• Childhood Kidney Diseases
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• v.23 no.2
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• pp.93-99
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• 2019

C3 glomerulopathy is a renal disorder involving dysregulation of alternative pathway complement activation. In most instances, a membranoproliferative pattern of glomerular injury with a prevalence of C3 deposition is observed by immunofluorescence microscopy. Dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are subclasses of C3 glomerulopathy that are distinguishable by electron microscopy. Highly electron-dense transformation of glomerular basement membrane is characteristic of DDD. C3GN should be differentiated from post-infectious glomerulonephritis and other immune complex-mediated glomerulonephritides showing C3 deposits.

• Childhood Kidney Diseases
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• v.17 no.1
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• pp.1-5
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• 2013

C3 glomerulonephritis (C3GN) is a recently described entity that shows a glomerulonephritis on light microscopy, bright C3 staining and the absence of C1q, C4, and immunoglobulins on immunofluorescence microscopy and mesangial and/or subendothelial electron-dense deposits on electron microscopy. The term 'C3 glomerulopathy' is often used to include C3GN and dense deposit disease (DDD), CFHR5 nephropathy, those of which result from dysregulation of the alternative pathway of complement. C3GN shares some aspects of atypical hemolytic uremic syndrome, MPGN, late stage of post infectious glomerulonephritis and other glomerulonephrtis. When C3GN is considered, measurement of serum complement proteins including C3, CFH, CFI, CFB and testing for the presence of C3 nephritic factor, anti-factor H autoantibodies are necessary. To screening for mutations, genes that encode complement regulators should be evaluated. This disorder equally affected all ages, both genders, and typically presented with hematuria and proteinuria. In both the short and long term, renal function remained stable in the majority of patients.

• Childhood Kidney Diseases
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• v.25 no.1
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• pp.29-34
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• 2021

C3 glomerulonephritis (C3GN), a rare condition associated with dysregulation of the alternative pathway of the complement system, is histopathologically characterized by isolated or dominant C3 deposition in the renal glomeruli. We report a case of C3GN associated with anti-complement factor H (CFH) autoantibodies and CHF-related protein deficiency in an adolescent male. A 16-year-old adolescent male was admitted to a hospital with a 1-month history of generalized edema prior to presentation. Persistent microscopic hematuria and low serum C3 levels were incidentally detected at 7 and 10 years of age, respectively. Laboratory test results revealed hypoalbuminemia, nephrotic-range proteinuria, microscopic hematuria, and normal serum creatinine levels. The serum C3 and C4 levels were 17 mg/dL (normal 80-150 mg/dL) and 22 mg/mL (17-40 mg/mL), respectively. Renal biopsy showed typical features of C3GN. Further investigations revealed positive results on plasma anti-CFH autoantibody testing and a homozygous deletion of CFHR1 and CFHR3, which encode CFH-related proteins 1 and 3, respectively. Proteinuria persisted despite treatment with intravenous methylprednisolone, mycophenolate mofetil, and angiotensin-receptor blocker; however, his renal function remained stable. In conclusion, anti-CFH autoantibodies serve as important contributors to C3GN. This is the first case report that describes C3GN in an adolescent Korean male with anti-CFH autoantibodies and homozygous CFHR1 and CFHR3 deletion.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.159-165
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• 2004

Purpose: This study aimed to compare the clinical outcome of acute poststreptocaccal glomerulonephritis (APSGN) between patients who had presented with gross hematuria and those with microscopic hematuria. Methods: Thirty-nine patients with acute poststreptococcal glomerulonephritis, who were diagnosed from January 2000 to April 2003 were enrolled. Results: The mean age was 8.85 years and the male to female ratio was 1.1:1 Seventeen patients presented with gross hematuria at diagnosis(group A) and twenty-two patients had microscopic hematuria only(group B). There were no significant differences between the two groups in the incidence of edema, fever or history of respiratory infection and oliguria. But hypertension was more frequent in group B. Laboratory data showed decreased C3 and C4 level in group B. Spot urine protein/creatinine ratio and 24hr urine protein showed higher levels in group A. Conclusion; Patients with gross hematuria at diagnosis had lower incidence of hypertension and a higher rate of nephrotic range of proteinuna than patients with microscopic hematuria. However, no difference in the duration of admission or complication rate was observed. All patients had clinical improvement during follow-up. We conclude that gross hematuria is not a significant prognostic factor for poststreptococcal glomerulonephritis.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.192-195
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• 1998

[ $\beta$ ]-streptococci have been reported as an etiology of Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP), but the role of $\beta$-streptococci in HSP is not yet confirmed. Our report of two HSP patients is as follows : Although the two patients' clinical symptoms resembled those symptoms of HSP, whereas serum $C_3$ decreased in one case, ASO titer increased in both cases and a histological examination of renal biopsy specimens revealed the fetures commonly described in poststreptococcal glomerulonephritis.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.93-98
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• 2008

Rare cases of IgG associated mesangial glomerulonephritis(IgG GN) defined by exclusive or predominant mesangial IgG deposits were reported first by Sato et al.(1993). and subsequently 10 pediatric cases were reported by Yoshikawa et al.(1994). Previous reports suggested that the prognosis of IgG GN is relatively benign course but recent report suggested that prognosis of IgG GN is highly variable. Also the recurrence of IgG GN in a renal transplant was reported by Fakhouri et al. (2002). Such a recurrence highlights the specificity of this type of glomerulonephritis. We experienced two pediatric cases of IgG GN proven by renal biopsy. Case 1. 4-year-old girl with nephrotic syndrome admitted because of general edema. The patient's urinalysis showed proteinuria and microscopic hematuria. Renal biopsy was performed because of relapsed nephritic syndrome. Light microscopic finding was nonspecific with almost normal histology. Immunofluorescent findings showed diffuse segmental IgG(+) and IgM(+) deposits in the capillary walls, and focal segmental spotty C4(trace), C1q(trace) deposits. Electron microscopic findings showed focal portion of mesangial electron dense deposits without mesangial widening. Case 2. 11-year-old girl admitted for evaluation of microsopic hematuria detected through mass school urinary screening program. Renal biopsy was performed for exact diagnosis. Immunofluorescent findings showed focal segmental IgG(+), IgM(+/-) and C3(+/-) deposits. Electron microscopic findings showed focal portion of mesangial electron dense deposits without mesangial widening.

• Pediatric Infection and Vaccine
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• v.11 no.1
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• pp.126-130
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• 2004

Neisseria meningitidis is a leading cause of meningitis and septicemia in children and young adults. Meningococcemia, the most severe form of infection, represents a part of the various spectrum of the illness, and its clinical manifestations varied from mild fever and occult bacteriemia to fulminant catastrophic events(e.g., sepsis, shock, and death) within a few hours after onset of symptoms. Complement deficiencies, either congenital or acqired, increase the risk for invasive meningococcal disease. Since C3 plays a critical role in the complement cascade, congenital C3 deficiency or conditions that decrease C3(e.g., systemic lupus erythematosus, cirrhosis, nephritis, C3 nephritic factor) increase the risk for invasive disease due to pyogenic bacteria including N. meningitidis. We had experienced a case of meningococcemia and meningococcal meningitis presenting with mild fever, petechiae, and purpura. The patient was a 7-year old male. He had mild fever, vomiting, headache, nuchal rigidity, petechiae and purpura on both lower extremities, which spreaded to the whole body. His blood culture grew Neisseria meningitidis. The diagnosis of meningococcal meningitis was confirmed by examination of cerebrospinal fluid. The clinical symptoms of the illness were improved after treatment of antibiotics(cefotaxime) for 12 days. However the patient had developed generalized edema, microscopic hematuria & proteinuria on the third day after admission. High ASO titer and low serum C3 level were also noted. The microscopic hematuria returned to normal about 2 months after discharge. The C3 level returned to normal range about 3~4 months after discharge. we report a case of combined acute poststreptococcal glomerulonephritis and acute invasive meningococcal disease with reference review.

• Clinical and Experimental Pediatrics
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• v.48 no.6
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• pp.606-613
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• 2005

Purpose : Acute poststreptococcal glomerulonephritis(APSGN) is a common form of glomerulonephritis in children. Most patients recover completely after the acute phase but a few patients have acute complications or progress to chronic renal disease. In recent years, the frequency of APSGN has been was decreasing but is still common in children. So we studied the clinical characteristics of APSGN from 1994 to 2003 and compared it with past studies. Methods : We studied 105 patients who were diagnosed with APSGN in the Department of Pediatrics, Asan Medical Center between January 1994 and December 2003, with a retrospective chart review. Results : The mean age was $8.5{\pm}2.6$ years. The male to female ratio was 2 : 1. Average annual incidence was $10.5{\pm}4.9$ most patients(60.0 percent) occurred from October to January. Edema was seen in 82 cases(78.1 percent), gross hematuria in 70 cases(66.7 percent), hypertension in 50 cases (47.6 percent) and oliguria in 22 cases(20.9 percent). Microscopic hematuria was seen in 105 cases (100 percent), positive ASO in 99 cases(94.2 percent), proteinuria in 67 cases(63.8 percent) and azotemia in 38 cases(36.2 percent). Serum complement 3(C3) level decreased in 96 cases and returned to normal within eight weeks in 70 patients(75.3 percent). Kidney biopsy was carried out in 22 cases. Most acute symptoms subsided within 2 weeks of onset. Conclusion : We concluded that there was no significant difference between clinical features of recent and past APSGN in children, and short term prognoses were excellent.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.45-51
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• 2006

Hypocomplementemia is found in all types of membranoproliferative glomerulonephritis (MPGN) but not in all patients. Hypocomplementemia can be ascribed to at least two circulating complement reactive modalities. The activation of the classical pathway produced by circulating immune complexes and the presence in the blood of anticomplement autoantibodies, called 'nephritic factor'(NF). The activation of the classical pathway by circulating immune complexes is probably the major mechanism responsible for hypocomplementemia in idiopathic MPGN type I. Nephritic factors have been shown to be responsible for the hypocomplementemia in both MPGN type II and III. NFa is probably the major mechanism responsible for the hypocomplementemia of idiopathic MPGN type II. NFt appears to be solely responsible for the hypocomplementemia in MPGN type III. Judging from the complement profile, NFt also may be present in some patients with MPGN type I. Although infection by meningococcus has been associated with deficiency of any of the plasmatic proteins of complement, it more commonly involves deficiency of the terminal components of the complement pathway(C5-C9). We experienced a patient who had MPGN and meningococcal meningitis. We examined the complement level and significantly lower levels of C3, C5 were found persistently. C7 was low at first and it returned to normal range after 2 months. C9 was normal at first, and was low after 2 months. This is the first reported case in which MPGN with meningococcal meningitis occurred.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.73-76
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• 2003

A girl aged 21 months with $Henoch-Sch\"{o}nlein$ purpura(HSP) developed heavy proteinuria with hematuria 8 days after the appearance of purpuric rash, swelling and tenderness of both ankle joints. Her clinical and laboratory features demonstrated nephrotic and nephritic syndrome. The percutaneous renal biopsy revealed diffuse mesangial proliferative glomerulonephritis. Unlike usual HSP nephritis, immunoglobulin A deposition was not detected in the mesangium or the capillary of the glomeruli. Instead, numerous subepithelial electron-dense deposits('humps') mimicking acute poststreptococcal glomerulonephritis were found.