• Childhood Kidney Diseases
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• v.3 no.2
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• pp.227-231
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• 1999

Hemolytic uremic syndrome is a clinical syndrome with various etiology and pathogenesis. And pneumococcal neuraminidase has been known to play a pathogenetic role in some cases with this syndrome. We experienced two children with hemolytic uremic syndrome complicated by pneumococcal infection. One was 21-month-old girl with pneumococcal pneumonia, and the other was 7-month-old girl with pneumococcal meningitis and sepsis. Both of them showed typical clinical manifestations of hemolytic uremic syndrome with prolonged anuria during the course of pneumococcal infection. The renal functions of both cases did not recovered after resolution of acute hemolytic episode and chronic renal failure developed.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.104-108
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• 1999

Atypical hemolytic uremic syndrome associated with neuraminidase-producing Streptococcus pneumoniae usually associated with invasive infection such as fulminant pneumonia, sepsis, and meningitis and may occur earlier in lift and has a higher mortality rate than typical hemolytic uremic syndrome. We have experienced a 22-month-old female patient with hemolytic uremic syndrome associated with S. pneumoniae pneumonia and empyema. The patient was treated with ceftriaxone and washed red blood cell transfusion. As the disese course could be aggravated by the use of blood products containing anti-Tomsen-Friedenreich antigen, early recognition and sensible use of blood products such as washed RBC might lead to the improved outcome.

• Tuberculosis and Respiratory Diseases
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• v.72 no.2
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• pp.207-211
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• 2012

Hemolytic uremic syndrome (HUS) is a rare disorder characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. HUS arises from a wide spectrum of conditions, and chemotherapeutic agents have been reported to be associated with HUS, including Mitomycin, Cisplatin, Bleomycin, and Gemcitabine. A 76-year-old man treated with Gemcitabine due to non-small cell lung cancer developed clinical and laboratory findings compatible with HUS. Gemcitabine was ceased and hemodialysis and plasma exchange were utilized and he recovered. A high level of suspicion for HUS is necessary when cancer patients are treated with Gemcitabine, and prompt recognition and treatment are also essential.

• Childhood Kidney Diseases
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• v.10 no.2
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• pp.249-256
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• 2006

Diarrhea-associated hemolytic uremic syndrome(D+ HUS) is induced by enterohemorrhagic Escherichia coli(EHEC) and is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. The disease is usually transmitted by meat and water contaminated by excreta of domestic animals. We report a son and his mother with diarrhea-associated hemolytic uremic syndrome that spread within the family.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.38-45
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• 2005

Purpose : The purpose of this study was to analyze the therapeutic effect of plasmapheresis in various pediatric diseases. Methods : Therapeutic plasmapheresis was performed by COBE Spectra centrifugation. Nine cases were included in this study. The number an[;. method of plasmapheresis, together with the progress and prognosis of each case were retrospectively reviewed. Results : The patients' ages ranged from 26 mont]Is to 16 years of age, and the mean age was 9.9 years. There were S males and 4 females. The underlying diseases requiring plasmapheresis included 2 cases of hemolytic uremic svndrome(HUS), 1 case of lupus nephritis, 2 cases of rapidly Progressive glomerulonephritis(RPGN), 1 case of focal segmental glomorulosclerosis(FSGS), 1 case of systemic vasculitis after pulmonary hemorrhage, 1 case of acute renal failure associated with pulmonary hemoIThage, and 1 case of acute rejection after renal transplantation. The average number of plasmapheresis performed was 6.2 times with a range of 3 to 13 times. The patients with HUS, lupus nephritis, ANCA positive systemic vasculitis induced by pulmonary hemorrhage and ARF-associated pulmonary hemorrhage showed a good response to therapeutic plasmapheresis, but the patients with RPGN, refractory FSGS, and acute rejection after renal transplantation were not responsive to treatment. The most common side effect was hypocalcemia which was rarely symptomatic. Vital signs were not compromised. Conclusion : Although it is presumptuous to generalize the therapeutic effects of plasma pheresis in different diseases due to the small number of study subjects, this study shows that plasmapheresis may be an effective therapeutic modality in various pediatrics diseases and should be considered as a therapeutic option.

• Clinical and Experimental Pediatrics
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• v.50 no.10
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• pp.931-937
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• 2007

The hemolytic uremic syndrome (HUS) is a rare disease of microangiopathic hemolytic anemia, low platelet count and renal impairment. HUS usually occurs in young children after hemorrhagic colitis by shigatoxin-producing enterohemorrhagic E. coli (D+HUS). HUS is the most common cause of acute renal failure in infants and young children, and is a substantial cause of acute mortality and morbidity; however, renal function recovers in most of them. About 10% of children with HUS do not reveal preceding diarrheal illness, and is referred to as D- HUS or atypical HUS. Atypical HUS comprises a heterogeneous group of thrombomicroangiopathy (TMA) triggered by non-enteric infection, virus, drug, malignancies, transplantation, and other underlying medical condition. Emerging data indicate dysregulation of alternative complement pathway in atypical HUS, and genetic analyses have identified mutations of several regulatory genes; i.e. the fluid phase complement regulator Factor H (CFH), the integral membrane regulator membrane cofactor protein (MCP; CD46) and the serine protease Factor I (IF). The uncontrolled activation of the complement alternative pathway results in the excessive consumption of C3. Plasma exchange or plasma infusion is recommended for treatment of, and has dropped the mortality rate. However, overall prognosis is poor, and many patients succumb to end-stage renal disease. Clinical presentations, response to plasma therapy, and outcome after renal transplantation are influenced by the genotype of the complement regulators. Thrombotic thrombocytopenic purpura (TTP), another type of TMA, occurs mainly in adults as an acquired disease accompanied by fever, neurologic deficits and renal abnormalities. However, less frequent cases of congenital or hereditary TTP associated with ADAMTS-13 (a disintegrin and metalloprotease, with thrombospondin 1-like domains 13) gene mutations have been reported, also. Recent advances in molecular genetics better allow various HUS to be distinguished on the basis of their pathogenesis. The genetic analysis of HUS is important in defining the underlying etiology, predicting the genotype-related outcome and optimizing the management of the patients.

• Childhood Kidney Diseases
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• v.18 no.1
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• pp.42-46
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• 2014

Kawasaki disease (KD) is a systemic vasculitis that can affect many organ systems. Renal manifestations include pyuria, hematuria, proteinuria, tubulointerstitial nephritis, acute renal failure, hemolytic uremic syndrome, or renal scarring. Although its precise pathogenesis remains unknown, it is considered an autoimmune disease. In the literature, it has been reported that KD may develop in conjunction with urinary tract infections. However, many of these previous studies did not use imaging methods such as renal sonograms, dimercaptosuccinic acid renal scans, and voiding urethrocystograms. We report a case of an 8-month old male infant with high grade vesicoureteral reflux, who developed incomplete KD after recurrent pyelonephritis. Acute pyelonephritis can be an early manifestation of KD. Such cases require the evaluation of urinary tract anomalies according to the guidelines for the management of urinary tract infections.

• Pediatric Infection and Vaccine
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• v.4 no.1
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• pp.73-78
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• 1997

Purpose : Escherichia coli O157 can produce diarrhea as well as hemorrhagic colitis and hemolytic uremic syndrome. In many parts of North America, E. coli O157 often is the second or third most commonly isolated enteric bacterial pathogens. Recently, intakes of fast food, including hamburgers have increased in Korea. Therefore, E. coli O157 infection in Korea are likely to be increased. Methods : Stool samples from 317 pediatric diarrheal patients were analyzed by culture on sorbitol-MacConkey agar. Sorbitol-negative colonies were teated by E. coli O157 latex agglutination test. Results : Of the 317 specimens, one (0.3%) were E. coli O157:NM that not produced Shiga toxin. The 7 year old male patient who had complained of abdominal pain, vomiting and non-bloody diarrhea for 2 days. The patient was improved for 2 days after admission. Conclusions 1 Only one (0.3%) of all fecal samples were isolated E. coli O157 that not produced Shiga toxin. Therefore, routine stool culture for the isolation of E. coli O157 was not likely to be neccessary so far.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.48-56
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• 2000

Purpose: Alport SD., the most common herectitary rephriris, is a renal disease with rapid progression. Deafness, ocular abnormalities and a specific EM finding may be associated in addition to a family history. We have aralyged retrospectively. Methods: We observed 12 children with Alport syndrome who were diagnosed at Dept. of pediatrics in Kyunghee Univ., College of Medicine, from Apr. 1991 until Jun. 1999. We used four criteria for diagnosis: renal disease, family history, deafness or eye abnormalities, and a specific finding in electron microscopy Results: 2 of 12 patients had all features of the four diagnostic criteria. We could not trace an exact family history in 3 patients, and 6 patients did not exhibit deafness or eye abnormality. One could not have renal biopsy because offer chronic renal failure. Other three criteria were observed in her. The ratio of male to female observed was 1:2 respectively and the mean age of initial renal symptom was 5.6 years. 9 of 12 patients had a family history of renal disease. In the audiogram and ocular examination for 11 of 12 cases, sensorineural hearing loss was observed in 6 and ocular abnormality in 2 cases. In electron microscopic finding, irregular thickness of the capillary basement membranes with lamination of lamina densa and foot process obliteration was noted in 9 of 11 and thin basement membrane with splitting and foot process obliteration was noted in the other 2. The mean period of follow-up was 3 6/12 years. And one patient developed the chronic renal failure until now and had kidney transplantation. Conclusion: For the diagnosis of Alport syndrome, the following four diagnostic criteria are very important : renal disease, family history, deafness or eye abnormalities, and a specific finding on electron microscopy. We expect that more patients can be detected through the analysis of these characteristics.

• 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)