• Childhood Kidney Diseases
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• v.3 no.1
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• pp.95-99
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• 1999

Idiopathic membranous nephropathy is one of the most common causes of nephrotic syndromes in adults but rare in childhood. The occurrence of crescentic glomerulonephritis and membranous nephropathy in a patient is rare. This report describes a patient who initially was diagnosed as a membranous glomerulonephropathy at age 12 years and subsequently developed a crescentic, rapidly progressive glomerulonephritis at age 19 years. The patient responded to methylprednisolone pulse therapy and he maintained with partial remission and normal renal function with residual proteinuria.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.231-236
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• 2005

Juvenile rheumatoid arthritis(JRA) is the most common major connective tissue disease in children. Renal involvement in JRA is rare. Among the renal lesions that have been reported in JRA, amyloidosis and drug-induced nephropathy are the most common. Crescentic glomerulonephritis in JRA has rarely been reported. We report a case of ANCA-associated pauci-immune crescentic glomerulonephritis in JRA. The patient was a 15-year old boy with a 3-year history of JRA. He presented with gross hematuria, proteinuria, positive p-ANCA and elevation of BUN and creatinine. Pathologic findings revealed focal necrotizing and crescentic glomerulonephritis. There were no significant immunoglobulin or complement deposits. His renal function recovered after intravenous methylprednisolone pulse therapy and oral steroid use. In Korea, this is the first reported case of pauci-immune crescentic glomerulonephritis in JRA. (J Korean Soc Pediatr Nephrol 2005;9:231-236)

• 대한소아신장학회:학술대회논문집
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• 2007.04a
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• pp.3-4
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• 2007

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.82-85
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• 1997

The incidence of nephritis associated with Henoch-Sch nlein purpura varies, but glomerulonephritis consistently accounts for most of the associated morbidity and mortality. A very small number of Henoch-Sch nlein purpura develop rapidly progressive glomerulonephritis. A three-year old male patient who showed acute nephritic nephrotic syndrome developed abdominal pain, arthralgia and multiple purpurae on lower extremities later. Peritoneal dialysis was done at the 6th hospital day and continued for 7 months. Renal biopsy disclosed crescentic glomerulonephritis (with 81% crescent formation) and methylprednisolone pulse therapy was done. These days, his general condition is good, but serum creatinine levels are 1.2-1.3 mg/dL. This case was reviewed briefly with the literatures.

• Journal of Yeungnam Medical Science
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• v.34 no.1
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• pp.140-145
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• 2017

Glomerulonephritis (GN) is sometimes associated with infective endocarditis (IE). Bartonella endocarditis is difficult to diagnose because it is rare and cannot be detected by blood culture. This is the first report of cytoplasmic anti-neutrophil cytoplasmic antibody-positive subacute endocarditis-associated GN caused by Bartonella infection in South Korea. A 67-year-old man was hospitalized due to azotemia. He complained of weight loss and anorexia for 6 months. A diagnosis of IE was made based upon echocardiographic detection of vegetations on the mitral and aortic valves and a Bartonella antibody titer of 1:2,048. Renal histology identified focal crescentic GN. Azotemia and proteinuria improved after doxycycline and rifampin treatment combining with steroid therapy.

• Childhood Kidney Diseases
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• v.10 no.2
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• pp.119-124
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• 2006

Purpose : The prognosis of acute poststreptococcal glomerulonephritis(APSGN) has been reported to be favorable. However, several studies have reported that patients with nephrotic range proteinuria in the acute phase or persistent proteinuria may progress to chronic renal failure. To elucidate this further, we analyzed the features of proteinuria and its prognosis in pediatric patients with APSGN. Methods : A total of 48 children with APSGN admitted to our hospital between Jan. 2000 and Dec. 2004 were included. After discharge from the hospital, patients were regularly followed up every month by clinical evaluations and laboratory tests including routine urinalysis and quantification of proteinuria. Results : Age of the patients ranged from 3 to 15 years(median 5.8 years) at the time of disease onset. Proteinuria was present in 34(70.8%) patients and 5 of them showed heavy proteinuria. Proteinuria normalized within one month in most patients(82.3%) and there was no one with proteinuria after 6 months. Cyclosporine A(5 mg/kg/day in two divided doses) was given to 3 patients with massive proteinuria that lasted longer than 2 months and the result was complete remission within 4 months. Conclusions : Our data indicated that the prognosis of APSGN during childhood is excellent. Children with severe proteinuria or subnormal renal function in poststreptococcal glomerulonephritis had favorable prognosis without chronic renal failure, and children with crescentic formation also had favorable prognosis. Three patients who continued to have heavy proteinuria for more than 2 months received cyclosporine A and remission of proteinuria was achieved within a couple of months.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.4-12
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• 1997

Purpose: To evaluate the prevalence and clinical manifestations of various glomerulonephritis (GN) in children, a clinicopathological anlysis of 310 biopsied cases were performed. Method: We conducted retrospective study with review of histopathologic findings and clinical manifestations of the 310 cases diagnosed as glomerulonephritis by percutaneous renal biopsy which were done between January 1986 and December 1996 at department of pediatrics, Pusan Paik hospital. Results: 1) Male to female ratio was 1.54:1 and the range of age was from 13 months to 15 years 10 months. 2) Among these, 217 (70.0%) patients were belong to primary GN and 93 (30.0%) patients were belong to secondary GN. As a whole, the most common pathologic diagnosis was minimal change lesion (MC, 32.6%), which was followed by IgA nephropathy (IgAN, 15.8%), $Henoch-Sch\"{o}nlein$ purpura nephritis (HSPN, 13.5%), Poststreptococcal glomerulonephritis (PSAGN, 8.1%). 3) Clinical manifestations of patients were asymptomatic urinary abnormality (43.2%), nephrotic syndrome (41.0%), acute glomerulonephritis (14.2%), chronic glomerulonephritis (1.0%), rapidly progressive glomerulonephritis (0.6%). 4) In primary GN, the most common pathologic diagnosis was MC (46.5%), IgAN (22.6%), thin glomerular basement membrane (GBM) disease (7.8%), membranoproliferative glomerulonephritis (MPGN, 5.5%), mesangial proliferative glomerulonephritis (MesPGN,4.6%), focal segmental glomerulosclerosis (FSGS, 4.6%), membranous nephropathy (MN, 0.9%), sclerosing glomerulonephritis (SCGN, 0.9%), crescentic glomerulonephritis (CreGN, 0.5%) and non-specific glomerulonephritis (NonspGN, 6.0%). 5) Major causes of secondary GN were HSPN (45.2%), PSAGN (26.9%), hepatitis B associated glomerulonephritis (HBGN, 17.2%), lupus nephritis (LN, 6.5%), Alport syndrome (2.2%), hemolytic uremic syndrome (1.0%), fibrillary glomerulonephritis (1.0%) in descending order. Conclusions: There are some differences of the results of clinicopathological stuidies of glomerulonephritis in children because of its different indications of renal biopsy, pathologic classification of renal disease and methods of analysis among investigators. In order to establish more reliable data of incidence and classification of childhood glomerulonephritis in Korea, multicenter cooperative study were necessary.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.99-104
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• 2008

Microscopic polyangiitis(MPA) is a systemic necrotizing vasculitis that involves many organ systems including the skin, joint, kidneys, and lungs. In spite of early diagnosis and intensive care, the five-year actuarial patient and kidney survival rates are 65% and 55%. We experienced a case in 7-year-old girl of microscopic polyangiitis presenting with rapidly progressive glomerulonephritis which was confirmed by renal biopsy and positive serum perinuclear antineutrophil cytoplasmic autoantibodies(p-ANCA). The diagnosis of patients first renal biopsy was MPA, p-ANCA-associated crescentic glomerulonephritis. The patients second renal biopsy was done 5 years 6 months later since first renal biopsy, and pathologic diagnosis was chronic sclerosing glomerulonephritis, advanced, due to MPA. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor, angiotensin II receptor blocker, and cyclophosphamide were used until now and the patients current age is 14 years old. On admission, the patients laboratory findings showed BUN 117 mg/dL and Cr 2.3 mg/dL, while on the hospital day BUN and Cr values fell to 20.8 mg/dL and 1.6 mg/dL. But renal function was progressed to chronic failure with latest laboratory data BUN 51.7 mg/dL and Cr 3.2 mg/dL. ACE inhibitor, angiotensin II receptor blocker and small dose of immunosuppressant with close observation is the key to maintain the patient survival.

• 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)