• Childhood Kidney Diseases
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• v.2 no.1
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• pp.34-40
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• 1998

Present study was conducted to determine the frequency, clinical characteristics and long-term outcome of children with idiopathic hypercalciuria. Study patients consisted of 150 children with isolated hematuria (recurrent gross or persistent microscopic), and hypercalciuria was defined as urinary calcium excretion over 4 mg/kg/day. During follow-up period up to $6{\sim}8$ years, serial check-up of renal sonogram for stone formation and Dipstick examination for hematuria were done. Forty-four (29%) out of 150 cases were diagnosed as idiopathic hypercalciuria, and in hypercalciuric children compared to normocalciuric children boys were more common than girls (9:35) and gross hematuria was more common than microscopic hematuria (37:7) (P<0.05). Oral calcium loading test showed renal type in 29 cases, absorptive type in 8 cases and in 7 cases type could not be definable. Among 3 types no differences could be found in 24 hour urinary calcium excretion and in clinical or laboratory data. Urolithiasis developed in 4 out of 44 cases (2 at the time of initial diagnosis and 2 within $1{\sim}2$ years of follow-up periods) and these children showed lower chronologic age ($3.7{\pm}2.7\;vs\;7.2{\pm}2.9\;yr$) and more girl than boys (3:1 vs 6:34) (P<0.05) compared to the rest of the hypercalciuric children. Follow-up urinalysis showed disappearance of hematuria in 56, 50, 66 and 75% of children at $1{\sim}2,\;2{\sim}4,\;4{\sim}6$ and $6{\sim}8$ years after initial diagnosis respectively. In conclusion, present study demonstrates that idiopathic hypercalciuria is a major cause of isolated hematuria in children so that in these children 24 hour urinary calcium excretion test seems to be an essential test to be performed. And serial renal sonography should be done to detect development of nephrolithiasis. However, clinical significance of dividing hypercalciuric children into two pathophysiologically distinct subtypes by oral calcium loading test seems to be in doubt and further study is needed to solve this problem.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.23-29
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• 2003

Purpose : This study was performed to determine the natural history of histologically confirmed IgA nephropathy in pediatric patients who presented with hematuria and proteinuria. Patients and Methods : We reviewed the clinical course of 57 patients diagnosed with IgA nephropathy at the age of 15 years or younger from 1981 to 2000. All patients presented with hematuria or minimal proteinuria($<40\;mg/m^2/day$) and had normal renal function and blood pressure at the time of renal biopsy. Based on the clinical and pathological findings at the time of diagnosis, we sought for complications of IgA nephropathy such as heavy proteinuria(${\ge}40\;mg/m^2/day$), hypertension, and chronic renal failure. Results : The mean age at presentation was $9.5{\pm}2.8$ years(4 to 15 years) and 42(74%) were male. Isolated gross hematuria was observed in 20 patients(35%), microscopic hematuria in 3(5%), minimal proteinuria in 4(7%), both gross hematuria and minimal proteinuria in 15(26%), and both microscopic hematuria and minimal proteinuria in 15(26%). During a median follow-up of $7.0{\pm}3.5$ years, 38(67%) had complete resolution of hematuria and proteinuria, 12(21%) had persistently abnormal urinalysis without development of adverse events. Only 7(12%) developed adverse events : 4(7%) developed severe proteinuria, 1(2%) became hypertensive, and 2(3%) developed Impaired renal function. By univariate analysis using the chisquare test, the age at presentation(>10 years)(P<0.01) and poor histological classes of the Lee or Haas classification at onset(P<0.05) were significantly correlated with adverse events, whereas sex and clinical signs at onset were less concordant. Conclusion : We can conclude that the prognosis of IgA nephropathy diagnosed in early childhood is better and a good correlation exists between the clinical manifestations of this disease and the histological classes.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.219-224
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• 2001

Antiphospholipid syndrome is a thrombotic disorder characterized by the association of arterial and venous thrombosis with the antibodies directed toward phospholipids. The presence of these antibodies in systemic lupus erythematosus(SLE) has been shown to be related to several clinical and analytical alterations. We experienced one case of lupus nephritis with positive antiphospholipid antibodies in a 10-year-old girl whose chief complaint was persistent microscopic hematuria. We report this case with a brief review of related literatures. (J. Korean Soc Pediatr Nephrol 2001 ;5 : 219-24)

• Clinical and Experimental Pediatrics
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• v.56 no.4
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• pp.159-164
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• 2013

Purpose: Indoxyl sulfate and p-cresyl sulfate are important protein-bound uremic retention solutes whose levels can be partially reduced by renal replacement therapy. These solutes originate from intestinal bacterial protein fermentation and are associated with cardiovascular outcomes and chronic kidney disease progression. The aims of this study were to investigate the levels of indoxyl sulfate and p-cresyl sulfate as well as the effect of probiotics on reducing the levels of uremic toxins in pediatric patients on dialysis. Methods: We enrolled 20 pediatric patients undergoing chronic dialysis; 16 patients completed the study. The patients underwent a 12-week regimen of VSL#3, a high-concentration probiotic preparation, and the serum levels of indoxyl sulfate and p-cresyl sulfate were measured before treatment and at 4, 8, and 12 weeks after the regimen by using fluorescence liquid chromatography. To assess the normal range of indoxyl sulfate and p-cresyl sulfate we enrolled the 16 children with normal glomerular filtration rate who had visited an outpatient clinic for asymptomatic microscopic hematuria that had been detected by a school screening in August 2011. Results: The baseline serum levels of indoxyl sulfate and p-cresyl sulfate in the patients on chronic dialysis were significantly higher than those in the children with microscopic hematuria. The baseline serum levels of p-cresyl sulfate in the peritoneal dialysis group were significantly higher than those in the hemodialysis group. There were no significant changes in the levels of these uremic solutes after 12-week VSL#3 treatment in the patients on chronic dialysis. Conclusion: The levels of the uremic toxins p-cresyl sulfate and indoxyl sulfate are highly elevated in pediatric patients on dialysis, but there was no significant effect by probiotics on the reduction of uremic toxins in pediatric dialysis patients. Therefore, studies for other medical intervention to reduce uremic toxins are also necessary in pediatric patients on dialysis.

• 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.16 no.2
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• pp.132-137
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• 2012

Microscopic polyangiitis (MPA) is systemic small vessel vasculitis that is very rare in childhood. MPA is characterized by pauci-immune necrotizing small vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation. Approximately 90% of patients have glomerulonephritis that is accompanied by a variety of other organ involvement. A 10-year-old girl visited our clinic with clinical manifestations suggestive Henoch-Sch$\ddot{o}$nlein purpura nephritis such as purpuric skin rash, abdominal pain, arthralgia on both knees, massive proteinuria and microscopic hematuria. So initially we suspected Henoch-Sch$\ddot{o}$nlein purpura nephritis. However, later her perinuclear-antineutrophil cytoplasmic antibodies(p-ANCA) test was positive, and her renal biopsy was consistent with microscopic polyangiitis. We began steroid therapy, combined with cyclophosphamide, ACE inhibitor. Currently she is a 12-year old, and until now she has been regularly examined in the outpatient. We report a case of microscopic polyangiitis initially suspected with Henoch-Sch$\ddot{o}$nlein purpura nephritis.

• 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.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.31-37
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• 2005

Puruose : Thin glomerular basement membrane disease(TGBMD) is found in patients with family history of hematuria. TGBMD is autosomal dominant and is known to be one of the commonest causes of asymptomatic hematuria. This study was conducted to evaluate the histological and clinical features of patients with TGBMD. Methods : 150 cases diagnosed with TGBMD by renal biopsy while admitted in the department of pediatrics, Kyungpook National University Hospital between January 1999 and December 2003 comprised the study group. The following parameters were retrospectively anaIyzed age of onset, hematuria pattern, existence of proteinuria, process of diagnosis, laboratory findings, thickness and character of basement membrane and family history. Results : The mean age at the time of diagnosis was 7.9 years. The male to female ratio was 65:77. 94 patients or 66% visited the hospital with a chief complaint of persistent microscopic hematuria. Gross hematuria accounted for 13 cases or 9%. 78 cases(55%) were found to have hematuria for the first time from a routine school urinalysis screening. The renal biopsy showed the thickness of basement membrane to be 186$\pm$36 nm. Focal lamellation of the basement membrane was found in eight cases. In the family history, hematuria was shown in 10 cases on the Paternal side, 13 on The maternal side and none on both sides. In seven cases, hematuria was shown among siblings. No significant differences were found among the laboratory test results which were conducted at an average interval of fifteen months. Conclusion : TGBMD is one of the major causes of asymptomatic hematuria in children, which was diagnosed in increasing numbers since school urinary mass screening test started in 1998. In cases with familial progressive renal disease or focal duplication in the basement membrane Alport syndrome should be considered.

• Childhood Kidney Diseases
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• v.16 no.1
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• pp.46-50
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• 2012

C1q nephropathy is a distinct clinicopathologic entity, characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence for systemic lupus erythematosus. Clinically it may present as nephrotic syndrome and non-nephrotic proteinuria per se or associated with microscopic hematuria, gross hematuria, hypertension, or renal insufficiency. So far there is only one report about a familial case of C1q nephropathy (in two sisters). We present two cases of familial C1q nephropathy with nephrotic syndrome which was steroid resistant, but partially remitted with cyclosporine.

• Childhood Kidney Diseases
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• v.26 no.1
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• pp.31-39
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• 2022

Alport syndrome (AS) is a progressive hereditary nephritis that is often accompanied by sensorineural hearing loss and ocular abnormalities. It is inherited in three modes of X-linked AS (XLAS), autosomal recessive AS (ARAS), and autosomal dominant AS (ADAS). XLAS is caused by pathogenic variants in COL4A5, while ARAS and ADAS are caused by those in COL4A3 or COL4A4. There is currently no curative treatment for AS; however, angiotensin-converting enzyme inhibitors (ACEi) can improve the outcome of AS. In the past decade, multiple studies have shown that early intervention with ACEi upon isolated microscopic hematuria or microalbuminuria could delay disease progression, and early diagnosis is crucial for early treatment. Therefore, a new classification of AS based on molecular diagnoses has been proposed, including the paradigm shift of re-classifying female "carriers" to "patients" and "thin basement membrane nephropathy" to "ADAS." In addition, with the detection of COL4A mutations in some patients with biopsy-confirmed IgA nephropathy, focal segmental glomerulosclerosis, and chronic kidney disease of unknown origin, it is suggested that the phenotype of AS should be expanded. In this review, we highlight the landmark studies and guidelines published over the past decade and introduce strategies for early diagnosis and treatment to improve the outcomes of AS.