, '22081920': 1, '12218335': 1, '24339644': 2} | {} |
164,370 | 3841496-1 | 24,339,645 | noncomm/PMC003xxxxxx/PMC3841496.xml | Fatal overdose of iron tablets in adults | A 25-year-old lady allegedly consumed 200 tablets of ferrous sulphate with suicidal intent. The total dose amounted to 13.5 g of elemental iron (270 mg/kg). She had abdominal pain with vomiting and was given gastric lavage along with supportive care at a primary health center. She was referred to our hospital after 24 h of ingestion for further management. At presentation, she was hemodynamically stable with a normal pulse rate and blood pressure. Complete blood count (CBC) showed hemoglobin-14.1 g/dl, total white blood cell (WBC) count-19,300/cu mm (90% neutrophils) and platelet count-283,000/cu mm. Serum iron level as measured by spectrophotometric analysis was 73 μg/dl (normal range 40-145 μg/dl) and the total iron binding capacity was 350 g/dl (normal range 250-350 μg/dl). Liver function tests (LFT) showed marked elevation in liver enzymes (serum glutamic oxaloacetic transaminase [SGOT]-11,000 U/L and serum glutamic pyruvic transaminase [SGPT]-11,420 U/L), total bilirubin (2.6 mg%), direct bilirubin (1.1 mg%), protein (4.6 g%), albumin (2.8 g%) and alkaline phosphatase (161 U/L). Serum creatinine was 1.9 mg%, bicarbonate was 15 mmol/l and blood sugar level was 81 mg/dl. Chelation therapy with desferrioxamine was started as an intravenous infusion of 15 mg/kg/h and increased to 25 mg/kg/h. 24 h after admission, she developed severe gastro-intestinal (GI) bleeding and went into a state of circulatory shock. Hemoglobin dropped from 14.1 to 7 gm%. Activated partial thromboplastin time (aPTT) was 71.3 s and prothrombin time (PT) was 24.5 with an international normalized ratio (INR) of 1.9. Arterial blood gas analysis showed severe metabolic acidosis with a pH of 6.96. She became progressively drowsy with un-recordable blood pressure, for which she was intubated and ventilated and supported with multiple inotropes. Aggressive blood product supports were given and hemodialysis was initiated for metabolic acidosis. Despite these measures, she continued to deteriorate and died within 36 h of admission due to multi-organ failure. | [[25.0, 'year']] | F | {'30598567': 2, '7599407': 1, '30218980': 1, '6151497': 1, '11778670': 1, '3669120': 1, '26600863': 1, '2197323': 1, '16323571': 1, '12824662': 1, '24339645': 2} | {'3841496-2': 2, '6259445-1': 1} |
164,371 | 3841496-2 | 24,339,645 | noncomm/PMC003xxxxxx/PMC3841496.xml | Fatal overdose of iron tablets in adults | A 17-year-old lady allegedly consumed 300 tablets of ferrous sulfate amounting to a total dose of 20 g of elemental iron (400 mg/kg). She was initially taken to a local hospital with abdominal pain and vomiting and gastric lavage given. Further treatment details are unknown. After 48 h, she developed multiple episodes of hematemesis and melena and progressively became oliguric and drowsy. She was intubated and referred to our hospital 48 h after ingestion. At presentation, pulse and blood pressure were not recordable and resuscitated with colloids. CBC profile showed hemoglobin-2.8 g/dl, total WBC count-4,500/cu mm, platelet count-52,000/cu mm. LFT showed marked elevation in liver enzymes (SGOT-4,250 U/L and SGPT-3,908 U/L), total bilirubin (0.7 mg%), direct bilirubin (0.5 mg%), protein (2.2 g%), albumin (0.7 g%) and alkaline phosphatase (72 U/L). Serum iron levels were not measured as the patient presented more than 48 hours after ingestion. Serum creatinine was 2.4 mg%, bicarbonate was 10 mmol/l, blood sugar level was 27 mg/dl, aPTT was >3 min and PT was >2 min with an INR of >10. Aggressive supportive management along with desferrioxamine as a 15 mg/kg/h infusion was initiated. Hemodialysis was planned, but could not be instituted as the patient had refractory hypotension and had a cardiac arrest within 2 hours of presentation. Despite ventilator and inotropic supports, she continued to deteriorate with circulatory collapse and could not be resuscitated. She died within 3 h of presentation to our hospital. | [[17.0, 'year']] | F | {'30598567': 2, '7599407': 1, '30218980': 1, '6151497': 1, '11778670': 1, '3669120': 1, '26600863': 1, '2197323': 1, '16323571': 1, '12824662': 1, '24339645': 2} | {'3841496-1': 2, '6259445-1': 1} |
164,372 | 3841497-1 | 24,339,646 | noncomm/PMC003xxxxxx/PMC3841497.xml | A case of pulmonary alveolar proteinosis treated with whole lung lavage | A 54-year-old male, non-smoker, non-alcoholic, known diabetic and hypertensive, presented with fever, dry cough, and progressive breathlessness for one month. Initial evaluation in a different hospital confirmed hypoxia and PFT (pulmonary function tests) was suggestive of a restrictive pattern. A CT chest demonstrated characteristic crazy-paving pattern. []. Sputum for PAS was negative. He was labeled as having interstitial lung disease and was discharged on oxygen and steroids.\nHis symptoms worsened gradually and he presented to our Institution on high flow oxygen and was found to be PaO2-42 on FIO2 of 1. There was no history suggestive of a connective tissue disorder. On examination, he had bilateral basal crepitations on the chest. Patient was admitted in ICU, blood gases showed type I respiratory failure with a paO2 of 53 mm of Hg on FIO2 of 0.6. Rest of the routine investigations were normal. The chest X-ray [] showed bilateral reticular shadows affecting the lower zones (left > right). Bronchoscopy with lavage was done; the patient did not consent to lung biopsy. BAL microbiology was negative. After counseling, a repeat bronchoscopy with TBLB was done, which showed pulmonary alveolar proteinosis on histopathology (PAS stain was strongly positive) []. As the patient was deteriorating, it was decided to treat him with whole lung lavage (WLL).\nPatient was intubated with a double-lumen endotracheal tube for WLL. The left lung was lavaged first with 15.5 L of warmed (at 37°C) normal saline using aliquots of 500 mL. The procedure was terminated once the effluent had cleared significantly. The effluent fluid was collected in sequentially numbered glass bottles []. It had characteristic opaque, milky appearance with dense sediments on standing, which were strongly positive for PAS stain.\nPatient's oxygen requirement decreased to 2-3 liters/min post-extubation. ABG showed pH-7.44, PaO2-62 on FIO2 of 0.4, and PaCO2- 44. CXR showed marked clearing of left side []. Subsequently, the procedure was repeated on the right lung with 20 liters saline. Patient had marked improvement symptomatically and objectively. His PaO2 on room air was 68 mm of Hg. Repeat HRCT showed marked clearance. Blood samples were sent to University of Cleveland (USA) for anti-GM-CSF antibody titers, which were reported to be highly positive, so recombinant GM-CSF was started. The patient was discharged on N-acytyl cystine nebulization and rGM-CSF therapy for 3 months. His chest X-ray [] after 8 months was almost normal. His oxygen saturation on room air was found to be 92%, and he was carrying out his routine activities including work without getting breathless. | [[54.0, 'year']] | M | {'27890991': 2, '10731299': 1, '9041997': 1, '14695413': 1, '28031836': 1, '9042125': 1, '21632797': 1, '13552931': 1, '11179134': 1, '6879484': 1, '10189465': 1, '27186004': 2, '12119235': 1, '24339646': 2} | {'5112819-1': 1, '4857576-1': 1} |
164,373 | 3841498-1 | 24,339,647 | noncomm/PMC003xxxxxx/PMC3841498.xml | Posterior reversible encephalopathy syndrome-an under recognized manifestation of Chronic Kidney Disease | A 17-year-old female was admitted for living donor (LD) RTx. Her earlier medical history included renal biopsy proven malignant hypertension, and end stage renal disease (ESRD). She was on maximum dosage of three types of antihypertensive drugs [nifedipine, clonidine, metoprolol] to control blood pressure. She was on regular hemodyalisis of 4 hrs duration two to three times per week since three months.\nShe developed headaches, altered consciousness, visual disturbances and seizures. A seizure was the presenting manifestation. Seizures were generalized tonic clonic leading to status epilepticus. It was succeeded by visual blurring. The headache was typically constant, nonlocalized, moderate to severe, and unresponsive to analgesia. Altered consciousness ranged from mild somnolence to confusion and agitation, progressing to stupor/coma. The deep tendon reflexes were brisk with Babinski signs present. The patient had weakness and incoordination of the limbs. No other focal neurologic deficits were present. Physical assessment revealed blood pressure of 210/110 mmHg, temperature 38.3°C, respiratory rate of 30 breaths per minute, heart rate of 100 beats per minute.\nArterial blood gas showed PaO2 137 mm of Hg, PCO2 43 mm of Hg, pH 7.37 and bicarbonate 24.6 mmol/L, potassium 4.3 mmol/L, sodium 143 mmol/L, choride 103 mmol/L, anion gap 19 mmol/l, calcium 1.12 mmol/l, glucose 108 mg/dl, lactic acid 3.4 mmol/l.\nThe fundoscopic examination revealed hypertensive retinopathy without papilledema. A non contrast brain computed tomography revealed bilateral symmetrical white matter hypodensity in the parietal, temporal, and occipital regions suggesting PRES []. No other abnormalities were noted. There was no history of cytotoxic immunosuppressive therapy or eclampsia. Laboratory investigations revealed hemoglobin, 9.2 gm/L; total white cell count, 5.65 × 103/μl (differential count: 55% neutrophils, 42% lymphocytes, 2% monocytes, and 1% eosinophils); platelet count, 1.5 × 105/μl; serum creatinine (SCr), 7.2 mg/dl; blood urea, 92 mg/dL; serum calcium, 8.7 mg/dl; serum phosphorus, 5.7 mg/dl; serum magnesium, 2 mg/l; alanine aminotransferase, 34 units/l (normal range: 0-40 units/l); aspartate aminotransferase, 30 units/l (normal range: 5-34 units/l); serum bilirubin, 1 mg/dl; and serum albumin, 3.8 gm/dl. Multiple blood, urine, and sputum cultures were sterile. With control of hypertension, the patient improved dramatically.\nIntravenous lorazepam was given for acute control of seizures and she went on to require phenytoin, valproic acid and levetiracetam. She was started on more frequent HD. She required five different types of antihypertensive drugs to control blood pressure. Initially she was started on intravenous nitroprusside and nytroglycerine to rapidly control blood pressure. Cardiac monitoring, frequent measurement of blood pressure, and pulse oximetry were instituted. She recovered clinically within one week. The resolution of findings on neuroimaging occurred gradually. Subsequently, she underwent successful LD RTx without recurrence of PRES. | [[17.0, 'year']] | F | {'21043390': 1, '20450101': 1, '18661248': 1, '20630137': 1, '8559202': 1, '20979955': 1, '22453710': 1, '21556718': 1, '22390203': 1, '21898031': 1, '24339647': 2} | {} |
164,374 | 3841499-1 | 24,339,648 | noncomm/PMC003xxxxxx/PMC3841499.xml | Neem oil poisoning: Case report of an adult with toxic encephalopathy | A 73-year-old man with the medical history of diabetes mellitus type 2 and psoriasis presented to the emergency department with complaints of vomiting and drowsiness, 1 hour after accidental ingestion of 20 ml of Neem oil. The patient's current medication included human Mixtard insulin (30/70), 16 U before breakfast and 10 U before dinner.\nAt presentation, the patient was drowsy with Glasgow coma scale of 13/15. His pulse rate was 110/min, respiratory rate 28/min, oxygen saturation of 95% at room air, and blood pressure 150/90. Physical examination was unremarkable. Systemic examination showed: bilateral equal and reacting pupils with no meningeal signs and no focal neurological deficits. Respiratory, Cardiovascular and abdominal examination were unremarkable.\nIn the emergency department, the patient developed generalized convulsions with loss of consciousness. He was intubated and managed initially with intravenous lorazepam. In the intensive care unit, he was managed with insulin and symptomatic treatment.\nInvestigations at presentation showed: Hemoglobin 13.3 gm/dl, leukocyte leukocyte count 16800/μl, red blood cell count 4.41 million/cmm, platelet count 375000/μl, blood sugar 298 mg/dl, serum osmolality 277 mosmol/kg, and normal serum lactate level. An arterial blood gas analysis showed metabolic acidosis: pH 7.34, pO2 81, pCO2 24, HCO3 16, and pO2/FiO2 385. He had normal urine analysis, serum electrolytes, liver, renal, and thyroid function. MRI scans of brain showed chronic ischemic changes due to small vessel disease. ECG and radiograph of the chest were normal.\nInvestigations repeated on day 2, 3, and on discharge showed: Normal electrolytes, arterial blood gas, liver, and renal function. During the course of illness, he remained drowsy for 4 days and recovered without any complications. He was discharged after 1 week. | [[73.0, 'year']] | M | {'10836141': 1, '6110100': 1, '34693242': 1, '32581815': 1, '2259944': 1, '20448012': 1, '33841143': 1, '18250509': 1, '20932102': 1, '7141505': 1, '24339648': 2} | {} |
164,375 | 3841515-1 | 24,339,525 | noncomm/PMC003xxxxxx/PMC3841515.xml | Successful renal transplantation from a brain-dead deceased donor with head injury, disseminated intravascular coagulation and deranged renal functions | A 19-year-old male patient with head injury following a road traffic accident was brought in emergency to a private trauma care hospital. On admission, he was found to be comatose, had fractured right tibia and left femur. On day of admission, his renal function and liver function tests were normal with serum creatinine (SCr), 1.40 mg/dL. However, he had deranged coagulation profile with activated partial thromboplastin time: 32.7 s, (control: 27.9 s), Prothrombin time: 20 s (control: 10.6 s) and international normalized ratio was 1.89. His hemoglobin was 7.21 g/dL, total leukocyte count 1.52 × 103/μl and platelet count was 2.08 × 105/ μl. He had hypotension and fall in hemoglobin level; hence, he was transfused 4 units of whole blood and started on vasopressors dopamine at 10 μg/kg/min and noradrenaline at 10 μg/ min. Blood pressure was maintained around 110-120 systolic and 80-90 mm of Hg diastolic. On 2nd day, he developed extensive petechiae and purpura all over the body and oozing of blood through wounds hence 6 units of fresh frozen plasma were transfused. However, he further deteriorated with increase in purpuric spots, further derangement in coagulation profile, decreasing platelet count and Hemoglobin level, low Serum fibrinogen level and D-DIMER >4000 ng/ml suggestive of extensive DIC. Detaillaboratory parameters of the donor are shown in . Computed tomography brain was suggestive of changes of diffuse hypoxia. Electroencephalogram was suggestive of alpha alpha coma. Patient was declared brain-dead by neurophysician and neurosurgeon at the interval of 6 h. Ultrasonography of the abdomen showed normal sized kidneys. Blood and urine culture were sterile. The relatives were explained about organ donation. Since, they agreed he was shifted to our institute. His renal function was deranged with SCr, 3.57 mg/dL and blood urea 92 mg/dL. His hemoglobin had dropped to 7.3 g/ dL and platelet count was 5.5 × 104/μl. Blood pressure was 100/70 mmHg on dopamine and noradrenalin support. Both kidneys were harvested and transplanted in two recipients with favorable complement-dependent lymphocytotoxicity cross-match.\nRecipient 1-was a 25-year-old male on maintenance hemodialysis for, 55 months. He received induction therapy with rabbit anti-thymocyte globulin (r-ATG) 1.5 mg/kg and 3 doses of methyl prednisolone 500 mg/ day. His hematological, coagulation and renal function profile remained within normal range throughout the post-operative period and follow-up period of 1 month. SCR normalized on 5th post-operative day to 1.22 mg/ dL and he was discharged on 7th post-operative day with SCR, 1.18 mg/dL on maintenance immunosuppression of Tacrolimus 0.08 mg/day, Mycophenolate sodium 720 mg twice daily and Prednisolone, 20 mg/day. There was no evidence of graft dysfunction throughout the follow-up period of 1 month.\nRecipient 2 was a 30-year-old female with post-partum cortical necrosis. She was on maintenance hemodialysis since 44 months. She received the same induction and maintenance immunosuppression therapy as recipient 1. On first post-operative day, her platelet count decreased from 1.44 × 105/μl to 9.5 × 104/μl. However, it recovered gradually and normalized on 4th post-operative day without any treatment. Coagulation profile performed performed on 1st and 4th post-operative day was normal and there was no evidence of bleeding diathesis. SCR normalized on 8th post-operative day to 1.27 mg/dL. She was discharged on 12th post-operative day with SCR of 0.87 mg/dL with same maintenance immunosuppression as that of recipient 1. Demographic profile and laboratory parameters of both recipients are shown in . Sr. Tacrolimus level of both transplant recipients in immediate post-transplant period and throughout the follow-up period of 1 month was maintained in the range of 7-10 ng/ml. | [[19.0, 'year']] | M | {'4554877': 1, '17175278': 1, '17235277': 1, '19857287': 1, '22390203': 1, '15992668': 1, '10451465': 1, '21355766': 1, '331129': 1, '9474270': 1, '4809569': 1, '4604477': 1, '8434402': 1, '2658065': 1, '12811244': 1, '1539297': 1, '3529530': 1, '13402886': 1, '21372214': 1, '14576251': 1, '25061532': 2, '5065327': 1, '13394690': 1, '21427617': 1, '6472592': 1, '2206888': 1, '2656108': 1, '9665067': 1, '4572392': 1, '24339525': 2} | {'4100278-1': 1} |
164,376 | 3841516-1 | 24,339,526 | noncomm/PMC003xxxxxx/PMC3841516.xml | Distal renal tubular acidosis and amelogenesis imperfecta: A rare association | A 10-year-old female, resident of Tamil Nadu born of second degree consanguineous marriage was admitted with inability to move all 4 limbs since early morning. The weakness had evolved over a period of 20 days initially presenting as calf muscle pain, progressing to difficulty in getting up from bed, and eventually involving all four limbs.\nThere was no prior history of fever, loose stools, vomiting, trauma or drug intake. No significant past medical history could be elicited except for polyuria and polydipsia for last 1 month. Antenatal, perinatal, and development history was unremarkable. There was no family history of periodic paralysis, cardiac, thyroid, renal or auto immune disorders. On examination, child had flaccid areflexic quadriparesis with power 2/5 in all limbs and no muscle wasting. Her higher mental functions, cranial nerves, sensory system and autonomic system examination were normal. Systemic examination was unremarkable. Her vitals were within normal range. Serum level of potassium was 1.5 meq/l and electrocardiogram showed prolonged QT interval, ST depression, T wave flattening in V1-V4, and the presence of ‘U’ waves. Child was given potassium replacement therapy and recovered from paralysis.\nShe had severe growth retardation with a weight of 15 kg and height of 110 cm (both below 5th centile). Intraoral examination revealed poor oral hygiene; the dentition was consistent with the chronological age. Enamel was hypoplastic with brownish orange discoloration and irregular mottling of occlusal surface of molar teeth. Based on the history, clinical and radiographic presentation provisional diagnosis of AI, hypoplastic type was made []. Routine lab investigations including renal and liver function tests were within normal range. Serum electrolytes showed hypocalcemia (7.2 mg/dl), hypophosphatemia (2.8 mg/dl) with normal serum magnesium and sodium. Her arterial blood gas revealed normal anion gap metabolic acidosis with respiratory alkalosis []. Thyroid and lipid profile were normal. Urine culture and sensitivity showed no growth and urine metabolic screening were negative. There was no glycosuria or proteinuria. The urinary potassium level was 24 meq/l, which indicates renal potassium wasting (exceeding 20 meq/l).\nIn view of persisting metabolic acidosis with normal anion gap, hypokalemia, and polyuria (2.5 l/m2/24 h) a possibility of distal RTA was considered, which was confirmed by necessary investigations. Three consecutive early morning urine pH was 7.5 and the urine pH after frusemide challenge was 7.5. Urine anion gap was positive and urinary calcium/creatinine ratio was 2. 24 h urinary calcium was 280 mg (increased), fractional excretion of bicarbonate was 4.5% and urine blood pCO2 difference was 4 mmHg (decreased). The ultrasound scan of the abdomen revealed bilateral renomegaly with increased cortical echoes. There was no evidence of nephrocalcinosis. Skeletal survey did not show any evidence of rickets. She was investigated for secondary causes of RTA and all investigations including her antinuclear antibody and auto immune profile were negative.\nThe child was managed symptomatically with potassium replacement therapy initially intravenously followed by oral supplementation with correction of acidosis and electrolyte abnormalities during the ICU stay and upon the diagnosis of distal RTA was started on long- term oral potassium citrate supplementation. The patient is currently under follow-up and her weakness and polyuria have improved dramatically. | [[10.0, 'year']] | F | {'7600222': 1, '28553046': 2, '31959358': 1, '12617253': 1, '9870488': 1, '21069676': 1, '4812435': 1, '15891923': 1, '11225229': 1, '4504766': 1, '30779877': 1, '4894504': 1, '18085734': 1, '3872071': 1, '6597532': 1, '12597691': 1, '16304440': 1, '17944757': 1, '30199559': 2, '44068': 1, '17410104': 1, '25097344': 1, '24339526': 2} | {'6788841-1': 1, '5434692-1': 1} |
164,377 | 3841517-1 | 24,339,527 | noncomm/PMC003xxxxxx/PMC3841517.xml | Chronic type B aortic dissection in association with Hemolyticuremic syndrome in a child | A 9-year-old male child weighing 23.5 kg was admitted in December 2012 with complains of vomiting, oliguria, edema face and pallor for 4 days. There was no history of fever, diarrhea and frank hematuria, pain in abdomen, pain in chest, dyspnea, dysuria or joint pain. Child had a history of similar complains in February 2012 when he was admitted in our institute and was diagnosed to have hemolytic uremic syndrome (HUS). He had undergone six sessions of plasma exchanges and alternate day hemodialysis (HD) during his stay in hospital. He was discharged on continuous ambulatory peritoneal dialysis (CAPD) with tablet nifedipine- retard 10 mg twice-a-day, tablet clonidine (0.1 mg) half-tablet twice-a-day and tablet sodium bicarbonate (500 mg) twice-a-day. In May 2012, his CAPD catheter was removed when his urine output was increased to 1.5 L/day and he was maintaining serum creatinine of 0.6 mg/day. He was continued on anti-hypertensive medication and he was on monthly follow-up evaluation.\nOn examination, child was well-built, well-nourished, intelligent with normal skin texture without any bruises on joints, having a height of 130 cm, upper segment 66 cm, lower segment 64 cm with an arm span of 126 cm without any abnormal phenotypic feature. His pulse was 100 beat/minhaving bilateral symmetry, blood pressure 200/100 mm of Hg in both upper limb in a sitting position and 206/100 mm of Hg in both lower limbs in lying down position. Femoral, popliteal and dorsalis pedis arteries were equally palpable on both sides. There were no parasternal murmurs on auscultation. Respiratory system was normal on examination and fundus examination was normal. On investigation, urine examination showed albumin +2, sugar nil, 30-40 dysmorphic red blood cells/high power field, hemoglobin 13.1 g/dL, platelet-100,000/cm, peripheral smear showed many schistocytes suggestive of hemolysis. Direct Coomb's test was negative. Blood urea was 154 mg/dL, serum creatinine-5.36 mg/dL, lactate dehydrogenase (LDH) 1317 U/L, high-sensitivity C-reactive protein 2.7 μg/mL (reference range 1-3 μg/mL), serum sodium 138 meq/L, serum potassium 4.8 meq/L, serum C3 level 68 mg/dL (reference level 90-207 mg/dL) serum C4 level 30 mg/dL (reference level 17.4-52.4 mg/dL), ultrasound abdomen showed right kidney 8.4 cm × 3.1 cm and left kidney 8.7 cm × 3.1 cm with increased echogenicity without stone or hydronephrosis. Right internal double lumen catheter (DLC) was inserted for management and X-ray chest was taken to confirm the position of DLC, which showed widening of the mediastinum and well-defined soft-tissue opacity in left paratracheal and left paracardiac region and shadow of aortic knob and descending aorta was not visualized. Contour of right cardiac border was normal and catheter tip was in the right atrium. X-ray chest of previous admission was brought out and it was showing normal cardiac shadow []. Transthoracic echocardiography showed normal aortic valve, ascending aorta and cardiac chambers. Doppler of the abdominal aorta and computed tomography with contrast (CT angio) showed dissection of aorta arising distal to the left subclavian artery and extending up to bifurcation of the aorta with a diameter of descending thoracic aorta 4 cm and abdominal aorta 38 mm with central patent lumen of 2.4 cm and intimal flap within with false lumen diameter of 0.4 cm with partial peripheral thrombus in true lumen [Figures and ]. CT angio showed two left renal arteries and one right renal artery of normal caliber without any aortic branch vessel involvement []. As the child is asymptomatic and has type B dissection of the aorta with a diameter of less than 4 cm, he was started on conservative management with beta blocker and other anti-hypertensive medications along with CAPD and other supportive medications on surveillance monitoring. For his relapse of HUS child was given alternate day hemodialysis and five sessions of plasma exchange each of 1L volume when his platelet count reached 320,000/cu mm and LDH 240 U/L. His oliguria persisted to 300 mL/day and serum creatinine remained 4.28 mg/dL. Renal biopsy was performed, which showed acute on chronic HUS []. CAPD catheter was again inserted and peritoneal dialysis cycles were restarted and his anti-hypertensive requirement included tablet atenolol (50 mg/day), tablet minoxidil (2.5 mg twice a day), tablet prazosin (slow release) (5 mg/day), tablet losartan (50 mg twice a day) and he is maintaining blood pressure of 110/70 mm of Hg at the time of discharge. His father was trained to measure blood pressure at home and keeps a record of same and he has been informed about warning symptoms and child is prohibited from weight lifting. | [[9.0, 'year']] | M | {'18083364': 1, '5458238': 1, '20971366': 1, '6702637': 1, '9164331': 1, '12900496': 1, '384280': 1, '10807810': 1, '10685714': 1, '3772661': 1, '22236596': 1, '17101856': 1, '26550312': 1, '831420': 1, '11511117': 1, '21144696': 1, '7261533': 1, '24339527': 2} | {} |
164,378 | 3841535-1 | 24,339,661 | noncomm/PMC003xxxxxx/PMC3841535.xml | Unusual causes of Hematuria in dialysis patients: Diagnostic dilemma, risks and management | A 63-year-old man suffering from ESRD on maintenance HD for the past 2 years, was admitted with right flank pain and intermittent hematuria for one month. Previous ultrasound showed bilaterally small kidneys, each measuring about 6 cm. There was a fall of hemoglobin of 3 g/dL with development of postural symptoms and he also noticed a new onset mass in the right upper abdomen. Patient had a session of HD about a week back. On examination, he was pale, heart rate was 90/min, respiratory rate 18/min and his blood pressure was 130/90 mmHg. Abdomen was soft, non-tender, a 10 × 12 cm hard palpable lump was occupying the right hypochondrium and lumbar regions. There was no free fluid in the abdomen. Abdominal ultrasound revealed heteroechoic mass in the right kidney with multiple septations. Abdominal CT scan revealed a 10 × 10.3 × 11 cm well-defined predominantly hypodense mass with mean density of 19 HU, replacing the upper and mid-pole of the right kidney. There was peripheral wall enhancement with solid enhancing component within it (56 HU). Multiple enhancing septae were seen within it. Right renal vein and inferior venacava were normal []. Blood investigation revealed hemoglobin of 8.3 g/dL, hematocrit 28%, platelets 1,24000 /μL, INR 1.06, albumin 3.8, urea 73mg/dL, creatinine 8.4 mg/dL and serum potassium of 6.2 mEq/dl. The patient received 2 units of packed red blood cells during hemodialysis. On considering all available reports, a diagnosis of right renal mass, probably of renal cell carcinoma was made. He underwent open right radical nephrectomy and gross specimen revealed a solid renal mass []. On cutting the specimen whole pelvicalyceal system (PCS) was filled with blood clots [] and no growth was detected in PCS []. A detailed histopathological evaluation revealed changes of chronic glomerulonephritis with no evidence of malignancy, vascular malformations or PAN. Postoperatively, the patient is on HD and awaiting renal transplantation. | [[63.0, 'year']] | M | {'11427653': 1, '835942': 1, '1899744': 1, '3537463': 1, '10992385': 1, '11257671': 1, '12814684': 1, '3941443': 1, '3493660': 1, '8740036': 1, '24339653': 1, '3339720': 1, '11912370': 1, '25536394': 1, '24339661': 2} | {'3841535-2': 2} |
164,379 | 3841535-2 | 24,339,661 | noncomm/PMC003xxxxxx/PMC3841535.xml | Unusual causes of Hematuria in dialysis patients: Diagnostic dilemma, risks and management | A 50-year-old male with ESRD, on hemodialysis since 14 months, awaiting renal transplantation presented with complaints of progressively increasing acute left flank pain, and was referred to the emergency from another centre. During evaluation for the cause of chronic kidney disease, bilateral shrunken kidneys were noted. No biopsy was performed and patient was declared ESRD and initiated on maintenance HD. There was no history of hematuria. This patient was hypertensive and diabetic for more than 10 years. Hemoglobin fell by 4 g% inspite of 6 units of blood transfusion. He presented to the emergency department with features of shock. On evaluation, patient was pale with a blood pressure of 86/60mm of Hg. Platelet count was 2.85/μL and coagulation parameters were normal. After fluid resuscitation and stabilization, CT scan revealed large perirenal hematoma with compressed atrophic kidney []. Pain and lump gradually increased with progressive fall in hemoglobin, which led to exploration and nephrectomy with removal of perirenal hematoma. Operative finding revealed a large perirenal hematoma with atrophic kidney []. On gross examination of the specimen, outer surface showed scars and focal areas of granularity. On cut-section cortico-medullary junction was indisntinct and PCS was filled with blood clots. Section of the kidney examined showed globally and focally sclerosed glomeruli with atrophic tubules. Interstitium was studded with lymphocytic infiltrates at places. No ulceration or granulomas were noted. | [[50.0, 'year']] | M | {'11427653': 1, '835942': 1, '1899744': 1, '3537463': 1, '10992385': 1, '11257671': 1, '12814684': 1, '3941443': 1, '3493660': 1, '8740036': 1, '24339653': 1, '3339720': 1, '11912370': 1, '25536394': 1, '24339661': 2} | {'3841535-1': 2} |
164,380 | 3841536-1 | 24,339,662 | noncomm/PMC003xxxxxx/PMC3841536.xml | Pulmonary embolism: An abdominal pain masquerader | A 48-year-old male presented to the emergency department with a 1-year history of intermittent right upper quadrant abdominal pain. He had been seen by his primary care physician 1 week earlier for the same pain and had an ultrasound performed. The patient stated he was unaware of the results and that today the pain became acutely worse. The patient denied any other associated symptoms. He denied any personal history or family history of medical problems. The patient was a smoker, drank up to a six-pack of beer daily, and used marijuana. He did not take any medications.\nThe vital signs were as follows: Blood pressure 112/52, pulse 82, respiratory rate 16, oxygen saturation 99% on room air. He was afebrile. Physical exam revealed a well appearing male in moderate distress secondary to pain. His abdominal examination was remarkable for severe right upper quadrant pain to palpation without guarding or rebound. There was no costovertebral angle tenderness. His lung sounds were clear bilaterally. The patient remarked that the abdominal pain was made worse with deep inspiration. His cardiac exam was normal.\nLaboratory analysis revealed a normal complete blood count, complete metabolic profile, urinalysis, and lipase. A right upper quadrant ultrasound examination was performed, which demonstrated a positive sonographic Murphy's sign and slight dilation of the common bile duct at 0.66 cm [Figure and ]. A chest radiograph was significant for a right lower lobe infiltrate in the lung periphery []. At this time, it was felt that this could represent a Hampton's hump; however, the patient had a low pre-test probability for having a PE. A d-dimer test was added and was positive (1.0 mg/mL fibrinogen equivalent units, normal <0.05) at which point a computed tomography angiogram (CTA) of the chest was performed. On CT a large central PE was identified in the right main pulmonary artery [Figure and ]. The right lower lung lobe contained a peripheral based wedge-shaped opacity consistent with pulmonary infarct []. Anticoagulation was started in the emergency department and the patient was admitted. A formal echo done during the patient's admission showed a small atrial septal defect with left to right shunting that did not require closure. No other etiology for thromboembolism was identified. | [[48.0, 'year']] | M | {'34600641': 1, '13459102': 1, '22934143': 1, '26495343': 2, '31130721': 1, '1909617': 1, '17904458': 1, '27390523': 1, '8885008': 1, '10715347': 1, '32789064': 2, '30083629': 2, '15726055': 1, '14615562': 1, '31689854': 2, '24339662': 2} | {'4614616-1': 1, '6075479-1': 1, '7417130-1': 1, '6946195-1': 1} |
164,381 | 3841537-1 | 24,339,663 | noncomm/PMC003xxxxxx/PMC3841537.xml | Post-partum sequential occurrence of two diverse transfusion reactions (transfusion associated circulatory overload and transfusion related acute lung injury) | A 29-year-old female suffered post-partum hemorrhage following a normal vaginal delivery after, which she was referred to our institute on the second post-partum day. She had no co morbidities. On admission to the high dependency unit, she was in shock (blood pressure [BP] 80/50 mm Hg), had tachycardia (pulse rate 120/min), was restless and had severe pallor. Her baseline hemogram results were — hemoglobin 4 g/dl, Leucocyte count of 7800/ml and platelet count of 88 × 103/ml. Abdominal ultrasound was suggestive of placenta accreta. Immediate resuscitation commenced under anesthetist's supervision and packed red blood cells (PRBC's), fresh frozen plasma (FFP), platelet concentrates, and cryoprecipitate were arranged. Under ultrasound guidance and local anesthesia left internal jugular vein (for central line) and left radial artery (for arterial line) were cannulated. When conservative measures such as blood product transfusion (2 units PRBC and FFP each), uterine massage, administration of oxytocin, methylergometrine, prostaglandin and balloon tamponade failed to control the hemorrhage, the patient was rushed to the operation theater for an emergency hysterectomy. During the surgery, the patient lost around 1500 ml of blood for which she was transfused 3 units each of PRBC, FFP and platelets along with 1500 ml of hydroxyethyl starch over 3 h. Intraoperative central venous pressure (CVP) was maintained between 8 mm Hg and 10 mm Hg. She also required ionotropic support (dopamine at the rate of 8 μg/kg/min) transiently. At the conclusion of surgery, arterial blood gases (ABG) showed metabolic acidosis. (pH: 7.20, P/F ratio: 310, HCO3: 12, BE: −6.5), but ionotropic support could be tapered off. The patient was shifted to the intensive care unit (ICU) for elective mechanical ventilation. Within an hour of ICU admission, the patient developed an elevated CVP (14 mm Hg), jugular venous distension, and bilateral crepitations. Urgent work-up revealed hypoxemia (PO2: 70 mm Hg on FiO2 of 0.4) and cardiomegaly and lung congestion on chest X-ray []. Ultrasound chest showed B lines []. Electrocardiography and Troponin T levels were within normal limits. Echocardiography carried out promptly, showed a dilated left ventricle (left ventricular end diastolic diameter: 6 cm) with an ejection fraction of 50%. Plasma B-type natriuretic peptide (BNP) levels was measured, which was found elevated (255 pg/dL). A presumptive diagnosis of TACO was made and ventilator support was augmented by increasing the oxygen concentration (FiO2: 0.6), Positive End Expiratory Pressure (PEEP) (8 cm H2O), and pressure support (17 cm H2O). Diuretic therapy (furosemide 40 mg intravenous loading dose followed by 20 mg intravenously 8 h) was also initiated. Repeat chest X-ray after 24 h showed a reduction in cardiomegaly and clearing of congestion. Subsequently, her condition resolved and she could be extubated on the 2nd day of ICU stay. The next 2 days were uneventful with the patient receiving supportive therapy and making a gradual recovery. Her investigations and clinical features were within normal limits during this period. On the 3rd day from the event, oozing was observed from her central line insertion site, which prompted an emergency coagulogram. Her investigations revealed a platelet count of 115 × 103/ml, prothrombin time of 13 s, fibrinogen level of 252 mg/dl and an elevated activated partial thromboplastin time of 46 s. For correction of the same, she received 4 units of FFP over the next 5 h. Within 2 h of the last transfusion, she developed breathlessness, tachypnea and bilateral crepitations at the lung bases. At this point, the CVP measured was 5 mm Hg. Considering an allergic reaction, she was immediately administered steroids and antihistaminics, but was not relieved. An urgent chest X-ray revealed new onset bilateral pulmonary infiltrates []. ABG showed hypoxemia (PaO2 of 58.5 on FiO2-.35). A plasma sample for BNP was sent, which resulted in a value of 80 pg/dL. Echocardiography showed an ejection fraction of 55% without any valvular defects (left ventricular end diastolic diameter: 4.8 cm). Computed tomography angiography ruled out embolic phenomenon. As no other causes of acute lung injury (ALI) could be elicited, TRALI was considered a diagnosis of exclusion and the sample was sent back to the blood bank informing about the event with requisitioning for donor human leucocyte antigen (HLA) antibody testing. Ventilatory support was initiated (non-invasive ventilation) with the following settings: Mode Continous Positive Airway Pressure (CPAP), pressure support-16 cm H2O, PEEP-8 cm H2O and FiO2-0.5. After receiving ventilator support, the patient became comfortable. Repeat ABG's showed an improving trend and the ventilator support could be discontinued by the next 24 h. After this episode, further complications were not observed and the patient was discharged from the ICU on the 7th day of her surgery. A week later, the result of donor plasma came out to be positive for HLA antibody. | [[29.0, 'year']] | F | {'15818095': 1, '15248168': 1, '11136955': 1, '19224781': 1, '17626910': 1, '21512622': 1, '9588412': 1, '17676434': 1, '15987348': 1, '9973048': 1, '15209881': 1, '15584994': 1, '3380985': 1, '24339663': 2} | {} |
164,382 | 3841538-1 | 24,339,664 | noncomm/PMC003xxxxxx/PMC3841538.xml | A case of emphysematous pyelonephritis induced by Citrobacter freundii infection | A 79-year-old female with diabetes mellitus and hypertension had general fatigue, high fever and vomiting, was transferred to our hospital. She was experiencing a state of systemic inflammatory response syndrome and had lower abdominal tenderness. A computed tomography (CT) scan for detection of the septic focus suggested acute emphysematous pyelonephritis (EPN) []. She was treated conservatively, however, her condition did not improve, so an urgent right nephrectomy was performed on the 2nd hospital day. The results of the blood and urine culture showed the presence of Citrobacter freundii infection. The patient's post-operative course was uneventful and discharged on foot.\nThis case is the second reported case of EPN induced by C. freundii.[] Bacteremia induced by C. freundii infection typically results in a high mortality rate. In this case, early diagnosis of EPN using CT and immediate medical treatment, including urgent elective nephrectomy, was key to the favorable outcome. | [[79.0, 'year']] | F | {'18353396': 1, '20840327': 1, '20814181': 1, '8766145': 1, '24339664': 2} | {} |
164,383 | 3841539-1 | 24,339,665 | noncomm/PMC003xxxxxx/PMC3841539.xml | Timely management of penetrating neck trauma: Report of three cases | A 20-year-old male was admitted to our emergency room in respiratory distress. He had been stabbed in the neck in a fight. A laceration in the submental area had been sutured at another hospital. After being followed for 1 hour in the first hospital, he was discharged. Subsequently, he developed swelling in the submental region and then progressively worsening respiratory distress. Indirect laryngoscopy showed a mass in the vallecula, and his vocal cords could not be seen clearly. There was an approximately 7-cm-long horizontal skin incision in the submental area. Neck ultrasonography showed a hematoma in the submental region. Under orotracheal general anesthesia, we opened the sutures and extended the incision by 1 cm at both ends. We drained the hematoma and saw that a branch of the lingual artery had been cut. We ligated the bleeding vessel and followed the incision to the right vallecula. On direct laryngoscopy, we saw a small laceration on the right anterior vallecula surface and no other pathology. We used a Hemovac tube for drainage. Then, we sutured the cut muscles, platysma, and cutaneous tissue on both sides. He was extubated without any problem and did not need a tracheotomy. | [[20.0, 'year']] | M | {'1637536': 1, '11308235': 1, '8782468': 1, '2027695': 1, '14106707': 1, '21103111': 1, '15891890': 1, '20009310': 2, '19213388': 1, '8298918': 1, '7288808': 1, '28754171': 2, '11571970': 1, '1795619': 1, '20145502': 1, '22945428': 1, '22111076': 2, '24339665': 2} | {'3841539-2': 2, '3841539-3': 2, '2776368-1': 1, '3214866-1': 1, '3214866-2': 1, '5534056-1': 1} |
164,384 | 3841539-2 | 24,339,665 | noncomm/PMC003xxxxxx/PMC3841539.xml | Timely management of penetrating neck trauma: Report of three cases | A 53-year-old male was admitted to our emergency room with a laceration in the left neck. The laceration started in the sternocleidomastoid muscle, a third of the way anterior to the midpoint and continued to the anterior neck midline horizontally []. The injury occurred when he was being shaved by barber who had epilepsy and had a seizure while he was shaving him. When we examined the patient initially in the emergency room, he was not in respiratory distress. The flexible laryngoscopy examination was normal. His hemodynamic condition was stable. Doppler ultrasonography did not show any pathology of the vascular system. Under orotracheal intubation, we extended the wound at both ends by 1 cm. We saw that the lower third of the sternocleidomastoid muscle, thyrohyoid muscle, and omohyoid muscle had been cut horizontally. The anterior jugular vein was also cut. We stopped the bleeding with ligation and sutured the ends of the sternocleidomastoid, thyrohyoid, and omohyoid muscles together. We used a Hemovac tube for drainage. He was extubated without any problem and did not need a tracheotomy. | [[53.0, 'year']] | M | {'1637536': 1, '11308235': 1, '8782468': 1, '2027695': 1, '14106707': 1, '21103111': 1, '15891890': 1, '20009310': 2, '19213388': 1, '8298918': 1, '7288808': 1, '28754171': 2, '11571970': 1, '1795619': 1, '20145502': 1, '22945428': 1, '22111076': 2, '24339665': 2} | {'3841539-1': 2, '3841539-3': 2, '2776368-1': 1, '3214866-1': 1, '3214866-2': 1, '5534056-1': 1} |
164,385 | 3841539-3 | 24,339,665 | noncomm/PMC003xxxxxx/PMC3841539.xml | Timely management of penetrating neck trauma: Report of three cases | A 53-year-old male was admitted to our emergency room with a deep stab wound in his neck after attempting suicide. The wound started to the left of the medial head of the sternocleidomastoid muscle and continued horizontally to the right of the medial head of the right sternocleidomastoid []. The laceration was at the level of the lower third of the thyroid cartilage. The wound continued deep into the larynx. The thyroid cartilage was cut horizontally and away from the supraglottic region, and the damage continued to the hypopharynx and ended anterior to the prevertebral mucosa []. When initially examined in the emergency room, he was in moderate respiratory distress. There was no massive bleeding. His larynx was obviously badly damaged. Shortly after admission, he developed respiratory distress and started bleeding, preventing a radiological examination. We did not attempt orotracheal or nasotracheal intubation because of the badly damaged larynx. Under local anesthesia, we made a tracheotomy and then induced general anesthesia. Using his self-inflicted wound, when we dissected the neck, we saw that the thyroid ala, both laryngeal ventricles, and the lower third of the epiglottis were cut horizontally. The supraglottic and infraglottic regions were separated horizontally. We consulted a vascular surgeon in the operating room. The patient has no significant carotid or venous injuries, although there were some minor venous injuries. We reconstructed all of the damaged structures and sutured the sinus piriformis mucosa, epiglottis petiole, and thyroid cartilage alae. We used Prolene sutures to reconstruct the epiglottic and thyroid cartilages. After 5 days’ follow-up, he was transferred to the psychiatric ward. We closed his tracheotomy after 3 months. He has not had any voice, swallowing, or breathing problems since his tracheotomy was closed. There is some residual damage to the epiglottis on the right side [].\nWe gave all patients second-generation antibiotics for surgical prophylaxis. After 5 days’ follow-up, the first and second patients were discharged without any complications. | [[53.0, 'year']] | M | {'1637536': 1, '11308235': 1, '8782468': 1, '2027695': 1, '14106707': 1, '21103111': 1, '15891890': 1, '20009310': 2, '19213388': 1, '8298918': 1, '7288808': 1, '28754171': 2, '11571970': 1, '1795619': 1, '20145502': 1, '22945428': 1, '22111076': 2, '24339665': 2} | {'3841539-1': 2, '3841539-2': 2, '2776368-1': 1, '3214866-1': 1, '3214866-2': 1, '5534056-1': 1} |
164,386 | 3841542-1 | 24,339,668 | noncomm/PMC003xxxxxx/PMC3841542.xml | An unusual stab wound to the buttock | A 47-year-old diabetic man had a stab wound to his right buttock inflicted by his roommate using a kitchen knife. On examination, his pulse was 75 beats/min and his blood pressure (BP) was 100/50 mmHg. The abdomen was soft and not tender. A single wound was seen on the medial side of the right gluteal area superior to the intertrochanteric plane []. No neurological deficits were detected. Arterial pulses of the lower limbs were well-felt. The wound was sutured in the Emergency Department and patient was scheduled for discharge. Patient felt dizzy, complained of fresh bleeding per rectum and fainted before being sent home. At this stage, his pulse rate was 105 beats/min and his BP had dropped to 65/40 mmHg. Patient was resuscitated with crystalloids and he became hemodynamically stable. Rigid sigmoidoscopic examination was performed under general anesthesia and showed blood clots in the rectum with no active bleeding. No obvious injury to the rectal wall was seen despite the injection of methylene blue through the gluteal wound. Patient was admitted to the surgical ward for close observation. He was kept fasting, had intravenous fluids and antibiotics were administered. Two days later, patient complained of lower abdominal pain. Fecal matter passed through the gluteal wound. Patient had a temperature of 38°C. There was tenderness and guarding in the lower abdomen. Abdominal computed tomography (CT) scan with oral, rectal and intravenous contrast was performed. It showed contrast leakage from the posterior wall of the rectum, intraperitoneal free air and air in the mesentery around the rectum [Figures and ]. Exploratory laparotomy was performed. No intraperitoneal fecal soiling was seen. Nevertheless, there was a significant amount of air in the retroperitoneal space and in the bowel mesentery []. The rectum was mobilized, but the extraperitoneal perforation could not be seen. Hartmann's procedure was performed. Gluteal wound exploration revealed a deep laceration lateral to the edge of the sacrum. Debridement of the necrotic tissue was performed. Post-operatively, patient had slow recovery because of intestinal ileus. The colostomy started to function on the fourth post-operative day. Patient recovered completely at day 10 and was scheduled for reversal of the Hartmann's procedure 12 weeks after surgery. He decided to travel overseas to his home country to continue his treatment. | [[47.0, 'year']] | M | {'1558492': 1, '11303168': 1, '18308283': 1, '17033545': 1, '19152899': 1, '20628100': 1, '15589933': 1, '7108989': 1, '21995834': 1, '29066647': 1, '3175731': 1, '9783600': 1, '17901288': 1, '24339668': 2} | {} |
164,387 | 3841544-1 | 24,339,670 | noncomm/PMC003xxxxxx/PMC3841544.xml | Urgent-setting magnetic resonance imaging allows triage of extensive penoscrotal hematoma following blunt trauma | A healthy 40-year-old male arrived to Emergency Department after a bicycle fall. At physical examination, the most prominent finding was a painful, extensive ecchymosis and hematoma involving the penis and ventral aspect of the scrotum. The patient urinated without difficulty, macroscopic hematuria was absent and urinalysis excluded significant abnormalities.\nPlain radiographs of the pelvic skeleton (not shown) did not disclose bony fractures. In consideration of the association of an atypical history of trauma on a flaccid penis associated with extensive regional hematoma and marked tenderness, the attending Urologist requested emergency magnetic resonance imaging (MRI) to be performed, including focused multiplanar T1-, STIR and T2-weighted sequences acquired with the penis in a neutral position []. A diffuse, subcircumferential T1- and T2-hyperintense subacute hematoma involved the superficial skin and subcutaneous planes of the entire penis, perineal region and anterior aspect of the scrotum. The low-signal tunica albuginea was recognizable along the penile shaft, without appreciable discontinuities suggesting a possible penile fracture. No signs of hematoma were noted in the spongiosum and cavernosal bodies. The testes (as confirmed by ultrasound, not shown) showed normal size and shape, homogeneous signal intensities and good visualization of their tunica albuginea. The absence of signs of penile and testicular rupture led to conservative treatment option, with resolution of clinical abnormalities after two weeks and no functional complaints. | [[40.0, 'year']] | M | {'18936025': 1, '10992029': 1, '11598264': 1, '18480487': 1, '11868085': 1, '16132913': 1, '17374858': 1, '24339670': 2} | {} |
164,388 | 3841562-1 | 24,339,550 | noncomm/PMC003xxxxxx/PMC3841562.xml | Mosaic double aneuploidy: Down syndrome and XYY | Patient was a 17-month-old boy with growth retardation and developmental delay, the third child of non-consanguineous parents. The father was 37-year-old and the mother was 31-year-old at conception. The antenatal period was uneventful. The child weighed 2.2 kg at birth. The two older siblings were normal and there was no significant family history.\nClinical examination revealed an interactive and social child. His height at admission was 76 cm and weight, 8.65 kg, which were within the normal limits for Down syndrome charts. The phenotype was consistent with Down syndrome with flat facies, hypertelorism, mongoloid slant of the eyes, epicanthal folds and flat nasal bridge, low-set posteriorly placed ears, small abnormal pinnae, short neck and increased sandal gap. Vision and hearing screening tests were normal. He had hypotonia with exaggerated deep tendon reflexes, hyperextensible elbows, knees and fingers and a flexible calcaneovalgus deformity. He was able to sit with support, grasp objects, recognize parents and vocalize. Neurodevelopmental assessment revealed a developmental age of 7 months. There were no other significant physical findings. Blood investigations including thyroid screening were normal.\nConventional cytogenetic analysis of phytohemagglutinin-stimulated peripheral blood showed trisomy 21 in all 25 metaphases. Six metaphases showed double aneuploidy with an associated XYY [], which was confirmed by fluorescence in situ hybridization (FISH) analysis [] using the centromeric probes for chromosomes X and Y (Abott Molecular, Abbott Park, IL, USA). The karyotype of the child was 47, XY,+21[19]/48, XYY,+21[6]; in situ hybridization XYY (DXZ1 × 1, DYZ1 × 2). Parental karyotypes were not available. | [[17.0, 'month']] | M | {'13642856': 1, '6419709': 1, '10545600': 1, '155969': 1, '24339550': 2} | {} |
164,389 | 3841563-1 | 24,339,551 | noncomm/PMC003xxxxxx/PMC3841563.xml | Poland syndrome a rare congenital anomaly | An eight-year-old girl first seen at the Paediatric Out-Patient Department of Aminu Kano Teaching Hospital, Kano, with left anterior chest wall deformity since birth and recurrent loss of consciousness of two years duration. There was no history of breathlessness, paroxysmal nocturnal dyspnea or orthopnea. About a year ago, she developed a swelling on the left side of the jaw which was incised by a traditional barber and a thick whitish secretion was drained. However since then mother noticed that the site had been discharging clear fluid intermittently whenever She laughed, coughed, ate or cried. There was history of syncopal attacks lasting for few minutes noticed whenever she was carried on the back and the left side of the chest is compressed and also during play when she is being hit or pushed on the chest. She was a product of full-term gestation and pregnancy was not supervised. The pregnancy and delivery were not adversely eventful. The child was the fourth of the mother's five children in a polygamous family setting of a non-consanguineous marriage. There was no family history of congenital anomalies.\nExamination findings revealed a girl with normal growth parameters and good mental status. She had an actively discharging tract at the region of the angle of the left mandible which was about 2 cm below the left earlobe []. A rudimentary accessory nipple was seen on the left side with depression of the left hemithorax. Pectoralis major muscle, the third, fourth and fifth ribs were absent on the left side []; the left scapula was hypoplastic and displaced upward [], but the left latissimus dorsi was well preserved. No limbs abnormalities were seen. She had normal cardiovascular examination findings.\nShe had a chest radiograph which showed absence of the left 3rd, 4th and 5th ribs []. Abdominal ultrasound, electrocardiogram and echocardiogram were normal. A diagnosis of Poland syndrome, esophago-cutaneous fistula and recurrent cardiac syncope was made. The patient and parents were counseled, and she is awaiting reconstructive surgery for the chest defect. | [[8.0, 'year']] | F | {'3008556': 1, '18582821': 1, '10823612': 1, '16532987': 1, '19169181': 1, '9632447': 1, '15759092': 1, '20927162': 1, '19533787': 1, '2560355': 1, '17030703': 1, '9409872': 1, '19183988': 1, '24339551': 2} | {} |
164,390 | 3841565-1 | 24,339,553 | noncomm/PMC003xxxxxx/PMC3841565.xml | Genetic analysis of a family with complete androgen insensitivity syndrome | A 10-year-old girl, product of consanguineous marriage was referred for evaluation of palpable gonads in the inguinal region. She was born at term after an uneventful pregnancy by normal vaginal delivery with a birth weight of 2.6 kg and length of 48 cm. Bilateral inguinal gonads with short and blind ended vagina were detected at birth. She was followed-up over a period of 8 months when assignment of the female gender was decided; corrective procedure for inguinal gonads was planned, but did not occur. On physical examination, she had typical female external genitalia. Palpable gonads were found bilaterally in the inguinal region [], pubertal stage was B4P5. The uterus was absent under pelvic sonograms. Hormonal evaluation revealed follicle stimulating hormone (5.4 mIU/mL; normal: 1.5-12.4), luteinizing hormone (21.2 mIU/mL; Normal: 1.7-8.6) and total testosterone (15 ng/mL; Normal: 2.86-8.1); chromosomal analysis showed diploid 46 XY karyotype. Testosterone and DHT synthesis defects were excluded by the normal rise of T and DHT after human chorionic gonadotrophin (HCG) stimulation (basal T: 15 ng/mL; basal DHT: 9.3 ng/mL, T/DHT ratio after HCG stimulation: 7:9). Gonadectomy was performed a few months later and histological analysis revealed bilateral testes with no evidence of malignancy. She subsequently underwent vaginoplasty and received therapy with estrogens. Three maternal aunts presented with primary amenorrhea with an adequate breast and pubic hair development and palpable gonads in the age group between 15 and 20 years and were treated with bilateral gonadectomy, vaginal reconstruction and estrogen supplementation.\nPeripheral blood samples were obtained from the girl and her maternal family members for molecular analysis. Genomic DNA was extracted with the use of polymerase chain reaction (PCR) amplification of AR exonic fragments 1-8 followed by direct sequencing analysis of the PCR products was performed. It revealed C 2754 to T transition in exon 6 []. The same mutation was confirmed in her mother and the 3 maternal aunts [], maternal grandmother []. Her mother and maternal grandmother were fertile carriers with 46 XX karyotype. | [[10.0, 'year']] | F | {'15146455': 1, '10995865': 1, '2626022': 1, '1752359': 1, '15724799': 1, '17334586': 1, '28670533': 1, '16888681': 1, '9245853': 1, '17161333': 1, '24339553': 2} | {} |
164,391 | 3841566-1 | 24,339,554 | noncomm/PMC003xxxxxx/PMC3841566.xml | Type 2 diabetes mellitus: An unusual association with Down's syndrome | A 28-year-old male patient with a body mass index (BMI) of 25.1 kg/m2 and positive family history of DM reported to the out-patient clinic of the department of Endocrinology, Medwin hospital with the clinical features of polyuria and polydipsia for last 1 year. There was no history of weight loss or any significant past medical history. Physical examination revealed typical mongolian facies and other features of DS such as short stature (145 cm), brachycephaly, short neck and pot belly, small mouth with protruding tongue, wide occipital region and characteristic small eyes of DS. Thyroid was not palpable. Laboratory data revealed diabetes with fasting plasma glucose of 256 mg/dl, postprandial plasma glucose of 375 mg/dl and glycated hemoglobin (HbA1C) level of 9.9%. Urine was negative for ketone bodies. C-peptide assay showed fairly well-preserved pancreatic β cell function: Fasting values of 1.3 pmol and stimulated values of 2.4 pmol/ml (normal values in non-diabetic subjects, fasting >1.5 pmol/ml and stimulated >4.0 pmol/ml). Glutamic acid decarboxylase (GAD) antibody testing (3.2 IU/ml) was negative. These values were suggestive of type 2 DM. He also had primary hypothyroidism with a thyroid stimulating hormone level of 54 mIU/l and T4 value of 3.5 μg/dl. Other investigations (hemogram, renal and liver function parameters, serum electrolytes and lipid profile) were within the normal limits. He was initiated on basal glargine insulin at a dose of 15 units subcutaneously at night along with oral hypoglycemic agent (glimepiride 2 mg/metformin 1000 mg) once daily and L-thyroxine 100 μg daily. Later, the doses of insulin were reduced and subsequently withdrawn completely and he is maintained well on oral hypoglycemic agents alone. | [[28.0, 'year']] | M | {'11578024': 1, '28461836': 1, '28130199': 1, '9507919': 1, '16575558': 1, '12637977': 1, '15823385': 1, '2526634': 1, '23014256': 1, '23153973': 1, '9875020': 1, '15963228': 1, '9301372': 1, '31019277': 1, '24339554': 2} | {'3841566-2': 2} |
164,392 | 3841566-2 | 24,339,554 | noncomm/PMC003xxxxxx/PMC3841566.xml | Type 2 diabetes mellitus: An unusual association with Down's syndrome | A 26-year-old female patient with a BMI of 33.4 kg/m2 with strong family history of DM were admitted in the in-patient Department of Endocrinology, Medwin Hospital for proper control of hyperglycemia. She gave a history of polyuria, but denied any weight loss. Physical examination revealed features of DS (short stature, obesity, brachycephaly, gynecomastia, protruding tongue, simian crease, pot belly, short neck and acanthosis nigricans, wide occipital region with characteristic small eyes). The laboratory evaluation revealed fasting and postprandial sugar levels as 175 mg/dl and 240 mg/dl, respectively HbA1c of 8.9%. Urine was negative for ketones. His fasting and stimulated C-peptide levels were 1.4 pmol/ml and 2.5 pmol/ml with negative GAD-antibody test (4.1 IU/ml). Other investigations were within normal limits. Glycemic control was achieved with gliclazide 40 mg with metformin 500 mg twice daily. | [[26.0, 'year']] | F | {'11578024': 1, '28461836': 1, '28130199': 1, '9507919': 1, '16575558': 1, '12637977': 1, '15823385': 1, '2526634': 1, '23014256': 1, '23153973': 1, '9875020': 1, '15963228': 1, '9301372': 1, '31019277': 1, '24339554': 2} | {'3841566-1': 2} |
164,393 | 3841567-1 | 24,339,555 | noncomm/PMC003xxxxxx/PMC3841567.xml | A case of primary amenorrhea with 46+XY genotype from Kashmir Valley | A 19-year-old female student residing in central urban district of Kashmir was referred to our genetic counseling center by the local Gynecologist. The patient had been diagnosed with failure to reach menarche and PA. Her height was 5.8 ft and weight 54 kg. Secondary sexual characters of the patients were also under developed, with breast buds instead of well-developed ones. Interview with the patient revealed the female gentile organs. Furthermore, patient was a product of consanguineous marriage – both parents were paternally related []. The patient was the first among four children arisen from the marriage and only one to survive the gestation period prior to three consecutive abortions. The facial features of the patient were similar to those of male individuals with high rise brows, prominent fore head and jaw line. The patient was advised to undergo regular ultrasonographic (USG) examination. USG of the patient revealed the complete absence of the uterus and small atretic ovaries []. It also revealed mullarian agenesis. The hormonal profile of the patient revealed the lower levels of follicle-stimulating hormone (15.15 mIU/ml), but elevated levels of luteinizing hormone and prolactin 33.71 mIU/ml and 5.6 mIU/ml respectively. Later on estradiol of the patient was found to be <12.5 ng/ml, which is in the same range as in menopausal adult. Our genetic counselors diagnosed the patient with possible turner syndrome clinically and advised cytogenetic analysis.\nWe carried out the cytogenetic evaluation of the patient for the karyotyping to detect the possible chromosomal abnormality. Cytogenetic analysis was carried out for patient as well as parents using blood as the source of chromosomes. Whole blood was cultured in 5 ml of Roswell Park Memorial Institute 1640 medium with 10% fetal calf serum. Lymphocytes were stimulated by adding phytohemagglutinin. Cultures were incubated at 37°C for 48-72 h. The cell proliferation was arrested by colchine/colcemid, slides were prepared after hypotonic treatment and fixation with Carnoy's fixative. G-banding was done with trypsin and metaphase plates were scored and karyotyped using an image analysis system. The chromosomal evaluation revealed the female to possess only one X chromosome instead if two and the presence of one Y chromosome. Thus, genotype of the patient was a confirmed male one [Figures and ]. | [[19.0, 'year']] | F | {'3105755': 1, '16669559': 1, '2255934': 1, '31007464': 1, '24339555': 2} | {} |
164,394 | 3841568-1 | 24,339,556 | noncomm/PMC003xxxxxx/PMC3841568.xml | Hypoparathyroidism-retardation-dysmorphism syndrome | A 13-year-old Hindu male child 4th born to a consanguineously married couple with uneventful perinatal event, having delayed social and personal development was brought with spasms of the upper and lower limbs. He has been suffering from similar episodes of spasms frequently since the age of 5 years. He also had developmental bilateral cataract for which he was operated at the age of 6 years. At admission, he had carpopedal spasm and his vitals were normal. His weight was 29 kg (<5th centile), height 132 cm (<5th centile) and head circumference 49 cm (<3rd centile). On examination, he had facial dysmorphism features like long face, prominent forehead, bilateral dropping of eyelids, preauricular tag on the right side, bilateral pseudophakia with posterior capsular opacification (secondary), beaked nose, depressed nasal bridge, long philtrum, high arched palate, maloccluded teeth, micrognathia and microcephaly []. Latent tetany signs Trousseau sign and chvostek sign were positive. Intelligent quotient assessment revealed mild mental retardation. Other systems examinations were normal. One of his sisters also had operated for developmental cataract at the age of 4 years, having delayed social and personal development, similar facial dysmorphism [] but no tetany or latent tetany signs. Both parents and other two sibs were phenotypically normal.\nHis investigations revealed normal blood counts and peripheral smear. His serum calcium was 6.8 mg/dl, serum phosphorus 7.1 mg/dl, serum magnesium 2.2 mg/dl, serum albumin 3.2 g/dl, alkaline phosphatase 124 U/L and intact PTH was 5.46 pg/ml (N = 15-65 pg/ml). Blood urea, blood sugar, serum creatinine and electrolytes were normal. Urine chromatography was normal and urinary pH 8. His urine for sugar and ketones was absent. Chest X-ray, humerus X-ray and ultrasound abdomen was normal. Computerized tomographic (CT) scan of the head showed bilateral globus pallidus calcification. Audiogram showed mild hearing loss on the left side with normal hearing on the right side. He was started on intravenous calcium gluconate slow infusion for 2 days followed by calcium tablets. His facial dysmorphic sister serum calcium, phosphorus, magnesium, albumin, alkaline phosphatase, intact PTH and CT head were normal. | [[13.0, 'year']] | M | {'15645691': 1, '15005702': 1, '9634513': 1, '12389028': 1, '19554981': 1, '20653155': 1, '31857291': 1, '10691411': 1, '17257873': 1, '24339556': 2} | {} |
164,395 | 3841569-1 | 24,339,557 | noncomm/PMC003xxxxxx/PMC3841569.xml | A novel ABCB11 mutation in an Iranian girl with progressive familial intrahepatic cholestasis | A nine-year-old girl with cirrhosis was referred for genetic counseling. The patient was affected by icterus and pruritus from newborn period. Her brother and sister displayed the same symptoms; they died at the age of 1.5 and 7 years, respectively. Her parents had a non-consanguinity marriage. The patient's serum biochemical analysis indicated high in total and direct bilirubin and S.G.P.T (ALT). The copper level was raised in serum biochemical result. In CT scan examination, liver and gallbladder had normal sizes but spleen was significantly enlarged. Microscopic examination from a liver biopsy specimen showed significant pathological characterizations of cholestasis. Hepatocytes indicated extensive pseudo acinar transformation; ballooning degeneration, cholestasis and bile plague. Portal tracts contain moderate lymphocytic infiltration and also necrosis in focal confluent. Fibrosis expansion of portal tracts with marked bridging and focal bile duct damage were observed. From the pathological examination of the hepatocytes and clinical demonstrations, the changes were compatible with progressive familial intra-hepatic cholestasis type 1 or 2. The liver biopsy result from the expired sister had the same pathological characteristics of the proband and ended with cirrhosis.\nThe diagnosis of the kind of PFIC can be difficult because PFIC type 1 has initial clinical and laboratory findings similar to those of PFIC type 2.[] Pathological features may differ. In PFIC type 1 bland canalicular cholestasis with variable fibrosis is found. In PFIC type 2, variable features include canalicular cholestasis and a neonatal hepatitis pattern, with hepatocellular swelling and giant cell transformation.[] Immune staining is a useful diagnostic tool for PFIC type 2 since most patients with ABCB11 mutations and hepatobiliary disease of onset in infancy have no canalicular BSEP expression.[] So pathological study was performed on proband. Hepatocellular swelling with multinucleated canalicular and hepatocellular cholestasis, portal lymphocytic infiltration and fibrosis, newly formed liver nodules, bridging necrosis, bile duct proliferation, giant cells and micro vesicular fat droplet can be seen in proband liver biopsy []. | [[9.0, 'year']] | F | {'12717091': 1, '18376240': 1, '18692205': 1, '19750581': 1, '15975683': 1, '18395098': 1, '19133130': 1, '11815775': 1, '7912266': 1, '15946126': 1, '24339557': 2} | {} |
164,396 | 3841570-1 | 24,339,558 | noncomm/PMC003xxxxxx/PMC3841570.xml | Genetic mutations in Gorlin-Goltz syndrome | A 12-year-old female patient was referred to dental clinic with a chief complaint of swelling on the left side of the upper jaw since 4 months by a local medical practitioner. Patient was concerned about the facial deformity and denied any other associated symptoms. Patient's medical, family, dental and personal history was noncontributory.\nOn general physical examination, the patient was moderately built and nourished, presenting with normal gait and satisfactory vital signs. There were multiple palmar pits brownish black in color present on the palms of both her hands []. There was a diffuse swelling in the left middle third of the face with no secondary changes noted over it. On palpation, there was local rise in temperature; the swelling was tender and soft in consistency. A solitary left submandibular lymph node was palpable, tender, soft in consistency and mobile.\nThere was a swelling in the left buccal vestibule causing vestibular obliteration in region of 22 and 63 extending distally unto 26. Mucosa over the swelling showed no secondary changes. On palpation the swelling was tender and soft in consistency with areas of decortication. Aspiration yielded thin straw-colored fluid.\nBased on the history and clinical findings, a provisional diagnosis of dentigerous cyst in relation to 63 was given and a differential diagnosis of odontogenic keratocyst considered. The patients was subjected to the following radiographic examination. Intraoral periapical radiograph in the region of 22, 63 and 26 showed a well-defined radiolucency with sclerotic borders in the periapical region extending from 22 to 26. The radiolucency was not associated with impacted teeth. The radiograph also showed missing 23, 24, 25. Anterior maxillary occlusal radiograph showed similar well-defined radiolucency in the region of 22, 63, 26 [].\nOrthopantomograph revealed multiple, unilocular well-defined radiolucencies with sclerotic borders located in maxilla and mandible []. The presence of multiple cysts in the jaws, associated with unerupted teeth, raised a suspicion of Gorlin syndrome and other relevant investigations were done. The patient was then evaluated systemically for other anomalies of the skeletal, cardiovascular or central nervous system. Ophthalmologic examination incidentally revealed Bitot spots in both the eyes. An incisional biopsy of the swelling in left side of maxilla was advised. Histopathological examination of specimen revealed stratified squamous parakeratinized epithelium with palisading pattern of columnar cells along with keratin flakes suggestive of odontogenic keratocyst []. Since the criteria of multiple cysts in the jaws (one of them being odontogenic keratocyst), multiple palmar pits, were present, a final diagnosis of Gorlin syndrome was given. | [[12.0, 'year']] | F | {'26604574': 1, '2442330': 1, '8665318': 1, '11987666': 1, '34703144': 2, '15050066': 1, '10332131': 1, '16419085': 1, '28951717': 1, '25610614': 2, '24339558': 2} | {'8491327-1': 1, '8491327-2': 1, '4299365-1': 1} |
164,397 | 3841592-1 | 24,339,571 | noncomm/PMC003xxxxxx/PMC3841592.xml | Cerebral revascularization with superficial temporal – middle cerebral artery anastomosis for complete carotid occlusion: An emerging modality for preventing recurrent stroke | A 60-year-old man presented with recurrent episodes of transient ischemic attacks (TIA) in the form of weakness involving the left side of the face, upper limb and lower limb since 3 months. He had recently been detected to be hypertensive and was on antihypertensive medications. He had no other risk factors for stroke (diabetes, hypercholesterolemia, smoking, alcoholism, hyperhomocystenemia). He continued to have episodes of hemodynamic TIA despite best medical management. These were not related to exertion or valsalva maneuver and were thought to be postural, related to hemodynamic factors. While under evaluation in the hospital, he developed a major stroke involving the left upper and lower limbs from which he recovered only partially (power 3/5).\nRoutine blood investigations were normal. Computed tomography (CT) brain revealed multiple infarcts in the right cerebral hemisphere []. Echocardiogram and electrocardiography were normal. Color Doppler scan of the carotid arteries revealed total occlusion of the right ICA. This was confirmed with CT angiogram (CTA) and MR angiogram (MRA). DSA revealed total occlusion of the right ICA at its origin. There was poor collateral flow to the right hemisphere from the left ICA (across the anterior communicating artery) and from vertebrobasilar system due to an incomplete circle of Willis. There was no evidence of thrombus or embolus in any IC vessel []. This led to an inadequate perfusion of the right cerebral hemisphere resulting in recurrent TIAs. After the major stroke in the hospital, a repeat CT brain revealed a right middle cerebral artery (MCA) territory infarct. Single Photon Emission CT (SPECT) CT of the brain also revealed ischemia of the entire right cerebral hemisphere.\nIt was decided that medical management alone would not be sufficient to improve the cerebral vascularity of the right cerebral hemisphere and prevent further ischemic events. Hence a decision was made to revascularize the right cerebral hemisphere by performing a bypass procedure from superficial temporal artery (STA) to the MCA. Pre-operative trans-cranial Doppler (TCD) could not pick up any flow in the MCA. The STA was localized using Doppler and superficial temporal-MCA (ST-MCA) anastomosis performed in the standard fashion [Figures and ].\nPost-operative recovery was uneventful. Aspirin was continued throughout the peri-operative period to prevent graft thrombosis. Post-operative CT and MRA and DSA confirmed good flow across the graft []. Post-operative TCD was not performed so as not to apply any pressure over the anastomotic area that would lead to thrombosis of the anastomosis. He did not develop any further episodes of TIA and was discharged after 10 days of hospital stay. At 2 months follow-up, he was asymptomatic and was improving from his previous deficits with grade 4/5 power in the left upper and lower limbs and was ambulant. SPECT is planned at the 6 monthly follow-up visit. | [[60.0, 'year']] | M | {'7191494': 1, '7349004': 1, '15678866': 1, '5462239': 1, '11134377': 1, '15232124': 1, '11872890': 1, '508123': 1, '3929672': 1, '7745417': 1, '10408660': 1, '15767504': 1, '2406995': 1, '3982632': 1, '24339571': 2} | {} |
164,398 | 3841593-1 | 24,339,572 | noncomm/PMC003xxxxxx/PMC3841593.xml | Hemorrhage and ischemia in different hemispheres in a child with moyamoya disease: Case report and review of literature | A 6-year-old female presented with sudden onset of weakness of the right side of the body, along with headache. Prior to this, she was asymptomatic, and had normal developmental milestones. On examination, she was conscious, alert and oriented to time, place and person; and had right hemiplegia. Computed tomography (CT) scan of the head revealed hemorrhage in the thalamus, posterior limb of the internal capsule on the left side, without any intraventricular extension []. Magnetic resonance imaging (MRI) brain revealed a small hematoma in the left thalamus, posterior limb of the internal capsule, without any intraventricular extension. Magnetic resonance angiogram revealed occlusion of bilateral supraclinoid ICA, with multiple basal leptomeningeal collaterals. There were no infarcts on diffusion-weighted MRI; however, there were multiple small hyperintensities on T2W1 in the white matter in the fronto-parietal region []. Patient was evaluated with a digital subtraction angiogram, which revealed occlusion of bilateral ICAs, along with leptomeningeal collaterals, suggestive of Suzuki stage 4 MMD []. There were pial-pial collaterals supplying the MCA and ACA territory bilaterally from both PCAs. There were no aneurysms in the leptomeningeal vessels. The hemorrhage was thought to be from the rupture of fragile leptomeningeal collaterals. In view of the severe disease, she was advised bilateral revascularization surgery after 6 weeks, to allow for improvement of her neurological deficits. She improved gradually, and after 2 months, was completely asymptomatic. Her motor power improved to normal, and she was neurologically normal.\nTwo months after her hemorrhage, she had a sudden onset of weakness of left lower limb. There was no history of headache or seizures. There was no evidence of prior dehydration, crying or any other precipitating factor. On examination, she was conscious, alert and oriented. Power in the left lower limb was 2/5 proximally and 0/5 distally. Cranial CT scan revealed a well-defined hypodensity in the right medial frontal region, which was not enhancing on contrast, suggestive of infarct in right ACA territory. There was complete resolution of the previous bleed in the left ganglionic region, along with encephalomalacia in the same region.\nThe patient underwent bilateral superficial temporal artery-MCA bypass, first on the right side, followed by on the left side after 3 weeks. Middle meningeal arteries were preserved bilaterally. She had hyperperfusion syndrome, with transient aphasia following surgery on the left side, which improved completely at the time of discharge. CT following both surgeries did not show any evidence of infarct. At 6-month follow-up, she is asymptomatic, and did not have any further TIA or stroke. Digital substraction angiography carried out after 6 months showed patent grafts with good revascularization to bilateral hemispheres []. | [[6.0, 'year']] | F | {'12217680': 1, '19035718': 1, '16648003': 1, '18035479': 1, '17566207': 1, '21805358': 1, '22412278': 2, '11593973': 1, '24339572': 2} | {'3299077-1': 1} |
164,399 | 3841599-1 | 24,339,578 | noncomm/PMC003xxxxxx/PMC3841599.xml | Benign occipital lobe seizures: Natural progression and atypical evolution | A 13-year-old boy presented with a history of seizures since the age of 5 years. Seizures initially started as brief episodes of blurring of vision. Corrective glasses for refractory error did not stop the episodes. Single seizure involving the left side prompted commencement of treatment with valproate syrup [], which made him seizure free for 2 years, but recurred. At the time, seizures were characterized by fear and cry, saying “save me” followed immediately by few seconds of blindness and then automatism of the hands. The boy would be responsive during the brief episodes, but less responsive during prolonged episodes with automatism. There is no available documentation of loss of vision post-event.\nAlthough there is no cry now, semiology is similar for brief episodes. Prolonged episodes have additional clenching of both fists with tonic posturing of arms more than legs. Sometimes, there is forced head turn mainly to the left accompanied by continuous eyelid fluttering and this could evolve into secondary generalized seizures. The duration lasts from 1-3 min. With secondary generalization, postictal confusion lasts for 20-30 min without any blindness.\nSeizures have gradually increased in frequency over the years from once every month to once every week and now to everyday seizures occurring in clusters leading to postictal drowsiness. The hallmark feature is the blurring of vision during these seizures.\nThroughout this course, his general behavior has been normal, but school performance was below average []. As the boy continued to have daily seizures, parents withdrew him from school. He was initially treated with sodium valproate (VAP) alone then levetiracetam was added followed by lamotrigine (LTG) after withdrawing VAP. He was also treated with oxcarbazepine during the initial periods, but stopped due to unknown reason [].\nIn 2012, when he presented to us with history of daily seizures, he was on levetiracetam, LTG and clobazam [].\nCarbamazepine 200 mg BD was added as part of the polytherapy. Now his seizures are less frequent, 1-2/week without secondary generalization, but not fully controlled. Patient was diagnosed as occipital seizures in 2009. Imaging studies have not shown evidence for any focal lesions including positron emission tomography (PET) computed tomography (CT) [].\nThe boy is more interactive with better seizure control and is willing to start school again. | [[13.0, 'year']] | M | {'12614400': 1, '6802526': 1, '3148690': 1, '16822650': 1, '17442007': 1, '16950946': 1, '15519916': 1, '10386532': 1, '12773298': 1, '10403229': 1, '17442005': 1, '10756414': 1, '6810202': 1, '17888075': 1, '16338676': 1, '6410006': 1, '16105750': 1, '1889978': 1, '24339578': 2} | {'3841599-2': 2, '3841599-3': 2} |
Subsets and Splits
No community queries yet
The top public SQL queries from the community will appear here once available.