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165,400 | 3895529-2 | 24,466,524 | noncomm/PMC003xxxxxx/PMC3895529.xml | Diffusion Tensor Tractography in Two Cases of Kernohan-Woltman Notch Phenomenon | A 25-year-old woman presented to our hospital after being assaulted. Initial Glasgow Coma Scale was 10/15. Initial CT scan revealed a large amount of acute left frontotemporoparietal SDH and frontal intracranial hemorrhage with uncal herniation to the right. An emergent craniectomy and hematoma removal was performed (). Physical examination showed left side hemiparesis: Motor grade, 2/5; Fugl-Meyer assessment, upper 8+lower 12=20/100; Manual Function Test, left 16/32. After the operation, her cognitive function coincided with a Rancho Los Amigos level of function VI. MRI was obtained 10 days after injury to find the cause of the ipsilateral motor weakness, and abnormal signal change of the right cerebral peduncle contralateral to the primary lesion was detected. The round-shaped region of decreased intensity in the right crus cerebri on T1-weighted images and high signal intensity on T2-weighted images were observed (). DTI scanning was performed 8 weeks after injury using the same protocol as stated in case 1.\nDTT did not reconstruct right CST around the midbrain due to the lower level of lesion in the T1-weighted image. Also, the CST from the cerebral cortex to the PLIC was not reconstructed. However, CST disrupted in the midbrain level was reconstructed after using different ROI method: one ROI was placed on the CST portion of the anterior lower pons and the other was placed on the CST portion of PLIC (). The FA and ADC values were obtained segmentally as in case 1 on both sides (). Decreased FA value and increased ADC value were noted on the right cerebral peduncle and anterior pons area compared to those on the left side. Four weeks after rehabilitation program, she was independent in ambulation and the motor weakness was improved: Motor grade 3/5. Her cognitive function improved to level VII with Rancho Los Amigos Scale. We re-evaluated the functional status 15 months after the onset of injury. The patient showed gradual improvement of functional abilities. She was able to grasp and release small objects and to ambulate independently: Fugl-Meyer assessment, upper 58+lower 31=89/100; Manual Function Test, left 30/32. However, we could not obtain a follow-up DTI because the patient refused the evaluation. | [[25.0, 'year']] | F | {'9161379': 1, '18940992': 1, '3257624': 1, '22083801': 1, '21812586': 1, '19699493': 1, '2385337': 1, '2928523': 1, '11113240': 1, '24466524': 2} | {'3895529-1': 2} |
165,401 | 3895530-1 | 24,466,525 | noncomm/PMC003xxxxxx/PMC3895530.xml | Sciatic Nerve Injury Caused by a Stretching Exercise in a Trained Dancer | An 18-year-old female patient who had performed dance training for one year presented with right foot drop and weak push-off on gait after a routine groin stretching exercise. Long adductor stretch with both legs apart and knees straight was performed while sitting to fully stretch the inner thigh muscles once anteroposteriorly and once laterally. The patient's dancing tutor helped her stretch by compressing her torso forward, which is the routine procedure. Immediately, after stretching, she felt a blunt pain and numbness, followed by weakness of both feet. Two hours later the weakness of left foot was improved, but pain and numbness had remained. She visited an orthopedic clinic and was treated with medication for pain control, non-steroidal anti-inflammatory drug-based lotion, use of hot packs, and neuromuscular stimulation for 3 months. The symptoms were not improved by treatment and she was referred to our rehabilitation clinic for electromyography. The patient was alert and cooperative. In her medical history, there was no systemic or neurologic disease including compression neuropathy in any extremity. Her family history was negative for peripheral nerve or neuromuscular disease.\nBased on initial physical examination, using the Medical Research Council scale, strength was 4/5 in right knee flexors, 5/5 in right knee extensors, 3/5 in right ankle dorsiflexors, and 2/5 in right ankle plantarflexors. Deep tendon reflexes were 2+/4 for bilateral quadriceps, 1+/4 for the right Achilles, 2+/4 for the left Achilles, and 2+/4 for bilateral elbow flexors. The Babinski sign was not present, and sensation was diminished to pinprick and light touch on the entire right lower limb below the knee. Position sense was normal. Tibial pulses were 2+ bilaterally and capillary refill was normal. Little peripheral edema remained in the right lower leg. Commonly encountered systemic peripheral nerve disorders were ruled out by evaluation using thyroid studies, serum/urine electrophoresis, and a vitamin B12 assay.\nThree months after the injury, electrodiagnostic nerve conduction studies were performed in both lower limbs. Sural sensory nerve action potentials (SNAP) were absent on the right side. The amplitude of compound muscle action potentials (CMAP) of the right deep peroneal nerve was within normal range, but its conduction velocity was lower than the left side. The amplitude of CMAP on the right tibial nerve was one-third lower than on the left side, implying incomplete tibial nerve injury (, ).\nA needle electromyographic examination showed abnormal spontaneous activity in the form of positive sharp waves and fibrillation potentials with polyphasic motor unit configuration and reduced recruitment in muscles innervated by the right sciatic nerve including the right long head of the biceps femoris, peroneus longus, semimembranosus, tibialis anterior, medial, and lateral gastrocnemius. The right gluteus maximus, quadriceps, tensor fascia lata, and paraspinal muscles were normal ().\nMagnetic resonance imaging (MRI) studies of the lower legs and the lumbar area showed swelling and strong high signal intensity in the right sciatic nerve the entire way from the ischial spine to the proximal common peroneal nerve and the distal tibial nerve. Mild edema of the right proximal hamstring muscle suggested muscle sprain without definite fascial thickening or hematoma associated with the sprain. No definite mass lesion compressing or contacting the right sciatic nerve was found. Also, partial tear and fluid accumulation was seen at the left internal obturator muscle around the ischial spine, and avulsion tear was found at the left hamstring tendon origin on the ischial tuberosity of the left leg. However, the left sciatic nerve still contacted the above lesion, but was not enlarged or edematous ().\nShe was diagnosed with right sciatic neuropathy based on the above data. Conservative rehabilitation therapy, such as muscle strength exercise, transcutaneous electrical nerve stimulation, and hot pack was continued.\nAt 5 months post-injury, right sural SNAP were evoked (). There was no interval change, except a measured strength 5/5 in the right knee flexors. At 12 months, increased amplitude of CMAP of the right tibial nerve from 10.5 to 17.5 mV showed axonal regeneration (). Also, strength was graded as 5/5 in right ankle dorsiflexors, which was indicative of full motor recovery and 4/5 in right ankle plantarflexors. These findings provided evidence of axonal regeneration in right sciatic neuropathy mainly in the tibial division. | [[18.0, 'year']] | F | {'9655133': 1, '33515412': 1, '28255938': 1, '16881066': 1, '28758137': 1, '29666444': 1, '15860140': 1, '24466525': 2} | {} |
165,402 | 3895531-1 | 24,466,526 | noncomm/PMC003xxxxxx/PMC3895531.xml | Management of Severe Bilateral Ptosis in a Patient With Midbrain Infarction: A Case Report | A 73-year-old woman with a previous history of hypertension and myocardial infarction was admitted to the neurosurgery department via the emergency room because she could not open her eyes and had altered consciousness. She was diagnosed as acute infarction on the bilateral paramedian midbrain including bilateral cerebral peduncle by magnetic resonance imaging ().\nAfter conservative management, her conscious level improved but she was still unable to open her eyes. Examination revealed pronounced bilateral ptosis, with left and right pupil diameter of 6 mm and 3 mm, respectively. The left pupil was not reactive to light. There was marked limitation of adduction, moderate limitation of depression and elevation in the movement of both eyeballs. Abduction was normal. There were no other findings of central nervous system disease that could cause ptosis from brain imaging, and no evidence of other orbital disease causing ptosis in an ophthalmologic examination. Muscle strength rated using the Medical Research Council Scale was grade 3/5 in both upper limbs and 4/5 in both lower limbs. She was transferred to the department of rehabilitation medicine for intensive rehabilitation management. Participation in the rehabilitation programs was difficult due to continuing inability to open her eyes.\nWe tried taping her lids with Micropore tape and used lubricants to prevent exposure keratopathy. However, this method was less efficient due to blinking. Eye-putti eyelash glue-mediated fixation of the upper eyelid to the supraorbital structures was ineffective. The patient became frustrated and depressed, and refused to participate in the rehabilitation programs.\nAfter about 6 months from the stroke attack, bilateral ptosis was not improved at all. Consultations were held with the department of plastic surgery with the aim of considering surgical treatment, with the thinking that it would be hard to anticipate spontaneous recovery. Plastic surgeons planned the surgical correction of bilateral ptosis and the Muller's muscle procedure was done, considering the benefits and losses.\nAfter surgical correction, the upper lid height was improved from 7.0 to 4.0 mm on the right side and from 7.0 to 4.5 mm on the left side. The palpebral fissure height was 3.5 mm on the right side and 3.0 mm on the left side (). The patient was quite contented with the effect of the operation and actively participated in the rehabilitation programs. She was able to perform activities of daily living and mobility with minimal assistance. The effect of surgical correction has persisted for at least 6 months. | [[73.0, 'year']] | F | {'15824351': 1, '9574483': 1, '18309144': 1, '20222904': 1, '12925861': 1, '22189277': 1, '3833733': 1, '7969955': 1, '24466526': 2} | {} |
165,403 | 3895532-1 | 24,466,527 | noncomm/PMC003xxxxxx/PMC3895532.xml | Thoracic Outlet Syndrome Caused by Schwannoma of Brachial Plexus | A 47-year-old woman visited the department of rehabilitation medicine complaining of pain, dysesthesia, and mild weakness in the intrinsic muscles of her right hand, each of which had persisted for approximately 1 year. The patient specifically reported dysesthesia and pain in her right upper arm, the medial part of her right forearm, and the fourth and fifth fingers of her right hand. There were no changes in color or pulse, and no indications of swelling or muscle atrophy. Plain cervical radiography and magnetic resonance imaging (MRI) were performed to rule out cervical or thoracic radiculopathy, but no specific abnormal findings were noted. Electrodiagnostic studies (EDS) showed that sensory and motor nerve conduction was normal. However, the F-wave study indicated slightly delayed onset latency at the right median and right ulnar nerves (right median, 27.8 ms; right ulnar, 27.8 ms). Needle electromyographic examinations of the right upper limb showed increased amplitude at the abductor pollicis brevis (APB) and reduced recruitment patterns at the APB and first dorsal interosseous (FDI). Results are presented in -. On physical examination, the Adson provocative test, hyperabduction maneuver, and elevated arm stress test were all positive. TOS was diagnosed. In the outpatient department, the patient received several forms of conservative management, including posture education, stretching exercises, trigger point injections, cervical stellate ganglion block, and medication. Despite this, her symptoms did not improve.\nNine months after receiving her first outpatient department treatment, the patient's symptoms were slightly aggravated. Motor power of the right finger intrinsic muscles was fair. The patient complained of a fixed and tender growing mass at the upper margins of her right clavicle. Ultrasonography revealed a 1.9×1.3×2.1-cm, well-marginated heterogeneous echoic mass at the lateral side of the right sternocleidomastoid muscle (). As ultrasonographic findings were strongly suggestive of a tumor, we performed enhancing right brachial plexus MRI, which revealed a 3.0×1.8×1.7-cm, well-marginated peripheral enhancing ovoid mass between the inferior trunk and the anterior division of the brachial plexus. T1-weighted images showed heterogeneous low signal intensity and T2-weighted images showed generally high signal intensity, with partial low signal intensity ().\nSubsequently, the patient was referred to the orthopedic department, where she underwent surgical mass removal. A biopsy was performed during surgery. On microscopic examination, hematoxylin and eosin staining revealed a strongly stained spindle cell neoplasm displaying Antoni A and Antoni B areas with Verocay bodies, which are characteristic findings of schwannoma (). After removal of the schwannoma, the weakness in the patient's right hand did not progress any further and her neuropathic pain gradually reduced. However, dysesthesia at the right C8 and T1 dermatome did not improve.\nTwenty-eight months after surgery, the patient underwent follow-up EDS. Needle electromyographic examinations showed increased amplitude and reduced recruitment patterns at the right APB, FDI, and abductor digiti minimi. Motor nerve conduction study showed that compound muscle action potential (CMAP) amplitudes of the right median and ulnar nerves were increased as compared with the previous test results (right median, 10.9 mV; right ulnar, 11.0 mV). The F-wave study for the right median and ulnar nerves indicated normal onset latency (right median, 24.8 ms; right ulnar, 26.7 ms). | [[47.0, 'year']] | F | {'19008742': 1, '30431732': 1, '18989247': 1, '13323047': 1, '19741351': 1, '8672574': 1, '15005383': 1, '22211049': 1, '24466527': 2} | {} |
165,404 | 3895533-1 | 24,466,528 | noncomm/PMC003xxxxxx/PMC3895533.xml | Atypical Supernumerary Phantom Limb and Phantom Limb Pain in a Patient With Spinal Cord Injury: Case Report | A 43-year-old man was admitted to our hospital for intensive rehabilitation 115 days after a traffic accident. Cervical computed tomography (CT) and magnetic resonance imaging (MRI) on the day of the injury had shown a C6 vertebral body fracture as well as a cervical cord signal change on T2-weighted MRI images (). He had undergone a C5-6-7 lateral mass fusion on the day of the injury. Six days after injury, he was transferred to the rehabilitation department. A subsequent C5-6-7-T1 anterior and posterior fusion was additionally performed 35 days after injury due to spinal instability detected on follow-up cervical spine X-ray (). There was no concomitant brain injury on brain CT scans and all cognitive evaluations including cognitive score subsets of the Functional Independence Measure showed intact cognition. Other than a past history of type 2 diabetes mellitus, no other significant medical history including psychiatric history existed. The patient also had no history of drug or alcohol misuse.\nAccording to the International Standards for Neurological Classification of Spinal Cord Injury, the patient had a complete lesion (American Spinal Injury Association impairment scale A [ASIA A]) with a C6 neurologic level of injury. According to neurologic exams performed 6 days after injury, the initial upper extremity motor score was 27 and the lower extremity motor score was 0. These scores improved to 31 and 0, respectively, at 115 days after injury when he was transferred to our hospital. Total light touch scores were initially 21 for the right side and 21 for left side and improved to 28 and 29, respectively, at 115 days follow-up. Total pin prick scores were initially 23 for the right side and 24 for the left side, but improved to 27 and 27, respectively on follow-up. There was no interval change in the neurologic exam at 6 months follow-up. Electrodiagnostic studies performed 2 months after injury revealed complete myelopathy and bilateral symmetric peripheral sensorimotor polyneuropathy. Due to concurrent diabetes related peripheral polyneuropathy, sensory nerve conduction studies were done, which showed low amplitudes in bilateral ulnar, superficial peroneal, and sural nerves. However, conduction velocities of all examined nerves and bulbocavernosus reflex latencies were within normal range. Median somatosensory evoked potentials (SEP) stimulating the median nerves at the wrist bilaterally showed prolonged N20 latency in both sides. Tibial and pudendal SEPs were un-evoked with stimulations to the tibial nerves at the ankle and pudendal nerve at penile shaft.\nSPL sensations appeared 6 days after trauma onset. The patient felt the presence of an additional pair of legs that originated at the hip joints and extended medially, at equal lengths to the paralyzed legs (). The patient described the presence of both knee and ankle joints in an extended position. SPL perceptions persisted throughout the day but its intensity fluctuated with more vividness during the afternoon and evening. Body posture also altered the intensity of SPL sensations with more prominence in the supine position and waning during sitting. The patient reported that he could feel but could not see the additional limbs and was aware that this feeling was an illusion. Visualization of his legs did not diminish or alter the intensity of SPL sensations. The supernumerary legs were also associated with severe lancinating pain that measured 8 points on the numeric rating scale. The patient also complained of severe neuropathic pain of the paralyzed legs that worsened when the SPLs were in the flexed position. However, he reported that he had no voluntary control over the phantom limbs and the movements of the SPLs were unintentional. Psychiatric evaluation showed no evidence of delusion, hallucination or any other specific psychiatric abnormality, and the patient showed good insight regarding his various clinical status related to his SCI. The intensity of the SPLs was equal to his paralyzed limbs.\nCombinations of oral gabapentin, pregabalin, baclofen, tramadol, and duloxetine improved the neuropathic pain in both the real and supernumerary limbs that measured 6 points on the numeric rating scale, but failed to affect changes in the intensity of the SPLs.\nThe patient underwent visual-tactile stimulation treatment where he would first visually identify his paralyzed limbs and then gently tap his legs with a wooden stick (). Although his legs were anesthetic to both pain and light touch, tactile stimulation evoked painful tingling sensations in his paralyzed legs. Thus, he was able to receive both visual and sensory feedback and reconfirm the existence of his paralyzed legs. Wooden stick stimulation also evoked painful tingling in the SPLs but visually he was able to recognize the absence of the SPLs and thus differentiate the sense as erroneous. Visual-tactile stimulation was performed three times daily for 10-15 minutes per session with approximately one tap per second. The patient was then asked to rate the intensity of the SPLs on a 10-point numeric analog scale relative to the paralyzed real limb. The presence of the SPLs were felt at an equal intensity to the real limbs initially but improved to half the intensity of the real paralyzed limbs after 8 days of daily visual-tactile stimulation. This improvement continued over the 2 months of inpatient treatment at our hospital and the presence of the SPLs was reduced to 20% of the real paralyzed legs.\nNine months after the SCI, the patient reported persistence of the illusory limb sensations, but the sensations had improved to an extent where it no longer affected the patient's quality of life. Through a telephone interview conducted 13 months post-trauma, the patient reported a telescoping pattern of the SPL with total length shortening to levels half of the original. However, although shorter in length, the knee and ankle joints were still present and the limb continued to remain in the extended position. Although SPL sensation and subsequent total motor and sensory scores at 6 months follow-up improved, motor and sensory scores pertaining to the lower extremities as well as the ASIA A neurologically complete injury state remained unchanged. Additional lower limb SEP studies and imaging studies were not preformed due to patient refusal. However, considering the lack of change in both motor and sensory scores as well as ASIA grades, these studies did not appear to be clinically significant or essential. | [[43.0, 'year']] | M | {'8437005': 1, '19557858': 1, '12027341': 1, '21079624': 1, '28886692': 2, '14604017': 1, '1192653': 1, '14662516': 1, '31624763': 2, '24466528': 2} | {'6795722-1': 1, '5591514-1': 1} |
165,405 | 3895534-1 | 24,466,529 | noncomm/PMC003xxxxxx/PMC3895534.xml | Diagnosis With Manometry and Treatment With Repetitive Transcranial Magnetic Stimulation in Dysphagia | A 52-year-old male patient visited the emergency department in our hospital due to sudden dizziness and dysphagia. The patient was diagnosed with lateral medullary infarction using brain magnetic resonance imaging. Three days after onset, he was referred to the department of rehabilitation medicine for the rehabilitative treatment of dysphagia. The patient had right blepharoptosis and no muscle weakness except for ataxia of the right lower extremity. He was being fed by a gastric feeding tube. Water swallowing test demonstrated a typical wet voice and laryngopharyngeal retention despite the small amount of food intake. VFSS was conducted to identify the causative factors of dysphagia 4 days after the onset. The functional dysphagia scale score was 60 points, with no particular abnormality in the anterosuperior elevation of the oral cavity and larynx, atresia of the epiglottis, definite contraction of the upper pharynx, and retention of almost all foods in the pyriformis sinus and vallecular fossa after swallowing, with no passage past the upper esophageal sphincter (). During the test, compensation techniques, such as Mendelsohn maneuver, head turn, and head lean, were also conducted, but no particular difference was observed. The patient underwent rehabilitative treatments that included electrical stimulation for dysphagia, Shaker exercise, and training concerning a compensation technique including the Mendelsohn maneuver. An assessment was conducted using esophageal manometry 32 days after onset to more accurately identify the cause of dysphagia. By setting the upper esophageal sphincter as a reference point, the pressure changes in the pharynx above the upper esophageal sphincter, upper esophageal sphincter, and esophageal body were measured. Normal pressure change in the upper pharynx, decreased contraction of the lower pharynx, definitely decreased pressure upon the relaxation of the upper esophageal sphincter, retention in the upper esophageal sphincter due to the impairment of the coordination with the contraction of the lower pharynx, and no pressure changes caused by esophageal peristalsis were evident (). About four weeks later (33 days after onset), VFSS was conducted again. The functional dysphagia scale score was again 60 points (). As the patient was refractory to the one-month dysphagia rehabilitative treatment including electrical treatment for dysphagia, Shaker exercise, and the training of compensation technique, another treatment was applied.\nAs several previous studies reported that dysphagia was improved by increasing the neurotransmission and plasticity of the corticobulbar tract via rTMS of the cerebral cortex associated with swallowing [,], rTMS was used. The International 10-20 system and a bandana with lattice positioned at intervals of 1 cm were used to determine the stimulation site. The surface electrode was used to determine the site with the lowest motor threshold of the pharyngeal-laryngeal muscles, which was identified as an area 2 cm anterior and 5 cm lateral from the vertex of the head. rTMS was conducted with a frequency of 5 Hz and intensity of 120% of the motor threshold of bilateral hemisphere, and 10 cycles of 10 seconds stimulation and 50 seconds relaxation for 10 days (five times/week for 2 weeks) followed by VFSS and manometry to assess the result of the 2-week TMS treatment. The functional dysphagia scale was improved to 20 points. Compared to the result of the preceding VFSS, which showed that most foods were retained in the lower pharynx because of the inability to passing by the upper esophageal sphincter, at the follow-up most foods were able to passed by the upper esophageal sphincter. A small quantity of food was retained in the pyriformis sinus and vallecular fossa sinus after swallowing, and the laryngeal delay time was significantly shortened (, ). Meanwhile, compared to previous manometry results, the follow-up revealed a prolonged relaxation time of the upper esophageal sphincter of 2 seconds, which was more than twice the time of the previous results. This would be sufficient enough to enable foods to pass by the upper esophageal sphincter after the contraction of the lower pharynx. The pressure change was observed in the esophageal phase due to peristalsis ().\nAfter 2 weeks of rTMS, the patient began to consume small amounts of food in accordance with the schedule set by the authors. Regular chest X-ray and blood test revealed no complications, such as aspiration pneumonia, were observed. When the patient was able to consume 1,650 calories per day, he was discharged. He has been followed-up in our out-patient clinic and has been taking a regular diet without any complication for 2 years. | [[52.0, 'year']] | M | {'11137665': 1, '32654058': 1, '19427312': 1, '16754707': 1, '6100081': 1, '11606849': 1, '21376629': 1, '2484181': 1, '12417783': 1, '19828479': 1, '24466529': 2} | {} |
165,406 | 3895535-1 | 24,466,530 | noncomm/PMC003xxxxxx/PMC3895535.xml | Medial Antebrachial Cutaneous Nerve Injury After Brachial Plexus Block: Two Case Reports | A 62-year-old female patient presented to the Department of Anesthesiology of this hospital with the chief complaints of 1-month duration of pain and numbness in her right medial antebrachial region. She was referred to the Department of Rehabilitation for electrodiagnostic examinations. According to the patient's past history, she had undergone right carpal tunnel release 8 months before she visited our hospital. However, this did not relieve the complaint of right finger numbness that she had before the surgery. Therefore, she had undergone the surgery again one month earlier. The symptoms in the medial antebrachial region occurred thereafter. During the surgery, she was anesthetized by axillary brachial plexus block using ultrasound guidance. First, in a supine position, her arm was externally rotated by 90°, her elbow joint was flexed by 90°, and her head was turned to face the side opposite to the surgery. The ultrasonic probe was placed on the axillary region to find the axillary artery, the surrounding structures were checked, and a 23-G needle was inserted to inject a solution comprising a mixture of 2% lidocaine, 0.5% bupivacaine, and epinephrine (1:200,000) into the axillary sheath. Physical examinations revealed no muscle weakening but right thenar muscle atrophy. In sensory tests, the patient complained of decreased sensation and numbness in the right first, third, and fourth fingers, and in the medial antebrachial region. In particular, she reported that she suffered sleep disturbance due to the pain in the medial antebrachial region. The upper extremity deep tendon reflexes were all normal, but positive responses were obtained in the median nerve compression test. The symptoms were not accompanied by cervical and upper extremity pain, or decreased sensation. According to the electrodiagnostic examinations conducted 4 weeks after the onset of the symptom, the median and ulnar nerves were normal in motor nerve conduction studies. Sensory nerve conduction studies revealed a 4.10 ms delay in latency of the right median nerve and no response from the right MABC nerve (). Needle electromyography revealed no abnormal spontaneous activities in the abductor pollicis brevis muscle, which is innervated by the median nerve in the right-hand region.\nBased on the results of the physical and electrodiagnostic examinations, the patient was diagnosed with right MABC nerve injury. Pregabalin 75 mg, clonazepam 0.5 mg, and tramadol 50 mg were orally administered as drug treatment, and stellate ganglion block was performed. However, the patient's symptoms were not relieved. We decided to conduct outpatient follow-ups and periodic electrodiagnostic examinations. Eight weeks after the onset of the symptoms, the patient's pain was relieved slightly. In follow-up sensory nerve conduction study of the right MABC nerve, the latency on the right side was delayed by 5.00 ms compared to 2.50 ms on the unaffected left side, and the amplitude on the right side showed an abnormally reduced value of 2.9 µV compared to 10.1 µV on the left side. In addition, in the somatosensory evoked potential, the N20 latency of the right MABC nerve was delayed by 22.60 ms compared to 17.70 ms on the left unaffected side. The N20-P25 amplitude on the right side was 1.30 µV, showing a reduction of at least 50% compared to the left side (3.85 µV) (). | [[62.0, 'year']] | F | {'26495085': 2, '25566484': 1, '18426569': 2, '21896172': 1, '842295': 1, '11726453': 1, '10195536': 1, '27365965': 1, '32698279': 1, '2766143': 1, '21857274': 1, '9972761': 1, '24466530': 2} | {'3895535-2': 2, '2365945-1': 1, '4610944-1': 1} |
165,407 | 3895535-2 | 24,466,530 | noncomm/PMC003xxxxxx/PMC3895535.xml | Medial Antebrachial Cutaneous Nerve Injury After Brachial Plexus Block: Two Case Reports | A 30-year-old female patient was treated in the Department of Anesthesiology of this hospital for pain and numbness in the right medial antebrachial region that had begun 8 weeks earlier. She was referred to the Department of Rehabilitation for electrodiagnostic examinations. She had been receiving conservative treatment for an enchondroma following a fracture of the third proximal phalanx of her right hand suffered 6 months earlier during a fall. She had undergone curettage and a bone graft under ultrasound-guided axillary brachial plexus block 8 weeks prior to the present visit. She experienced numbness in the right medial antebrachial region immediately after the surgery. Her past medical history was unremarkable. Physical examinations revealed no particular finding other than decreased sensation and numbness of the right medial antebrachial region. According to the electrodiagnostic examinations conducted 8 weeks after onset, the median nerve and the ulnar nerve were both normal in motor nerve conduction studies. In sensory nerve conduction studies, the latency of the right median nerve was 1.80 ms and 1.95 ms on the unaffected left side. The amplitude on the right side was 8.6 µV, showing a slight reduction compared to the left side (10.6 µV), although the difference was not clinically significant (). In needle electromyography, no abnormal spontaneous activities were observed in the muscles innervated by the median nerve or in the ulnar nerve of the right-hand region.\nBased on the results of the physical examinations and the electrodiagnostic examinations, right MABC nerve injury was suspected. Pregabalin 75 mg, clonazepam 0.5 mg, and tramadol 50 mg were orally administered, and stellate ganglion block was performed. However, the treatment did not relieve the patient's symptoms. In follow-up sensory nerve conduction study of the right MABC nerve 13 weeks after the onset of the symptoms, the amplitude had increased slightly to 10.6 µV, and the patient reported that the numbness and pain in the right medial antebrachial region were considerably relieved. In the somatosensory evoked potential, the N20 latency of the MABC nerve on the right side was 16.80 ms and 15.90 ms on the left side. The N20-P25 amplitude on the right side was 1.30 µV and 1.56 µV on the left side (). | [[30.0, 'year']] | F | {'26495085': 2, '25566484': 1, '18426569': 2, '21896172': 1, '842295': 1, '11726453': 1, '10195536': 1, '27365965': 1, '32698279': 1, '2766143': 1, '21857274': 1, '9972761': 1, '24466530': 2} | {'3895535-1': 2, '2365945-1': 1, '4610944-1': 1} |
165,408 | 3895550-1 | 24,466,541 | noncomm/PMC003xxxxxx/PMC3895550.xml | Rhabdoid Carcinoma of the Rectum | A 49-year-old Caucasian male presented to the outpatient clinic with symptoms of tenesmus, abdominal pain, blood and mucus in stool, and weight loss of over 40 kg during the previous 6 months. A circular ulcerated and stenosing tumor was discovered on colonoscopy and was located 13 cm above the anal verge. A partial total mesorectal excision (TME) with a resection of the right ureter and urinary bladder (tumor penetrated these structures), as well as dissection of the right lateral pelvic node, was performed; 27 of 51 harvested lymph nodes contained metastases. No distant metastases or other tumors were discovered at the time of surgery.\nGross examination demonstrated a broadly ulcerated, deeply infiltrative tumor within the rectal wall, measuring 7 cm in greatest dimension, growing through the adventitia into the adjacent urinary bladder that was removed together with a segment of bowel. Microscopically, the lesion was composed of two components: over 99% of it showed a rhabdoid morphology with discohesive solid sheets of pleomorphic epithelioid cells, little stroma and few inflammatory cells in between. Abundant eosinophilic cytoplasm with a large excentric nucleus and a single prominent nucleolus was present in the majority of the tumor cells. Numerous mitotic figures, including atypical forms, were present (). No "signet-ring" cells were identified. The second minor component constituted a poorly-differentiated adenocarcinoma and was present only in one tissue block section located at the periphery of the main tumor mass just underneath the intact colonic mucosa (). It was composed of irregular, cribriform glands lined by large atypical cells with prominent nucleoli and scant cytoplasm, surrounded by a desmoplastic inflammatory stroma.\nLymphovascular invasion was present. Metastatic disease was identified in multiple mesenteric lymph nodes and constituted a spread of pure rhabdoid cells without an adenocarcinoma component. Assessments of the surgical resection margins raised the possibility of microscopic residual disease.\nRhabdoid cells demonstrated positivity for pancytokeratin, vimentin, and epithelial membrane antigen, and were nonreactive to cytokeratin 7 (CK7), cytokeratin 20 (CK20), thyroid transcription factor-1, homeobox protein CDX-2 (CDX-2), desmin and myogenic differentiation 1 (MyoD1). The minor adenocarcinoma component vanished on deeper sections; nevertheless, pancytokeratin, vimentin and CDX-2 stains were positive whereas CK7 and CK20 stains were both negative. INI1 antibody stained all neoplastic nuclei within the rhabdoid component (). A microsatellite analysis was undertaken by applying markers to MLH1, MSH2, MSH6, and PMS2 and showed no loss of expression of all 4 of them in tumor cells.\nMutation analysis of the rhabdoid tumor component revealed a wild-type (not mutated) KRAS gene and BRAF V600E mutation.\nRectal lesions are strongly believed to represent a primary neoplasm based on their overall gross appearance and clinical presentation. They are circular, dominant rectal tumor with no other lesions being identified at the initial workup, making metastatic disease in the rectum almost impossible. The immunophenotypic pattern was informative with respect to conclusively confirming the rhabdoid morphology; however, it was not suggestive of organ specificity to help rule out secondary dissemination to the rectum. Identification of a minor adenocarcinoma component was most useful. It strongly argued for the lesion to represent a primary rectal composite rhabdoid tumor. With the rhabdoid morphology in mind, an epithelioid sarcoma was also on the differential list; however, given that both rhabdoid tumors and epithelioid sarcomas share almost identical histological, immunohistochemical and molecular patterns, separation of these two entities may have been impractical and nearly impossible []. Again, the presence of a minor adenocarcinoma component strongly favored a rhabdoid tumor, as it would not be expected to occur in an epithelioid sarcoma.\nA month past surgery, a computed tomography (CT) scan disclosed multiple metastases within abdominal lymph nodes, iliac vessel invasion, and probable disease spread to the liver. Intensive chemotherapy was initiated: 4 cycles according to the chemotherapy regimen, consisting of folinic acid (leucovorin), fluorouracil (5-fluorouracil), and irinotecan (FOLFIRI) regimen were administered. The therapy course was complicated by development of bilateral pneumonia and ascites that were treated with antibiotics and drainage. Three months into the postoperative course, a repeated CT scan demonstrated disease progression despite aggressive chemotherapy. The patient received four additional cycles of chemotherapy according to the chemotherapy regimen, consisting of folinic acid (leucovorin), fluorouracil (5-fluorouracil), and oxaliplatin (FOLFOX4) regimen; however, again, no improvement was observed, metastatic disease rapidly spread to leptomeninges, neurological symptoms developed, and the patient eventually passed away 7 months after initial disease presentation. | [[49.0, 'year']] | M | {'21315413': 1, '20390315': 1, '18251752': 1, '17913953': 1, '16198956': 1, '11679945': 1, '28643792': 1, '17707262': 1, '206343': 1, '8927435': 1, '17913954': 1, '8545590': 1, '29452605': 2, '31455041': 1, '2543225': 1, '18317218': 1, '33903966': 2, '21791485': 1, '21934399': 1, '24466541': 2} | {'5816521-1': 1, '8076409-1': 1} |
165,409 | 3895551-1 | 24,466,542 | noncomm/PMC003xxxxxx/PMC3895551.xml | Pneumoretroperitoneum After Procedure for Prolapsed Hemorrhoid | A previously healthy 27-year-old female who had a grade-3 prolapsing hemorrhoid was treated with PPH at a local private hospital. She was discharged on the second postoperative day without any specific symptoms. She visited the Emergency Department of our institution on the evening of the discharge day due to a sudden onset of abdominal pain and fullness. Physical examination showed a diffuse abdominal tenderness, but the degree was not as severe as that seen in a typical case of peritonitis. On rectal digital examination, the staple line was noted at approximately 4 cm above the anal verge, and the defect was located at the right side of patient. There was no bleeding evidence in either the patient's history or on examination. Vital signs were stable except for a mild fever (37.5℃). The white blood cell count was 13 × 106/mL. A plain abdominal radiograph and a computed tomography (CT) scan showed extensive retroperitoneal gas ( and ). Air bubbles were also found at the subcutaneous layer of the anterior abdominal wall on the CT scan. A laparoscopic evaluation was performed at 55 hours after the original surgery. Exploration of the peritoneal cavity and the retroperitoneal space revealed no necrosis, abscess or fecal material. The retro-rectal tissue, however, was markedly edematous and vascular. A closed suction drain was placed at the retroperitoneal space, and a loop sigmoid colostomy was created.\nThe rectal staple line was inspected after the abdominal procedure. Partial staple-line dehiscence, which was consistent with the preoperative digital examination, was noted at the right side of the patient (). The defect was closed with 4-0 vicryl after massive irrigation. An overstitching was done throughout the staple line to prevent possible future dehiscence. The postoperative course was uneventful, and oral feeding was resumed on the second postoperative day. At the 2-month follow-up, a gastrografin enema showed the absence of leaks, and the colostomy was closed. During follow-up, she has had no residual problems, including staple-line stenosis. | [[27.0, 'year']] | F | {'20847627': 1, '28289657': 1, '15785901': 1, '16969619': 1, '17665254': 1, '33207111': 1, '28289661': 1, '12790985': 1, '18512007': 1, '15622575': 1, '17032319': 1, '22418745': 1, '19091821': 1, '15701493': 1, '17380367': 1, '16323166': 1, '24466542': 2} | {} |
165,410 | 3896286-1 | 24,470,768 | noncomm/PMC003xxxxxx/PMC3896286.xml | Misdiagnosed spontaneous intracranial hypotension complicated by subdural hematoma following lumbar puncture | A 34-year-old man without any headache history, cardiovascular risk factor, fever, or antecedent trauma had since September 3, 2010 acute postural headache that occurred within 15 minutes of assuming an upright position; it began as a pain over the vertex, and rapidly became holocephalic, with photophobia, nausea, and vomiting. The headache resolved within 10 minutes of assuming the supine position. The patient did not have aura, visual change, or other neurological symptoms. Clinical examination was normal, and did not found any hyperflexibility of skin or joints. The first cerebral computed tomography scan was normal. A brain MRI with angio-sequences did not show cerebral thrombophlebitis, and was interpreted as normal (). Lumbar puncture was performed, without a measure of cerebrospinal fluid (CSF) pressure, showed hyperproteinorachy at 2 g/L, and six cells with lymphocytosis. The headache became very intense. At admission, clinical examination was normal. Ophthalmological examination did not show any abnormalities. A second brain MRI showed bilateral subdural hematoma with tonsillar descent, simulating Chiari type I malformation (). The patient was treated by surgical drainage of the subdural hematoma and bed rest. The blood patch was not realized. The headache was completely resolved 2 days after surgery. There was no recurrence after 3 years of follow-up. | [[34.0, 'year']] | M | {'14984683': 1, '15305844': 1, '14676045': 1, '16981936': 1, '33014673': 1, '9671999': 1, '27453759': 1, '15557521': 1, '11723293': 1, '16705110': 1, '16514502': 1, '25324976': 2, '14979299': 1, '18395303': 1, '30278562': 1, '24470768': 2} | {'4199149-1': 1} |
165,411 | 3896311-1 | 24,531,165 | noncomm/PMC003xxxxxx/PMC3896311.xml | Ocular findings in two siblings with Joubert syndrome | A 6-year-old boy presented with ptosis and strabismus of his left eye along with an abnormal head posture. He had delayed motor and language development with episodic hyperpnea–apnea spells and hypotonia in infancy. JS diagnosis was made on the basis of the characteristic MTS on MRI () and neurological findings observed in the pediatric clinic. He showed mild facial dysmorphism in the form of forehead prominence and bilateral epicanthic folds. He had ptosis, hypotropia, and exotropia in his left eye (). His abnormal head posture included a head tilt to the left and turning his face to the left. On ophthalmic examination, the best-corrected visual acuity in his right eye was 0.8 with +0.25 D −1.00 D ×90°, and 0.5 with +0.50 D −2.00 D ×60° in his left eye. The prism cover test demonstrated that there was 20 prism diopter hypotropia and 35 prism diopter exotropia of his left eye. The patient was unable to maintain his eyes in a central position by convergence, and he had horizontal pendular nystagmus. Slit-lamp examination showed no other abnormalities in either of his eyes. Funduscopy showed mild chorioretinal discoloration at the peripapillary region in both eyes (, arrows). Additionally, a small coloboma was detected at the inferior region of his right optic disc (, arrowhead). OCT (RS-3000; NIDEK, Gamagori, Japan) showed that the foveal architecture was preserved in both eyes, although there was poor recording due to nystagmus (). | [[6.0, 'year']] | M | {'32169553': 1, '27571019': 1, '16155189': 1, '20615230': 1, '23870701': 1, '19928742': 1, '10385844': 1, '15473174': 1, '9373798': 1, '22606509': 1, '19461662': 1, '1341417': 1, '17377524': 1, '2470340': 1, '20592197': 1, '18286952': 1, '19041481': 1, '576733': 1, '5816874': 1, '21506742': 1, '30518138': 1, '10488903': 1, '17896309': 1, '28228833': 1, '24531165': 2} | {'3896311-2': 2} |
165,412 | 3896311-2 | 24,531,165 | noncomm/PMC003xxxxxx/PMC3896311.xml | Ocular findings in two siblings with Joubert syndrome | An 11-year-old boy presented with ptosis and strabismus of his left eye along with an abnormal head posture. He had delayed motor and language development with episodic hyperpnea–apnea spells and hypotonia in infancy. JS diagnosis was made on the basis of the characteristic MTS on MRI and neurological findings observed in the pediatric clinic. He showed mild facial dysmorphism in the form of forehead prominence and bilateral epicanthic folds. He had ptosis, hypotropia, and exotropia in his left eye, and he was unable to maintain his eyes in a central position by convergence. He also had horizontal pendular nystagmus. On ophthalmic examination, best-corrected visual acuity in his right eye was 1.2 with −1.00 D, and was 1.2 with −1.25 D −1.50 D ×50° in his left eye. Slit-lamp examination showed no abnormalities in either of his eyes. Funduscopy showed coloboma in his right eye and a colobomatous optic disc in his left eye (, arrows). OCT showed that the foveal architecture was preserved in both eyes (). In addition, on the basis of the Early Treatment Diabetic Retinopathy Study sectors, the thickness of center (central fovea), inner ring (1–3 mm from the central fovea), and outer ring (3–6 mm from the central fovea) in his right eye were 283 μm, 327.5 μm, and 272.0 μm, respectively. In his left eye, these measurements were 284 μm, 315.0 μm, and 270.0 μm, respectively. | [[11.0, 'year']] | M | {'32169553': 1, '27571019': 1, '16155189': 1, '20615230': 1, '23870701': 1, '19928742': 1, '10385844': 1, '15473174': 1, '9373798': 1, '22606509': 1, '19461662': 1, '1341417': 1, '17377524': 1, '2470340': 1, '20592197': 1, '18286952': 1, '19041481': 1, '576733': 1, '5816874': 1, '21506742': 1, '30518138': 1, '10488903': 1, '17896309': 1, '28228833': 1, '24531165': 2} | {'3896311-1': 2} |
165,413 | 3896650-1 | 24,465,264 | noncomm/PMC003xxxxxx/PMC3896650.xml | Novel Partial Anomalous Pulmonary Venous Connection Associated with Brain Abscess | A 47-year-old woman presented with worsening headache and neck pain over 4 days associated with nausea and vomiting. She denied subjective fevers or chills. She reported traveling to the Dominican Republic 1 month prior to presentation. She had no other medical history and was not on any medications. She denied any recent illnesses or dental procedures.\nHer temperature was 38.0℃, heart rate was 70 beats/min, respiratory rate was 18/min, and blood pressures were 140/72 mm Hg. The patient had no evident toxicity and was alert and oriented to person and time but not to place. An oral examination showed good dentition with no notable abscess, pain, or gingival disease. A cardiovascular examination revealed no abnormalities, including murmurs. There was no evidence of skin infections or lesions. She had mild expressive aphasia with clear, unpressured speech. Reception and comprehension were intact. Her cranial nerves and the results of motor and sensory examinations were normal. However, she did have a shuffling gait with loss of balance to the right and clonus at the right ankle.\nThe laboratory parameters of the patient at admission were remarkable for leukocytosis with a left shift (17.8×109 WBCs per liter and 90% neutrophils). The results of electrocardiogram and chest X-ray examinations were normal. Computed tomography (CT) of the brain without contrast agent demonstrated a left thalamic mass with a hypodense center and hyperdense periphery. A mass effect was noted on the left lateral and third ventricles. Well-aerated paranasal sinuses and mastoid cells were noted. Magnetic resonance imaging of the brain with and without contrast agent revealed a 2.4×2.2×1.9-cm lesion with mild surrounding hyperintense T2-weighted and fluid-attenuated-inversion-recovery (FLAIR) signals compatible with edema (). The enhanced rim demonstrated a hypointense T2-weighted signal. The internal contents of the lesion were hyperintense on T2-weighted and FLAIR signals with corresponding restricted diffusion. These imaging findings were highly suggestive of a brain abscess.\nStereotactically guided drainage was applied immediately to the patient. Five milliliters of foul-smelling, purulent fluid was aspirated. The patient was initially started on vancomycin, ceftriaxone, and metronidazole after cultures were obtained. Streptococcus mitis grew in these cultures, and so the antibiotic coverage was appropriately narrowed.\nThe patient subsequently underwent transthoracic echocardiography (TTE) and transesophageal echocardiography with agitated-saline contrast agent injected into the right arm; the results were unrevealing. A CT scan of the chest was performed with contrast agent on postoperative day 13 due to a transient episode of hypoxia, which revealed an incidental finding of an anomalous connection between the left brachiocephalic vein and the left superior pulmonary vein. No PAVMs were noted.\nDiagnostic cardiac catheterization via the left basilic vein confirmed the anomalous venous connection and the presence of a predominantly left-to-right shunt based on measurement of the oxygen saturation. TTE with agitated saline injected into the left arm this time demonstrated the passage of a large amount of bubbles into the left side of the heart. The patient recovered well after abscess drainage and was discharged to a rehabilitation facility with plans for close follow-up, and eventual percutaneous closure of the anomalous connection. | [[47.0, 'year']] | F | {'20127903': 1, '11061251': 1, '9356788': 1, '16337045': 1, '17683907': 1, '8273750': 1, '1383854': 1, '17170340': 1, '18182759': 1, '2029923': 1, '20858807': 1, '11440444': 1, '24465264': 2} | {} |
165,414 | 3896651-1 | 24,465,265 | noncomm/PMC003xxxxxx/PMC3896651.xml | Unilateral Reversible Posterior Leukoencephalopathy Syndrome after Coiling of an Aneurysm | A 36-year-old woman was admitted with acute severe headache. She had also experienced an epileptic seizure and had a slightly lowered consciousness at admission. Nonenhanced computed tomography (CT) showed subtle signs of subarachnoid hemorrhage (SAH) with some frontobasal subarachnoid blood (). CT angiography revealed a 4-mm aneurysm of the left anterior cerebral artery (ACA). At admission her blood pressure was 128/55 mm Hg. On the first day of admission her mean arterial pressure (MAP) varied between 81 and 99 mm Hg, with a mean of 88 mm Hg. She had no history of hypertension, was no taking any medication, and exhibited a good neurological condition (Hunt and Hess grade of 1).\nThe aneurysm was coiled on the same day. The procedure took longer than usual (2 hours) because of the smallness of the aneurysm, which made it difficult to enter it safely with the microcatheter. During the procedure, both internal carotid arteries were catheterized to optimize depiction of the local vascular anatomy (). However, a microcatheter was only advanced in the left ACA to reach the aneurysm. According to the local protocol, 5000 IE of heparin was given during the procedure; no other medication was given during the procedure. The aneurysm was successfully packed with two small coils (3 cm long; 2-mm UltraSoft, Boston Scientific, Natick, MA, USA) using a left approach. No complications were noted and the patient experienced no focal neurological symptoms.\nAfter the procedure the patient developed periods of bradycardia with sinus arrest in the intensive care unit (ICU) but without clinical symptoms. There were assumed to be secondary to the SAH, and were treated conservatively. Transcranial Doppler (TCD) performed during the following week showed mildly increased flow velocities in the middle cerebral artery (MCA) that were just within the normal range (111 and 82 cm/s in the left and right MCAs, respectively), suggesting mild vasospasm. Oral nimodipine at 30 mg taken 12 times daily and triple-H therapy were started according to the local protocol. On day 4 of the triple-H therapy the MAP reached 72-108 mm Hg (mean 93 mm Hg). Slight lung edema was treated with furosemide. TCD performed 5 days later showed normal MCA velocities, although the velocities in the MCA and ACA continued to be higher on the left than on the right, albeit still within the normal range. Triple-H therapy was subsequently stopped. During the hospital stay the clinical status of the patient improved and her headaches gradually diminished. The patient was discharged in good clinical condition after 12 days with a prescription for oral nimodipine (60 mg taken six times daily for 20 days). The MAP at discharge was 100 mm Hg.\nThe evening after taking the last prescription of nimodipine, which was 21 days after coiling and 15 days after the cessation of triple-H therapy, the patient presented at the emergency room with progressive headache, right-sided homonymous hemianopsia, right-sided tactile extinction, and a slight paresis of the right arm. Her MAP during the first day of re-admission was 81-120 mm Hg (mean 108 mm Hg). The cerebrospinal fluid pressure measured via a lumbar puncture was elevated at 42 cmH20. Spinal fluid showed residual signs of the recent SAH but no signs of meningitis. Renal function was normal with serum creatinine, sodium, and potassium levels of 47 µmol/L, 140 mmol/L, and 4.4 mmol/L, respectively. Nonenhanced CT performed 21 days after the first onset of the SAH revealed subcortical hypodense areas in the left hemisphere occipitally and around the insula. Magnetic resonance imaging (MRI) showed vasogenic edema mostly subcortically in the entire left hemisphere, with a preference for the watershed areas (). No susceptibility artifacts were seen within the parenchyma. Diffusion-weighted MRI images showed no signs of ischemia. After gadolinium administration there was patchy enhancement in the lesions (). CT angiography did not show vasospasms or vascular occlusions.\nThe combination of headache, confusion, visual disturbances, and vasogenic edema on MRI was suggestive of RPLS. Further clinical analysis revealed no signs of infection, and dural sinus thrombosis was ruled out with CT venography. Because late-onset secondary brain ischemia was initially suspected clinically, oral nimodipine was restarted at a daily dose of 180 mg. This improved the clinical status of the patient, with headache being the most prominent symptom; this was reduced by prescription of dexamethasone at 2 mg twice daily. Slow tapering of dexamethasone led to the return of slight headaches, but they could now be controlled with acetaminophen. Because the initial clinical suspicion of ischemia was ruled out by the imaging workup, a diagnosis of RPLS was eventually suggested on the fifth day of re-admission based on clinical signs and MRI findings. By the sixth day of re-admission the MAP had decreased to 82-100 mm Hg (mean 89 mm Hg), possibly due to the nimodipine treatment, and so no antihypertensive medication was administered in our patient. The patient was discharged and nimodipine was gradually reduced.\nAt three months after SAH the patient had no neurological symptoms apart from slight balance problems. The MRI abnormalities had diminished but were not completely resolved. However, MRI performed 7 months after the first presentation showed further reduction of the lesions, with a single small area of signal enhancement remaining in the left frontal lobe (). | [[36.0, 'year']] | F | {'18356474': 1, '25788891': 2, '16159094': 1, '25045386': 1, '31360275': 1, '16629742': 1, '19249960': 1, '21964775': 1, '11142628': 1, '17885064': 1, '8559202': 1, '17698535': 1, '16757832': 1, '31687449': 1, '21327576': 1, '15705136': 1, '27322924': 1, '25045385': 1, '20488715': 1, '20435835': 1, '24465265': 2} | {'4349179-1': 1} |
165,415 | 3896652-1 | 24,465,266 | noncomm/PMC003xxxxxx/PMC3896652.xml | Clinical and Molecular Study of the Extracellular Matrix Protein 1 Gene in a Spanish Family with Lipoid Proteinosis | The index patient was a 35-year-old woman born to unrelated parents who developed a progressively hoarse voice from 9 months of age, and vesicular and bullous lesions that appeared after minimal trauma. A physical examination revealed deposition of hyaline material in the larynx, epidermolysis bullosa, multiple yellowish warty skin papules on the body, and diffuse acneiform scars over her face, with blisters in the mouth, eyes, and areas subject to friction. Neurological symptoms included unilateral paresthesia, dizziness, and memory loss associated with depression, in addition to a decline in verbal fluency and impaired coordination of movement from the age of 19 years. Computed tomography revealed bilateral and symmetric amygdaloid-uncal calcifications (). Gradient refocused-echo T2*-weighted MRI images () revealed marked hypointense bilateral rounded symmetric lesions in the mesial temporal lobes, involving the amygdaloid complex. A routine EEG (awake and sleep) recording was slightly unstable, but no epileptiform activity was evident. A cognitive event-related potential P-300 wave revealed a reaction-time delay during development of the Posner task, with a normal latency of the P3 component during the oddball task. The results of a neurological examination were unremarkable, and a neuropsychological evaluation revealed a decrease in information processing speed and disturbances in episodic memory and the amnesic evocation process. These findings could be explained by her depressed mood. The results of a blood workup were unremarkable.\nA skin biopsy () of the granuloma annulare revealed sweat glands and small nerves with deposition of strongly periodic-acid-Schiff-positive homogeneous, eosinophilic, hyaline-like material and diastase resistant granules around dermal blood vessels, revealing its glycoproteic nature, which is consistent with LP.\nGenomic DNA was purified from blood samples using a salting-out procedure, and stored in TE buffer. ECM1 was amplified by a polymerase chain reaction (PCR) with primers spanning all ten exons and intronic flanking regions. Genomic DNA (100 ng) was amplified in 20-µL reaction volumes containing 20 pmol of primers (), 1.5 nmol of MgCl2, 4 nmol of dNTP, and 1 U of AmpliTaq DNA polymerase (Applied Biosystems, Warrington, UK). The PCR products were separated by 1.5%-agarose-gel electrophoresis and purified with the Qiaquick Gel Extraction Kit (Qiagen, Crawley, UK). Amplified fragments were run and sequenced in an ABI 3130 genetic analyzer.\nSequencing of exon 7 revealed the presence of a single base substitution at position 1076 of cDNA (GenBank accession no. NM_004425.3). The c.1076G>A transversion causes a premature termination codon, p.Trp359*, that affects the Ecm1a isoform. The patient was found to be homozygous for the mutation, while her parents were heterozygous carriers (). No other changes or polymorphisms were found in the ECM1 coding region. The mutation causes the loss of a restriction target for NlaIII endonuclease, which was therefore used as a screening test. The mutation was not present in 106 chromosomes of unrelated control subjects.\nFour different polymorphic dinucleotide microsatellites were analyzed in this study: D1S2344, D1S2345, D1S305, and D1S2624. The primer sequences were obtained from the uniSTS database of the National Center for Biotechnology Information. The sizes of the microsatellites were determined by direct sequencing in the ABI 3130 genetic analyzer.\nThe four markers allowed determination of the segregation phase and the haplotypes associated with the c.1076G>A transversion. The parents' haplotypes differed significantly in the mutated chromosomes, although double recombination events cannot be ruled out (). | [[35.0, 'year']] | F | {'11917104': 1, '18200062': 1, '25045357': 2, '16274456': 1, '15990087': 1, '16512877': 1, '15738681': 1, '16481424': 1, '33441084': 2, '11165938': 1, '28680266': 1, '2155760': 1, '17927570': 1, '10733679': 1, '9367673': 1, '11826121': 1, '27824015': 1, '12472532': 1, '12603844': 1, '24465266': 2} | {'4089944-1': 1, '7805162-1': 1, '7805162-2': 1} |
165,416 | 3896653-1 | 24,465,267 | noncomm/PMC003xxxxxx/PMC3896653.xml | Myasthenia in Acquired Neuromyotonia | A 19-year-old man presented with generalized muscle stiffness and cramps of 5 weeks duration. He also noticed moderate limb weakness, and complained of difficulty climbing stairs, elevating his arms, and handwriting. Repeated exertions relieved the stiffness, but made the weakness worse. He had paresthesia and hyperhidrosis in both hands and feet. Neurological examination revealed mild limb weakness with remarkable fatigability (), diffuse fasciculation, and myokymia. Repetitive nerve stimulation (RNS) of the ulnar nerve at low frequencies revealed marked reductions in compound muscle action potential (CMAP) amplitudes (), but there was neither postexercise facilitation nor abnormal increase at a stimulation frequency of 50 Hz. The findings of a conventional nerve conduction study were normal except for abnormal stimulation-induced afterdischarges (). Needle electromyography revealed varying degrees of neuromyotonic () and myokymic discharges with normal motor unit potentials. Antibody tests against VGKC, acetylcholine (ACh) receptor, and muscle-specific kinase were all negative. Computed tomography of the chest revealed no evidence of thymus abnormalities. All other laboratory tests yielded no other unusual findings.\nWith a presumed diagnosis of seronegative generalized MG superimposed on NMT, this patient was treated with high-dose intravenous immunoglobulin (IVIg) along with oral carbamazepine and a low dose of pyridostigmine bromide (30 mg three times per day). Within 2 days, even before the IVIg treatment was completed, the patient had responded so dramatically that not only the muscle stiffness but also the weakness had improved to a near-premorbid state. However, a follow-up nerve conduction study at that time was not fully consistent with the clinical recovery, revealing the occurrence of repetitive CMAPs (repCMAPs) along with more prominent afterdischarges (). The repCMAPs disappeared after discontinuation of pyridostigmine, but the abnormal afterdischarges persisted in the peroneal and tibial nerves. Abnormal CMAP decrements continued to be present in a follow-up RNS study, but to a lesser degree with larger CMAPs (). The patient continued to receive carbamazepine treatment only, and his clinical improvement was maintained during the next 2 years of follow-up. | [[19.0, 'year']] | M | {'8647024': 1, '33927677': 1, '9689026': 1, '8761268': 1, '16843723': 1, '9553750': 1, '16570308': 1, '6842232': 1, '24465267': 2} | {} |
165,417 | 3897032-1 | 24,516,497 | noncomm/PMC003xxxxxx/PMC3897032.xml | Pityriasis rubra pilaris following exposure to dolomite | A 30-year-old man was referred to St-Alzahra Hospital dermatology clinic, Isfahan, Iran in 2010 because of erythematous, scaly, and slightly itchy eruption all over the body. The eruption had started about 4 months ago from the scalp and progressed to involve other parts of the body in the cephalocaudal pattern.\nIn physical examination, numerous papules with central keratotic pulg were found on the upper chest, upper back, neck, arms, and face [].\nPalms and soles were hyperkeratotic with yellow-orange hue and the nails were hyperkeratotic and thickened [].\nThe patient stated that the lesions developed following contact with dolomite as he was truck driver in a dolomite factory.\nHa had no family history of pityriasis rubra pilaris (PRP) and in past medical history, he revealed no history of the recent infection or fever or use of any medication.\nA clinical diagnosis of PRP was suggested for the patient and a biopsy was performed from the hand.\nIn histological examination, psoriasiform dermatitis along with parakeratosis and orthokeratosis and focal acantholytic changes consistent with PRP was found.\nHe was treated with Oral acitretin (Actavis UK Ltd) (50 mg/day) with minimal improvement and therefore oral cyclosporine (Novartis, UK) (300 mg/day) was added to his regimen.\nAfter 3 months, (about 70%) of his lesions resolved but he returned to his job in dolomite factory and his lesions recurred again.\nThis time, in addition to acitretin and cyclosporine, Narrow Band UVB (starting dose of 50 (mJ/cm2)) was started for the patient and the patient was advised not to have more contact with dolomite. He was visited monthly during treatment course.\nFollowing this treatment protocol, the lesions cleared almost completely during 15 weeks (30 sessions of Narrow band UVB). | [[30.0, 'year']] | M | {'20184391': 1, '2689476': 1, '18833084': 1, '20886435': 1, '24516497': 2} | {} |
165,418 | 3897033-1 | 24,516,498 | noncomm/PMC003xxxxxx/PMC3897033.xml | A significant response to sunitinib in a patient with anaplastic thyroid carcinoma | A 79-year-old man with a medical history of hypertension and diabetes was diagnosed with a localized papillary thyroid carcinoma was admitted at the Ramón y Cajal University Hospital Madrid (Spain). There was no evidence of distant metastasis at diagnosis. The patient underwent a total thyroidectomy and a suspicious right-cervical lymph node was also resected. The pathological stage after surgery was pT2 pN1b M0. Following surgery, thyroid ablation therapy with 150 mCi of radioactive iodine (131I) was given. One year later, although no evidence of distant spread of the disease was observed in a whole body scan, serum thyroglobulin levels were 24 ng/ml (normal range <3 ng/mL), despite suppressive thyroxin therapy. Therefore, a second course of 131I was administered, achieving a total dose of 350 mCi of 131I. Two years after surgery, during a follow-up visit, it was noted that the patient had recurrence of his disease, due to a rapid appearance of a midline hard, rigid, painful, and violet neck mass, just below the scar of previous thyroidectomy, and bilateral cervical lymph nodes [] together with moderate dyspnoea. Macroscopic neck mass size was initially measured as 6.5 × 3.5 cm. Multiple and bilateral lung metastasis were detected in a computer tomography (CT) scan. Fine needle aspiration cytology of the thyroid mass revealed anaplastic thyroid carcinoma cells. Based on recent reported data with multi-target tyrosine kinase inhibitors in iodine-refractory thyroid cancer patients added to the age, performance status, and comorbidities of the patient, we decided to start treatment with sunitinib (SUTENT®, Pfizer Inc, NY) as a single agent under “off-label” use request from a clinical trial. The patient signed the appropriate informed consent and local legal procedures were followed. Sunitinib was administered orally at 50 mg per day for 4 weeks followed by 2 weeks of rest. A weekly follow up was undertaken and at the first week of treatment a reduction in neck tumor bulk was noted (6.0 × 3.0 cm) with clinical improvement of pain and less violet neck mass []. After the start of the treatment for 4 weeks the tumor mass clearly showed a reduction in size (3.0 × 1.5 cm) and skin color was almost normal []. The patient reported neither pain nor dyspnoea at this time and Eastern Cooperative Oncology Group (ECOG) performance status was 0. After the end of second cycle, 12 weeks from the beginning of treatment, a complete macroscopic response of the ATC in the neck was achieved [] and it was maintained for 8 more weeks. Tolerability of sunitinib was acceptable with grade 2 asthenia and grade 2 neutropenia with only one week of rest needed to recover after third cycle. However, response in tumor size over neck mass did not correlate with lung metastasis shrinkage that were stable after 12-20 weeks of treatment. Unfortunately, the patient died as a result of a massive upper gastrointestinal bleeding 5 months after the start of sunitinib treatment and while the patient was still on treatment. | [[79.0, 'year']] | M | {'26680454': 1, '22285864': 1, '32970704': 1, '25276134': 1, '20418080': 1, '18987168': 1, '32055496': 1, '21296855': 1, '23113752': 1, '33292371': 1, '18541897': 1, '22397650': 1, '21081783': 1, '27048161': 1, '19586800': 1, '20847059': 1, '21220477': 1, '32636733': 1, '24516498': 2} | {} |
165,419 | 3897038-1 | 24,497,692 | noncomm/PMC003xxxxxx/PMC3897038.xml | Transitional cell carcinoma with glandular differentiation metastatic to the inguinal lymph node from the urinary bladder | A 58-year-old male patient was admitted to our clinic with complaints of hematuria and right flank pain. Contrast-enhanced computed tomography (CT) revealed diffuse bladder wall thickening and grade 2 hydroureteronephrosis in the right kidney. Transurethral resection of the bladder tumor was reported as a high-grade (G3), muscle-invasive TCC (pT2) transitional cell carcinoma. No metastasis was detected on CT and magnetic resonance imaging (MRI). The patient underwent radical cystoprostatectomy with a Studer ileal bladder substitute for orthotopic diversion with bilateral extended pelvic lymph node dissection. Histopathology confirmed muscle-invasive TCC (pT2) []. Surgical margins were free and lymph node metastasis was not detected. No complications occurred and the right kidney hydroureteronephrosis decreased.\nSix months after cystoprostatectomy, the patient was admitted with complaints of pain in the left inguinal region. Ultrasonography (USG) showed a left inguinal mass 38 cm × 25 cm × 26 mm in diameter. In addition, pelvic MRI showed a mass 1.5 cm in diameter in the left inguinal region. Positron emission tomography (PET) showed increased fluorodeoxyglucose (FDG) uptake in the left inguinal area and the maximum standardized uptake value was 9 (SUV max: 9). Left inguinal lymph node excision was planned but curative resection could not be performed because of dense adhesions to the adjacent vital structures, including the left femoral vein. Excisional biopsy was taken from the left inguinal region which was reported as adenocarcinoma, including transitional cell variants []. The patient underwent gastrointestinal evaluation to search for the primary lesion of adenocarcinoma; however, no primary site could be identified. Carcinoembryonic antigen (CEA) and carbohydrate antigen (CA) 19-9 were within the normal range. Tumor cells were positive for Cytokeratin CK7 [] and negative for CK20. Based on these immunohistochemical findings, it is unlikely that the tumor had originated in the gastrointestinal tract. After four courses of chemotherapy with a combination of cisplatinum, mitomycin-c, etoposide and tegafur-/uracil,[] the metastatic tumor remained unchanged. Radiotherapy was also performed, but achieved minimal remission. | [[58.0, 'year']] | M | {'11942328': 1, '21520635': 1, '11260340': 1, '16977301': 1, '32992094': 1, '9850170': 1, '11668468': 1, '16363710': 1, '16452061': 1, '9354957': 1, '7299915': 1, '12204054': 1, '24497692': 2} | {} |
165,420 | 3897039-1 | 24,497,693 | noncomm/PMC003xxxxxx/PMC3897039.xml | The nested variant of urothelial carcinoma arising in a fibroepithelial polyp: Report of a case and review of literature | A 30-year-old male presented with pain in the hypogastric region. X-rays and ultrasound revealed a stone in the bladder. While removing the stone surgically, a sessile polypoidal mass measuring 1.8 cm was seen at the posterosuperior wall of bladder, which was resected and sent for histopathology. On gross examination, it was a reddish-brown sessile polyp measuring 1.8 cm × 1.5 cm × 1.3 cm, which was submitted entirely for histopathological evaluation. Microscopy revealed a polyp covered by unremarkable epithelium [Figures and ]. The hyalinized vascular stroma was interspersed with many multinucleated bizarre stromal cells []. There were closely packed nests of bland-looking cells extending from the epithelium to the underlying muscularis propria infiltrating in between the smooth muscle and nerve bundles. The cells showed minimal atypia. The base of the polyp showed scattered bizarre stromal cells with occasional nests of the same bland-looking tumor cells.\nSpecial stains including Von Gieson and Masson's trichrome were done, which highlighted the transecting smooth muscle bundles clearly depicting the infiltrating nature of the tumor cells. Immunohistochemisty showed the cells strongly positive for pankeratin (CK AE1/AE3) [] and high molecular weight keratin (34BE12), which confirmed their epithelial nature. The cells were also positive for p53 expression [] and ki 67 index was high (50% approx.), predicting an aggressive course of the tumor. | [[30.0, 'year']] | M | {'1495929': 1, '19800100': 1, '3753860': 1, '15767798': 1, '16799712': 1, '22511434': 1, '12873196': 1, '14681330': 1, '17979494': 1, '32008408': 2, '2712189': 1, '24497693': 2} | {'7113808-1': 1} |
165,421 | 3897040-1 | 24,497,694 | noncomm/PMC003xxxxxx/PMC3897040.xml | A rare occurrence of hepatic portal venous gas in emphysematous pyelonephritis | A 65-year-old hypertensive diabetic female patient developed colicky left flank pain radiating to the groin. Ultrasonography revealed an 8.0 mm mid-ureteric calculus for which she underwent ureterorenoscopic lithotripsy and double-J stenting. The stent was removed after 6 weeks. A week after stent removal, she developed continuous left flank pain, progressive abdominal distension, obstipation, and high-grade continuous fever. A large ovoid left lumbar mass was palpable. She had anemia (hemoglobin 8 g/dL), polymorphonuclear leukocytosis (total leucocyte count 42,600/mm3) and raised blood glucose (400 mg/dL), blood urea (55 mg/dL) and serum creatinine (2.7 mg/dL) levels. Non-contrast computerized tomography (CT) revealed a grossly enlarged left kidney (15.5 cm × 12.0 cm × 19.0 cm) distended with fluid and air. Air was also noted in the peritoneal cavity, retroperitoneum, right inguinal canal and hepatic portal venous radicals [Figures and ]. Retroperitoneal and mesenteric lymphadenopathy was present. A diagnosis of emphysematous pyelonephritis, Wan type 2, was made. Emergency percutaneous nephrostomy drained 1150 ml of pus, which grew Escherichia coli on culture. The patient was managed successfully with central venous pressure-guided intravenous fluids, insulin and broad-spectrum antibiotics. Renal scintigraphy revealed non-functioning left kidney. The patient underwent left subcapsular nephrectomy with uneventful recovery. Histopathology of the resected kidney suggested “end-stage kidney” with chronic pyelonephritis. | [[65.0, 'year']] | F | {'8596845': 1, '19931783': 1, '22567427': 1, '18203942': 1, '10737279': 1, '8254788': 1, '19558985': 1, '23096514': 1, '23526850': 1, '20609107': 1, '19653334': 1, '26715900': 1, '24497694': 2} | {} |
165,422 | 3897041-1 | 24,497,695 | noncomm/PMC003xxxxxx/PMC3897041.xml | Acute pancreatitis and development of pancreatic pseudo cyst after extra corporeal shock wave lithotripsy to a left renal calculus: A rare case with review of literature | A 21-year-old gentleman was referred to us with the history of intermittent left flank pain of 2 weeks duration. He had no previous history of biliary lithiasis, alcohol consumption or abdominal surgeries. Computed tomography (CT) Kidneys, Ureter, Bladder showed a left renal pelvic calculus of 10 mm × 8 mm without any hydronephrosis []. No other abnormality was detected on the CT scan. His blood chemistry and urine analysis were normal. SWL (Dornier Compact Delta, Germany) was performed at 15 kv with 60 shocks per minute for a total of 2700 shock waves, using fluoroscopic guidance.\nApproximately 24 h after SWL, he developed severe pain in the epigastrium and peri-umbilical regions with persistent vomiting. On physical examination, he had tachycardia and was tachypnoeic. Blood pressure was 130/80 mmHg. His abdomen was distended and mild guarding in the epigastrium and periumbilical region was present. On biochemical evaluation, he had an increase in white blood cells count (17 × 103 μ/L), renal and liver function tests were within normal limits. X-ray abdomen and X-ray chest were normal. Ultrasound showed minimal peri-pancreatic fluid collection. Rest of the abdomen was normal. Serum amylase was 1165 μ/L (normal range: 30-110 μ/L) and serum lipase was 8625 μ/L (normal range 15-322). CT scan of abdomen done 48 h after the onset of pain showed features of acute pancreatitis with inflammatory exudate in the peri-pancreatic space, sub-hepatic space and pelvis and minimal left pleural effusion [Figures and ]. There was no evidence of pancreatic necrosis in the contrast enhanced CT scan. Blood and urine culture reports revealed no growth.\nHe was treated conservatively with nasogastric tube insertion, bowel rest, parenteral nutrition, intravenous antibiotics and intravenous somatostatin. His pancreatic enzymes returned to normal within 5 days and follow-up CT scan performed 2 months after the acute episode showed the resolution of features of acute pancreatitis with the formation of pseudo cyst in the body and fail of pancreas measuring 12 cm × 8 cm × 8 cm []. No residual fragment of renal calculus was found in the follow up CT scan. Later he underwent surgery for persistent pseudocyst of pancreas. | [[21.0, 'year']] | M | {'11020904': 1, '34136213': 2, '9225923': 1, '12475688': 1, '10710251': 1, '2731762': 1, '29201688': 1, '9126103': 1, '24497695': 2} | {'8178083-1': 1} |
165,423 | 3897042-1 | 24,497,696 | noncomm/PMC003xxxxxx/PMC3897042.xml | Striated pattern on scrotal ultrasonography: A marker for Non-hodgkins lymphoma of testis | A 52-year-old hypertensive male presented with progressively increasing painless, bilateral scrotal swellings of 6 months duration. Physical examination revealed a cachectic patient with moderately enlarged, uniformly firm testes with loss of testicular sensation on either side. Abdominal examination did not reveal any organomegaly. Basic biochemical investigations were normal. Serum alpha fetoprotein and beta human chorionic gonadotropin were normal. Serum lactate dehydrogenase was minimally elevated.\nUS of the scrotum showed striated pattern of both enlarged testicles []. Increased vascularity was seen in color Doppler study []. Chest X-ray and abdominopelvic computed tomography (CT) scan were normal. He underwent bilateral high inguinal orchidectomy. On the third post-operative day, he became unconscious and CT scan of the brain showed evidence of metastasis with mass effect. The patient expired on the same day.\nHistopathological examination and immunohistochemical staining (CD20 +ve, CD79 +ve, CD3 −ve, CD5 −ve) confirmed bilateral B-cell type NHL. | [[52.0, 'year']] | M | {'2036761': 1, '10650105': 1, '11906874': 1, '8827805': 1, '8648792': 1, '24497696': 2} | {} |
165,424 | 3897043-1 | 24,497,697 | noncomm/PMC003xxxxxx/PMC3897043.xml | Magnetic resonance-retrograde pyelography: A novel technique for evaluation of chyluria | A 35-year-old woman with refractory chyluria underwent Gd-RGP-MRI using 1.5 T MR Scanner (Signa; GE Healthcare, Milwaukee, USA). Coronal fat suppressed T2 and T1 sequences were taken before instillation of 2.5 ml of gadolinium MR contrast (0.5 mmol MultiHance; Bracco, Milan, Italy) mixed in 5.0 ml of normal saline through the ureteric catheter. Post-gadolinium injection abdominal angiography sequences were acquired and maximum intensity projection (MIP) images were reconstructed []. | [[35.0, 'year']] | M | {'18601756': 1, '21161658': 1, '34584798': 2, '28978586': 1, '32685073': 1, '20842157': 1, '8420087': 1, '15328382': 1, '26150622': 1, '24497697': 2} | {'8457537-1': 1} |
165,425 | 3897063-1 | 24,497,691 | noncomm/PMC003xxxxxx/PMC3897063.xml | Cutaneous metastasis from testicular germ cell tumour | A 50-year-old male presented to us in August 2004 with a history of left-sided testicular swelling. On examination, there was a hard 10 × 8 cm left-sided testicular lump with a scar of a previous testicular biopsy on the skin of the left side of the scrotum. Baseline levels of serum lactate dehydrogenase (LDH), alfa fetoprotein (AFP) and beta subunit of human chorionic gonadotropin (beta-HCG) were within normal limits. Chest X-ray was normal and ultrasonography of the abdomen revealed para-aortic lymphadenopathy. He underwent a left high inguinal orchidectomy and left hemiscrotectomy. The histopathology was non-seminomatous malignant mixed germ cell tumor consistent with embryonal carcinoma (97%) with element of teratocarcinoma (3%). Following this, he completed four cycles of adjuvant chemotherapy with Cisplatin (20 mg/m2/day: Days 1-5) and Etoposide (100 mg/m2/day: Days 1-5). Bleomycin was not given due to the previous history of smoking. Subsequent evaluation showed residual para-aortic lymphadenopathy, for which the patient was advised surgery. However, he defaulted treatment and was lost to follow-up.\nSix years later, he presented in November 2010 with a 2-month history of an ulceroproliferative growth over the shin of the right lower limb. On examination, there was an ulceroproliferative growth 3 cm in diameter with satellite lesions over the shin []. Biopsy of this lesion revealed a metastatic malignant germ cell tumor. Further evaluation showed normal levels of tumor markers (LDH, AFP and beta-HCG), with no evidence of intraabdominal or intrathoracic disease. He received three cycles of salvage second-line chemotherapy with Vinblastine (0.11 mg/kg on Days 1 and 2) Ifosfamide (1200 mg/m2/day on Days 1-5) and Cisplatin (20 mg/m2/day on Days 1-5). There was reduction in size of the lesion and he underwent wide local excision of the tumor and skin grafting []. The surgical biopsy was reported as metastatic malignant germ cell tumor. Because the tumor was close to the deep resection margin, he received adjuvant local external radiotherapy to the tumor bed (66 Gy in 33 fractions). He was asymptomatic and was subsequently on regular three-monthly follow-up till May 2012, when he presented with hoarseness of voice. Computed tomography (CT) of the thorax showed a large 4 × 5 cm mediastinal nodal mass with multiple lung metastases. CT-guided biopsy of the mediastinal nodal mass was showed a malignant germ cell tumor with yolk sac component (Pan Cytokeratin and AFP positive on immunohistochemistry) and tumor markers (LDH, AFP and beta-HCG) were within normal limits. He has completed six cycles of salvage third-line chemotherapy with Carboplatin (AUC 5, on Day 1), Paclitaxel (175 mg/m2 on Day 1) and Gemcitabine (1000 mg/m2 on Days 1 and 8), and the repeat CT of the thorax showed minimal decrease in the size of mediastinal lymphadenopathy and lung nodules. He is currently on best supportive care. | [[50.0, 'year']] | M | {'12506179': 1, '17290059': 1, '18006893': 1, '8465631': 1, '15186430': 1, '9053482': 1, '1703917': 1, '7537800': 1, '8275469': 1, '8989940': 1, '30249738': 1, '32015625': 1, '15223965': 1, '23032847': 1, '24497691': 2} | {} |
165,426 | 3897079-1 | 24,497,866 | noncomm/PMC003xxxxxx/PMC3897079.xml | Duplication of the inferior vena cava and thrombosis: A rare case | Our patient was an 18-year-old girl with recent onset right leg pain and swelling with extension to her inguinal area. She also had dyspnea with respiratory distress. At the time of presentation, she was ill and febrile. She also had pitting edema of right leg and significant size difference between circumferences of her two legs. On the day of admission, she suddenly presented with dyspnea and severe thoracic pain on the right side. All other physical examination was normal.\nShe had no predisposing factor for venous thrombosis. Besides, she had no known a history of DVT, clotting disorders, recurrent abortion, peripheral vascular disease, non-healing ulcerations on the extremities, cerebrovascular accidents or taking oral contraceptive pills.\nHer laboratory data showed only mild normochromic, normocytic anemia, elevated erythrocyte sedimentation rate (ESR) to 75 (normal range less than 25) and CRP = 16 (normal less than 8). Other laboratory test including renal, electrolytes, liver, and urine analysis and culture were normal.\nColor duplex ultrasound study demonstrated deep venous thrombi in the right popliteal, superficial and common femoral veins, with external iliac vein.\nIn addition, hypercoagulable workup was revealed no abnormal finding and also negative for antiphospholipid syndrome and lupus anticoagulant antibodies.\nA multi-detector computed tomography (CT) pulmonary angiography of the chest defined pulmonary emboli in the territory of right main and segmental branch pulmonary artery and suggesting extensive infarction of the right middle pulmonary lobe. She was subsequently started on a low molecular weight heparin (Clexane®: 60 mg, every 12 h SC). Subsequently, CT of the abdomen and CT angiography with IV contrast injection carried out after the procedure confirmed duplication of the IVC []. Both two IVC originate in the pelvis, run parallel to each other, bordering the aorta and left-sided IVC drained into the left renal vein. There was no other pathologic finding in thoracic and abdominopelvic CT studying.\nAfter fully anticoagulated with low molecular weight heparin for 5 days and starting warfarin international normalized ratio (INR) range was between 2 and 3, she was discharged from hospital and was not symptomatic in her follow-up until next 3 months. According to the previous studies, for this patient we planned to continue anticoagulant therapy for 6 months.[] | [[18.0, 'year']] | F | {'19055711': 2, '16034513': 1, '30619987': 1, '34061818': 1, '12591684': 1, '11564079': 1, '19551299': 1, '22082518': 1, '24497866': 2} | {'2637295-1': 1} |
165,427 | 3897080-1 | 24,497,867 | noncomm/PMC003xxxxxx/PMC3897080.xml | Cervicoisthmic choriocarcinoma mimicking cesarean section scar ectopic pregnancy | In August 2012, a 30-year-old Chinese woman was admitted to Pescia Hospital for irregular vaginal bleeding of 10 days duration and sovrapubic pain.\nShe was gravida 4 para 2. Her first and third pregnancy ended in full term delivery by cesarean section (C-section) at 40 and 39 gestational week respectively. Her second and last pregnancy ended in the first trimester abortion in 2007 and 2010 respectively.\nAfter 17 weeks of amenorrhea, serum beta human chorionic gonadotropin (β-hCG) was 40796 mUI/ml. Trans-vaginal ultrasound scan failed to identify an intrauterine pregnancy and showed the presence of an extremely vascularized cervical mass, localized in the isthmic region of the cervix, just over the scar of the previous C-section. The patient was considered to have an ectopic cesarean scar pregnancy and then was referred to our clinic, Careggi University Hospital as a third level hospital, for a better diagnostic and therapeutically assessment.\nWe repeated trans-vaginal ultrasound scan that confirmed the diagnosis [Figures and ]. Serum β-hCG level continued to grow reaching the value of 60,000 mUI/dl in 3 days. The patient had no any other symptoms than little vaginal bleeding.\nSince, there is no standard protocol in the treatment of cesarean scar pregnancy, we discussed with the patient three treatment options: Expectant management, medical therapy or conservative surgical therapy. The decision fell on surgery and the patient underwent explorative laparotomy. The cervical lesion appeared as a large, friable highly vascularized mass that occupied the whole thickness of cervical wall. Intra-operative conversion to hysterectomy became necessary since the unfeasibility of conservative uterine surgery. The patient made an uneventful recovery. She was discharged on postoperative day 4, with serum β-hCG of 4885 mUI/ml.\nDefinitive pathologic evaluation of surgical specimen led to the diagnosis of: Primary gestational cervical choriocarcinoma with the presence of syncitiotrophoblastic and cytotrophoblastic cells [Figures and ]. Imunohistochemical staining was positive for β-hCG.\nNo metastases were detected by post-surgical whole-body computed tomography (CT).\nThe FIGO (Fédération Internationale de Gynécologie et d’Obstétrique) 2000 criteria showed a stage I choriocarcinoma (confined to the uterus) with a FIGO score of 8:[] Abortion before choriocarcinoma (1 point), delay between pregnancy and choriocarcinoma of over 12 months (4 points), and β-hCG blood level of between 10,000 and 100,000 (2 points), largest tumor size of 3, 7 cm (1 point).\nAccording to the FIGO classification, the patient began a combination chemotherapy using etoposide, methotrexate, actinomycin D, alternating with cyclophosphamide and oncovine (EMA-CO).\nAfter the first cycle of chemotherapy, the serum β-hCG decreased to normal range (<5 mUI/dl). Eight weeks after the β-hCG level was still negative and treatment ended with the third cycle of EMA-CO.\nOne month later whole body CT scan revealed no abnormality. | [[30.0, 'year']] | F | {'27803848': 1, '28352705': 1, '12065144': 1, '32368549': 2, '28099907': 1, '18649986': 1, '34794477': 1, '13678743': 1, '9038276': 1, '32883223': 2, '22696813': 1, '19260253': 1, '8455223': 1, '17309671': 1, '24497867': 2} | {'7190942-1': 1, '7469604-1': 1, '7469604-2': 1} |
165,428 | 3897091-1 | 24,459,336 | noncomm/PMC003xxxxxx/PMC3897091.xml | Microsurgical reconstruction of major scalp defects following scalp avulsion | A 27 year old female presented with total scalp avulsion (stage III defect) and intact periosteum. We did skin grafting immediately. Early post operative results were good with 100% graft take []. | [[27.0, 'year']] | F | {'6498399': 1, '10672308': 1, '8996993': 1, '20390420': 1, '26557946': 1, '5778460': 1, '358230': 1, '8468702': 1, '4551236': 1, '16482784': 1, '8628792': 1, '34912678': 1, '708984': 1, '34307094': 1, '643955': 1, '16163072': 1, '6703607': 1, '940866': 1, '14732639': 1, '1090960': 1, '7122753': 1, '24459336': 2} | {'3897091-2': 2, '3897091-3': 2, '3897091-4': 2, '3897091-5': 2} |
165,429 | 3897091-2 | 24,459,336 | noncomm/PMC003xxxxxx/PMC3897091.xml | Microsurgical reconstruction of major scalp defects following scalp avulsion | A 30 year old lady presented late (after 30 days) with bare skull after scalp avulsion injury. Dead and necrotic calvarium was removed and reconstruction was done using omental free flap with STSG. There were no major complications in the post operative period apart from post operative ileus and partial necrosis of the flap []. | [[30.0, 'year']] | F | {'6498399': 1, '10672308': 1, '8996993': 1, '20390420': 1, '26557946': 1, '5778460': 1, '358230': 1, '8468702': 1, '4551236': 1, '16482784': 1, '8628792': 1, '34912678': 1, '708984': 1, '34307094': 1, '643955': 1, '16163072': 1, '6703607': 1, '940866': 1, '14732639': 1, '1090960': 1, '7122753': 1, '24459336': 2} | {'3897091-1': 2, '3897091-3': 2, '3897091-4': 2, '3897091-5': 2} |
165,430 | 3897091-3 | 24,459,336 | noncomm/PMC003xxxxxx/PMC3897091.xml | Microsurgical reconstruction of major scalp defects following scalp avulsion | A 32 year old lady presented with total scalp loss with bare skull bone. The patient also had fractured right forearm bones and mild pleural effusion right side. After management of associated trauma, patient was taken for scalp reconstruction. After removal of necrotic calvaria, LDSA flap with skin paddle based on a cutaneous branch arising from thoracodorsal-subscapular trunk composite free flap was done. As arterial flow was found to be low in superficial temporal vessels, pedicle was anastomosed to facial vessels using vein graft, tunneled at preauricular region. Flap healed well in the postoperative period with stable cover []. | [[32.0, 'year']] | F | {'6498399': 1, '10672308': 1, '8996993': 1, '20390420': 1, '26557946': 1, '5778460': 1, '358230': 1, '8468702': 1, '4551236': 1, '16482784': 1, '8628792': 1, '34912678': 1, '708984': 1, '34307094': 1, '643955': 1, '16163072': 1, '6703607': 1, '940866': 1, '14732639': 1, '1090960': 1, '7122753': 1, '24459336': 2} | {'3897091-1': 2, '3897091-2': 2, '3897091-4': 2, '3897091-5': 2} |
165,431 | 3897091-4 | 24,459,336 | noncomm/PMC003xxxxxx/PMC3897091.xml | Microsurgical reconstruction of major scalp defects following scalp avulsion | A 35 year old lady presented to us with scalp avulsion injury from thresher machine. Scalp was reconstructed with latissimus dorsi muscle with STSG, but flap necrosed because of venous thrombosis. The patient again planned for surgery and LDSA with skin paddle, a composite free flap was done. This time vein was anastomosed with external jugular vein using vein graft tunneled at the preauricular region. There were no postoperative complication and flap recovery was uneventful []. | [[35.0, 'year']] | F | {'6498399': 1, '10672308': 1, '8996993': 1, '20390420': 1, '26557946': 1, '5778460': 1, '358230': 1, '8468702': 1, '4551236': 1, '16482784': 1, '8628792': 1, '34912678': 1, '708984': 1, '34307094': 1, '643955': 1, '16163072': 1, '6703607': 1, '940866': 1, '14732639': 1, '1090960': 1, '7122753': 1, '24459336': 2} | {'3897091-1': 2, '3897091-2': 2, '3897091-3': 2, '3897091-5': 2} |
165,432 | 3897091-5 | 24,459,336 | noncomm/PMC003xxxxxx/PMC3897091.xml | Microsurgical reconstruction of major scalp defects following scalp avulsion | A 40 year old lady presented with scalp avulsion injury from a thresher machine. Scalp reconstruction was done using LDSA flap along with skin paddle. There were no postoperative complications []. | [[40.0, 'year']] | F | {'6498399': 1, '10672308': 1, '8996993': 1, '20390420': 1, '26557946': 1, '5778460': 1, '358230': 1, '8468702': 1, '4551236': 1, '16482784': 1, '8628792': 1, '34912678': 1, '708984': 1, '34307094': 1, '643955': 1, '16163072': 1, '6703607': 1, '940866': 1, '14732639': 1, '1090960': 1, '7122753': 1, '24459336': 2} | {'3897091-1': 2, '3897091-2': 2, '3897091-3': 2, '3897091-4': 2} |
165,433 | 3897098-1 | 24,459,343 | noncomm/PMC003xxxxxx/PMC3897098.xml | Facial contour deformity correction with microvascular flaps based on the 3-dimentional template and facial moulage | A 19-year-old girl presented with progressive flattening of the right side of the face in the region of cheek, posterior mandibular region and upper lip. The progressive deformity started at the age of 8 years and become stable 3 years prior to the surgery. She had mild deviation of the chin to the ipsilateral side without occlusal cant. Assessment of the deformity and planning was done with the facial moulage. Free de-epithelialised groin flap from the opposite side was used for the reconstruction. Submandibular approach was used for pocket creation and anastomosis. Donor site was closed primarily. Patient had uneventful post-operative and was discharged on 20th day. Patient was under follow-up for 48 months and further augmentation of the upper lip with dermal-fat graft was done [Figures –]. | [[19.0, 'year']] | F | {'15252256': 1, '33456697': 1, '8175994': 1, '16808566': 1, '18708310': 1, '4011779': 1, '2249386': 1, '16217457': 1, '18537169': 1, '29922550': 1, '2762408': 1, '18520863': 1, '8559818': 1, '366052': 1, '5059334': 1, '11910221': 1, '8911463': 1, '20856027': 1, '9924400': 1, '24459343': 2} | {'3897098-2': 2, '3897098-3': 2, '3897098-4': 2, '3897098-5': 2} |
165,434 | 3897098-2 | 24,459,343 | noncomm/PMC003xxxxxx/PMC3897098.xml | Facial contour deformity correction with microvascular flaps based on the 3-dimentional template and facial moulage | A 21-year-old girl presented with unilateral deformity of the left side of the face. She and her parents were extremely anxious about the progressive flattening of the face. On examination, she was found to have significant soft-tissue atrophy of the malar and zygomatic regions on the left side of the face. There was also thinning of the upper lip with excessive show of dentition. The soft-tissue correction was done with de-epithelialised radial forearm flap. The flap was chosen based on the required tissue thickness of the template. The donor site needed a skin graft for the closure. During the 5th post-operative day, haematoma developed between the flap and skin causing marginal loss of the overlying skin of the nasolabial region, which was debrided and sutured secondarily. Flap vascularity was undisturbed. This also necessitated minor soft-tissue revisions at follow-up. At 3 year follow-up, the flap had settled well with good contour correction [Figures –]. | [[21.0, 'year']] | F | {'15252256': 1, '33456697': 1, '8175994': 1, '16808566': 1, '18708310': 1, '4011779': 1, '2249386': 1, '16217457': 1, '18537169': 1, '29922550': 1, '2762408': 1, '18520863': 1, '8559818': 1, '366052': 1, '5059334': 1, '11910221': 1, '8911463': 1, '20856027': 1, '9924400': 1, '24459343': 2} | {'3897098-1': 2, '3897098-3': 2, '3897098-4': 2, '3897098-5': 2} |
165,435 | 3897098-3 | 24,459,343 | noncomm/PMC003xxxxxx/PMC3897098.xml | Facial contour deformity correction with microvascular flaps based on the 3-dimentional template and facial moulage | A 21-year-old male presented with progressive unilateral deformity of the right side of the face and asymmetric chin with deviation. He was concerned about the flattened appearance of the malar and zygomatic regions on the right side of the face. Owing to this, he suffered low self-esteem and had led to discontinuation of education. He underwent an augmentation of the right side of the face with an ALT flap with additional genioplasty to correct the chin asymmetry. He had an uneventful recovery. Pressure garments were used for 6 months. At 17 months follow-up, the correction was stable, which significantly restored self-confidence and he resumed attending classes [Figures –]. | [[21.0, 'year']] | M | {'15252256': 1, '33456697': 1, '8175994': 1, '16808566': 1, '18708310': 1, '4011779': 1, '2249386': 1, '16217457': 1, '18537169': 1, '29922550': 1, '2762408': 1, '18520863': 1, '8559818': 1, '366052': 1, '5059334': 1, '11910221': 1, '8911463': 1, '20856027': 1, '9924400': 1, '24459343': 2} | {'3897098-1': 2, '3897098-2': 2, '3897098-4': 2, '3897098-5': 2} |
165,436 | 3897098-4 | 24,459,343 | noncomm/PMC003xxxxxx/PMC3897098.xml | Facial contour deformity correction with microvascular flaps based on the 3-dimentional template and facial moulage | A 26-year-old lady presented with unilateral contour deformity of the left side of the face. She was extremely worried about the infraorbital depression and the flattening of her left side of the face. On examination in addition to the flattening, the skin was hyperpigmented and a coup-de-sabre was seen extending along the nasolabial fold, corner of the mouth and chin. Patient was closely followed for 1 year prior to the surgery for any progression of the deformity. Upon ensuring non-progression of the disease she underwent a soft-tissue augmentation with an ALT flap. The flap was extended across the midline beneath the lower lip. Her post-operative course was uneventful. Although she achieved good contour correction, hyperpigmentation persisted possibly due to atrophy of the overlying skin [Figures –]. | [[26.0, 'year']] | F | {'15252256': 1, '33456697': 1, '8175994': 1, '16808566': 1, '18708310': 1, '4011779': 1, '2249386': 1, '16217457': 1, '18537169': 1, '29922550': 1, '2762408': 1, '18520863': 1, '8559818': 1, '366052': 1, '5059334': 1, '11910221': 1, '8911463': 1, '20856027': 1, '9924400': 1, '24459343': 2} | {'3897098-1': 2, '3897098-2': 2, '3897098-3': 2, '3897098-5': 2} |
165,437 | 3897098-5 | 24,459,343 | noncomm/PMC003xxxxxx/PMC3897098.xml | Facial contour deformity correction with microvascular flaps based on the 3-dimentional template and facial moulage | A 21-year-old lady presented with left sided contour deformity in the pre-auricular, cheek, posterior mandibular region with swaying of the jaw towards left. She underwent resection of the condyle along with soft-tissue 3 years prior for the sarcoma of the mandible condyle. After CT scan imaging bony and soft-tissue defect was defined. The bony defect of 3 cm was reconstructed with vascularised fibula and soft-tissue augmented with 6 cm × 5 cm de-epithelialised cutaneous paddles. The donor defect was closed primarily. The depressed contour and chin deviation on mouth opening was corrected restoring the symmetry. Post-operative course was uneventful [Figures –]. | [[21.0, 'year']] | F | {'15252256': 1, '33456697': 1, '8175994': 1, '16808566': 1, '18708310': 1, '4011779': 1, '2249386': 1, '16217457': 1, '18537169': 1, '29922550': 1, '2762408': 1, '18520863': 1, '8559818': 1, '366052': 1, '5059334': 1, '11910221': 1, '8911463': 1, '20856027': 1, '9924400': 1, '24459343': 2} | {'3897098-1': 2, '3897098-2': 2, '3897098-3': 2, '3897098-4': 2} |
165,438 | 3897106-1 | 24,459,351 | noncomm/PMC003xxxxxx/PMC3897106.xml | Double flap from amputated opposite lower limb for cover of plantar and dorsal surfaces of a crushed foot | A 45 year old male patient who was run over by a train resulting in a right leg amputation at the level of the knee and a crush injury of the left foot. He was brought to our hospital about 2 h after the accident. The right lower limb had a severe comminution and bone loss at the knee joint, with the loss of skin and soft- tissue and crushing of muscle above and below the knee [Figures and ]. The left forefoot was completely degloved and all the toes were crushed and degloved as well [Figures and ].\nThe right lower limb was deemed not replantable as the knee joint was severely damaged and not salvageable, In addition, debridement of crushed and devitalized tissues would result in a 15-20 cm shortening and a limb that was at least 15 cm short with fused knee joint would not be functionally useful and primary insertion of prosthetic knee joint was not considered to be feasible by the attending orthopaedic surgeon.\nFocus was then shifted to the crushed left foot with a view to perform immediate debridement and early soft-tissue cover, to salvage as much of the foot as possible and get it fully healed and weight bearing at the earliest. Stable and sensate skin cover were vital, as this would be the only surviving foot. A large defect such as this would require a large distant flap or even two flaps. Best replacement for skin on the foot was anatomically identical skin from the opposite foot, which in this case was provided by the well-preserved amputated limb. The general condition of the patient was stable and he had no other life-threatening injuries. Immediate double free tissue transfer from the amputated limb was done. A plantar flap based on the posterior tibial vessels [] and a dorsal flap based on the dorsalis pedis vessels [], with the communication between the dorsal and plantar systems left intact through the deep branch of the 1st dorsal metatarsal artery. Satisfactory inset of dorsal flap [], and of plantar flap [] was achieved. Post-operatively the patient developed a hematoma below the dorsal flap. Since flaps were harvested from the amputated limb, small blood vessels that could not be seen were probably left unligated. Re-exploration and evacuation of hematoma was performed. Both flaps survived completely [Figures and ]. | [[45.0, 'year']] | M | {'1564265': 1, '8289646': 1, '3399566': 1, '7208703': 1, '25408784': 1, '3763731': 1, '18580505': 1, '27833281': 1, '8479984': 1, '9234885': 1, '6693746': 1, '10490181': 1, '24459351': 2} | {} |
165,439 | 3897107-1 | 24,459,352 | noncomm/PMC003xxxxxx/PMC3897107.xml | Short-term augmentation of venous drainage with extra-corporeal shunt and simultaneous auto-transfusion, for salvaging a congested free flap | A 48-years-old male presented with history of road traffic accident. He had sustained head injury, fracture of right femur and Gustillo type IIIc fracture of both bones right leg. He had anterior tibial artery injury and extensive crushing of anterior compartment muscles along with a soft tissue defect spanning across upper and middle thirds of leg and extending into the upper part of lower third of leg [Figure –]. Associated head injury and patient's hemodynamic instability did not allow early surgical intervention. As the management of wound was difficult due to multiple unstable fractures, after 48 hours of injury he was taken up for a short procedure of fracture fixation. His fractures were stabilised with external fixator, spanning across the knee. Soft tissue cover was not possible at this stage because of his general condition.\nAfter getting clearance from neurosurgery and the intensive care specialists, he was taken up for elective micro-vascular free tissue transfer on the fourth post injury day. On exploration of the wound, posterior tibial artery was found intact but both accompanying deep veins of both anterior and posterior tibial vessels were found thrombosed. The short saphenous vein was injured; the great saphenous vein (GSV) was patent and uninjured. Gentle irrigation with heparin saline into the vein (GSV) was possible without much resistance and the intima appeared healthy under microscope, hence it was selected as recipient vein for anastomosis.\nAfter confirming the recipient vessels, Vastus lateralis myo-cuteneous flap of dimensions 30 × 15 cm was harvested from the left thigh and was transferred to recipient site for coverage of the fracture site. After partial insetting, anastomosis was performed. The artery was anastomosed to the posterior tibial artery in an end to side fashion with 9/0 nylon. One of the flap veins was anastomosed to the great sephanous vein in an end to end manner. Due to non-availability of recipient veins in the vicinity, the second flap vein was clipped at the end of the procedure after confirming good flow. In setting was completed and good bleeding was observed from the flap at the end of the surgery. The patient was shifted to postoperative ward.\nAn hour after the surgery, the flap became congested. Clinically, it was evident by brisk and congested bleeding on scratching the flap with needle. The flap was explored immediately suspecting some compromise in the venous flow. On exploration, after exposing the anastomotic site, the vein anastomosis was found patent. Both the anastomosed flap vein and the GSV were distended and tense. The ligated second vein was also found to be tense and distended []. The clip at the end of the second vein was released to let out some blood, in order to decongest the flap. This manoeuvre improved the flap colour and bleeding after some time. The flap drainage through a single vein was probably inadequate, so nearby areas were re-explored for another recipient vein. We could not find a suitable vessel for the second anastomosis.\nAs there was no possibility of getting a second vein for anastomosis, it was decided to let the blood out periodically to decongest the flap. The blood flow through the second vein was checked and was found to be very high (22 ml/min); letting out so much blood in a patient with multisystem injury was not a suitable option. Collecting the blood and then transfusing it was another option but had risk of contamination and sepsis. After carefully considering all the options and weighing the risks, it was decided to drain the blood through an IV catheter and auto-transfuse it to the patient simultaneously. The idea was to create a closed system with lesser risk of contamination. One IV cannula was inserted over patients forearm and the other one in the second vein of the flap through the edge of the flap. In the leg the flap vein was ligated over the cannula under appropriate pressure with 7/0 polypropylene, also the position of cannula was secured over the skin so as to prevent it from accidently getting dislodged. The cannulas were connected with an extension tubing (100 cm) as shown in . A three way system was attached at the proximal end of the extension tube through which heparin (5000U in 500 ml of normal saline) infusion was started at a rate of 20 micro-drops per minute to keep the extension tube patent [].\nThe drainage of the flap vein through the external shunt was checked every 2 hours as shown in the accompanying video []. The patient was also quite comfortable with this position and was even able to take his hand to the mouth. Considering the fact that cannulating the flap vein and transfusion of blood from the site of injury directly into systemic circulation might lead to sepsis, stringent wound hygiene was maintained. As a precautionary measure the venous cannula draining the flap vein was changed once, after 3 days of drainage. By seventh day the flap developed enough collateral venous drainage and there was no congestion on blocking the venous cannula. At this time the drainage was stopped and the second vein was ligated.\nThe flap survived, except a small area of necrosis at the distal edge []. The final insetting was completed after 2 weeks. The distal necrotic portion was debrided and the residual raw area was managed with VAC and was covered with skin graft at a later date. Complete wound healing was achieved in 4 weeks []. | [[48.0, 'year']] | M | {'24501461': 1, '19790180': 1, '9727436': 1, '11083558': 1, '2201050': 1, '4083725': 1, '8942909': 1, '17572562': 1, '21437862': 1, '18241999': 1, '15074725': 1, '11207131': 1, '2648429': 1, '2249394': 1, '24459352': 2} | {} |
165,440 | 3897108-1 | 24,459,353 | noncomm/PMC003xxxxxx/PMC3897108.xml | Touraine Solente Gole syndrome: The elephant skin disease | A 27-year-old male presented with complaints of progressive thickening of facial skin for past 5 years. Thickened forehead skin subsequently developed deep transverse furrows []. Associated broadening of fingers and toes with painless clubbing of nails were noticed []. Recurrent episodes of pain and swelling over knee and ankle joints had occurred over the past 1 year, for which he was prescribed steroid and non-steroidal anti-inflammatory medications causing symptomatic relief. There were no significant complaints pertaining to cardiac respiratory or gastrointestinal systems, any history of fever or recent weight loss. No other member of his family exhibited similar complaints.\nOn examination, the patient showed coarse facial features with increased facial skin thickness, seborrhoeic hyperplasia, deepening of nasolabial folds and furrowing of forehead skin into multiple transverse folds. Scalp skin showed furrows oriented parallel to the sagittal plane, with normal hair distribution and no fixity of skin to the underlying calvarium [].\nOn investigation, his haemogram, blood counts, erythrocyte sedimentation rate, peripheral smear, platelet counts, liver and renal function tests and serum electrophoresis were within the normal limits. Serum uric acid value was 4.8 mg/dl. Anti-nuclear antibody and rheumatoid factor were negative, with elevated C reactive protein value of 41.6 mg/L. Human growth hormone assay showed 1.34 ng/ml using chemiluminescence immunoassay method. Age and sex matched insulin-like growth factor 1 level was also within normal limits.\nX-rays of long bones showed minimal skeletal changes with periosteal reaction involving diaphysis of fibula, without any evidence of joint effusions []. Magnetic resonance imaging showed diploic space enlargement in calvarium indicating marrow hyperplasia []. Sella turcica and pituitary glands were visualised normally. Skin biopsy showed increased pilosebaceous structures and minimal increase in perivascular and periappendageal inflammatory infiltration. Bone marrow biopsy showed minimal erythroid hyperplasia.\nA frontal rhytidectomy through a browlift incision, with dissection into forehead in the subgaleal and subfrontalis planes was done. A spindle shaped excision of scalp skin and frontalis muscle behind hairline was performed. Skin was sutured without excessive tension, frontalis muscle was repaired with tacking sutures to periosteum. This procedure caused considerable improvement in his forehead furrows, though he is expected to require revision surgeries in future with progression of the disease []. | [[27.0, 'year']] | M | {'21046885': 1, '1623675': 1, '14264474': 1, '30847204': 2, '28112140': 2, '24459353': 2} | {'5322714-1': 1, '6389490-1': 1} |
165,441 | 3897109-1 | 24,459,354 | noncomm/PMC003xxxxxx/PMC3897109.xml | Total upper and lower eyelid reconstruction using deltopectoral flap | A 24 year-old woman, who had undergone a right orbital exenteration with total upper and lower eyelid excision for orbital haemangioma, presented to us for reconstruction of eyelids, after 3 weeks of the above procedure. At presentation right socket was covered with healthy granulation tissue []. At the first stage STSG done to cover the raw area of right orbital socket []. At 2nd stage 3 weeks after the first procedure delay of DP was done beyond DP groove on the right side. The dimension of the flap was 24 cm × 8 cm. 1 week later DP was raised; inset given to skin margin at upper orbital rim and inner surface of the flap at the distal part, which forms upper and lower eyelid in the future, is covered with STSG []. The donor area covered with STSG. 3 weeks later flap division was done and lower inset of the given to skin margin at lower orbital rim [] and part of proximal flap after division is returned to donor area. 3 weeks later flap is divided into horizontally in centre to form upper and lower eyelids. The palpebral aperture was 15 mm, whereas the cavity inside was approximately 32 mm at the end of the procedure []. In co-ordination with prosthodontic department ocular prosthesis was made and fitted into ocular defect []. Spectacles were used to camouflage the scarred tissue. We are publishing the results after 2 years follow-up []. | [[24.0, 'year']] | F | {'4940060': 1, '11932593': 1, '729342': 1, '10654746': 1, '24987223': 1, '10594607': 1, '11176644': 1, '3966090': 1, '1924577': 1, '24459354': 2} | {} |
165,442 | 3897111-1 | 24,459,356 | noncomm/PMC003xxxxxx/PMC3897111.xml | Electrical burns of the abdomen | A 35-year-old male farmer came in contact with 11,000 volts high tension electric wire while irrigating his field. This patient was initially admitted to general surgery ward from main casualty for management as he was having open abdominal wound and was managed there for initial 3 days. He had full thickness burn wounds over the right scapula, right upper limb and anterior abdominal wall along with perforation of the intestine. There was prolapse of the intestinal loops through abdominal wound. Patient was referred to our plastic surgery unit after 3 days. He presented to us in septic shock with an abdominal drain in place. Details of the preliminary management were unavailable except mention of putting in catheter drain through the open abdominal wounds. There was necrosis of burned skin and muscles over the right shoulder and abdomen []. Patient was initially managed conservatively with intravenous fluids, parenteral nutrition, barrier dressing of the abdominal wound and culture specific antibiotics. Patient responded well to the conservative management and once he was stable, a thorough debridement of the necrotic skin over the right shoulder and upper arm was performed. The area was then split skin grafted. The patient developed enterocutaneous fistula, which healed over a period of 8 weeks. The wound over the abdomen was also covered with skin graft and patient was discharged after 18 days [].\nAbout 4 months after the discharge, the patient presented with ventral hernia and requested reconstruction of the abdominal wall. Repair of ventral hernia by synthetic mesh application and reconstruction of the abdominal wall with flap cover was planned. Per-operatively the abdominal skin graft was separated from the peritoneum and the underlying omentum and an inlay composite mesh was applied to secure the abdominal wall []. The graft was not de epithelialised. It was carefully separated from the peritoneum over the bowels. No perforation occurred. A free tensor fascia lata flap was applied over the mesh, but the flap failed. Then after debridement two random pattern transposition skin flaps, one from the right upper and another from the left lower abdomen were transposed over the abdominal wound and donor area was skin grafted []. Patient was discharged after 17 days with advice to wear an abdominal binder. He is presently in follow-up, it has been 18 months since surgery and there is no ventral hernia and abdominal wall is flat and intact. Patient is satisfied with the results [Figures and ]. | [[35.0, 'year']] | M | {'7880427': 1, '26807397': 1, '3779475': 1, '17161540': 1, '6263197': 1, '34585274': 2, '3886090': 1, '24459356': 2} | {'8479039-1': 1} |
165,443 | 3897146-1 | 24,497,716 | noncomm/PMC003xxxxxx/PMC3897146.xml | Aromatase excess syndrome presenting with prepubertal gynecomastia in an Egyptian child with type 1 neurofibromatosis | A 4-year-old Egyptian boy was referred to our clinic for evaluation of bilateral asymmetrical gynecomastia which slowly increased in size over the past 6 months. He was known to have NF1, but had no other medical history of note. Family history was negative for breast malignancies, but positive for NF1 in the father and prepubertal gynecomastia in two of his brothers. Mental development was normal. No drugs or dermal applications were used. There was no history of galactorrhea. Physical examination showed a healthy-appearing boy with multiple café-au-lait spots with diameter > 1.5 cm [Figures and ]; axillary and inguinal freckling. His height was 115 cm (>97th percentile) and weight 20 kg (90th percentile).\nPalpation of the both breast revealed a firm mobile mass measuring approximately 6 cm in diameter in left and 4 cm in the right corresponding to a female Tanner B3 stage[] [], with tenderness on deep palpation. The right nipple-areola complex was also enlarged, measuring 1.5 cm compared to 1 cm on the left. There was no sign of galactorrhea and his testicular volume was 4 ml bilaterally. The penis was infantile and there was no pubic or axillary hair. Ultrasound examination of both breast showed retroareolar glandular tissue with normal aspects and swelling containing subcutaneous fat tissue. Bone was advanced by 3 years.[] An abdominal and testicular computed tomography scan excluded any estrogen-producing tumor. Routine serum/blood chemistry was normal. Chromosome analysis in cultured peripheral blood lymphocytes was also normal. Endocrinological testing revealed that the child had persistently raised levels of estradiol (E2; 33, 40, and 37 pmol/l; normal: 15-35) in the presence of undetectable T levels of < 0.1 nmol/l (9.1-27.8). Basal levels of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) were normal (0.5 and 0.41 IU/l, respectively and 1.3 and 1.5 IU/l, respectively), with no change in response to stimulation by 100 mg of LH-releasing hormone intravenously. TSH, free thyroxine (FT4), and prolactin levels were normal. Genetic studies showed mutations of the CYP19A1 gene and presence of the allele (TTTA) 11 in a homozygous state and located in intron 4 of the CYP19A1 gene. Based on these findings, a diagnosis of AEXS was made. He was started on treatment with an aromatase inhibitor (tamoxifen 10 mg/day, orally). Our patient was seen 6 months after starting treatment. His growth velocity decreased from 7 to 5 cm per year and gynecomastia from 6 to 3 cm in diameter. E2 and T levels were near to normal levels. Our plan is to continue treatment and to follow-up every 6 months till improvement of gynecomastia. | [[4.0, 'year']] | M | {'8780094': 1, '16452534': 1, '1901626': 1, '22319526': 1, '33745030': 1, '874104': 1, '15212645': 1, '3924954': 1, '18317084': 1, '10023187': 1, '26668786': 1, '5440182': 1, '9543166': 1, '30411285': 1, '5785179': 1, '24497716': 2} | {} |
165,444 | 3897148-1 | 24,497,718 | noncomm/PMC003xxxxxx/PMC3897148.xml | Double heterozygous hemoglobin Q India/hemoglobin D Punjab hemoglobinopathy: Report of two rare cases | A 26-year-old male of Indian Punjabi origin, resident of Delhi, presented with complaints of weakness, epigastric pain, and jaundice for 2 weeks. General physical examination revealed icterus. Systemic examination was unremarkable. Liver function tests revealed predominantly unconjugated hyperbilirubinemia (total bilirubin: 3.2 mg/dL, unconjugated: 2.5 mg/dL). Transaminases and serology for viral hepatitis were normal. Molecular studies for Gilbert's syndrome were negative. In view of the unconjugated hyperbilirubinemia, a complete hemolytic work up was performed. Hb high performance liquid chromatorphy (HPLC) of proband performed on Bio-Rad variant; β thalassemia short program, showed four major peaks (retention time in minutes) which were identified as HbA (α2 β2) (2.30), HbD Punjab (α2 ββD Punjab) (4.11), HbQ India (ααQ India β2) (4.72), HbD Punjab/HbQ India (ααQ India ββD Punjab) (4.96) []. The variant α and β chain hybrid was seen to elute in the HbC window. The patient had normal Hb values with raised mean corpuscular hemoglobin concentration (MCHC) (Hb: 16.1 g/dL, mean corpuscular volume: 85.5 fL, mean corpuscular hemoglobin: 32.8 pg, MCHC: 38.3 g/dL, red cell count: 4.91 × 1012/L). The peripheral blood smear showed normocytic normochromic picture and reticulocyte count was 4%. Work up for other hemolytic anemia including glucose-6-phosphate dehydrogenase deficiency and osmotic fragility was normal. Agarose gel electrophoresis at alkaline pH showed bands in the A, S, and C positions []. This was interpreted as HbA in the A position, HbD Punjab in the S position, and HbD Punjab/HbQ India in the C position. | [[26.0, 'year']] | M | {'17486507': 1, '16572955': 1, '18236737': 1, '22178109': 1, '29398805': 1, '22965366': 1, '29075078': 1, '25408858': 1, '29622873': 1, '24497718': 2} | {'3897148-2': 2} |
165,445 | 3897148-2 | 24,497,718 | noncomm/PMC003xxxxxx/PMC3897148.xml | Double heterozygous hemoglobin Q India/hemoglobin D Punjab hemoglobinopathy: Report of two rare cases | A 29-year-old asymptomatic male of Indian Punjabi origin, resident of Meerut, was evaluated as part of family study, as his 1-year-old son had microcytic hypochromic anemia and associated HbQ India hemoglobinopathy. The Hb profiles and globin genotypes of the family members are summarized in . The Hb values and red cell indices were similar to case 1 []. Hb HPLC findings are depicted in . | [[29.0, 'year']] | M | {'17486507': 1, '16572955': 1, '18236737': 1, '22178109': 1, '29398805': 1, '22965366': 1, '29075078': 1, '25408858': 1, '29622873': 1, '24497718': 2} | {'3897148-1': 2} |
165,446 | 3897150-1 | 24,497,720 | noncomm/PMC003xxxxxx/PMC3897150.xml | Dyschromatosis universalis hereditaria: Infrequent genodermatoses in India | A 28-year-old unmarried woman from rural south India presented to us with chief complaints of multiple hyper- and hypopigmented lesions over arms, legs, trunk, and buttocks since she was 3-years-old, she was apparently normal prior to that age. The lesions had started over both the legs and gradually spread upwards towards the thighs. Few lesions had appeared during this time over the hands subsequently spreading upwards to the elbows. Later, lesions developed over the buttocks and trunk also. There was no history of photosensitivity or photophobia. There was no history of handling any chemical directly or any significant history of drug intake. Her mental status was subnormal with depression.\nPhysical examination revealed numerous asymptomatic, generalized, 0.5-1 cm hyperpigmented macules interspersed with spotty hypopigmented macules. The lesions were dense on the limbs and trunk. Palms and soles were not involved. Her face showed mild involvement. The hair, nails, teeth, and mucosae appeared normal. There was no apparent atrophy, erythema, or telangiectasia [Figures and ]. Systemic examination did not reveal any abnormality. Other routine investigations and chest X-ray were within normal limits. Venereal disease research laboratory (VDRL) and human immunodeficiency syndrome test was negative. There was no history of consanguinity among the parents. She reported that her father and sister had similar lesions, but in a milder form. She was depressed since she was not married due to the cosmetic disfigurement and felt worthless, we counseled the patient and also referred her for psychiatric consultation.\nA skin biopsy was taken from both the hyper- and hypopigmented lesions. Section studies shows epidermis in both the biopsies was mildly atrophic with hyperkeratosis. Basal layers showed mild vacuolar change, papillary dermis with melanin incontinence, and dilated dermal vessels. Hyperpigmented lesions showed increase in pigment extending into the stratum spinosum; whereas, in the hypopigmented lesions it showed decrease in pigment. Dermis in both the biopsies showed mild perivascular and periadnexeal lymphomononuclear infitrate [Figures and ].\nThe patient is currently being treated with narrowband ultraviolet B (NBUVB) therapy, has completed 20 sessions and feels her lesions have improved. Also, was put on escitalopram 10 mg in the morning and clonazepam 0.5 mg at night for depression. Patient feels after the counseling and with treatment, is happier with the cosmetic appearance of the lesions and has started working too. | [[28.0, 'year']] | F | {'12372090': 1, '16684309': 1, '18700119': 1, '12437556': 1, '29201043': 1, '23091589': 1, '14994234': 1, '12916015': 1, '15955093': 1, '20877065': 1, '24497720': 2} | {} |
165,447 | 3897151-1 | 24,497,721 | noncomm/PMC003xxxxxx/PMC3897151.xml | A case report of ectopia cordis and omphalocele | A 24-year-old, unbooked primigravida delivered spontaneously, a 34-week, male stillborn weighing 1,870 g at Kasturba Hospital, MGIMS, Sevagram. The mother was not suffering from any illness related to pregnancy. There was no history of intake of any teratogens or exposure to unusual environment in antenatal period. The family history was negative for congenital anomalies or genetic abnormalities. There was no history of consanguinity. An anterior thoracoabdominal defect with extrathoracic heart, a cleft sternum, and omphalocele were recognized at birth. The physical examination revealed an exposed heart totally outside of the thoracic cavity without pericardium protection. The abdominal wall defect that caused evisceration of liver, stomach, and the intestines []. Other abnormal features included asymmetrical face with medial epicanthal folds, low set ears, micrognathia, asymmetrical bossing of the skull, and high arched palate. Thorax was small and kyphoscoliosis (thoracolumbar region) of the spine was present. Left sided foot had talipes varus and right foot had valgus deformity. Skeletal survey revealed scoliosis and torsion of the spine. The cranial ultrasound examination was normal. Autopsy confirmed the above skeletal and USG abnormalities. Karyotyping was not done because parents refused to do any genetic testing. | [[24.0, 'year']] | M | {'8677109': 1, '9160135': 1, '12530621': 1, '34000488': 1, '2644176': 1, '7077480': 1, '1416141': 1, '30065917': 2, '7120001': 1, '3189400': 1, '12530497': 1, '25404984': 1, '27957373': 2, '24497721': 2} | {'5120199-1': 1, '6056637-1': 1} |
165,448 | 3897317-1 | 24,548,999 | noncomm/PMC003xxxxxx/PMC3897317.xml | Histopathological examination of Acanthamoeba sclerokeratitis | A healthy 46-year-old man was referred to our hospital with severely reduced vision and severe pain to have his eye examined. He had been diagnosed with herpetic disciform keratitis at a local clinic 6 months earlier because of a ring infiltrate. Because of complications of iridocyclitis, he was treated with topical and systemic steroids along with acyclovir ointment. However, the symptoms and findings worsened, and he was referred to our hospital.\nOn his first visit, we learned that he had been wearing 1-day disposable soft contact lenses for several days. The visual acuity in his right eye was hand motion, and the cornea had a ring-shaped opacity with extensive epithelial defects (). A nodule was observed beneath the highly injected conjunctiva at the superior sclera close to the limbus (, arrow). Cysts of Acanthamoeba were identified in cultures of samples of the corneal abscess, and the patient was diagnosed with ASK. He was treated with oral itraconazole and topical micafungin and chlorhexidine (0.02%) hourly. The corneal region was debrided several times; however, the injection of the conjunctiva and sclera worsened, leading to necrotizing scleritis 1 month after the first examination (). An 8 mm-diameter ring-shaped abscess developed in the corneal lesion, and soon after that the cornea perforated (). Because he complained of severe pain and requested removal of the right eye, the eye was enucleated under general anesthesia.\nParaffin sections of the enucleated eye were examined histopathologically. Cysts of Acanthamoeba and polymor-phonuclear leukocytes (PMNLs) were observed throughout the corneal stroma, indicating an active invasion of the protozoan (). The anterior sclera in the limbal area was acutely inflamed with an infiltration of PMNLs, and an abscess was observed at the limbus ( and ). However, the sclera close to the ciliary body appeared different: granulation tissue consisting of congested blood vessels surrounded by macrophages and lymphocytes was observed in this area (). Interestingly, the signs of inflammation and granulation were not observed in the sclera posterior to the insertion of the rectus muscles or in the retina or choroid. | [[46.0, 'year']] | M | {'8154529': 1, '10406160': 1, '9793944': 1, '9436874': 1, '20957062': 1, '3753282': 1, '32884549': 1, '31309100': 1, '8119418': 1, '12045063': 1, '18843498': 1, '32143731': 1, '17893557': 1, '24548999': 2} | {} |
165,449 | 3897635-1 | 24,466,402 | noncomm/PMC003xxxxxx/PMC3897635.xml | Intolerance to Sunitinib Treatment in Hemodialysis Patients With Metastatic Renal Cell Carcinoma | A 57-year-old man underwent right radical and left partial nephrectomy to treat stage III (pT3N0M0) and stage II papillary RCC (pT2N0M0), respectively, in January 2007. Thereafter, his renal function became impaired; his blood urea nitrogen level was 88 mg/mL and his creatinine level was 8.5 mg/mL. Hemodialysis was commenced, three times a week, in May 2007. Three years later, multiple metastatic lesions appeared in the lungs. He also developed additional lymph node metastases in the retroperitoneum and multiple lesions in the liver. Biopsies of the liver masses revealed the presence of mRCC. His prognostic risk category was high, as defined by the Memorial Sloan-Kettering Cancer Center (MSKCC) risk model, with a Karnofsky performance status of 70%, a serum hemoglobin level of 12.1 mg/mL, a serum-corrected calcium level of 10.7 mg/mL, and a serum lactate dehydrogenase (LDH) concentration of 568 U/L. Twenty-five milligrams of sunitinib was administered orally, on a daily basis, for 4 weeks of every 6, commencing in July 2010. Several toxicities, including facial edema, yellowish skin pigmentation, mucositis, hypertension, and general weakness, were manageable (grades 1-2). However, no attempt was made to increase the dose because of a gradual increase in anemia (from grades 1-4) associated with a requirement for frequent packed red blood cell infusion and increasingly problematic chronic fatigue and general weakness. There was no evidence of hypothyroidism. Computed tomography conducted after two cycles of treatment showed that his disease was progressive. Sunitinib was discontinued in November 2010. | [[57.0, 'year']] | M | {'17367763': 1, '18801884': 1, '17215529': 1, '19707503': 1, '12967694': 1, '18436521': 1, '19136449': 1, '19167044': 1, '20948925': 1, '16314617': 1, '28424553': 2, '24466402': 2} | {'3897635-2': 2, '5344426-1': 1, '5344426-2': 1, '5344426-3': 1, '5344426-4': 1} |
165,450 | 3897635-2 | 24,466,402 | noncomm/PMC003xxxxxx/PMC3897635.xml | Intolerance to Sunitinib Treatment in Hemodialysis Patients With Metastatic Renal Cell Carcinoma | An 80-year-old female patient underwent left radical nephrectomy after a diagnosis of stage II clear cell carcinoma of the left kidney (pT2N0M0) in 2002. The patient was put on a hemodialysis program (three times per week) 6 years after nephrectomy. Her medical history included essential hypertension, which had been controlled with amlodipine (5 mg/d), for 10 years. In February 2009, she complained of pelvic and lower extremity pain. Abdominopelvic magnetic resonance imaging (MRI) revealed a lytic lesion with dimensions of 7 cm×6 cm at the sacrum. Systemic screening did not reveal any additional metastatic site. Histopathologic evaluation of the lesion showed clear cell RCC metastasis. Although radiotherapy was delivered to the sacrum, the patient was referred to our department for further evaluation because the lytic lesion became enlarged 8 months after radiotherapy. The prognostic risk category was intermediate, as defined by the MSKCC risk model, with a Karnofsky performance status of 90%, a serum hemoglobin level of 10.6 mg/mL, a serum-corrected calcium level of 9.4 mg/mL, and an LDH level of 216 U/L. Twenty-five milligrams of sunitinib was prescribed daily, commencing in March 2010. On the 15th day of treatment, acute pulmonary edema developed as a result of uncontrolled hypertension. Sunitinib could not be continued, but 15 days later, MRI showed that the disease was stable. She refused further oncologic treatment and 10 months after sunitinib withdrawal, she has not developed any new metastatic lesion or progressive disease in the sacrum. | [[80.0, 'year']] | F | {'17367763': 1, '18801884': 1, '17215529': 1, '19707503': 1, '12967694': 1, '18436521': 1, '19136449': 1, '19167044': 1, '20948925': 1, '16314617': 1, '28424553': 2, '24466402': 2} | {'3897635-1': 2, '5344426-1': 1, '5344426-2': 1, '5344426-3': 1, '5344426-4': 1} |
165,451 | 3897636-1 | 24,466,403 | noncomm/PMC003xxxxxx/PMC3897636.xml | Ureteral Substitution Using Appendix for a Ureteral Defect Caused by a Retroperitoneal Rhabdomyosarcoma in a Child | A 7-year-old boy was referred to our outpatient clinic for evaluation of his abdominal mass, which was found at a local clinic by use of abdominal ultrasound. He underwent laparoscopic biopsy of his abdominal mass and the mass was finally diagnosed as an embryonal rhabdomyosarcoma in the retroperitoneum. The size of the mass was 18 cm×16 cm×7 cm, and after 4 cycles of chemotherapy for 5 months, the mass size was reduced to 5 cm×2 cm×3 cm. Surgical resection of the mass was then performed and the patient was followed up for 2 years. Abdominal computed tomography showed no remnant mass of the tumor. We found no evidence of recurrence of the tumor for 1 year after surgery. After 1 year, however, follow-up computed tomography showed a newly developed mass (3.3 cm×3.0 cm in size) surrounding the right lower ureter along the right iliac artery, resulting in hydronephrosis of the right ureter (). We diagnosed the mass as a recurrent retroperitoneal rhabdomyosarcoma and we decided to perform surgical resection. The operation was performed through a midline vertical incision and the anatomy was confirmed by direct inspection. The mass had severe adhesions with the surrounding tissues and it completely encircled the right lower ureter along the right iliac artery. After resection of the mass, direct end-to-end anastomosis of the ureter was not possible owing to the length of the resected segment (). Therefore, we decided to perform ureteral substitution by using the appendix to repair the ureteral defect. The cecum and the right colon were mobilized. The vermiform appendix was assessed. It was 6 cm in length from the base to the tip and no macroscopic abnormalities were noted. The appendix was divided at its base and the stump was ligated and conventionally inverted. Care was taken to preserve the appendicular artery, which was identified as running in the short triangular mesoappendix. The isolated appendix was mobilized to the retroperitoneum and positioned in an antiperistaltic manner to avoid strangulation. Tension-free end-to-end anastomosis with 5-0 Vicryl was performed between the spatulated end of the ureter and the tip of the appendix (). A ureteral stent was placed by using a guide wire before completing the anastomosis. The resected mass was 4.5 cm×3.0 cm×1.0 cm in size and it grossly revealed no central necrosis. The mass was finally diagnosed microscopically as retroperitoneal rhabdomyosarcoma. The patient's postoperative recovery was uneventful. The ureteral stent was removed 6 weeks postoperatively and retrograde pyelography revealed a patent appendiceal interposition and no extravasation. Intravenous urography showed no definite stricture point and improvement of the hydronephrosis that was seen before surgery (). This patient died at 6 months postoperatively as the result of multiple metastases of rhabdomyosarcoma. | [[7.0, 'year']] | M | {'2754579': 1, '10799226': 1, '7408090': 1, '2724435': 1, '2810509': 1, '3244090': 1, '3563176': 1, '24466403': 2} | {} |
165,452 | 3897696-1 | 24,465,150 | noncomm/PMC003xxxxxx/PMC3897696.xml | Anti-LGI1 Limbic Encephalitis Presented with Atypical Manifestations | A 62-year-old woman presented with confusion and memory loss in August 2012. She developed confusion with disorientation to the time and place and had no memory of a recent family trip before 2 days of admission. Upon admission, her serum sodium was 120 mmol/L, which was corrected to 134 mmol/L after 3 days. A brain MRI and EEG failed to indicate any abnormality, and no focal deficit was found on neurological examinations. After correction of hyponatremia, she was discharged without significant problems, except for memory disturbance. Her Korean Mini-Mental State Examination (K-MMSE) score was 25 at the time of discharge (8/10 on time and place orientation and 0/3 on memory recall).\nThe patient was readmitted to our hospital 12 months after her initial admission. Since two months before readmission, she was experiencing recurrent chest discomfort that she described as squeezing and dull in nature, typically lasting 10~30 seconds. The frequency of these episodes was initially once a day, but progressed to more than 20 times a day after 2 months. She reported that her memory disturbance was aggravated and she had developed intermittent confusion with disorientation. She repeatedly asked, "Where am I?" and, "Why am I here?" and sometimes lost her sense of direction. On the day before her readmission, she experienced chest discomfort and subsequently lost consciousness and collapsed with facial grimacing and abnormal arm-twisting movements. She regained consciousness a few minutes later, but was disoriented to the time and place. Definite convulsive movements were not observed. At admission, she was alert, oriented and showed no focal deficits on neurological examination. Her K-MMSE score was 24 (8/10 on time and place orientation, 4/5 on attention and calculation and 0/3 on memory recall), and she showed no difficulties with daily activity during her admission. Routine blood tests and a brain MRI were normal; a routine EEG failed to show definite abnormalities. For her chest discomfort, transthoracic echocardiography, CT coronary angiography, and 24-h electrocardiography were all normal. Supine blood pressure was 130/60 mmHg, and orthostatic blood pressure at 0, 1, 3, and 5 min was 120/60, 125/60, 130/60, and 125/65 mmHg, respectively. Esophagogastroduodenoscopy and esophagogram with esophageal manometry revealed normal findings.\nInitially, we diagnosed the patient with anxiety disorder and administered a selective serotonin reuptake inhibitor and an anxiolytic. However, her chest discomfort persisted. On video-EEG monitoring, ictal discharges were observed in the right temporal area during her chest discomfort (), whereas FBDS or other seizures were not reproduced. FDG PET revealed no abnormalities. After administering oxcarbazepine, the frequency and duration of her chest discomfort reduced to a few times a day and lasted only a few seconds, whereas her memory disturbance persisted. Thus, we suspected autoimmune causes for the limbic encephalitis, and anti-LGI1 antibody was detected in both serum and CSF (). After treatments with IV immunogloblin (0.4 g/day for 5 days) and antiepileptic drugs (oxcarbazepine and levetiracetam), the chest discomfort and memory disturbance markedly improved. Her follow-up K-MMSE score was 27 (8/10 on time and place orientation and 4/5 on attention and calculation). She has been symptom free for a month after her discharge. | [[62.0, 'year']] | F | {'15704018': 1, '11134380': 1, '23525443': 1, '26889260': 1, '17848866': 1, '1630984': 1, '15045602': 1, '1963440': 1, '3946434': 1, '29514833': 1, '22713136': 1, '32850103': 2, '31780881': 1, '29163505': 1, '5723018': 1, '23060110': 1, '23486322': 1, '21416487': 1, '30108593': 1, '20580615': 1, '24465150': 2} | {'7427439-1': 1} |
165,453 | 3897766-1 | 24,465,254 | noncomm/PMC003xxxxxx/PMC3897766.xml | Blonanserin-induced Mood Alteration in Schizophrenia and Schizoaffective Disorder: Two Cases | A 25-year-old male with schizophrenia developed a hypomanic episode 1 week after the initiation of blonanserin at 8 mg/day. He was first diagnosed with schizophrenia in 2003 at the age of 16 years with symptoms of auditory hallucination and delusions of reference and persecution and has been on various antipsychotic medications for 9 years. The patient did not achieve symptomatic remission, and his course was characterized by intermittent worsening and softening of the referential delusion that others were talking behind his back. He has been on a stable regimen of olanzapine 5-20 mg (final dose 10 mg), aripiprazole 15 mg, clonazepam 0.5 mg, and benztropine 1 mg since 2010. In February 2011, he experienced increased appetite and weight gain; thus, fluoxetine (20 mg) was added to his regimen for 7 months until September 2011 with no mood change.\nIn March 2012, the patient's ideas of reference began to increase and in May, he complained that his neighbors said bad things about him and that the rumors had spread throughout the community. Following this exacerbation, blonanserin 8 mg/day was added to his regimen. Ten days later, the intensity of his delusions decreased, but he developed new symptoms indicative of a hypomanic episode, such as euphoria, elated mood, grandiosity, and hyperactivity. The patient expressed his plan to become a psychiatrist and he wrote poems for several hours per day with the intention of becoming a professional poet. He had a score of 19 on the Young Mania Rating Scale (YMRS). One week later blonanserin was withdrawn and the episode ended 4 weeks after discontinuation (YMRS score: 4). | [[25.0, 'year']] | M | {'15554769': 1, '20030420': 1, '15289815': 1, '20661022': 1, '27790460': 1, '19659567': 1, '728692': 1, '23131856': 1, '20460137': 1, '16442194': 1, '22122647': 1, '24465254': 2} | {'3897766-2': 2} |
165,454 | 3897766-2 | 24,465,254 | noncomm/PMC003xxxxxx/PMC3897766.xml | Blonanserin-induced Mood Alteration in Schizophrenia and Schizoaffective Disorder: Two Cases | A 38-year-old male developed a manic episode after the initiation of blonanserin treatment. He first visited our outpatient department in May 2012 with complaints of persecutory and referential delusions that had developed 2 weeks earlier. He claimed that his colleagues at work bullied him and had hatched a plot to get him fired. His family reported that he accused his brother of being a spy from the company and called his father a North Korean spy. Furthermore, he showed bizarre behaviors such as wandering and picking up garbage. Schizophreniform disorder was suspected, and he was initially administered blonanserin 6 mg, alprazolam 0.375 mg, and benztropine 2 mg/day. Shortly after receiving the medication, he displayed a slightly argumentative attitude and increased activity, but his behavior was considered to be within the normal range. Two weeks later, at a dose of 8-mg blonanserin, the patient reported that his persecutory and referential delusions were no longer valid, and he displayed a slight euphoria and increased psychomotor activity that met the criteria for a hypomanic episode.\nTwo weeks later, at 16 mg/day blonanserin, the patient showed full-blown manic symptoms including talkativeness, hyperactivity, irritable and expansive mood, and flight of idea. He was considered for hospitalization but refused. He scored 27 on the YMRS. Valproate 500 mg/day was added to his regimen, and the dose of blonanserin was reduced to 12 mg/day. His manic episode continued for 2 additional weeks; however, when blonanserin was discontinued his manic symptoms disappeared within 2 weeks (YMRS score: 2). | [[38.0, 'year']] | M | {'15554769': 1, '20030420': 1, '15289815': 1, '20661022': 1, '27790460': 1, '19659567': 1, '728692': 1, '23131856': 1, '20460137': 1, '16442194': 1, '22122647': 1, '24465254': 2} | {'3897766-1': 2} |
165,455 | 3897767-1 | 24,465,255 | noncomm/PMC003xxxxxx/PMC3897767.xml | Successful Early Clozapine Trial in the Treatment of First-episode Schizophrenia: A Case Report | A 31-year-old man with no history of drug or alcohol use, was brought to the hospital by his parents and police. He had no history of psychiatric treatment or significant medical illness. He had good marks in high school, but he had been shy and reserved throughout his life. His mother had been treated for longstanding psychotic depression.\nThe patient studied education at college, but failed the certification exam three years in a row. He worked as a school instructor for several months; however, his parents wanted him to prepare for the examination. He became more socially withdrawn than he had been in the previous year and spent all day at home for a year. A month before his hospitalization, he was admitted to a community college to study electrical engineering, but soon began to hear commanding voices of unknown origin telling him to "build a nuclear power station and save the world." At times, he felt that his head was being controlled by someone and often stood alone muttering to himself.\nThe patient scored 110 on the Positive and Negative Syndrome Scale (PANSS), and his severity subscore on the Clinical Global Impressions (CGI) scale was 6.\nThe initial diagnosis was schizophrenia. A pharmacological therapy based on the current treatment algorithm was initiated targeting auditory hallucination, delusion, autistic and aggressive behavior, and blunted affect.\nIn his first antipsychotic trial, olanzapine was administered across a low-, medium-, and high-dose range (titrated between 10 and 30 mg). However, 4 weeks after hospitalization and treatment onset, the constant, commanding hallucination persisted. Moreover, the patient had begun banging his head against the door and refused meals nearly every day. At that point, his PANSS score was 102, and only a slight clinical improvement was observed. Because of his poor treatment response to prior medication, the rising frequency and intensity of his self-injurious behavior, and the poor clinical outcome, it was necessary to switch his antipsychotic medication. A clozapine trial was started based on its high response and remission rates in first-episode schizophrenia. The patient and his family were fully informed about the benefits and possible adverse events associated with clozapine. All laboratory tests and electrocardiogram (EKG), electroencephalogram (EEG), and brain magnetic resonance imaging were within normal limits. No evidence of encephalopathy was found. Complete blood cell counts were monitored weekly during dose titration. Olanzapine treatment was discontinued, and we initiated clozapine at 25 mg and increased the dose to 200 mg following the recommended dose titration schedule.\nOn day 60, the patient reported experiencing few auditory hallucinations and he began socializing with other patients. Moreover, he showed marked improvement in conceptual disorganization and bizarre content of thinking (PANSS score, 70). We observed no adverse side effects or physical compromise during the treatment regime. Three months after clozapine initiation, the patient was discharged from the hospital, and his psychopathology remained stable (PANSS score, 60).\nThe patient remains on a daily maintenance dose of clozapine (200 mg), and he is living with his parents while working and continuing his college education. Despite residual emotional and passive social withdrawal, the patient accepts the necessity for treatment, which has prevented further psychiatric decompensation. | [[31.0, 'year']] | M | {'10372610': 1, '23870807': 1, '23006236': 1, '3616518': 1, '15660702': 1, '2772122': 1, '30218445': 1, '16187768': 1, '23377634': 1, '33846298': 1, '9384923': 1, '24465255': 2} | {} |
165,456 | 3898454-1 | 24,498,504 | noncomm/PMC003xxxxxx/PMC3898454.xml | Fatal Disseminated Mucormycosis in an Immunocompotent Patient: A Case Report and Literature Review | A 64-year-old, previously healthy male was transferred to Alzahra Hospital (Isfahan University of Medical Sciences, Isfahan, Iran) in August 2011. 15 days before, he had a stroke following a cardiac surgery (mitral valve replacement and coronary artery bypass graft). The patient had no history of diabetes mellitus, alcohol abuse and malignancy. Upon admission physical examination revealed confusion, dysarthria, left side hemiparesis and extensive bedsores in the sacral area. Initial laboratory study showed leukocyte count: 15,000/mcl, hemoglobin: 10 mg/dl. During hospitalization, the neurological state of the patient gradually ameliorated; however, in the 7th day of admission, he developed dyspnea, productive cough and jaundice. Physical examination revealed fever, tachypnea, generalized jaundice and course crackles in the base of the left lung. New laboratory study showed leukocyte count 9700/mcl, hemoglobin 10 mg/dl, total bilirubin 6 mg/dl, (direct bilirubin 1 mg/dl), aspartate aminotransferase 154 U/L, alanine aminotransferase 122 U/L and alkaline phosphatase 1150 U/L. Human immunodeficiency virus antibody test was negative. Chest X-ray showed focal pneumonic infiltration in the left lower lobe. Abdominal ultrasonography showed no abnormality. Considering the probability of pneumonia, empirical antibiotic therapy was started. However, his condition deteriorated and without any response to the treatment he died within 24 h.\nIn the autopsy, multiple hemorrhagic foci on the pancreas and extensive perforations of spleen were two significant findings. Following the autopsy, each organ was separately put into 10% buffered formalin and abnormal macroscopic findings for each organ were recorded by the pathologist. Then, small slices with at least 0.5 cm were cut and the formalin solution was changed for the fixing overnight. The next day, proper cut for paraffin embedded tissue blocks were prepared. Two routine staining procedures including hematoxylin and eosin and periodic acid schiff were performed for tissue sections.\nHistopathological study of tissue sections revealed neutrophilic infiltration, massive coagulative necrosis and thrombotic vessels that some of them contained fungal hyphae in the left lung, pancreas, spleen and brain. On high power microscopic evaluation these hyphae appeared as ribbons which were often twisted and collapsed with a variable width. Septa in the most structures were absent. Some hyphae revealed swollen segments in cross-section [Figures –]. Hence, the histological findings were compatible with the diagnosis of mucormycosis. Despite making several cuts, no considerable finding was discovered in the other organs. | [[64.0, 'year']] | M | {'21331168': 2, '3684839': 1, '16961579': 1, '21625316': 1, '22121366': 1, '19305057': 1, '20571164': 1, '9455544': 1, '26288745': 1, '12068148': 1, '20191205': 1, '19754751': 1, '9114146': 1, '34367936': 1, '10707707': 1, '16940368': 1, '19788806': 1, '26644901': 2, '26365402': 1, '19561989': 1, '24498504': 2} | {'4649276-1': 1, '4649276-2': 1, '4649276-3': 1, '3034994-1': 1} |
165,457 | 3898455-1 | 24,498,505 | noncomm/PMC003xxxxxx/PMC3898455.xml | Rhabdomyolysis in Scrub Typhus: An Unusual Presentation | A 50-year-old male, farmer by occupation presented with a history of high grade fever for 8 days with pain in the calf and thigh muscle region for 4 days, pedal edema, and decreased urine output for 1 day. There was no history of rash, cough, jaundice or history suggestive of urinary tract infection. Patient presented to a rural tertiary care institute with a history of treatment for 2 days at a local health center and the details of treatment were not available at the time of presentation to our institute. Later the details of treatment given at local health center revealed that, patient had received a course of antimalarial (Chloroquine), analgesics and intravenous fluids. Patient was referred to our institute in view of persisting fever and hematuria. At the time of presentation, patient had myoglobinuria []. On examination, pulse was 90 beats/min, blood pressure was 130/80 mm Hg and there were no signs of pallor, icterus, clubbing or lymphadenopathy. The eschar was seen in the right medial aspect of the shoulder [] and there was bilateral pitting pedal edema. The systemic examination was within the normal limits.\nThe details of laboratory examination are shown in .\nThe renal parameters were deranged and the muscle enzyme creatine phosphokinase total was elevated. The liver function tests were normal. The complete hemogram showed a normal total leukocyte and platelet count and no malarial parasites. Electrocardiogram revealed tall-tented T waves with a rate of 88 beats/min. The chest X-ray and ultrasonography of the abdomen were normal. The blood and urine culture were sterile. The Widal test leptospira, and dengue serology were negative.\nIn view of rhabdomyolysis and ARF, urgent hemodialysis was planned. Meanwhile, patient was started on doxycycline 100 mg and received treatment for hyperkalemia. The repeat sample for potassium after correction revealed a value of 7 mE/l. In view of persistent hyperkalemia patient was immediately taken to hemodialysis. However, within 2 h of hospital admission, patient sustained cardiac arrest probably due to hyperkalemia and could not be revived with cardiopulmonary resuscitation. | [[50.0, 'year']] | M | {'20649092': 1, '18003808': 1, '20207857': 1, '30404620': 2, '11504834': 1, '17291879': 1, '10906171': 1, '14717236': 2, '24498505': 2} | {'4531640-1': 1, '6223005-1': 1} |
165,458 | 3899553-1 | 24,497,756 | noncomm/PMC003xxxxxx/PMC3899553.xml | Gastric Outlet Obstruction from Duodenal Lipoma in an Adult | A 40-year-old man presented to our institution with a 3 months history of projectile, copious vomiting (which consisted of recently ingested food), epigastric fullness, constipation and abdominal discomfort. There was no history of hematemesis, melena or change in bowel habit. He had a 7 months history of dyspepsia.\nClinical examination, showed a chronically ill-looking man, emaciated, pale and dehydrated. Visible peristalsis was noted at the upper abdomen with demonstrable succussion splash. He had no ascites, organomegaly or a palpable mass. Barium meal showed a dilated stomach with a large amount of residual food debris mixed with barium sulfate []. An extrinsic pressure effect was seen in the pyloric region of the stomach with resultant gastric outlet obstruction (GOO). The duodenum was not demonstrated. An abdominal ultrasound scan showed a large and prominent stomach, with the gastric lumen harboring large food debris with near absence of gastric emptying. An area of fusiform bowel thickening was noted near the duodenal bulb. He could not be investigated further due to financial constraints. We were, therefore left with no option than to carry out an exploratory laparotomy, after fluid and electrolyte resuscitation. The finding was a pedunculated, submucosal lipoma arising from the second part of the duodenum that extends to occlude the distal duodenum and proximal jejunum []. A duodenotomy with wedge resection of the pedunculated lipoma and primary repair was done. A sleeve of duodenal mucosa was taken along with the lipoma because of a suspicious nodule on the mucosa []. The post-operative course was uneventful and the patient was discharged home on the 7th day after the surgery. Final histopathological diagnosis of the specimen was a submucosal duodenal lipoma measuring 11 cm × 8 cm × 6 cm. | [[40.0, 'year']] | M | {'13949051': 1, '6016046': 1, '7129044': 1, '34414955': 1, '3984992': 1, '5662503': 1, '18327380': 1, '13871677': 1, '34507195': 1, '14864673': 1, '28892976': 1, '10049428': 1, '28496542': 2, '23129520': 1, '31718616': 2, '29797145': 1, '24497756': 2} | {'5412554-1': 1, '6852972-1': 1} |
165,459 | 3899554-1 | 24,497,757 | noncomm/PMC003xxxxxx/PMC3899554.xml | Non-bleeding Spontaneous Rupture of Hepatocellular Carcinoma | A 62-year-old non-diabetic, non-alcoholic male patient attended to our out-patient department with the complaint of dull aching pain in the right upper part of the abdomen. He was suffering from such pain since last 1 month. Pain responded to analgesic. He lost around 3 kg weight in last 1 month. He lost appetite also.\nAbdominal ultrasonography was performed, which suggested HCC with ascites. Triple phase contrast enhanced computer tomography (CECT scan) revealed HCC of 12 cm × 15 cm size in segments 6 and 7 of liver []. Computer tomography also showed typical radiological characteristics of HCC (arterial enhancement of liver mass and wash out on delayed phase, arterioportal shunt [Figures –], portal vein thrombosis). There was gross ascites. No extravasation of dye seen. Ruptured tumor through liver capsule [] was seen with necrosis and hemorrhage in the center of the tumor.\nPatient was admitted for evaluation. His blood pressure and heart rate was normal. Peritoneal tapping was performed for ascetic fluid analysis. Ascetic fluid was clear, exudative in nature with serum to ascetic fluid albumin gradient less than 1.1. Cytological examination ascetic fluid revealed no tumor cell.\nOn evaluation for chronic liver disease, all viral markers were negative. Upper gastrointestinal endoscopy showed non-bleeding grade II esophageal varices. On biochemical analysis of blood total and direct bilirubins were 1.98 and 0.48 respectively. Liver enzymes were raised (Aspartate transaminase-51 IU/ml and alanine amino transferase -55 IU/ml) with normal total protein and albumin. His hemoglobin was 12.4 g% and cell counts were normal. Alpha fetoprotein was high (424 ng/ml; normal range 0-8.5) and carcinoembryonic antigen was normal.\nThe disease was in advanced staged to be cured by surgery and portal vein thrombosis excluded TACE from treatment options; therefore, transarterial radioembolization was offered, but patient refused it. | [[62.0, 'year']] | M | {'11387009': 1, '21160956': 1, '11087643': 1, '9630270': 1, '12190677': 1, '31024207': 1, '11318532': 1, '16490898': 1, '18333020': 1, '27999693': 2, '30140457': 1, '27433178': 2, '14255982': 1, '29399732': 1, '24497757': 2} | {'5141538-1': 1, '4943902-1': 1} |
165,460 | 3899555-1 | 24,497,758 | noncomm/PMC003xxxxxx/PMC3899555.xml | Triplication of Deciduous Teeth: A Rare Dental Anomaly | A 10-year-old boy reported to the dental clinic with the chief complaint of large tooth in the maxillary anterior region of the jaw. No other member of the family was affected with similar dental anomaly and patient was mentally challenged. Intraoral examination revealed that the patient was in mixed dentition phase along with fusion of the maxillary right primary central incisor and primary lateral incisor with an additional (supernumerary) tooth []. Dental caries was evident on the incisal edge and proximal surface of supernumerary tooth. The child's dentition revealed Angle's class 1 molar relation on both right and left side. Maxillary right permanent central incisor was erupted, but it was in cross-bite relationship. A mid line diastema was also noted in maxilla. Clinical examination revealed missing right permanent lateral incisor and there was no history of previous extraction, of this tooth. Further investigation included intraoral periapical radiograph of maxillary anterior region []. Based on clinical and radiographical findings, the provisional diagnosis of fused triple teeth was made.\nThe treatment plan was aimed at removal of fused teeth. Under sedation, fused teeth were carefully extracted []. Macroscopically, root resorption was evident on apical portion of maxillary right primary lateral incisor. For histological examination, the extracted teeth were sectioned at three levels: The coronal, middle root and apical levels, respectively. These sections were visualized under a stereomicroscope at a magnification of ×50. Histological view of the coronal section revealed three teeth fused with each other with confluent dentin without intervening cementum and with separate pulp chambers []. The middle-third of the roots of three fused teeth also showed confluent dentin without intervening cementum []. The apical third of fused teeth showed merging of root canals of the fused teeth []. Based on these findings, a final diagnosis of incomplete fusion was confirmed.\nFurther treatment plan involved thorough oral prophylaxis and correction of anterior cross- bite. After oral prophylaxis and pit and fissure sealant application, a removable Hawley's appliance with Z-spring was fabricated for the correction of the anterior tooth cross-bite, but as patient was mentally challenged, cooperation from him was poor as the child was not ready to wear the appliance. Again Catalan's appliance was made and cemented on the lower incisors, but the attempt for the correction of cross- bite failed because of insufficient compliance of the patient as he was not occluding his teeth. | [[10.0, 'year']] | M | {'32695375': 2, '11985191': 1, '6584348': 1, '20927417': 1, '25743857': 1, '11688820': 1, '9161200': 1, '19055087': 1, '26877735': 1, '24497758': 2} | {'7364066-1': 1} |
165,461 | 3899588-1 | 24,459,537 | noncomm/PMC003xxxxxx/PMC3899588.xml | Adult Onset Still’s Disease Presenting with Acute Respiratory Distress Syndrome: Case Report and Review of the Literature | A 26-year-old African-American female with a history of type 2 diabetes mellitus presented to her primary care physician with sore throat and cough. She was noted to have a “viral exanthem”, and was started on amoxicillin-clavulanate. Her symptoms did not improve and she went to the emergency department (ED) for shortness of breath, cough and myalgias. Her vital signs were as follows: temperature 36.7 C, pulse rate 112, respiratory rate 18, and blood pressure 100/56 mm Hg. Physical examination was notable for coarse breath sounds bilaterally over lung fields with scattered crackles at the bases and tachycardia. Radiograph of the chest showed bilateral interstitial infiltrates suggestive of ARDS. Arterial blood gas (ABG) showed pH 7.28, pCO2 38 mmHg and pO2 58 mmHg. Her respiratory status worsened and she underwent endotracheal intubation requiring ventilator support, and was started on broad-spectrum antibiotics for presumed pneumonia.\nTwo days after admission to the intensive care unit (ICU), the patient developed daily spiking fevers up to 40.0 C. She became more difficult to oxygenate with increasing ventilator requirements. She became hypotensive to 70/49 mmHg and required vasopressors. Antibiotic therapy was broadened and changed multiple times, including treatment with vancomycin, ceftriaxone, azithromycin, piperacillin/ tazobactam, imipenem, metronidazole and fluconazole, without any clinical improvement.\nLaboratory analysis included an initial white blood cell (WBC) count of 20 x 103/mm3 (which increased to 49 x 103/mm3 within one week) with 91.8% neutrophils. Hemoglobin (Hb) was initially 10 g/dL (and dropped to 8.3 g/dL within one week), and platelet count 407,000/mL. Aspartate aminotransferase (AST) was 88 IU/L, alanine aminotransferase (ALT) 44 IU/L, and lactate dehydrogenase (LDH) 530 units/L. Autoimmune panel was negative for anti-nuclear antibodies (ANA) and rheumatoid factor (RF). C-reactive protein (CRP) was elevated at 7.19 nmol/L and erythrocyte sedimentation rate (ESR) was 39 mm/h. The ferritin level was 4,022 ng/mL.\nThe patient underwent further extensive evaluation during the first ten days of her admission. All cultures during fever spikes were negative, including seven sets of blood cultures. Computerized axial tomography (CT) scans of her neck and sinus were unremarkable. CT of the chest revealed bilateral pleural effusions and diffuse, patchy airspace opacities and interstitial infiltrates. She underwent bronchoscopy which was negative for bacterial, viral (including cytomegalovirus) or fungal infection. Hepatitis panel, urine legionella, serum histoplasma antigen, cryptococcal antigen, Clostridium dificile toxin, and acid fast bacilli were all negative. Transthoracic echocardiogram was normal with an ejection fraction of 55-65%. Bone marrow biopsy and flow cytometry were normal.\nShe was diagnosed with AOSD based on fulfillment of the Yamaguchi criteria with major criteria of fevers lasting greater than one week, leukocytosis with neutrophilia, and minor criteria of abnormal liver enzymes, sore throat, and negative RF and ANA []. In addition, she had hyperferritinemia and no evidence of infection or malignancy after extensive investigation. She was started on methylprednisolone 60 mg intravenously (IV) every 6 hours on day 13 of her hospital admission, and the next day, her maximum temperature improved to 38.2 C, down from 39.4 C. On day 16 she was given pulse methylprednisolone 1000mg daily for 3 days, followed by prednisone 60mg daily. She remained afebrile until her discharge on hospital day 19. She was weaned off vasopressors, antibiotics were discontinued, mental status was at baseline, and WBC count improved prior to discharge. She had developed an ICU polyneuropathy during her hospital course and required tracheostomy and transfer to a ventilator facility for weaning from ventilator support. At the ventilator facility she was continued on prednisone 60 mg daily except for one missed dose of prednisone the week prior to re-admission.\nTwo weeks after her discharge, she was re-admitted to the ICU with spiking fevers to 39.4 C, and again, broad-spectrum antibiotic therapy with vancomycin, tobramycin, and metronidazole was initiated. Two days after admission, she had a seizure followed by hypotension to 87/43 mmHg and required vasopressor support. Seizures were treated with lorazepam and phenytoin. Imipenem, metoclopramide, and metronizadole were discontinued, and meropenem and oral vancomycin were initiated. Laboratory testing revealed a WBC count of 15.1 x 103/mm3, Hb 6.9 g/dL, platelets 61,000/mL, AST 627 IU/L, ALT 507 IU/L, LDH 2,080 units/L, ferritin 110,065 ng/mL, fibrinogen 170 g/L, normal coagulation studies and ESR 16 mm/h.\nShe continued to have seizure activity, electromyogram showed moderate to severe axonal sensorimotor polyneuropathy, and electroencephalogram revealed epileptiform activity over the right parietal region and prominent sharp waves over bilateral temporal regions. Magnetic resonance imaging of the brain revealed multifocal stenosis of bilateral posterior cerebral arteries and a segment of the left anterior cerebral arteries. CT chest showed bilateral large pleural effusions with consolidation and airspace opacities consistent with ARDS. The patient was started on methylprednisolone 1000 mg IV daily for three days followed by prednisone 60 mg daily and cyclosporine 3mg/kg/day for presumed macrophage activation syndrome (MAS). Because of her continued deteriorating course, a repeat bone marrow biopsy was done over two weeks after cyclosporine therapy was begun and showed positive staining for cytomegalovirius (CMV) without evidence for hemophagocytosis. CD 163 staining to look for activated histiocytes was not performed. In addition, the cerebrospinal fluid (CSF) was positive for CMV by PCR and the patient was started on gancyclovir. The patient’s presentation was felt to meet criteria for CMV infection and secondary hemophagocytic syndrome despite bone marrow absence of true hemophagocytosis. The patient died despite treatment and supportive care measures six weeks after her initial presentation with ARDS and AOSD. | [[26.0, 'year']] | F | {'19273456': 1, '3778078': 1, '24387121': 1, '12673909': 1, '19947245': 1, '10411361': 1, '18078628': 1, '12195635': 1, '10461535': 1, '2849341': 1, '8832990': 1, '28723802': 1, '27472698': 1, '27588099': 1, '25604318': 1, '16540551': 1, '10378576': 1, '29676014': 1, '16283419': 1, '29168664': 2, '24459537': 2} | {'5703120-1': 1} |
165,462 | 3899732-1 | 24,475,343 | noncomm/PMC003xxxxxx/PMC3899732.xml | Severe inflammatory response and vasculitis leading to quadruple limb\namputations | A previously fit 33-year-old Somali woman returned from holiday in Canada with an\nurticarial rash on her upper arms accompanied by fever, dyspnoea, polyarthralgia, fatigue\nand headache. The rash resolved but the other symptoms continued, and after four weeks she\nreceived Clarithromycin for pharyngitis. A week later, she suffered a miscarriage at six\nweeks’ gestation (G4 P3) and was given Trimethoprim for a urinary tract infection. After a\nfurther week of persistent symptoms, she was admitted for treatment of pneumonia with IV\nMeropenem and Clarithromycin but deteriorated and was admitted to the intensive treatment\nunit (ITU) with type I respiratory failure. She was tachycardic with cool peripheries but\nnormotensive, and all peripheral pulses were palpable. She was pyrexial (38.9℃) and fully\nalert with no focal neurology. The differential diagnosis on ITU admission was systemic\ninflammatory response syndrome (SIRS) secondary to sepsis or pulmonary embolism (PE). She\ncontinued on IV antibiotics and was commenced on therapeutic low-molecular weight heparin\n(Clexane) until CT pulmonary angiogram excluded PE. Echocardiogram excluded endocarditis and\nevacuation of the uterus showed no retained products. All cultures were negative and blood\nresults revealed a white cell count of 25.1 × 109/L, CRP of 197 nmol/L and\nFerritin >40,000 ng/mL. All other investigations were unremarkable (). She soon required\nintubation and ventilation and the initiation of two vasopressor infusions (dopamine and\nnoradrenaline). After 48 h, she remained pyrexial and active cooling was commenced with\nhaemofiltration; this necessitated anticoagulation with IV unfractionated heparin. Within\n24 h, she developed a non-blanching, purpuric rash over her breasts, abdomen and all four\nlimbs which rapidly developed into large, tense bullae and full-thickness necrosis at the\nfingertips and toes. An abdominal skin biopsy revealed necrotizing small vessel vasculitis.\nShe received a three-day course of 1 g methylprednisolone; however, after 48 h the necrosis\nhad covered the limbs, breasts and abdomen, and peripheral pulses were no longer palpable.\nAfter a further week with continued skin loss and minimal response to steroids, it was\nconsidered that her condition may be due to adult Still’s disease. Other causes had been\nexcluded; she had a raised Ferritin level and met the Yamaguchi criteria. For this reason,\nshe was commenced on Anakinra (100 mg daily), which had shown some benefit in previous case\nreports of adult Still’s disease. She responded rapidly and within a week was extubated with\ndecreasing vasopressor requirements. On further investigation, an angiogram showed\nasymmetric iliac arteries with no flow beyond the popliteal arteries, and a muscle biopsy\nrevealed non-viable tissue below the knees. She underwent bilateral above-knee and\nbelow-elbow amputations and debridement of skin over her abdomen and breasts. Five months\nlater, she has undergone extensive skin grafting and is now receiving rehabilitation with\nlimb prostheses. | [[33.0, 'year']] | F | {'19718245': 2, '12637912': 1, '507970': 1, '20020138': 1, '1975175': 1, '18839267': 1, '10615196': 1, '16219707': 1, '15208567': 1, '11816242': 1, '24475343': 2} | {'2729264-1': 1} |
165,463 | 3899733-1 | 24,475,344 | noncomm/PMC003xxxxxx/PMC3899733.xml | A rare case of submandibular abscess complicated by stroke | A 33-year-old male presented to our institution with a left submandibular abscess secondary\nto dental infection. He had a three-day history of left jaw pain, lethargy, fever and\ndecreased PO intake. He had no significant medical history. Examination findings included\nmoderate to severe trismus and marked left submandibular swelling, and his 38th tooth was\ntender on palpation. His initial WCC was 18.7 with a neutrophilia and CRP was 153. Contrast\nCT demonstrated localized abscess in the left submandibular space.\nUltrasound reported a small hypoechoic mass on the medial aspect of the left submandibular\ngland. He was admitted and commenced on IV ceftriaxone, metronidazole and dexamethasone. His\nsymptoms and biochemical markers improved over the following three days and he was\ndischarged home on oral cephalexin and metronidazole. Drainage was initially considered\nunnecessary due to the small size of the collection and excellent clinical response.\nForty-eight hours after discharge, the patient re-presented to the same institution with\nright-sided weakness, confusion and sudden expressive aphasia. On examination, he was\nafebrile, had a BP of 159/92 and PR of 70. His GCS was 11, and he had a right upper motor\nneuron seventh nerve palsy and had decreased power in his right arm with hemi-sensory loss.\nHis WCC was 14, CRP 45 and ESR 61. A non-contrast head CT scan was normal.\nA CT angiogram of the head and neck was performed which demonstrated a tight stenosis\n(greater than 90%) involving the mid-left internal carotid artery over a distance of\napproximately 2 to 3 mm ().\nA 1 cm-segment of thrombus was noted in the superior branch of the left MCA at the level of\nthe anterior end of the Sylvian fissure and an abscess was seen involving the floor of mouth\nand submandibular space ().\nMRI showed acute left inferior frontal cortical infarct in the distribution of the left MCA\n(). It also showed further\nenlargement of the left submandibular space fluid collection, consistent with abscess\nformation with a focus of gas.\nThe patient was taken to theatre for a left transcervical incision and drainage of abscess\nand removal of the left submandibular gland. The findings at operation included a large left\nsubmandibular gland, adherent to the surrounding fascia. There was a large quantity of\nnecrotic tissue surrounding the gland with fistula extending to the gingival mucosa adjacent\nto the 38th tooth. The tooth was removed.\nSubsequent MRA of the neck performed one week postoperation demonstrated a stable stenosis\nof the distal left ICA at the level of C1 and, to a lesser extent, the right ICA. There was\nresolution of the left-sided submandibular fluid collection and the associated inflammatory\nchange. ECHO/Holter monitor screen, thrombophilia screen and cholesterol studies were\nunremarkable.\nTwo months later, the patient was admitted for an elective left carotid endarterectomy via\na combined transcervical and mandibular osteotomy approach. The findings at operation were a\nsmall internal carotid artery with an obvious stenosis, with intimal hyperplasia, 1 cm away\nfrom the base of the skull. His postoperative progress was complicated by a 10th, 11th and\n12th cranial nerve paresis secondary to postoperative oedema. This improved throughout his\npostoperative stay with some mild deficit remaining at discharge. | [[33.0, 'year']] | M | {'19136433': 1, '21197401': 1, '15580132': 1, '21541082': 1, '31426356': 1, '20346713': 1, '9039403': 1, '20642996': 1, '24475344': 2} | {} |
165,464 | 3900044-1 | 23,092,069 | noncomm/PMC003xxxxxx/PMC3900044.xml | Accidental digitoxin intoxication: an interplay between laboratory and clinical medicine | A married couple (a 61 year old man and his wife aged 60) arrived at the Maggiore della Carità Hospital (Novara, Italy) because of diarrhea, nausea and vomiting for 4 days; they also complained weakness, fatigue and visual hallucinations (orange and purple halo around lights). The subjects reported the ingestion of some leaves of a plant that they supposed to be “donkey ears” (Plantago minor; scientific name Plantago lanceolata) a week before; they collected these herbs still in leaf buds and used them for tea and for a pasta sauce.\nIn the emergency room physical examination showed bradycardia in both, most pronounced in the male (35/min) combined with hypotension (80/60 mmHg blood pressure). ECG examination disclosed sinus rhythm and repolarization abnormalities (scooping of the ST-T complex) in both and a 2:1 AV block was detected in the male patient. He was treated with atropine (two doses of 0.5 mg, i. v.) without any effect.\nECG pattern, history and clinical findings were consistent with digitalis toxicity (), but standard plasma digoxin assay was repeatedly negative for both patients (both at the admission and after 4 hours). Nonetheless, given the high probability of digitalis toxicity, even if not confirmed by a laboratory test, after consulting with the clinical pathologist that could not exclude or confirm at that time glycoside intoxication, the male patient was treated with anti-digoxin antibodies (Digifab®, Digoxin Immune Fab (Ovine), 3 vials containing 40 mg of antibody each, infused i. v. in 30 minutes), in addition to supportive care (–). The AV block was reverted in two hours after the beginning of treatment. Anti-digoxin antibodies were not administered to woman because she was clinically stable. The presence of cardiac glycosides in plasma of both patients was subsequently confirmed by Liquid Chromatography-Mass Spectrometry (LC-MS/MS) (,). Digitoxin was identified in both patients (woman: 50 ng/mL, man: 100 ng/mL) at toxic plasma levels compatible with the severity of the clinical picture (digitoxin therapeutic range: 10–30 ng/mL) (). Colchicines and protoveratine, as possible alternative toxic drugs, were excluded by LC-MS/MS patient plasma analysis. Digoxin was confirmed as negative by LC-MS/MS. After 48 hours of monitoring, both patients were discharged. Interestingly, a sample of the plants collected by the couple, brought to the Hospital some weeks later, was analyzed by an expert botanist and found to be Digitalis purpurea. | [[61.0, 'year']] | M | {'15554746': 1, '9299991': 1, '15862081': 1, '1997019': 1, '32550814': 1, '272436': 1, '9738088': 1, '2188752': 1, '21316177': 1, '3891290': 1, '9631936': 1, '19491701': 1, '31871798': 2, '2244414': 1, '23092069': 2} | {'6906804-1': 1} |
165,465 | 3900060-1 | 23,894,868 | noncomm/PMC003xxxxxx/PMC3900060.xml | Incorrect order of draw could be mitigate the patient safety: a phlebotomy management case report | A 45 years old male outpatient, with no apparent clinical complaints, wheelchair dependent, calls to our laboratory accredited by DICQ® (DICQ is a National System of Accreditation from Brazilian Society of Clinical Analyses based on ISO 15189 document), asking for a blood specimen collection at home, for performing a series of routine laboratory tests prescribed by his physician. The tests are the: total cholesterol (CHOL), HDL-cholesterol (HDL), triglycerides (TG), sodium (Na), potassium (K), total calcium (tCa), free calcium (fCa), urea, creatinine (CREA), total protein (TP), albumin (ALB), alkaline phosphatase (ALP) and routine blood count with platelets (CBC). The patient was asked to observe 12-hours overnight fasting and the blood collection was scheduled for 7 am, 3 work days after his call. Before blood collection, the median cubital vein was located on the left forearm using only a subcutaneous tissue transilluminator device (Venoscópio IV plus, Duan do Brasil, Sao Paulo, Brazil) –without tourniquet – to prevent interference from venous stasis (–), and blood samples were collected using a 20-G straight needle (BD Vacuntainer ® Becton, Dickinson and Company Franklin Lakes, NJ, USA) connected to the holder, directly into the vacuum tubes (Greiner Bio-One GmbH, Kremsmünster, Austria). The order of different types of tube filling was: a) clot activator and gel separator tube, b) K3EDTA tube for CBC and c) a blood gas dedicated-syringe (Pico 50® with 80 I.U. lyophilized electrolyte-balanced lithium heparin, Radiometer Medical ApS, Denmark) without needle, which was directly connected to the needle-holder system in order to avoid double vein puncture. After collection, the tubes were properly mixed and transported from patient’s house to the core laboratory by car at 20 ± 2 °C in upright position inside a transport box. The blood into syringe was transported at 4 ± 1 °C, as recommended by clinical laboratory standard institute C31-A2 document (). The blood specimens (serum tube, cell blood count tube and dedicated syringe) arrived in core laboratory about one hour after blood collection and were immediately processed and analyzed (< 15 min from the arrival time). The dedicated syringe was properly mixed and the free calcium was measured by ion-selective electrode on ABL 700® (Radiometer Medical ApS, Brønshøj, Denmark), according to the manufacturer’s specifications and using proprietary reagents. Whole blood for CBC was gently mixed and analyzed on Sysmex® XE-2100D, Automated Hematology Analyzer (Sysmex Corporation®, Kobe, Japan). Serum vacuum tube was centrifuged at 1500 x g for 10min at room temperature. In sequence, the laboratory tests on serum were performed on the primary blood tube on Cobas® 6000 < c501 > module (Roche Diagnostics GmbH, Penzberg, Germany), according to the manufacturer’s specifications and using proprietary reagents. All laboratory instruments had been previously calibrated against appropriate proprietary reference standard material and verified with the use of proprietary controls. Our evaluation of the within-run precision by internal quality control is shown as coefficients of variation (CVa). The results are shown in . The laboratory staff (clinical chemistry specialist) contacted the patient by phone because some results (i.e. potassium, total calcium and free calcium) of the sample collected in dedicated syringe were out of the critical limits established by Kost (,). The patient reported that he did not use topical medicines or intravenous solution in the last four months. A new blood collection (serum tube and dedicated syringe only) was scheduled for 12.30 pm of the same day. The venipuncture for the 2nd blood collection was performed, by the same phlebotomist, in cephalic vein on the left forearm. The drawing order was: a) clot activator and gel separator tube, b) blood gas dedicated-syringe; both of the same brand and lot used for the first blood collection. So, during the 2nd venipuncture the diagnostic blood specimen collection did not include a new EDTA vacuum tube. The following procedures were identical to the described above for the 1st blood specimen collection. The 2nd blood specimens arrived in core laboratory about 45 min after collection and were immediately processed and analyzed on the same analytical instruments. The results are shown in . | [[45.0, 'year']] | M | {'25351352': 1, '21414180': 1, '31839729': 1, '32256288': 1, '23160241': 1, '24266294': 1, '23092065': 1, '26981016': 1, '10667686': 1, '33867716': 1, '22135855': 1, '33927554': 1, '20595407': 1, '15222627': 1, '22888448': 1, '10171391': 1, '22669757': 1, '8368900': 1, '25351354': 1, '22440459': 1, '21510927': 1, '28694734': 1, '22838184': 1, '22558796': 1, '22838180': 1, '23057163': 1, '20716012': 1, '21778299': 1, '7074955': 1, '12425515': 1, '16948506': 1, '27812302': 1, '18768536': 1, '23617091': 1, '23894868': 2} | {} |
165,466 | 3900314-1 | 24,465,133 | noncomm/PMC003xxxxxx/PMC3900314.xml | Bilateral renal artery thrombosis in inherited thrombophilia: a rare cause of acute kidney injury | A 47-year-old male Caucasian farmworker presented with right-sided abdominal pain for the preceding 24 hours. Apart from mild nausea, there were no other associated symptoms. He was known to have antithrombin III deficiency, with functional antithrombin III having been previously quantified at 48% (normal pooled plasma activity 83%–128%). In addition, he was known to be heterozygous for the prothrombin (G20210A) gene mutation. He was taking aspirin 75 mg daily. He had no history of thromboembolism or hypertension. He had no vascular risk factors, including diabetes, dyslipidemia, or a history of smoking.\nOn examination, he was apyrexial. His blood pressure measured 159/88 mmHg. He had right-sided flank tenderness with no rebound or guarding. Bowel sounds were normal. Urine dip revealed 2+ protein but no blood. Creatinine was 1.02 mg/dL (90 μmol/L). Neutrophils were moderately raised at 9.4 × 109/L. CRP (C-reactive protein) was unremarkable at 1.7 mg/L. His pain was managed with paracetamol (acetaminophen) and a single dose of 50 mg diclofenac. Aspirin was withheld and he was given a daily prophylactic dose of enoxaparin 40 mg. Within 24 hours, his abdominal pain had localized to the right iliac fossa and he underwent appendectomy. Intraoperatively, his blood pressure fell to 100/50, requiring intravenous fluid resuscitation. Postoperatively, his abdominal pain settled. He was discharged home the following day with a blood pressure of 125/75 mmHg and a creatinine level of 1.17 mg/dL (103 μmol/L).\nThe patient was readmitted 4 days later with sudden-onset, severe left-sided abdominal pain. He was apyrexial. Blood pressure measured 150/80 mmHg. Examination revealed left-sided abdominal tenderness without rebound or guarding. Bowel sounds were normal. Urine testing revealed new microscopic hematuria. Inflammatory markers were raised with a neutrophil count of 11.7 × 109/L and a CRP level of 43.5 mg/L. Creatinine had increased to 1.62 mg/dL (143 μmol/L). Computed tomography with oral contrast revealed an 85 × 35 mm collection at the appendectomy site. Significant left-sided perinephric stranding was also seen (). He was treated for a postoperative intra-abdominal collection with intravenous co-amoxiclav and metronidazole. Prophylactic enoxaparin 40 mg was given daily. Despite treatment, his inflammatory markers continued to climb, with CRP peaking at 363.9 mg/L. In addition, his hematuria became macroscopic and his renal function declined to a creatinine level of 2.85 mg/dL (252 μmol/L), equating to an estimated glomerular filtration rate (GFR) of 22 mL/min/1.73m2. Anti-neutrophil cytoplasmic antibody (ANCA), double-stranded DNA (dsDNA) and anti-glomerular basement membrane antibody (anti-GBM) were negative. Immunoglobulins were normal and Bence Jones protein was not present in the urine. Nuclear medicine renography confirmed a significant kidney injury, showing a GFR of 21.72 mL/minute. It also demonstrated areas of poor uptake in the right kidney. Computed tomography angiography confirmed large bilateral renal infarcts with occluding thrombus visible in the left renal artery (). Computed tomography and echocardiography excluded left ventricular and aortic thrombi. The patient was commenced on life-long warfarin therapy.\nSix months later, his creatinine had stabilized to 1.98 mg/dL (175 μmol/L), equating to an estimated GFR of 36 mL/min/1.73m2. Blood pressure control required treatment with a combination of three agents. Urinary protein:creatinine ratio measured 698 mg/g and microscopic hematuria persisted. | [[47.0, 'year']] | M | {'12421140': 1, '7947234': 1, '16247095': 1, '31016074': 2, '34055700': 2, '27307798': 1, '18662160': 1, '27057875': 2, '28424376': 1, '7100022': 1, '2339350': 1, '19141163': 1, '19932950': 1, '12616091': 1, '28466834': 1, '20429882': 2, '7218490': 1, '10195932': 1, '11279641': 1, '2960305': 1, '1448834': 1, '24465133': 2} | {'6464458-1': 1, '8160427-1': 1, '2868876-1': 1, '4998791-1': 1} |
165,467 | 3900348-1 | 24,474,106 | noncomm/PMC003xxxxxx/PMC3900348.xml | Bullous pemphigoid in a 3-month-old infant: case report and literature\nreview of this dermatosis in childhood | White infant female, three months and fifteen days old, developed multiple tight bullae\nfive days ago, initially on palms and soles, with progressive involvement of trunk,\nlimbs and head, accompanied by papules and plaques associated or not to blisters.\nParents were not consanguineous, denied fever or use of medications, as well as other\ncomorbidities. Personal and family history of atopy, other cutaneous diseases or\nautoimmune disorders was negative. The patient was born vaginally, at term, without\ncomplications. Exclusively breastfed. The patient received the recommended vaccines,\nwith the last dose about 45 days previously.\nDermatological examination revealed the presence of multiple vesicles and widespread\ntight blisters with serous content and in different sizes, affecting areas of normal as\nwell as erythematous skin. She presented serohemorrhagic crusts, papules and annular\nurticarial erythematous plaques, some topped by vesicles and blisters. Lesions were\nlocated predominantly in the hands, feet, scalp, limbs, trunk and back. There were no\nmucosal lesions. Nikolsky sign was negative ( to ). Complete blood count showed\nmild anemia (hemoglobin: 10.8 g / dL), eosinophilia (2,300 eosinophils/mm3 or\n26% of total leukocytes) and thrombocytosis (585,000 platelets). Histopathology showed\nspongiotic dermatitis with subepidermal bullae rich in neutrophils and eosinophils. DIF\ndetected the presence of linear IgG and C3 deposits along the subepidermal basal\nmembrane zone. Immunohistochemical staining for type IV collagen was positive at the\nbase of the blister, demonstrating that cleaving occurred above the lamina densa.\nSaltsplit skin technique and IIFs (indirect immunofluorescence) could not be performed.\n( to )\nThe set of clinical and laboratory data confirmed the diagnosis of infantile bullous\npemphigoid. Treatment was started with prednisolone 1mg/kg. After five days, due to\npartial response, the dose of prednisolone was increased to 2mg/kg/day. Reassessment\nafter fifteen days showed good control of the lesions, with only sparse escape blisters.\nVaccinations were suspended on account of the immunosuppressive doses of\ncorticosteroids. In order to permit corticosteroid weaning, dapsone was initiated at 0.5\nmg / kg. Previous control exams had shown normal G6PD levels and no subsequent worsening\nof anemia occurred with medication. Corticosteroid therapy was reduced by 0.5 mg / kg\nevery two weeks until its full suspension, while dapsone was maintained at 0.5 mg / kg /\nday. Patient remained without skin lesions for three months, and resumed the use of\nvaccines without live antigens according to the national vaccination calendar. At nine\nmonths old, she received acellular conjugated vaccines (DTPA), anti hepatitis B, polio\nand meningococcal diseases, with subsequent display of new bullous lesions. Dapsone was\nincreased to a dose of 1mg/kg and prednisolone 1mg/kg was initiated for a week. With\nregression of the clinical symptoms dapsone was maintained for six months in which\nperiod no new lesions appeared, even with the use of vaccines, so, the medication was\ndiscontinued. The patient is followed-up in the outpatient dermatology service, and she\nhas been lesion-free for six months without any signs of recurrence to date. | [[3.0, 'month'], [15.0, 'day']] | F | {'19503978': 1, '29267440': 2, '22560135': 1, '22781589': 1, '1998369': 1, '22137222': 1, '9572692': 1, '21603823': 1, '17945382': 1, '20375426': 1, '22393540': 1, '20579597': 1, '21430884': 1, '16206462': 1, '22963966': 1, '20447089': 1, '17310001': 1, '21054491': 1, '21955378': 1, '1829723': 1, '19453790': 1, '30210165': 2, '17472685': 1, '22534318': 1, '24474106': 2} | {'6124229-1': 1, '5726671-1': 1} |
165,468 | 3900350-1 | 24,474,108 | noncomm/PMC003xxxxxx/PMC3900350.xml | Ectopic cutaneous schistosomiasis - Case report | A 23-year-old female patient, single, nurse, born in and from Maceió/AL reported the\nonset of pruriginous lesions on the abdomen about 2 months before the appointment, with\npartial regression and onset of new lesions on the right scapular region and back two\nweeks later. She mentioned having followed some treatments using topical and oral\nmedications (corticosteroids , antifungals , antibiotics) prescribed by dermatologists\nwithout success. She denied systemic complaints, history of river bathing in childhood\nand adolescence, the last occasion having happened four years ago. At the dermatological\nexamination were found papulous red-brown and shiny coalescent lesions, forming\nzosteriform plaques on the abdomen, back and right scapular region (, and ). Complementary exams: hemogram with eosinophilia\n(13%); biochemistry without alterations; negative parasitological stool exam with 3\nsamples (Hoffman and Kato-Katz); serology for schistosoma mansoni\n(indirect immunofluorescence) was non-reactive; ultrasound of total abdomen\nwithout alterations. Histopathological exam: granulomatous inflammatory infiltrate\ncomposed of lymphocytes, plasmocytes and histiocytes, with rare giant cells surrounding\nparasite eggs (s. mansoni). Interspersed eosinophiles can also be\nobserved. Epidermis without significant alterations (). Treatment: praziquantel 50 mg/kgm single dose. Evolution: total\nregression of lesions after a few months of treatment (). | [[23.0, 'year']] | F | {'25587473': 2, '10773705': 1, '15486639': 1, '963464': 1, '21173919': 1, '26598562': 1, '27447892': 1, '30753565': 1, '17420842': 1, '24474108': 2} | {'4283256-1': 1} |
165,469 | 3900351-1 | 24,474,109 | noncomm/PMC003xxxxxx/PMC3900351.xml | Cutaneous lesions and finger clubbing uncovering hypocomplementemic\nurticarial vasculitis and hepatitis C with mixed cryoglobulinemia | A 49-year-old woman presented with a 1-year history of highly pruritic generalized\ncutaneous lesions. She denied systemic symptoms. She was otherwise healthy and was on no\nregular medication. Physical examination revealed localized urticarial wheals and\npurpuric macules on the forearms, wrists, thighs, legs and dorsum of the feet; according\nto the patient, the lesions lasted longer than 24 hours and resolved with\nhyperpigmentation (-). Finger clubbing was also evident (). There were no other findings on physical examination.\nHistopathologic examination of a cutaneous lesion showed a perivascular inflammatory\ninfiltrate in the superficial and mid dermis, composed mainly of polymorphonuclear\nneutrophils and rare eosinophils, associated with fibrinoid necrosis of the vascular\nwall and leukocytoclasia (). These\nfeatures were consistent with a leukocytoclastic vasculitis. Laboratory tests disclosed\nincreased liver enzymes (ALT 90 U/L, normal 10-30, AST 96 U/L, normal 1036), positive\nanti-hepatitis C virus (HCV) antibodies with high titres of serum HCV RNA (720000 UI/L),\nlow complement levels (C1q 3.0 mg/dL, normal 12.220.8; C4 < 2 mg/dL, normal 12-42)\nand type II mixed cryoglobulinemia (cryoglobulins 187 mcg/mL, mixed type II\nIgG-IgM/Kappa). The remainder of the laboratory tests were negative or within the normal\nrange. Abdominal ultrasound showed diffuse hepatic steatosis and thorax x-ray was\nunremarkable. Altogether clinical, laboratory and histopathological findings allowed the\ndiagnosis of hypocomplementemic urticarial vasculitis and hepatitis C with myxed\ncryoglobulinemia. The patient started symptomatic treatment with hydroxyzine 25 mg PO\nbid, with partial control of the pruritus, and was referred to a gastroenterology\nconsultation for management of the hepatic condition. A liver biopsy was performed and\nshowed features of chronic hepatitis with scarce fibrosis. In the meantime, liver enzyme\nlevels returned to the normal range (ALT 22 U/L, normal 10-30, AST 28 U/L, normal\n10-36). This was accompanied by progressive clearance of cutaneous lesions and\nresolution of the pruritus; residual hypopigmentation persisted in the sites of previous\nscratching. Given the benign liver pathology and the lack of hepatitis C activity, it\nwas decided by the gastroenterology specialist not to start any kind of treatment but to\nkeep the patient under close surveillance instead. The cutaneous condition remains\nstable after one year of follow-up, similar to the hepatic disease course. | [[49.0, 'year']] | F | {'12637912': 1, '18284262': 1, '16426368': 1, '15928621': 1, '3040016': 1, '20227577': 1, '16879251': 1, '24474109': 2} | {} |
165,470 | 3900352-1 | 24,474,110 | noncomm/PMC003xxxxxx/PMC3900352.xml | Generalized Lichen Nitidus in a boy with Niemann-Pick disease type B | A 12-year-old boy presented with a 12-month history of asymptomatic papules that first\nappeared on his shoulders and gradually spread to involve most of the skin surface. The\npatient had Niemann-Pick disease type B, confirmed by genetic study at the age of 4.\nHepatosplenomegaly and dyslipidaemia were the main manifestations of the disease. He has\nbeen in the 10th percentile for both weight and height. His neurological state and\nintellectual ability were normal. There was no history of consanguinity between his\nparents. His 16 year-old sister was also affected by Niemann-Pick disease, although\nwithout any cutaneous manifestations.\nOn physical examination, there were numerous discrete, skin-coloured, flat-topped,\nmonomorphic, pinhead-sized papules grouped over the abdomen, umbilicus, trunk, penile\nshaft, upper extremities, knees and malleolar regions. On the lower back some of the\nlesions were grouped in linear array, possibly as a result of the Koebner phenomenon\n(). The face, oral mucosa and\npalmoplantar surfaces were not involved.\nHistopathological examination revealed a well circumscribed, lymphohistiocytic\ngranulomatous infiltrate in the papillary dermis. This infiltrate appeared to be\nclutched by elongated epidermal ridges resembling a "ball and claw" pattern (). The clinicopathological picture was\nentirely in-keeping with lichen nitidus. | [[12.0, 'year']] | M | {'9092753': 1, '22329413': 1, '22714765': 1, '7857847': 1, '24474110': 2} | {} |
165,471 | 3900354-1 | 24,474,112 | noncomm/PMC003xxxxxx/PMC3900354.xml | Bannayan-Riley-Ruvalcaba syndrome with deforming lipomatous hamartomas in\ninfant - Case report | We describe the case of a nine-month-old male child, who had fast growing and\nprogressive lesions on the trunk and abdomen since the age of 6 months. A previous\nevaluation by a pediatrician diagnosed first degree malnutrition and absence of\npsychomotor development deficit. At the examination, the child was thinner, presented\nmacrocephaly, voluminous abdomen with multiple masses of soft consistency, affecting\nalso the back and thorax, the larger of the lesions being located on his right flank\n(). He also presented brownish, round\nmacules on the penis, measuring around three millimeters in diameter ().\nGeneral physical examinations included lipid profile, without any alterations.\nComputerized tomography of the abdomen and pelvis revealed extensive lipomatosis in the\nright postero-lateral thorax-abdomen region at the T8 level, extending downward until\nthe perineal region with signs of controlateral paraspinal muscle invasion starting at\nT12. Lesional infiltration of vertebral, retrodural and psoas muscle epidural regions of\nthe spinal canal could also be bilaterally observed (). Magnetic resonance of the thoracic column revealed lipomatous\ndorsal lesion associated with enlargement of the spinal canal and spinal cord\ncompression in the low thoracic column, as well as nonenhanced lumbosacral medullary\ncompression after contrast.\nThe child underwent surgery for partial removal of the larger lesion, thus avoiding\nprogression of medullary compression and functional deficit; examination of the sample\nconfirmed the lipoma diagnosis (). After\nthe surgery, growth of the other lesions was observed, although without functional\ndeficit (). In order to detect chromosomal\nalterations in the adipocytic lesion, DNA was extracted, followed by CGH array. Loss of\napproximately 1MB of the long arm of chromosome 10, the region where the PTEN gene is\nlocated, was revealed. The same alteration was confirmed in the peripheral blood of the\npatient.\nThe authors present this case because of its exuberance and rarity. The child should be\nfollowed for early detection of intestinal polyps and possible neoplasms. | [[9.0, 'month']] | M | {'10640930': 1, '16198785': 1, '20697678': 1, '10400993': 1, '11332402': 1, '22252256': 1, '9467011': 1, '18781191': 1, '19321504': 1, '28401059': 2, '24474112': 2} | {'5385310-1': 1} |
165,472 | 3900355-1 | 24,474,113 | noncomm/PMC003xxxxxx/PMC3900355.xml | Dermatoscopic findings of urticaria pigmentosa | We report a 55 year-old female with asymptomatic brownish macules and papules in the\nneck, inframammary area, lower abdomen and thighs with a positive Darier`s sign since a\nyear ago (). She reported sporadic\nepisodes of epigastric pain, sweating, dizziness, iarrhea and headache. There was no\nhospitalization history for anaphylaxis and drug allergy. Dermoscopy showed a pigment\nnetwork anda light-brownish blot ( and\n). We performed complete blood count,\nhepatogram and kidney function, tryptase and abdominal ultrasonography which were all\nnormal. A biopsy taken from an abdominal lesion showed hyperpigmentation of the basal\nlayer and dermal grouping of lymphocytes and mast cells, identified with the Giemsa\nstaining, around vessels ( and ). | [[55.0, 'year']] | F | {'21844452': 1, '32695725': 1, '10392589': 1, '11377676': 1, '19060465': 1, '19416274': 1, '31544081': 2, '27613297': 1, '12734496': 1, '12174079': 1, '24474113': 2} | {'6743404-1': 1, '6743404-2': 1} |
165,473 | 3900359-1 | 24,474,117 | noncomm/PMC003xxxxxx/PMC3900359.xml | Case for diagnosis | A 68-year-old white female patient complained of an asymptomatic nodular lesion on the\nsecond left toe, with onset 7 years before and progressive increase in size since then.\nAt physical examination a voluminous nodular lesion was observed, with smooth\nerythematous surface, telangiectasias and 4cm in diameter (). There were no palpable swollen inguinal lymph nodes.\nA lesion biopsy was performed and the histopathological examination revealed cuboidal\ncell erytheagglomerates with basophilic round nucleus and eosinophilic cytoplasm\nadjacent to cartilaginous tissue ().\nA radiography of the left foot revealed increase in soft tissue without bone\ninvolvement. | [[68.0, 'year']] | F | {'16962023': 1, '17642601': 1, '15335266': 1, '12564820': 1, '20371021': 1, '19863434': 1, '12562350': 1, '24474117': 2} | {} |
165,474 | 3900360-1 | 24,474,118 | noncomm/PMC003xxxxxx/PMC3900360.xml | Case for diagnosis | A 1-month-old boy, born at term after an uneventful pregnancy, with a birth weight of\n3,480g. First child of healthy and unrelated parents. At birth his mother noticed a 1 x\n1.5 cm erythematous, infiltrated, perianal plaque that rapidly progressed to ulceration\n(). The patient was otherwise healthy.\nA second evaluation at age two and a half months showed a complete and spontaneous\nresolution of the lesion (). Serologic\ntest for syphilis (VDRL) was negative both for the mother and the patient.\nHistopathological exam revealed a dermal infiltrate with predominance of large, round\nhistiocytic cells with dense eosinophilic cytoplasm, with ground glass appearance, and\neccentric reniform nuclei ().\nImmunohistochemical staining was positive for S100 and CD1a ( and ). Routine\nlaboratory workup and radiographs of chest, skull, pelvis and long bones were within\nnormal ranges. The patient remains asymptomatic with no signs of recurrence. | [[1.0, 'month']] | M | {'16585988': 1, '15057004': 1, '28977321': 1, '17291304': 1, '16261594': 1, '15124855': 1, '21987150': 1, '16780478': 1, '21158911': 1, '17224372': 1, '21864117': 1, '24474118': 2} | {} |
165,475 | 3900361-1 | 24,474,119 | noncomm/PMC003xxxxxx/PMC3900361.xml | Case for diagnosis | Twenty-seven year old female patient, phototype V, presented a lesion in the right arm\nfor one and a half years, without associated symptoms. Dermatological examination\nrevealed a smooth-surfaced, brownish, painless nodule, movable within deep layers,\nmeasuring three centimeters and located in the right arm (). Pathological examination of the surgical specimen showed dense\nproliferation of large cells with granular-appearing eosinophilic cytoplasm, distinct\nmembranes, central or eccentric nuclei and slightly conspicuous nucleoli. The neoplastic\ncells formed syncytia in some areas and were inserted within the interstices of a\ndesmoplastic stroma, with scarce focal lymphocytic infiltration. The results of\nimmunohistochemical examination were positive for S100 protein, CD63 and CD68 (). | [[27.0, 'year']] | F | {'22420297': 1, '19445877': 1, '34480587': 1, '16133461': 1, '20471560': 1, '29127127': 1, '12041812': 1, '27148567': 1, '10503740': 1, '24474119': 2} | {} |
165,476 | 3900362-1 | 24,474,120 | noncomm/PMC003xxxxxx/PMC3900362.xml | Do you know this syndrome? | Woman, 65-year-old, with sensory-motor polyneuropathy for 15 years, worsening in the\nprevious 6 months. The patient had diffuse hyperpigmentation, dry skin and hemangiomas\n( and ). She reported hypertrichosis, edema of the legs, facial lipoatrophy,\nleukonychia and weight loss ( and ). Analytically, she presented polyglobulia,\nthrombocytosis, hypotestosteronemia, hyperalbuminemia and elevation of IgG in the\nliquor. The immunochemical study emphasized IgG monoclonal gammopathy of lambda\nchains.\nInvestigations showed small adenopathies, cardiomegaly, hepatosplenomegaly, generalized\nedema and extensive lytic lesion of L5. A biopsy of this lesion showed a lytic\nproliferation of plasma cells, CD138 +, К chains - chains λ +. The myelogram showed\nhypercellularity and eosinophilia. The electromyogram showed slowing of motor and\nsensory nerve conduction of several nerves. An excision of the right breast angioma was\nperformed and was compatible with capillary hemangioma. The patient has begun treatment\nwith dexamethasone, radiotherapy, bortezomib and is waiting for a bone marrow\ntransplant. | [[65.0, 'year']] | F | {'30902082': 2, '2164341': 1, '19077091': 1, '16781311': 1, '33530791': 2, '9255373': 1, '20500193': 1, '20566924': 1, '24474120': 2} | {'7871093-1': 1, '6431048-1': 1} |
165,477 | 3900363-1 | 24,474,121 | noncomm/PMC003xxxxxx/PMC3900363.xml | Do you know this syndrome? | A 17-year-old female patient was referred from the Endocrinology Department with\nyellowish papules on her arms and legs for about a year.\nOn examination, the patient presented acromegaloid fascies, generalized lipoatrophy,\nphlebomegaly, hepatomegaly, axillary acanthosis nigricans, clitoromegaly, and yellowish\ncoalescing and isolated papules with an erythematous halo in the upper and lower limbs\n().\nFrom birth, she presented a total absence of subcutaneous fat. At age 10, she had\nimpaired glucose tolerance which, because unchecked, had developed into diabetic\nketoacidosis in under one year. Subsequently, she presented type 2 diabetes mellitus\n(DM) associated with dyslipidemia and hepatic steatosis. These disorders were not\nresponsive to the usual pharmacological doses, and required the administration of high\ndoses of exogenous insulin, oral hypoglycemic and hypolipidemic agents. Despite therapy,\ntests showed fasting glucose level of 284 mg/dl; postprandial glucose, 423 mg/dl; total\ncholesterol, 579 mg/dl; and triglycerides, 3.194 mg/dl. Histopathological examination\nconfirmed clinical hypothesis of eruptive xanthoma (). | [[17.0, 'year']] | F | {'14065985': 1, '12165227': 1, '14444642': 1, '13130666': 1, '15629135': 1, '21508592': 1, '30559880': 1, '15028826': 1, '33411809': 1, '16722806': 1, '26931459': 1, '27766009': 1, '24474121': 2} | {} |
165,478 | 3901039-1 | 24,466,426 | noncomm/PMC003xxxxxx/PMC3901039.xml | Presensitization revisited: pitfalls of vascular allografts in transplant candidates | A 44-year-old woman previously immunized by several pregnancies developed ESRD and started hemodialysis in 2009. The first arteriovenous fistula soon occluded. Neither surgical revisions nor prosthetic grafts resulted in long-term patency. Menometrorrhagia led to blood transfusions in 2009 and 2011. Antibodies to several HLA class I molecules were detected in a Luminex single antigen assay before the first blood transfusions, but by August 2010 only reactivity to HLA-A24 was observed (a mean fluorescence index >1000 defined as positive). Overweight delayed by 2 years acceptance to the renal transplant ‘waiting list’. Meanwhile, graft thrombosis impaired dialysis access, necessitating insertion of a fresh arterial allograft from a deceased donor in September 2010. Antibodies to a broad range of HLA class I and II molecules were detected by Luminex single antigen assay in January 2012, but the panel reactive antibodies (PRAs), as determined by CDC cell screening, remained negative. Menometrorrhagia precluded anticoagulation. The arterial allograft was found obliterated at removal in February 2012 and replaced by another fresh arterial allograft. By April 2012, the PRA reactivity was 80% and Luminex analyses in June 2012 identified strong antibodies to almost all HLA class I and II molecules, including HLA-A1, -B60, -DR4 and -DQ8 present in the allograft. | [[44.0, 'year']] | F | {'11907435': 1, '23384346': 1, '31383839': 1, '19394535': 1, '20172310': 1, '21360466': 1, '17097949': 1, '33415125': 1, '12392854': 1, '15191518': 1, '8600626': 1, '16182785': 1, '11436087': 1, '23591442': 1, '21095143': 1, '19021970': 1, '27346143': 1, '9546235': 1, '24466426': 2} | {'3901039-2': 2} |
165,479 | 3901039-2 | 24,466,426 | noncomm/PMC003xxxxxx/PMC3901039.xml | Presensitization revisited: pitfalls of vascular allografts in transplant candidates | A man aged 55 received his first kidney transplant from an HLA-identical sibling in 2010 and was immunosuppressed with steroids and cyclosporine. Luminex screen test was negative in pretransplant sera, but positive (ratio >2.5) 1 year after transplantation. The Luminex single antigen assay then revealed multiple antibodies directed to HLA class I and II molecules. Since no blood transfusions had been performed, the only plausible immunizing event was an arterial interponate from a deceased donor inserted during the transplantation to prolong the donor renal artery. We suspected renal transplant artery stenosis as the patient developed hypertension requiring the addition of a selective alpha-1-receptor blocker to his preexisting regimen of beta-blocker, angiotensin-II-receptor antagonist and loop diuretics. Ultrasound investigations 13 and 19 months post-transplantation indicated moderate proximal renal transplant artery stenosis. A fluorodeoxyglucose (18F)-positron emission tomography (FDG-PET) scan was performed 19 months after transplantation in order to detect inflammation in the renal artery interponate, but the result was negative. Twenty-three months post-transplantation, magnetic resonance imaging confirmed a proximal narrowing of the transplant artery, but no significant stenosis. Taken together with stable graft function at an eGFR of 42, indication for intervention was not found. Three years after transplantation graft function, blood pressure and medication remain unaltered. | [[55.0, 'year']] | M | {'11907435': 1, '23384346': 1, '31383839': 1, '19394535': 1, '20172310': 1, '21360466': 1, '17097949': 1, '33415125': 1, '12392854': 1, '15191518': 1, '8600626': 1, '16182785': 1, '11436087': 1, '23591442': 1, '21095143': 1, '19021970': 1, '27346143': 1, '9546235': 1, '24466426': 2} | {'3901039-1': 2} |
165,480 | 3901585-1 | 24,474,926 | noncomm/PMC003xxxxxx/PMC3901585.xml | Primary Retroperitoneal Müllerian Adenocarcinoma: A Case Report and Literature Review | A 54-year-old primiparous woman presented with a 6-month history of mild abdominal pain and distension of the right portion of the abdomen. Her family history was not contributory. Her past surgical history included a total thyroidectomy for papillary carcinoma followed by radioiodine therapy 3 years before.\nPhysical examination revealed a slightly tender, ill-defined mass over the left abdomen. The remaining systemic examination did not reveal any coexistent lesion. Laboratory tests, including complete blood count, chemistry profile, urinalysis and chest X-ray, were all within normal limits. Serum tumor markers were negative.\nAn abdominal ultrasound (US) documented the presence of a coarse, partially cystic mass in the right retroperitoneal region. Abdominopelvic CT and MRI revealed a large unilocular cystic mass measuring 11 cm with an enhancing solid portion and partial water content, located in the retroperitoneal space adjacent to the lower pole of the right kidney, with wall calcifications (fig. ). There was no evidence of extracystic extension or distant metastasis. On suspicion of a hydatid cyst, serological tests were performed and yielded negative results. A gynecological examination, including hysteroscopy and Pap smear, was also performed. It was negative for utero-adnexal pathology.\nThe patient underwent laparotomy with subcostal transverse incision. On opening the peritoneal cavity, ascitic fluid was found and sampled. Cytological examination of the fluid did not reveal the presence of malignant cells. Abdominal cavity exploration revealed no abnormal findings in the liver, stomach, large bowel and spleen, and the uterus, ovaries, fallopian tubes and appendix were all normal. Sequential exploration of the small intestine revealed the presence of an exophytic lesion about 1 cm in diameter, which was removed and whose definitive histological examination was compatible with fibroma of the small intestine. A large, encapsulated cystic mass was found in the right retroperitoneum, below the lower pole of the right kidney that was displaced posteriorly and cranially to the mass. The cecum, right colon and right colic flexure were displaced medially to the mass. The mass was dissected from the surrounding structures. A fragment of the cystic wall was sent for extemporaneous histological examination and it was compatible with a fibrous wall denuded of lining epithelium. Complete excision of the cyst was performed. The postoperative period was uneventful, and the patient was discharged home 5 days after surgery.\nThe gross pathological findings showed a 12 × 10-cm cystic mass with a smooth surface. The cystic wall measured 0.3 cm at maximum thickness. The tumor was composed of malignant epithelial and stromal components. Microscopically, the fibrous wall of the cyst delimited an extensively necrotic lesion containing chronic inflammation, calcification, cholesterol crystals and hemorrhagic extravasation. On hematoxylin and eosin staining, the entire tumor showed cellular whorls dispersed amidst pale sarcomatous stroma. The cells in whorls were round to short, spindle shaped and had moderate mitotic activity. The sections, however, revealed whorled glandular areas.\nA panel of immunohistochemical tests was performed: whorled glandular areas were positive for cytokeratin (CK19) and negative for vimentin, confirming their epithelial nature; they were also negative for CK20 and calretinin. The stromal component of the tumor, highlighted on vimentin stain (vimentin), was positive for CD10. No immunoreactivity to estrogen and progesterone receptors was found. The presence of necrosis, cytonuclear atypia and immunophenotypic features were compatible with PRMA (fig. ).\nSix courses of adjuvant chemotherapy were administered with carboplatin at a dose of 5 AUC and taxol 175 mg/mq/ev. A follow-up abdominal CT, performed 9 months later, was negative for tumor recurrence. Written informed consent was obtained from the patient for publication of this case report and all accompanying images. | [[54.0, 'year']] | F | {'23800846': 1, '17224073': 2, '19593619': 1, '21244653': 2, '15099978': 1, '26137100': 1, '20952288': 1, '21139951': 1, '17079007': 1, '6054929': 1, '15371613': 1, '19697437': 1, '16445618': 1, '24474926': 2} | {'1783654-1': 1, '1783654-2': 1, '3224474-1': 1} |
165,481 | 3901586-1 | 24,474,931 | noncomm/PMC003xxxxxx/PMC3901586.xml | Orbital Cellulitis Presenting as a First Sign of Incomplete Kawasaki Disease | A 6-year-old boy was referred to the Department of Ophthalmology, Marmara University School of Medicine, with the diagnosis of orbital cellulitis. He had had mild fever, pharyngitis and conjunctivitis for 7 days. Four days prior to presenting at our department, he had been referred with complaints of irritation to an ophthalmologist. Consultation with a pediatrician due to his fever resulted in a diagnosis of conjunctivitis and pansinusitis and he was treated with ampicillin-sulbactam and ceftriaxone 100 mg/kg/day. In the following 3 days, restriction of upgaze in the right eye and inflammation of the eyelids began and he was referred to us with a diagnosis of orbital cellulitis. An initial examination revealed normal visual acuity, bilateral conjunctivitis with excessive secretion, periocular inflammation and edema, right preauricular lymphadenopathy and right restriction in the upgaze and his lips were dry and cracked (fig. ). Magnetic resonance imaging confirmed orbital cellulitis and pansinusitis (fig. ).\nHis erythrocyte sedimentation rate on the first day of hospitalization was 55 mm/h and the C-reactive protein level was 22.4 mg/dl. Laboratory findings included a white blood cell (WBC) count of 19,000 cells per mm3, with 81.5% neutrophils, 15.0% lymphocytes, 1.2% monocytes and 0.4% basophils. The antibiotherapy was changed to vancomycin 60 mg/kg/day and meropenem 60 mg/kg/day. After 10 days of hospitalization without a clear response to antibiotherapy, periungual desquamation of the fingers started (fig. ). Pediatric examination revealed a strawberry tongue. Repeated laboratory tests revealed a total WBC count of 15,300 cells per mm3 with 63.8% neutrophils, an elevated platelet count (650,000 mm3), an erythrocyte sedimentation rate of 52 mm/h and an elevated C-reactive protein level of 46.3 mg/dl. The serum transaminase urine examination was normal. Following the algorithm defined previously, we concluded a diagnosis of incomplete KD (table ; online , see ) []. With the first echocardiography, no coronary artery lesions were detected. Aspirin (100 mg/day) was administered and by the third day of its administration, the orbital cellulitis and fever had regressed and the patient was discharged from hospital with oral antibiotics and aspirin. After 6 weeks, his control echocardiography revealed dilatation of the right and left coronary arteries and the diagnosis of incomplete KD was confirmed. He was treated with a single dose of intravenous immunoglobulin, 2 mg/kg over 10 h. | [[6.0, 'year']] | M | {'33194239': 2, '34837997': 2, '10979916': 1, '12488668': 1, '20616737': 1, '15505111': 1, '4153258': 1, '15936970': 1, '17019412': 1, '10779274': 1, '15346907': 1, '12182382': 1, '24474931': 2} | {'7641693-1': 1, '8626717-1': 1} |
165,482 | 3901587-1 | 24,474,924 | noncomm/PMC003xxxxxx/PMC3901587.xml | Disposition of Erlotinib and Its Metabolite OSI420 in a Patient with High Bilirubin Levels | In this case report, we describe the therapeutic drug monitoring (TDM) of erlotinib in a patient with abnormally high bilirubin levels with the aim to gain additional information on potentially necessary dose modifications or cessation of drug medication in patients with heavily impaired liver function.\nThe case of a 62-year-old male patient of 77 kg body weight with an adenocarcinoma of the pancreas and metastases in liver and lung is presented. From July 2009 to January 2010, seven cycles of combination chemotherapy consisting of gemcitabine and oxaliplatin were administered. The best response reached under this treatment was a partial response. Thereafter, treatment was discontinued because of hepatic progression of the disease. From March to April 2010, the patient was treated within the frame of a phase I study with an aurora-kinase B inhibitor. Because of progression in form of an increase of pre-existing, and appearance of new, hepatic and pulmonary metastases, the patient did no longer take part in the study. In April 2010, an icterus developed, necessitating an endoscopic retrograde cholangiopancreatography as well as the insertion of a stent to allow recanalisation and elimination of bile and, simultaneously, bilirubin. Nevertheless, despite the attempt of recanalisation of the bile duct, disease progression led to a further increase in bilirubin accompanied by icterus and pruritus.\nAfter informing the patient in detail about the pros and cons of planning any further therapeutic step at all and after extensive discussion, erlotinib was used in the context of the now highly elevated bilirubin levels, with total bilirubin levels of 14.15 mg/dl (normal range 0.1–1.0 mg/dl) and direct bilirubin levels of 11.32 mg/dl (normal range 0.1–0.25 mg/dl), for which experience is lacking and intake of medication therefore not recommended. Written informed consent was obtained from the patient. The patient explicitly agreed to receive erlotinib 150 mg daily for 16 days and additional accompanying blood drawings in order to allow elucidating the pharmacokinetics in this peculiar situation of impaired liver function. The investigation had been approved by the ethical committee. | [[62.0, 'year']] | M | {'18391918': 1, '16014882': 1, '20094773': 1, '22927532': 1, '17483950': 1, '11432895': 1, '20493771': 1, '17625975': 1, '19850672': 1, '22285168': 1, '17228132': 1, '21783417': 1, '17452677': 1, '26168967': 1, '24474924': 2} | {} |
165,483 | 3901589-1 | 24,474,925 | noncomm/PMC003xxxxxx/PMC3901589.xml | Oxaliplatin-Induced Leukocytoclastic Vasculitis under Adjuvant Chemotherapy for Colorectal Cancer: Two Cases of a Rare Adverse Event | A 75-year-old man presented with a locally advanced adenocarcinoma of the rectum (37 cm above the anal verge, endoscopically staged as uT3 uN). As he had received interstitial brachytherapy (>145 Gy) 2 years before as a treatment for prostate cancer (cT2b, cNx, cM0, Gleason score 6), the standard treatment of neoadjuvant RCT with 50.4 Gy and concomitant 5-FU (+/− oxaliplatin) was not possible. An abdomino-perineal rectum exstirpation with implantation of a descendostoma was performed to remove the rectal cancer. The histopathological work-up revealed a pT3a, pN2b (21/31), cM0, L1, V0, G3, R0 (UICC IIIB) adenocarcinoma and thus adjuvant chemotherapy was strongly suggested according to the vote of the institutional interdisciplinary tumor board.\nEight weeks after surgery, the first cycle of adjuvant chemotherapy with 5-FU, leucovorin and oxaliplatin (FOLFOX-6) was scheduled. The cycle consisted of a bimonthly regimen with intravenous oxaliplatin (80 mg/m2) and leucovorin (400 mg/m2) administered on day 1. 5-FU 2,400 mg/m2 followed on day 1 as a continuous infusion for 46 h. During the following 3 days, the patient developed fever that resolved spontaneously. Fourteen days later, the patient showed disseminated petechiae with secondary hemorrhage, along with edema on both legs and interdigital ulceration. He reported that he had experienced arthralgia in his knees and elbows 4 days after the first dose of chemotherapy. His blood count showed microcytic, hypochromic anemia, leukocytosis of 13,900/μl, elevated C-reactive protein of 90.1 mg/l, and an increase in the retention parameters (urea 25 mg/dl), indicating an affliction of the kidneys. The urine analysis was normal apart from an alkaline pH of 7.5.\nThe chemotherapy was interrupted and the patient was admitted to the Department of Dermatology. On examination, there was palpable purpura with petechial hemorrhage on the lower limb in addition to massive edema on both lower legs (fig. ). Some petechiae were also found on the lower arms and the abdomen. We suspected drug toxicity to be the cause of the skin lesions. A skin biopsy confirmed the diagnosis of leukocytoclastic vasculitis. The patient did not complain about abdominal pain and 3 tests for occult blood in the stool were negative. There was no evidence for a systemic vasculitis reaction. After 2 days, the renal parameters were rising (creatinine 1.33 mg/dl; normal range 0.5–1.0 mg/dl) and the patient developed proteinuria of 1,200 mg/day in the 24-hour urine collection. Because of the putative renal involvement, therapy with a high-dose corticosteroid (prednisolone 80 mg/day), a proton pump inhibitor (pantoprazole 40 mg/day) and osteoporosis prophylaxis (calcium carbonate and cholecalciferol 500 mg and 10 μg b.i.d., respectively) was initiated. In addition, the patient was prescribed an ACE-inhibitor (ramipril 2.5 mg/day) for renal protection.\nAn infectious origin of the leukocytoclastic vasculitis was considered implausible because there were no respective clinical symptoms and serological tests were negative for hepatitis A, B and C as well as for an infection with streptococci. The rheumatoid factors, C3c (1.37 g/l), C4 (0.25 g/l), p-ANCA-IF (<1:20), and ANA-IF (>1:80), were not elevated and no circulating immune complexes were detected.\nA punch biopsy from the active area of the frontal aspect of the right lower leg showed typical features of leukocytoclastic vasculitis (fig. ). There were lymphocytic infiltrates with numerous neutrophils as well as ‘nuclear dust’ in the higher corium, extravasated erythrocytes, focal small-vessel edema, endothelial cell swelling within the vessels and fibrinoid deposition. Direct immunofluorescence revealed IgM, IgA, C3, and fibrinogen deposition in the small vessels of the dermis.\nIn our opinion, there was strong evidence for drug-induced toxicity as the cause of the vasculitis, so chemotherapy with oxaliplatin was discontinued. The patient was treated with prednisolone at a decreasing dosage for the next 6 weeks. Under the steroid therapy, the vasculitis lesions disappeared rapidly.\nThe patient's general practitioner continuously monitored creatinine clearance and proteinuria. After 2 weeks, there was evidence of hematuria (>20 erythrocytes) and proteinuria (745 mg/l). The urine analysis showed an increase in renal parameters with creatinine 2.41mg/dl, urea 43 mg/dl, eGFR 26.3, a proteinuria of 1,800 mg/day in a 24-hour urine collection, and a creatinine clearance of 40.1 ml/min. The patient was admitted to the Department of Nephrology with the clinical diagnosis of acute renal failure, presumably as a consequence of acute glomerulonephritis. The percutaneous renal biopsy showed a focal segmental extravascular necrotizing proliferating glomerulonephritis. Because GBM-antibodies (0.4 U/ml) or cryoglobulins (<0.2%) were not detectable, the most likely cause of renal failure was a renal manifestation of the leukocytoclastic vasculitis. Under high-dose steroid therapy (prednisolone 250 mg for 3 days followed by tapered doses), the renal function improved slowly.\nUnfortunately, during further follow-up, the patient had developed tumor progression with pulmonary metastases at the 12-month follow-up visit. Consequently, a palliative regimen with FOLFIRI (5-FU, leucovorin and irinotecan) was scheduled. Under this treatment, no side effects were apparent and the metastases remained stable for 8 months. Recent MRI and CT scans revealed a progressive disease with new metastasis-like lesions in the thoracic spine and suspect lymph nodes in the retroperitoneal, iliacal and inguinal region. | [[75.0, 'year']] | M | {'11085200': 1, '22450150': 1, '26029464': 1, '21558132': 1, '15496622': 1, '12783586': 1, '31131127': 1, '33596705': 1, '12860942': 1, '22203844': 1, '16111469': 1, '33194475': 2, '22529255': 1, '19350305': 1, '19076812': 1, '11432236': 1, '21331278': 1, '15865037': 1, '22627104': 1, '24474925': 2} | {'3901589-2': 2, '7657317-1': 1} |
165,484 | 3901589-2 | 24,474,925 | noncomm/PMC003xxxxxx/PMC3901589.xml | Oxaliplatin-Induced Leukocytoclastic Vasculitis under Adjuvant Chemotherapy for Colorectal Cancer: Two Cases of a Rare Adverse Event | We also treated a 71-year-old male patient with similar symptoms after chemotherapy with FOLFOX-6 and bevacizumab for colon cancer with hepatic metastases [pT3, pN0 (0/33), pM1 (hepatic), G2, R0]. Initially, he had received surgical treatment with a right hemicolectomy and an atypical liver resection of some of the lesions. After the subsequent postoperative chemotherapy with FOLFOX-6 and bevacizumab for 4 cycles, restaging was performed. Due to the remaining hepatic metastases, a right hemihepatectomy was performed. Postoperatively, the patient developed ubiquitous petechial hemorrhage. Except for increased S-ANA-IF activity (1:320), the rheumatoid factors were negative. A punch biopsy from an active hemorrhagic lesion again confirmed the diagnosis of a leukocytoclastic vasculitis. Shortly after diagnosis, the patient showed clinical symptoms of acute renal failure and his renal parameters increased to a serum creatinine of 2.5 mg/dl and an urea of 69 mg/dl, necessitating forced diuresis in addition to systemic corticosteroids for the next 2 months. The nephrotic syndrome with microhematuria persisted for 2 months of continued cortisone therapy.\nA renal biopsy was repeated 2 months later and showed an IgA nephritis with focal segmental and global glomerulosclerosis. As treatment for the chronic renal failure grade III, a continuous cortisone therapy was prescribed. Assuming that drug toxicity was the cause of the vasculitis, the supporting chemotherapy was prematurely terminated.\nWithin the following 12 months, the patient regretfully developed new metastases in the right lung segment and also showed bifocal liver metastases in the remaining left liver lobe. Therefore, he was treated with a modified FOLFIRI 14. The dose reduction was 75% and he received additional fluids for renal protection. Under this treatment, the patient again suffered from acute or chronic renal failure, which made hemodialysis necessary. The cortisone therapy was continued. | [[71.0, 'year']] | M | {'11085200': 1, '22450150': 1, '26029464': 1, '21558132': 1, '15496622': 1, '12783586': 1, '31131127': 1, '33596705': 1, '12860942': 1, '22203844': 1, '16111469': 1, '33194475': 2, '22529255': 1, '19350305': 1, '19076812': 1, '11432236': 1, '21331278': 1, '15865037': 1, '22627104': 1, '24474925': 2} | {'3901589-1': 2, '7657317-1': 1} |
165,485 | 3901590-1 | 24,474,920 | noncomm/PMC003xxxxxx/PMC3901590.xml | A Case of Malignant Peripheral Nerve Sheath Tumor with Rhabdomyoblastic Differentiation: Malignant Triton Tumor | A 24-year-old man presented with a subcutaneous mass in his right thigh, which gradually enlarged over 8 years, then grew rapidly 1 year prior to consultation. His medical and family histories were unremarkable. Physical examination revealed a firm, movable, painless, subcutaneous mass measuring 10 × 8 × 7 cm (fig. ). Palpation revealed no inguinal lymphadenopathy. The patient presented neither cafe-au-lait spots nor cutaneous neurofibromas. T2-weighted magnetic resonance imaging revealed a solid, subcutaneous mass containing a cystic lesion (fig. ). Computed tomography of the chest, abdomen and pelvis showed no evidence of metastasis. The tumor was totally excised under general anesthesia, with a 3-cm margin including the muscle surface. The resected margins were clear.\nThe resected tumor was not connected to the nerve tissue. Histopathological examination revealed the tumor's tissue origin as well as cystic degeneration and central necrosis. In 1 region, the tumor was composed of spindle cells occurring among collagen bundles or in a loose fibromyxoid stroma corresponding to a neurofibroma (fig. ). Elsewhere, it was composed of spindle cells with hyperchromatic nuclei, together with some rounded or oval and strap-like cells possessing deeply eosinophilic cytoplasm in variably interlacing fascicles or whorls with a herringbone appearance or nuclear palisadings (fig. ). Mitoses were also observed (fig. ).\nImmunohistochemically, the majority of spindle cells intensely expressed S-100. Cells with deeply eosinophilic cytoplasm are often positive for desmin (fig. ). The MIB-1 (Ki-67) labeling index was less than 1% in the region corresponding to the neurofibroma, but had increased to 25% in the most mitotically active portion of the tumor. Postoperative radiation therapy was administered to the affected area. However, the patient died of brain and lung metastases 10 months after surgery. | [[24.0, 'year']] | M | {'3922610': 1, '15104279': 1, '22627455': 1, '25879014': 1, '22253011': 1, '15474151': 1, '8651855': 1, '23084090': 1, '29089742': 2, '6235165': 1, '17149968': 1, '26114002': 1, '4198700': 1, '16185834': 1, '24474920': 2} | {'5649432-1': 1} |
165,486 | 3901594-1 | 24,474,902 | noncomm/PMC003xxxxxx/PMC3901594.xml | Laparoscopic Gastrectomy for Gastric Cancer with Peritoneal Dissemination after Induction Chemotherapy | A 53-year-old man had difficulty in swallowing. He underwent examination using a gastro fiberscope and was diagnosed with type 4 gastric cancer. A detailed examination of the patient was performed and included a CT scan, 18F-fluorodeoxyglucose positron emission tomography-CT (FDG PET-CT) and a barium meal; the final diagnosis was c stage IV (cT4aN3M0) gastric cancer. Peritoneal dissemination was suspected using FDG PET-CT, and consequently staging laparoscopy was performed. Peritoneal dissemination was confirmed (fig. ). With regard to induction chemotherapy, since HER2 was positive (3+), trastuzumab (first cycle 8 mg/kg, after two cycles 6 mg/kg on day 1) plus capecitabine (2,000 mg/m2 on days 1–14) combined with cisplatin (80 mg/m2 on day 1) was administered. After chemotherapy, we judged that the original tumor was resectable and that dissemination had disappeared on imaging, and we performed staging laparoscopy for a second time. Since peritoneal dissemination was not found during the second staging laparoscopy (fig. ), we carried out TLTG []. The patient commenced meal intake on the third day after the operation and was discharged 10 days later without complications. Adjuvant chemotherapy was initiated 21 days after surgery. To date, we have followed up this patient for 16 months after surgery and there has been no cancer recurrence. | [[53.0, 'year']] | M | {'23612763': 1, '24154716': 1, '22618718': 1, '22004901': 1, '18287387': 1, '16822992': 1, '23539255': 1, '15718321': 1, '34899944': 1, '22395984': 1, '22740972': 1, '22900159': 1, '23400787': 1, '22733199': 1, '21584835': 1, '23326149': 1, '24474902': 2} | {'3901594-2': 2, '3901594-3': 2} |
165,487 | 3901594-2 | 24,474,902 | noncomm/PMC003xxxxxx/PMC3901594.xml | Laparoscopic Gastrectomy for Gastric Cancer with Peritoneal Dissemination after Induction Chemotherapy | A 71-year-old man who suffered from abdominal pain was admitted to our hospital. Detailed examination included a CT scan, FDG PET-CT and a barium meal; the subsequent diagnosis was gastric cancer. We had planned to carry out TLTG, but when we inspected the abdominal cavity, ascites and peritoneal dissemination were evident. The patient was finally diagnosed with s stage IV (sT4aN3M0H0P1CY1) gastric cancer. 7 days after laparoscopic examination, S-1 (oral; 100 mg/day on days 1–21) combined with cisplatin (60 mg/m2 on day 8) were administered in three cycles as induction chemotherapy. After chemotherapy we performed a laparoscopic examination. No pathological dissemination was found during the operation. Thus, we judged that the cancer was resectable and undertook TLTG [, ]. The patient began food intake on the third day after surgery and was discharged without complications 10 days later. Adjuvant chemotherapy was begun 36 days after the operation. To date, we have followed up this patient for 14 months after surgery and cancer has not recurred. | [[71.0, 'year']] | M | {'23612763': 1, '24154716': 1, '22618718': 1, '22004901': 1, '18287387': 1, '16822992': 1, '23539255': 1, '15718321': 1, '34899944': 1, '22395984': 1, '22740972': 1, '22900159': 1, '23400787': 1, '22733199': 1, '21584835': 1, '23326149': 1, '24474902': 2} | {'3901594-1': 2, '3901594-3': 2} |
165,488 | 3901594-3 | 24,474,902 | noncomm/PMC003xxxxxx/PMC3901594.xml | Laparoscopic Gastrectomy for Gastric Cancer with Peritoneal Dissemination after Induction Chemotherapy | A 65-year-old man had stomach discomfort. He underwent a gastro fiberscope examination and was diagnosed with type 4 gastric cancer. He was admitted to our hospital and a detailed examination was carried out. The diagnosis was stage IV (T4aN0M0H0P1) gastric cancer. FDG PET-CT examination indicated possible peritoneal dissemination because of the presence of ascites. Staging laparoscopy revealed pathological peritoneal dissemination. S-1 (oral; 80 mg/m2 on days 1–21) combined with cisplatin (60 mg/m2 on day 8) was administered in two cycles as induction chemotherapy. After chemotherapy, dissemination had disappeared on imaging and we decided that the original tumor was resectable. We performed staging laparoscopy to confirm that the tumor was resectable and TLTG was carried out continuously []. The patient started to eat on the third day after the operation and was discharged without complications 10 days later. Adjuvant chemotherapy commenced 21 days after the operation. To date, we have followed up this patient for 10 months after surgery with no evidence of cancer recurrence. | [[65.0, 'year']] | M | {'23612763': 1, '24154716': 1, '22618718': 1, '22004901': 1, '18287387': 1, '16822992': 1, '23539255': 1, '15718321': 1, '34899944': 1, '22395984': 1, '22740972': 1, '22900159': 1, '23400787': 1, '22733199': 1, '21584835': 1, '23326149': 1, '24474902': 2} | {'3901594-1': 2, '3901594-2': 2} |
165,489 | 3901595-1 | 24,474,927 | noncomm/PMC003xxxxxx/PMC3901595.xml | Coincidence of Varicella-Zoster Virus Anterior Uveitis in a Patient with Chandler's Syndrome | A healthy, 40-year-old woman visited a private clinic with the complaint of hyperemia in her left eye and was referred to our hospital due to the presence of focal iris atrophy. At the initial examination, slit-lamp biomicroscopy of the left eye revealed localized iris atrophy with PAS at several sites, along with a hammered-silver appearance in the corneal endothelium. The corneal edema was absent and the IOP was 11 mm Hg (fig. ). The best corrected visual acuity was 20/20 in the affected eye. Anterior segment optical coherence tomography (OCT) documented the presence of PAS, with the corneal thickness being almost the same in both eyes (fig. ). Specular microscopy of the corneal endothelium in the left eye showed abnormal cells with a disrupted and irregular cellular border (fig. ). The corneal endothelium of the right eye was normal. Based upon these clinical findings, we diagnosed this patient as having Chandler's syndrome, and we decided to follow up without any treatment. However, PAS progressed rapidly at a somewhat unusual speed and, 3 months later, the IOP in the left eye was elevated to 32 mm Hg, while the anterior chamber was silent. Latanoprost (Xalatan®; P?zer Japan, Tokyo, Japan) and dorzolamide hydrochloride-timolol maleate ophthalmic solution (COSOPT®; Santen Pharmaceutical Co. Ltd., Osaka, Japan) were topically applied to control the IOP <20 mm Hg. Six months later, the patient started to complain of visual disturbance in the left eye. Slit-lamp biomicroscopy revealed the inflammatory cells in the anterior chamber to have white keratic precipitates (fig. ). There was no vitreous opacity or retinal exudate. Polymerase chain reaction (PCR) was performed on an aqueous humor sample in search of a panel of human herpes viruses (HHVs) including herpes simplex virus (HSV) type 1 or type 2, VZV, Epstein-Barr virus (EBV), cytomegalovirus and HHV-6, HHV-7 and HHV-8 [, ]. It was found to be positive only for VZV. At this stage, we strongly suspected that this patient had unilateral anterior uveitis due to zoster sine herpete, and so we started the treatment with 3% acyclovir eye ointment (Zovirax®; Santen Pharmaceutical Co., Ltd.) 5 times a day and 0.1% betamethasone eye drops (Rinderon®; Shionogi Pharmaceutical Co. Ltd., Osaka, Japan) 4 times a day, with oral administration of 20 mg/day of prednisolone and 1,500 mg/day of valacyclovir (Valtrex®; GlaxoSmithKline, Brentford, UK). The anterior chamber reaction subsided with this medication and was controlled with topical acyclovir and corticosteroids within 2 months. The iris atrophy and PAS further progressed with reduced corneal endothelial cell density (fig. ). As the IOP was elevated in spite of full medication with antiglaucoma agents, trabeculectomy was performed to control the IOP <15 mm Hg without medication. | [[40.0, 'year']] | F | {'20385952': 1, '14717277': 1, '6344293': 1, '2173898': 1, '6462621': 1, '7993217': 1, '18245272': 1, '22159687': 1, '24474927': 2} | {} |
165,490 | 3901601-1 | 24,474,923 | noncomm/PMC003xxxxxx/PMC3901601.xml | Low-Dose Bevacizumab Is Effective in Radiation-Induced Necrosis | The first case is a 51-year-old woman who was diagnosed with metastatic melanoma in the lungs, with an unknown primary tumor. She underwent left inferior lobectomy. Two years later, the patient developed 3 metastatic brain lesions. The largest lesion was surgically resected. After brain surgery, she was treated with whole-brain radiation and fotemustine. Soon after the radiation had been completed, the other 2 lesions were treated with stereotactic radiosurgery (SRS). After 17 months of SRS, the patient presented with severe drowsiness and was unable to ambulate by herself. An MRI of the brain with perfusion and spectroscopy showed radionecrosis. Therapy with bevacizumab, 5 mg/kg every 2 weeks, associated with dexamethasone, 4 mg p.o. q.i.d., was used, and important clinical benefits were noted after 3 days with a complete reversal of her symptoms. During the following 2 months, dexamethasone was discontinued and the patient remained asymptomatic. After 3 months, the dose and the schedule of bevacizumab was modified to 7.5 mg/kg i.v. every 4 weeks. Seventeen months after the first bevacizumab dose was administered, the patient remained neurologically stable and was able to lead a normal life (fig. ). | [[51.0, 'year']] | F | {'5435118': 1, '27438082': 1, '31632089': 1, '19189055': 1, '20694573': 1, '16517093': 1, '387205': 1, '12864928': 1, '28270886': 1, '33842309': 1, '32765892': 1, '19857784': 1, '30732625': 1, '22913802': 1, '33142986': 1, '23303838': 1, '33593308': 1, '24474923': 2} | {'3901601-2': 2} |
165,491 | 3901601-2 | 24,474,923 | noncomm/PMC003xxxxxx/PMC3901601.xml | Low-Dose Bevacizumab Is Effective in Radiation-Induced Necrosis | The second case is a 48-year-old woman who was initially diagnosed with cutaneous melanoma on the right deltoid region. Nine years later, she presented with subcutaneous and bone metastasis. She was treated with ipilimumab and achieved a complete systemic response. However, 3 weeks after her fourth dose of ipilimumab, a routine MRI of the brain showed a single brain metastasis. She underwent SRS followed by whole-brain irradiation. Three months later, the patient presented with partial seizures (facial tremor). A brain MRI with perfusion and spectroscopy showed severe vasogenic edema consistent with radionecrosis. A surgical approach to this lesion would have resulted in partial facial paresis; therefore, the patient refused the procedure.\nThe patient was started on dexamethasone, 4 mg p.o. t.i.d. for 3 months, with a partial seizure control, but during steroid taper, her seizures resumed. She was then started on bevacizumab, 5 mg/kg, with rapid clinical improvement. Due to her financial constraints, bevacizumab was scheduled every 6 weeks. Nonetheless, her symptoms disappeared and a repeat MRI of the brain showed an important reduction of the vasogenic edema (as shown in the FLAIR sequence of her MRI) (fig. ). After only 4 weeks since starting bevacizumab, dexamethasone was discontinued. | [[48.0, 'year']] | F | {'5435118': 1, '27438082': 1, '31632089': 1, '19189055': 1, '20694573': 1, '16517093': 1, '387205': 1, '12864928': 1, '28270886': 1, '33842309': 1, '32765892': 1, '19857784': 1, '30732625': 1, '22913802': 1, '33142986': 1, '23303838': 1, '33593308': 1, '24474923': 2} | {'3901601-1': 2} |
165,492 | 3901606-1 | 24,474,901 | noncomm/PMC003xxxxxx/PMC3901606.xml | Primary Systemic Amyloidosis with Extensive Gastrointestinal Involvement | A 42-year-old African American woman, born and raised in the United States, presented to the hospital with diarrhea, nausea and intractable vomiting with inability to tolerate oral feeding for 1 week. The patient had multiple prior visits to another institution with similar complaints, where a provisional diagnosis of gastroesophageal reflux disease was made from esophagogastroduodenoscopy. Upon further enquiry, she reported these symptoms to have started more than a year prior to the index presentation. Physical examination at the time of admission revealed a cachectic and disoriented young woman. Other pertinent positive findings of physical examination included hepatomegaly (20 cm) with ascites and bilateral pitting type of pedal edema.\nAn initial set of laboratory studies showed microcytic, hypochromic anemia (hemoglobin 8.4 g/dl), abnormal coagulation parameters (PT 17.1 s, aPTT 37.1 s), renal dysfunction (BUN 37 mg/dl, serum creatinine 2.0 mg/dl), abnormal liver enzymes (alanine transaminase 51 IU/l, aspartate transaminase 77 IU/l, markedly elevated alkaline phosphatase of 1,655 IU/l with normal bilirubin levels), profound serum hypoalbuminemia (1.5 gm/dl) and nephrotic range of proteinuria (urine dipstick protein assay showed 5+ proteinuria and the spot urine protein/creatinine ratio was 5.7). Additional work-up done for evaluation of abnormal liver enzymes including viral hepatitis panels (hepatitis A, B and C), ANA, anti-smooth muscle antibody, anti-liver kidney microsomal antibodies and anti-mitochondrial antibody was negative. Serum immunofixation revealed IgG lambda monoclonal protein. Urine immunofixation was negative. An echocardiogram revealed concentric left ventricular hypertrophy with a normal ejection fraction of 78%. A computed tomography scan of the abdomen showed pelvic ascites with mild, diffuse bowel wall thickening.\nThe patient's hospital course was marked by the persistence of diarrhea with minimal response to presumptive therapy with antibiotics (intravenous metronidazole and oral vancomycin). Stool work-up done for diarrhea revealed no infectious etiology. The patient had deteriorating renal function necessitating renal replacement therapy. She had a self-limited episode of hematemesis.\nThe patient underwent esophagogastroduodenoscopy and colonoscopy that revealed no gross mucosal lesions. Multiple random biopsies were taken from the stomach, duodenum, terminal ileum, colon and rectum. Biopsy results showed eosinophilic, acellular, globular deposits in the lamina propria and submucosa which stained positively with Congo, consistent with amyloidosis (fig. ). A subsequent bone marrow biopsy revealed normocellular bone marrow with trilineage maturation and diffuse extracellular eosinophilic, amorphous material consistent with amyloidosis (fig. ). A definitive diagnosis of primary systemic light-chain amyloidosis with gastrointestinal tract involvement was made. The patient was not considered for autologous stem cell transplantation, since the outcomes of autologous stem cell transplant in patients with multisystem involvement are often poor and fraught with a high mortality risk. She was subsequently referred to another institution for further management. | [[42.0, 'year']] | F | {'16830294': 1, '17063986': 1, '19724253': 1, '19626465': 1, '11705429': 1, '10319082': 1, '17344006': 1, '22733017': 1, '19242859': 1, '14606114': 1, '7878478': 1, '26979633': 1, '14572571': 1, '22100031': 1, '21247244': 1, '20958232': 1, '34307229': 2, '22863214': 2, '17428737': 1, '21869840': 1, '8244111': 1, '22037658': 1, '20791868': 1, '6979925': 1, '1558973': 1, '8331978': 1, '12887954': 1, '24474901': 2} | {'8214892-1': 1, '3438062-1': 1} |
165,493 | 3901616-1 | 24,474,929 | noncomm/PMC003xxxxxx/PMC3901616.xml | A Case of Functional (Psychogenic) Monocular Hemianopia Analyzed by Measurement of Hemifield Visual Evoked Potentials | A 14-year-old boy who was born deaf was referred to the emergency room at Kochi University Hospital on 30 January 2013 with a history of monocular temporal hemianopia of the right eye following headache, nausea and ague. He visited our department for the first time the next day. His visual acuity was 1.2 and 1.5 in the right and left eye, respectively. His intraocular pressure was normal, as were the anterior segment and macular as revealed by optical coherence tomography. No relative afferent pupillary defect was apparent, and a color perception test (Ishihara color test) was normal. Goldmann perimetry revealed a vertical monocular temporal hemianopia of the right eye (fig. ); this was also detected with a binocular visual field test. Computed tomography, magnetic resonance imaging (MRI) and MR angiography of the brain including the optic chiasm as well as orbital MRI revealed no apparent abnormality. On the basis of these findings, we diagnosed the patient's condition as functional monocular hemianopia.\nAt a follow-up 1 week later, the patient still manifested monocular temporal hemianopia of the right eye as revealed by Goldmann perimetry as well as a hemifield defect as revealed by the binocular field test. Pattern VEPs were then measured with a VikingSelect instrument (Natus Medical Inc., San Carlos, Calif., USA). The stimuli were checkerboard patterns with an individual check size of 1.5 degrees. The stimulus monitor was a cathode-ray tube of 14.3 × 18.5 degrees and the hemifield was of half size. The measurement distance was 100 cm and the reversal frequency was 0.7 Hz (transient VEP). Pattern VEPs according to the International Society for Clinical Electrophysiology of Vision (ISCEV) standard were within the normal range []. The latency and amplitude were 111.5 ms and 13.4 μV for the right eye and 107.5 ms and 11.9 μV for the left eye, respectively (fig. ). The hemifield pattern VEPs for the right eye were symmetrical, with a latency and amplitude of 114.0 ms and 9.1 μV for stimulation of the temporal field and 109.5 ms and 8.9 μV for stimulation of the nasal field, respectively (fig. ).\nOne month later, the visual field defect of the patient spontaneously disappeared. | [[14.0, 'year']] | M | {'7077338': 1, '3759417': 1, '19826847': 1, '3946540': 1, '444118': 1, '14664771': 1, '16966940': 1, '9693293': 1, '515946': 1, '889758': 1, '4014374': 1, '8343470': 1, '24474929': 2} | {} |
165,494 | 3901617-1 | 24,474,898 | noncomm/PMC003xxxxxx/PMC3901617.xml | Small Bowel Metastasis of Hepatocellular Carcinoma Detected by Capsule Endoscopy | A 60-year-old man with liver cirrhosis type B was treated with sorafenib chemotherapy for HCC (stage IVb) at Hiroshima University Hospital. He had been admitted for melena and anemia since October 2011. There was no obvious source of bleeding by both upper and lower gastrointestinal endoscopy. Therefore, CE was performed in November 2011. Physical findings were normal. Laboratory examination indicated a white blood cell count of 3,850/μl, a red blood cell count of 325 × 104/μl and a platelet count of 11.0 × 104/μl. The hemoglobin concentration was 9.2 g/dl. Liver function examination revealed the following levels: aspartate aminotransferase 61 IU/l (normal 2–39 IU/l), alanine aminotransferase 57 IU/l (normal 2–39 IU/l), total protein 7.7 g/dl (normal 6.5–8.1 g/dl), serum albumin 3.1 g/dl (normal 3.8–5.2 g/dl), alkaline phosphatase 722 IU/l (normal 96–310 IU/l), leucine aminopeptidase 177 IU/l (normal 45–71 IU/l), γ-glutamyl transpeptidase 138 IU/l, cholinesterase 0.10 IU/l (normal 0.6–1.2 IU/l), and total bilirubin 1.4 mg/dl (normal 0.1–0.4 mg/dl). With respect to renal function, the blood urea nitrogen level was 43.6 mg/dl and the creatinine level 2.54 mg/dl. The results for tumor markers were negative for carbohydrate antigen 19-9 and carcinoembryonic antigen; however, the levels of alpha fetoprotein and protein indicated by vitamin K absence (PIVKA II) were high at 86.5 ng/ml (normal ≤5 ng/ml) and 45,302 ng/ml (normal ≤10 ng/ml), respectively. The Child-Pugh score was 6 points (grade B).\nA computed tomography (CT) scan showed that the HCC consisted of a tumor in the liver (fig. ), a portal vein tumor thrombus (VP2) (fig. ) and multiple lung metastases (fig. ). CE showed a polypoid lesion with bleeding in the ileum, but there were no other findings in the small bowel (fig. ). DBE was performed after CE. The lesion was determined to be a pedunculated polyp in the ileum (fig. ). Histological examination of biopsy specimens showed tumor cells resembling HCC on hematoxylin and eosin stain (fig. ). Immunohistochemical examination showed tumor cells positive for hepatocyte antigen (fig. ) and glypican-3 antigen (fig. ).\nThe tumor was diagnosed as a small bowel metastasis of moderately differentiated HCC. We decided to perform prophylactic endoscopic mucosal resection (EMR) to control the continuous bleeding after receiving informed consent from the patient and his family. During EMR, the non-lifting sign was positive after glycerol injection (fig. ) however, we then tried to resect the tumor (fig. ). Histological examination of the resected specimen showed tumor cells resembling HCC on hematoxylin and eosin stain (fig. ). The patient had no melena or anemia and was well after EMR (hemoglobin increased from 9.2 to 11.5 g/dl) without transfusion. However, he died of liver failure 2 months later. | [[60.0, 'year']] | M | {'22817787': 1, '11174299': 1, '10847441': 1, '23247799': 1, '19136103': 1, '22580642': 1, '7328295': 1, '20658210': 1, '26419595': 1, '32723336': 2, '17674287': 1, '21113753': 1, '22207412': 1, '32274362': 2, '2162757': 1, '2155591': 1, '22503919': 1, '27904130': 1, '10839527': 1, '21960951': 1, '21626338': 1, '24474898': 2} | {'7389379-1': 1, '7104197-1': 1} |
165,495 | 3901621-1 | 24,474,934 | noncomm/PMC003xxxxxx/PMC3901621.xml | Alagille Syndrome Associated with Xerophthalmia | A 3-year-old boy was examined at our institution due to severe bilateral ocular-surface disease, more severe in the right eye. His medical history included neonatal cholestatic jaundice, which prompted us to make a thorough systemic evaluation. Liver biopsy revealed paucity of the intrahepatic bile ducts. Cardiac echography revealed a left-to-right shunt through an atrial septal defect and mild stenosis of the pulmonary arteries. Vertebral structure was normal on X-ray. The patient was first examined at our clinic at the age of 9 months, and a prominent posterior embryotoxon was noted bilaterally (fig. ). The combination of typical facial characteristics including a prominent forehead, bulbous nose and pointed chin together with systemic and ocular findings led to a clinical diagnosis of Alagille syndrome. His parents were reluctant to have genetic testing performed. During infancy, the main complaint was his failure to thrive, and a percutaneous endoscopic gastrostomy tube was inserted for added caloric supplementation along with the fat-soluble vitamins. The patient suffered from a significant developmental delay (e.g. he started walking only at the age of 2.5 years) and severe growth retardation under full caloric and pancreatic-enzyme supplementation (at the age of 3.5 years, he weighed 7.2 kg, which is below the 3rd percentile). He received chronic daily treatment with ursodeoxycholic acid and supplementation with the fat-soluble vitamins (AquADEKs, 1 ml until the age of 1 year, and then 2 ml; 1 ml contains vitamin A as 87% β-carotene and 13% palmitate, 5,751 IU which is 383% of the daily value for infants).\nSeveral months prior to examination at our clinic, he started complaining of severe bilateral ocular irritation. Because of constant eye-rubbing, it was assumed that he suffered from vernal keratoconjunctivitis, and he was treated with topical antihistamines. After failing to improve with this treatment, he was referred for our examination. At the initial evaluation, a central corneal ulcer was noted in the right eye, with marked opacity and central thinning of the cornea. The cornea in the left eye displayed mild opacities and diffuse superficial punctate keratitis. The fundus could not be seen in the right eye, but in the left eye, mild tilting and optic-disc pallor were noted as well as a few retinal pigmentary changes. A culture taken from the corneal ulcer in the right eye was positive for Staphylococcus mitis. The patient was treated with intensive topical cefamezine, garamycin and lubrication, and experienced a gradual improvement. Three weeks after his initial presentation to our clinic, the corneal infiltrate resolved, but a marked thinning remained. He underwent amniotic membrane transplantation with lower punctual occlusion (fig. ). Due to the clinical findings and his systemic condition, a suspicion of vitamin A deficiency was raised. This was confirmed by serum analysis of vitamin A, which measured 2 μg/dl; this is significantly lower than the normal range of 30–120 μg/dl. There were no Bitot's spots in either eye. It should be noted that, due to the patient's significant developmental delay, visual acuity could not be measured and he was unable to articulate any complaint regarding reduced vision or nyctalopia. The vitamin A supplementation was further increased and it resulted in a gradual improvement of the degree of ocular discomfort within the following months. The irritation had resolved, the patient was no longer rubbing his eyes, and the right cornea was adequately covered by conjunctival epithelium. | [[3.0, 'year']] | M | {'17721303': 1, '9207788': 1, '11064859': 1, '9951486': 1, '22087104': 1, '8501619': 1, '6796244': 1, '18097983': 1, '8259273': 1, '803282': 1, '3806290': 1, '20489600': 1, '12221259': 1, '23364653': 1, '7076119': 1, '24474934': 2} | {} |
165,496 | 3901624-1 | 24,474,919 | noncomm/PMC003xxxxxx/PMC3901624.xml | A Case of Autosomal Recessive Woolly Hair/Hypotrichosis with Alternation in Severity: Deterioration and Improvement with Age | A 3-year-old girl's parents brought her to our department complaining of hair loss and insufficient hair growth. Her delivery had been normal and her development otherwise unremarkable. She had no siblings, and both parents had apparently normal hair. Neither parent reported any family history of consanguinity. Physical examination showed fine, curly hair ranging in length from 3 to 5 cm and distributed uniformly sparsely. Her eyebrows and eyelashes were unaffected (fig. ). Her parents reported that her hair density and growth had been normal at birth (fig. ); however, significant hair loss had developed when she was 3 months old (fig. ) and had gradually reversed somewhat over the next 3 years. Laboratory tests, including thyroid hormone levels and autoantibody titers, showed no significant abnormality. Light microscopy showed hair shaft invaginations (fig. ), and polarized light microscopy revealed a damaged appearance of the hair cortex (fig. ). Scanning electron microscopy showed a partial defect of the medulla and fragile outer cortex of the hair shaft (fig. ). As the hair of both parents appeared normal, we suspected ARWH/H and performed direct sequencing of the LIPH gene on peripheral blood from the patient and her parents after obtaining the family's informed consent. The patient was found to be homozygous and both parents heterozygous for a missense mutation in exon 6 of LIPH (c.736T>A, p.Cys246Ser) (fig. ). Our patient was diagnosed with ARWH/H resulting from a homozygous mutation in LIPH. | [[3.0, 'year']] | F | {'19892526': 1, '17095700': 1, '29096685': 2, '18297072': 1, '19262606': 1, '18297070': 1, '21352330': 1, '12063250': 1, '32258056': 1, '12925199': 1, '18830268': 1, '24474919': 2} | {'5667512-1': 1, '5667512-2': 1, '5667512-3': 1, '5667512-4': 1} |
165,497 | 3901626-1 | 24,474,900 | noncomm/PMC003xxxxxx/PMC3901626.xml | A Large Hepatic Cyst with Obstructive Jaundice Successfully Treated with Single-Incision Laparoscopic Deroofing | A 72-year-old female patient was referred to our hospital due to right upper abdominal pain. Physical examination performed on admission revealed mild tenderness in the right epigastrium. Laboratory data revealed a serum bilirubin level of 1.6 mg/dl, an aspartate aminotransferase level of 226 IU/l, an alanine aminotransferase level of 164 IU/l and an alkaline phosphate level of 1,062 IU/l. A computed tomography (CT) scan disclosed two large hepatic cysts at segment 4 measuring 86 × 112 × 115 mm (fig. ) and 39 × 45 × 40 mm (fig. ), respectively. In addition, the intrahepatic bile ducts were slightly dilated due to compression of the hepatic hilum. Magnetic resonance cholangiopancreatography (MRCP) also showed intrahepatic duct dilatation (fig. ), although no communications between the cysts and the intrahepatic bile ducts were detected. Based on these findings, the provisional diagnosis was a symptomatic large hepatic cyst with compression of the intrahepatic bile duct. The patient was scheduled to undergo laparoscopic deroofing.\nThe patient was placed in the supine position and an entry hole into the abdomen was created using an umbilical incision (25 mm). The EZ ACCESSTM oval type (Hakko Co. Ltd.; fig. ) was placed at the umbilicus using a 25-mm incision as an access port with two 5-mm trocars. An additional 12-mm port was placed at the left epigastric region (fig. ). Cysts were detected in liver segment 4. A small incision was made in the cyst using an ultrasonically activated device, and serous fluid was evacuated. A vessel sealing system (Ligasure; Covidien, Mansfield, Mass., USA) was then used to excise the cyst wall. A thick peripheral bile duct was identified and clipped.\nThe patient's postoperative course was uneventful, and she was discharged on postoperative day 8. The pathological findings revealed a solitary cyst without malignant transformation. Four months after surgery, no recurrent symptoms were observed and liver regeneration was detected on abdominal CT. The regeneration volume of the liver was 153 cm3 (fig. ). | [[72.0, 'year']] | F | {'6603770': 1, '8661625': 1, '22688416': 1, '19959042': 1, '5684196': 1, '8129486': 1, '11813611': 1, '26416378': 1, '27358670': 1, '21876626': 1, '27610019': 1, '20421115': 1, '9171771': 1, '24474900': 2} | {} |
165,498 | 3901627-1 | 24,474,899 | noncomm/PMC003xxxxxx/PMC3901627.xml | Verrucous Carcinoma of the Esophagus: A Potential Diagnostic Dilemma | A 58-year-old female with a past history of squamous cell carcinoma of the larynx, treated with radiation therapy and surgery 13 years earlier, presented with dysphagia and weight loss of approximately 10 pounds; symptoms had started almost 2 months before. At the time of presentation, she was able to tolerate a liquid diet only and was referred to us for further diagnostic workup after two prior gastroscopy studies. Previous evaluation had revealed a large polyp-like mass involving the mid and distal esophagus. However, repeat biopsies from this lesion had been negative for malignancy. On repeat esophagogastroduodenoscopy, a large semi-pedunculated polyp was seen in the distal esophagus (fig. ). The polyp stalk seemed to blend into a background of nodular-appearing mucosa involving the distal esophagus with the gastroesophageal junction. On endoscopic ultrasound, a hypoechoic, irregular mass was found, involving the gastroesophageal junction with associated esophageal thickening, measuring 20 mm in thickness. There was sonographic evidence suggesting invasion into the deep adventitia layer (fig. ). Biopsies using large-capacity forceps demonstrated pseudoepitheliomatous hyperplasia, raising the suspicion of VCE. p16 immunohistochemical staining was positive for HPV.\nInitial positron emission tomography (PET) and computed tomography (CT) evaluation was consistent with an increase in hypermetabolic soft tissue fullness at the gastroesophageal junction. After discussion at the multidisciplinary conference, the decision was made to proceed with chemo-radiation with preoperative carboplatin and Taxol. Repeat PET/CT after 3 months demonstrated significant interval reduction in the size and metabolic activity of the distal esophagus near the gastroesophageal junction, consistent with a positive response to radiation therapy. Clinically, the patient had significant improvement in symptoms after initiation of chemo-radiation therapy. Afterwards, she underwent interval Ivor-Lewis esophagectomy with no residual mass seen on gross specimen. Final histology did not reveal any viable tumor (fig. ). | [[58.0, 'year']] | F | {'22272545': 1, '15229749': 1, '26734805': 2, '20626449': 1, '31229974': 1, '32759723': 1, '27721734': 2, '23195756': 1, '23067443': 1, '19555942': 1, '6073887': 1, '20405383': 1, '27036299': 2, '32774063': 1, '23039810': 1, '24474899': 2} | {'4755187-1': 1, '4818443-1': 1, '5043254-1': 1, '5043254-2': 1} |
165,499 | 3901628-1 | 24,474,932 | noncomm/PMC003xxxxxx/PMC3901628.xml | Late-Onset Capsular Block Syndrome: Unusually Delayed Presentation | A 75-year-old lady was referred to the eye department with a 6-month history of misty and fuzzy vision in her right eye. Her left eye was asymptomatic. Eight years earlier, she had undergone uncomplicated bilateral cataract surgery by phacoemulsification with lens implants. Upon examination, her LogMAR scale best-corrected visual acuity (BCVA) was 20/20 in both eyes. Refractive error was −1.50 DS for the right eye and +0.25 DS for the left eye, which suggested a myopic shift in the right eye. The left eye was normal, but there was a hazy appearance in the right eye in the retro-lenticular space between the posterior surface of the lens optic and the posterior capsule. The space was homogenously whitish and slightly opalescent, characteristic of a milky, turbid fluid (fig. ). There were no signs of inflammatory activity. On clinical examination, the lens implant did not appear to have shifted forward and the fundus examination was normal.\nShe agreed to undergo surgical removal of this fluid under local anaesthesia. Intra-operatively, a 30-gauge needle mounted on a 1-ml syringe was passed through the sclera and pars plana, 3.5 mm behind the limbus in the infero-temporal quadrant. The needle tip was gently passed through the posterior capsule and 0.2 ml of the turbid fluid was aspirated and then sent for microbiological culture and analysis. Intra-vitreal vancomycin (1 mg/0.1 ml) was administered at the end of the procedure. She was reviewed after 5 days and there were no signs of inflammation or endophthalmitis. In addition, her myopic shift had disappeared and she now had a +0.25 DS for both eyes, both still with a BCVA of 20/20. The microbiological report did not show any signs of indolent bacterial growth. Two months afterwards, at follow-up, she was asymptomatic and reported complete resolution of her misty vision. Examination showed a BCVA of 20/20, with a clear papillary axis and an intact posterior capsule and lens implant. Inferiorly, there was opacity of the posterior capsular area (fig. ). | [[75.0, 'year']] | F | {'21320976': 1, '9780116': 1, '9951681': 1, '10761406': 1, '9559740': 1, '9159693': 1, '28944190': 1, '2299584': 1, '9768398': 1, '34934563': 2, '3258419': 1, '28770106': 1, '9818349': 1, '24474932': 2} | {'8683699-1': 1} |
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