• Childhood Kidney Diseases
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• v.17 no.2
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• pp.149-153
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• 2013

We report the case of a 14-year-old girl, diagnosed with atypical thrombotic microangiopathy (TMA). The patient presented with persistent fever, nausea, and newly developed peripheral edema. Her laboratory findings indicated chronic anemia with no evidence of hemolysis, thrombocytopenia, or elevated serum creatinine level. A few days after hospitalization, acute renal failure and fever worsened, and proteinuria developed. On day 40 of hospitalization, she experienced a generalized tonic seizure for 5 min, accompanied by renal hypertension. Brain magnetic resonance imaging revealed posterior reversible leukoencephalopathy syndrome. After steroid pulse therapy, a renal biopsy was performed because of delayed recovery from thrombocytopenia. The biopsy findings showed features of thrombotic microangiopathic hemolysis with fibrinoid change restricted. Current diagnostic criteria for TMA have focused on thrombotic thrombocytopenic purpura and hemolytic uremic syndrome, and diagnosis is based on the clinical presentation and etiology, with the consequence that idiopathic and atypical forms of TMA can be overlooked. Developing effective tools to diagnose TMA, such as studying levels of ADAMTS13 or testing for abnormalities in the complement system, will be the first step to improving patient outcomes.

• Journal of The Korean Ophthalmological Society
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• v.59 no.11
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• pp.1097-1102
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• 2018

Purpose: To report a case of toxic optic neuropathy caused by chlorfenapyr ingestion accompanied by central nervous system involvement. Case summary: A 44-year-old female visited our clinic complaining of reduced visual acuity in both eyes for 7 days. She had ingested a mouthful of chlorfenapyr for a suicide attempt 2 weeks prior to the visit. Gastric lavage was performed immediately after ingestion at the other hospital. Her best-corrected visual acuity was finger count 30 cm in the right eye and hand motion in the left eye. Both pupils were dilated by 5.0 mm and the response to light was sluggish in both eyes. A relative afferent pupillary defect was detected in her left eye. Funduscopy revealed optic disc swelling in both eyes. Magnetic resonance imaging of the brain showed a symmetric hyper-intense signal in the white matter tract including the internal capsule, corpus callosum, middle cerebellar peduncle, and brainstem. The patient was diagnosed with toxic optic neuropathy induced by chlorfenapyr ingestion, and underwent high-dose intravenous corticosteroid pulse therapy. Three days later, the best-corrected visual acuity was no light perception in both eyes. Three months later, optic atrophy was observed in both eyes. Optical coherence tomography revealed a reduction in the thicknesses of the retinal nerve fiber layer and ganglion cell and inner plexiform layer in the macular area. Conclusions: Ingestion of even a small amount of chlorfenapyr can cause severe optic nerve damage through the latent period, despite prompt lavage and high-dose steroid treatment.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.20 no.10
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• pp.303-308
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• 2019

Non-traumatic acute myelopathy caused by cervical disc herniation is rare. To date, no case has been reported to be caused by extrusion cervical disc herniation, unrelated to patient posture during surgery. Here, we report the case of a 65-year-old male patient with cervical myelopathy who underwent subsequent arthroscopic rotator cuff surgery under general anesthesia; non-cervical spine surgery. Ed. Notes: I am unable to understand the insertion of the highlighted phrase. Please delete if not required, or revise the sentence appropriately. Patient showed acute postoperative tetraplegia in spite of optimal anesthetic management. He showed no limitation of neck movement at pre-operative airway evaluation, and had no history of trauma to the cervical spine. During surgery, there had been no overextension or twisting of the neck, including at the time of anesthetic induction by tracheal intubation. However, cervical disc herniation causing spinal canal cord compression was detected in the postoperative magnetic resonance imaging, which probably resulted in tetraplegia of the patient. Motor and sensory functions were recovered after 21 days of conservative treatment, including steroid pulse intravenous therapy without any surgical intervention. In this report, the disease is described after reviewing other reported cases; furthermore, we also discuss the pathophysiology of the disease. Based on our report, we propose that under general anesthesia, clinicians should pay attention to the possibility of pre-existing cervical disease, even in non-cervical spine surgeries of geriatric patients.

• Laboratory Medicine Online
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• v.1 no.2
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• pp.110-114
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• 2011

There have been a few reports of hemophagocytic lymphohistiocytosis (HLH) with chromosomal abnormalities. Clonal chromosomal abnormalities in HLH patients are usually found in association with hematologic malignancies and rarely with epstein-barr virus (EBV) infection. Here, we report a fatal case of HLH with clonal karyotype abnormalities. A 75-yr-old man was admitted with persistent anorexia and high fever. Laboratory data revealed pancytopenia, hypofibrinogenemia, hyperferritinemia, prolonged prothrombin time and activated partial thromboplastin time, and marked elevated level of serum transaminases. In real time-PCR using whole blood, EBV DNA was not detected but cytomegalovirus (CMV) DNA was detected. The bone marrow aspiration smear showed hyperplasia of mature histiocytes with prominent hemophagocytosis. In chromosomal analysis of bone marrow aspirates, complex chromosomal abnormalities were found. In spite of steroid pulse therapy and antibiotic treatment, he died of disseminated intravascular coagulopathy.

• Clinical and Experimental Pediatrics
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• v.45 no.6
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• pp.783-789
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• 2002

Purpose : We evaluated the epidemiologic characteristics and incidence of coronary artery sequele of children with KD according to treatment. Methods : We retrospectively analyzed 506 medical records of children with KD, who were admitted at Daejeon St. Mary's Hospital from Jan. 1987 to Dec. 2000. Results : The mean annual incidence was $36.1{\pm}11.1$ cases per year. There was a slightly higher occurrence in summer with no significant difference in monthly incidence. The mean age was $2.4{\pm}1.7$ years and 450 children(88.9%) were below four years of age. The male to female ratio was 1.7 : 1. When the 345 cases between 1987 and 1994 were divided into three groups according to treatment, incidences of the coronary abnormality(above grade II) of aspirin-treated(54 cases; 15.6%), divided-intravenous immunoglobulin(IVIG) treated($400-500mg/day{\times}4-5days$, 224 cases; 64.9%), and one-dose IVIG treated(2.0 g/day, 67 cases; 19.5%) groups were 8.3%, 6.0%, and 7.5%, respectively. Between 1995 and 2000, 143 cases were treated with only one-dose IVIG and 21 cases(14.7%) showed coronary artery abnormalities(grade I, 15 cases; grade II, two cases; and grade III, four cases). Among the 143 cases, 22 cases(15.1%) were retreated with IVIG and/or steroid pulse therapy. The incidence of coronary artery abnormality in this group was 50.0%. Incidences of cases in recurrence and among siblings were 0.6% and 0.4% respectively. There was no fatal case. Conclusion : In Daejeon, Korea, the epidemiologic feature of KD showed slight annual variations without monthly differences. The incidence of coronary abnormality with one-dose IVIG therapy was 14.7%. The nonresponse of this therapy was 15.1% with a coronary abnormality of 50.0%.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.78-86
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• 2001

Purpose: Rapidly progressive glomerulonephritis (RPGN) is characterized by the rapid increase in serum creatitnin and crescents formation involving more than $50\%$ of glomeruli. 10 patients who had been treated for RPGN were studied retrospectively for thier underlying diseases and clinical features Method: Cilinical review was performed on 10 children who were diagnosed with RPGN by clinical features and renal biopsy and followed up at department of pediatrics during tile last 10 years, from May 1990 to May 2000. Result: There were 6 males and 4 females between the ages of 2.1 and 14.3 years (mean $10.9{\pm}3.8$). 3 had Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis; 2, idiopathic rapidly progressive glomerulonephritis; 2, lupus nephritis; 1, hemolytic uremic syndrome; 1, membranous glomerulonephritis and 1, microscopic polyangiitis. The most common chief complaints were gross hematuria and oliguria. Initial clinical features included proteinuria, edema, hypertension, nausea and arthralgia. Mean serum BUN was $74.2{\pm}39.1\;mg/dL$ mean serum creatinin, $3.2{\pm}1.8\;mg/dL$ and mean creatinin clearance, $26.5{\pm}13.2\;mL/min/1.73m^2$. Antineutrophil cytoplasmic antibody was positive only in microscopic polyangiitis. ANA and Anti-DNA antibody were positive in two lupus nephritis patients. Serum complements were decreased in 4 patients. All patients except Hemolytic uremic syndrome received steroid pulse therapy and immunosupressive agents. 3 patients were performed acute peritoneal dialysis and 2 patients were given plasmapheresis. At the last follow up, 1 patient was dead, 4 patients had elevated serum creatinin, 2 of these 4 patients were on chronic ambulatory peritoneal dialysis and 6 patients had normal renal function. Conclusion: Rapidly progressive glomerulonephritis is a medical emergency that requires very rapid diagnosis, classification, and therapy. Appropriate therapy selected on the basis of underlying disease mechanism can substantially improve renal survival. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 78-86)