• Childhood Kidney Diseases
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• v.8 no.2
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• pp.244-249
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• 2004

Obesity-associated focal segmental glomeruloscleropis(OB-FSGS) has been known to progress into advanced renal insufficiency, and its clinicopathological features Include obesity, FSGS lesions with glornerulomegaly and, nephrotic-range proteinuria without edema. A 14 year old girl with Prader-Willi syndrome showed nephrotic-range proteinuria without hypoalbuminemia or edema. The renal biopsy revealed focal segmental glomerulosclerosis together with glomerular hypertrophy and an increased mesangial matrix. We report here a case of OB-FSGS as one of the renal problems of Pradel-Willi syndrome, and we came to the conclusion that Prader-Willi syndrome is one of the Possible disease entities that can lead to renal insufficiency through obesity.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.92-99
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• 2007

The posterior reversible encephalopathy syndrome(PRES) is characterized clinically by a combination of acute or subacute confusion, lethargy, visual disturbance, and seizures. PRES has been described in various clinical settings, including severe hypertension, chemotherapy, eclampsia, and seizure. We report a case of a 7-year-old girl who had taken cyclosporine for steroid resistant nephrotic syndrome. Twenty one days after the cyclosporine therapy, she was admitted due to generalized tonic clonic seizure and headache. Her blood pressure was 170/90 mmHg. Magnetic resonance(MR) imaging showed necrotic/cystic lesions involving the bilateral parieto-occipital region. After discontinuation of cyclosporine, and control of blood pressure, she had no more seizure and headache. The follow-up MR examination which was performed 6 months later showed the decreased extent of the lesion.

• Childhood Kidney Diseases
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• v.19 no.2
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• pp.98-104
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• 2015

Objective: To find out clinical features and long-term outcomes of idiopathic childhood nephrotic syndrome(NS) patients with late steroid resistance(LSR)/late steroid sensitiveness(LSS). Patients and Methods: A retrospective chart review was performed on 480 patients diagnosed with idiopathic childhood NS at Asan Medical Center Children's Hospital from 1990 to 2013. Twenty-four patients whose responsiveness to steroids changed over a minimum 2 year follow-up period (2-17.5 years) were investigated. All patients had undergone a renal biopsy. Results: Among 480 nephrotic children, 428 (89%) were sensitive to the first steroid course. Of those who initially responded, 11 (2.5%) developed resistance to steroid therapy after relapses. LSR mostly developed between 1 month and 1 year after the initial episode. Six patients showed a minimal change and five showed focal segmental glomerulosclerosis (FSGS). Nine (82%) responded to cyclosporine or methylprednisolone pulse therapy. Of these, two had no further relapse, whereas the other seven experienced several relapses that ranged in length from 1.1 to 13.9 years. Three of the nine who initially responded to immunosuppression went on to experience several changes in steroid responsiveness. Two (18%) with resistance to immunosuppressants, including steroids, eventually progressed to end stage renal disease. Among the 52 patients (11%) who were initially steroid resistant, 13 (23%) were converted to steroid sensitive at relapses. Among these, 9 showed minimal change and 4 showed FSGS. Two had no further relapse and the other 11 responded to steroids on subsequent relapses ranging in length from 1.3 to 9.4 years. All these patients have had no further changes in steroid responsiveness with normal renal function. Conclusions: In this study, 2.5% of initial steroid responders and 25% of initial steroid non-responders changed their responsiveness to steroids at subsequent relapses. Eighteen percent of LSR patients developed end stage renal disease. All of the LSS patients showed preserved normal renal function. Responsiveness to immunosuppressants seemed to be the most important factor determining longterm outcomes in LSR/LSS patients.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.42-47
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• 1999

Angiotensin-converting enzyme inhibitors lower urinary protein excretion in hypertensive and normotensive patients with renal disease. Most children with nephrotic syndrome have minimal change histology and the great majority of these patients respond to the treatment with oral prednisone. Here we have studied the effects of combination of Inhibace and oral prednisone in pediatric patients with nephrotic syndrome. 45 patients with nephrotic syndrome were selected. Of these, 29 patients were treated with prednisone(2mg/kg/day) and 16 children were treated with prednisone and Inhibace(2.5mg/day). Urinary protein, blood pressure, creatinine clearance, serum creatinine, serum albumin and serum cholesterol were measured in both control and Inhibace group. Also the duration to remission after treatment was compared in both groups. The amounts of proteinuria before and after treatment were not significantly different in both group. The duration to remission of proteinuria was significantly shorter in Inhibace group compared to that in control group. The changes of blood pressure and creatinine clearance were not significant in Inhibace group. In conclusion, the combination therapy of oral prednisone and ACE inhibitor have shortened the duration to remission of proteinuria in nephrotic syndrome of children.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.250-255
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• 2004

Wilson's disease is an autosomal recessive disorder characterized by degenerative changes in the brain, liver, and cornea. Treatment includes D-penicillamine, trientine, and zinc sulfate. D-penicillamine has been used frequently as first line therapy for Wilson's disease. However, nephrotoxicity can occur after D-penlcillamlne treatment. Among them membranous glomerulopathy is the most common histological abnormality but minimal change lesions have also been reported. Nephrotic syndrome is a late complication of D-penicillamine treatment but very rarely can occur within 2 months after treatment of D-penicillamine. We report the early development of minimal change nephrotic syn,frome in a 3-year-old'girl with Wilson's disease 3 weeks after initiation of D-penicillamine.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.26-33
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• 1998

This retrospective study was designed to evaluate the prognostic value and histologic correlation of sonography in childhood nephrotic syndrome. Sixty-nine patients with proteinuria over 2g per day at the time of presentation who were treated at the Korea University Hospital were included in this review. They were 1 to 15 years old(mean age, 7.8 years) with 49 males and 20 females. In each patient an ultrasound examination was done using SPA 1000(Diasonics, C.A., U.S.A.) on admission. Tissue specimens were obtained from 46 patients. The paraffin-embedded specimens were reviewed with special reference to interstitial edema, interstitial fibrosis, tubular atrophy, global sclerosis or inflammatory cell infiltrates. Biopsy proven renal disease were minimal change disease(n=20), focal segmental glomerulosclerosis(n=7), membranous glomerulonephritis(n=2), membranoproliferative glomerulonephritis(n=1), $Henoch-Sch\"{o}nlein$ purpura nephritis(n=6), IgA nephropathy(n=5), poststreptococcal glomerulonephritis(n=2), systemic lupus erythematosus(n=1) and Alport syndrome(n=2). There was a significant relationship between increased cortical echogenicity and global sclerosis or tubular atrophy(P<0.05). But no significant relationship was found between increased cortical echogenicity and interstitial fibrosis, interstitial edema, or inflammatory cell infiltration. In biopsy-proven primary nephrotic syndrome(n=30), no significant relationship was found between the increased conical echogenicity and the interstitial edema, interstitial fibrosis, global sclerosis, tubular atrophy or inflammatory cell infiltration. But there was a significant relationship between increased cortical echogenicity and resistance to corticosteroid (P<0.05). These results suggest that increased cortical echogenicity may be due to tubular atrophy or global sclerosis in patients with proteinuria and may be an effective indicator of resistance to corticosteroid in primary nephrotic syndrome.(J Korean Soc of Pediatr Nephrol 2:26-33, 1998)

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.13-16
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• 1997

Steroid induced bone loss is a serious problem in frequently relapsing nephrotic syndrome especially in growing children. In order to evaluate whether $1-(OH)D_3$ (IL Sung Pharma.Co.) is effective in preventing steroid induced bone loss, we gave $0.5{\mu}g$ of $1-(OH)D_3$ for one year to forty patients with frequently relapsing nephrotic syndrome, receiving longterm prednisolone therapy (mean duration $50.12{\pm}29.40$ months). We checked the following markers before and after $1-(OH)D_3$ therapy. i.e. bone mineral density(BMD) using dual energy X-ray absorptiometry(DEXA) at the 2nd to 4th lumbar spine, serum calcium, phosphorus, parathyroid hormone(PTH), osteocalcin and urine pyridinoline(U-PYD). BMD($g/cm^2$) was increased even steroid therapy from $0.71{\pm}0.0\;to\;0.73{\pm}0.0$ (p<0.05). Lumbar spine BMD is a sensitive marker for evaluating steroid induced bone loss in children receiving longterm corticosteroid therapy and that $1-(OH)D_3$ appears to be effective in treating and preventing steroid induced bone loss.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.123-129
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• 1997

Purpose : The Childhood Steroid Resistant Nephrotic Syndrome (SRNS) has a poor prognosis and there has been no effective therapy against SRNS of children. In 1990, Mendoza have reported that methylprednisolone pulse therapy was effective against SRNS of children. But in 1992, Waldo have reported that methylprednisolone pulse therapy was not as effective as in the report of Mendoza. So, retrospectively, we have studied 20 korean children with SRNS to evaluate the effect of methylprednisolone pulse therapy, Methods : Mothylprednisolone pulse therapy were given to 20 korean children with SRNS who admitted to Seoul National University Hospital from 1990 to 1995 and follow up was done Results : 1) During methylprednisolone pulse therapy, remission of nephrotic syndrome was induced in 45% of patients. 2) during follow up after the end of methylprednisolone pulse therapy, remission of nephrotic syndrome was maintained in 45% of patients. 3) 25% of patients has progressed to chronic renal failure. Conclusion : We think that the methylprednisolone pulse therapy is a effective therapy against SRNS of children with the 45%, remission rate of of SRNS in Korean Children

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.1-10
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• 1999

Purpose: Up to date there is no nationwide survey on epidemiological or clinical data of nephrotic syndrome, so we investigated about age of onset, sex, result of renal biopsy, treatment method, its results of treatment, its responsiveness, time of response to treatment and pattern of relapse in Korea. Methods: Between 1987 and 1997, 2193 patients with primary nephrotic syndrome diagnosed at 38 university hospital and general hospital in Korea were included. Of these 1655 were male and 538 were female. Incidence peaked at 1-5 years of age. Results: Results were as follows; 1) Among 2193 cases, male was 1655($75.5\%$), female was 538 cases($24.5\%$) and male to female ratio was about 3:1. Among 1752 patients with MCNS, male was 1338, female was 414 and male to female ratio was about 3.23:1. The most prevalent age group was 1-5 years of age. 2) Renal biopsy was done in 942 cases($43\%$), pathologic findings were as follows; MCNS 646 cases($68.6\%$), FSCS 149 cases($15.8\%$). 3) Regimen of treatment were as follows; prednisolone 1191 cases, Calcort 192 cases, cyclophosphamide 251 cases, cyclosporin A 223 cases, MPD pulse therapy 120 cases.4) Complete response to treatment were noted in 1597 cases($82.2\%$, n=1944). 5) Responsiveness according to result of renal biopsy were significantly different between MCNS and FSGS. Complete response were noted in $86.5\%$ among patients with MCNS, $35.8\%$ in patients with FSGS. 6) Time of response to treatment were noted between 1 and 4 weeks after treatment in 879 cases($67.4\%$, n=1305). 7) 994 cases($73.1\%$) relapsed during follow up, most frequently between 2 months and 6 months after response. Conclusion : Nationwide survey of epidemiological and clinical data were performed in childhood primary nephrotic syndrome. Most of the clinical and epidemiological data were similar to other reports from U.S.A. and from Europe, however male to female ratio is higher in Korean nephrotic syndrome(3:1 in contrast to 2:1).

• Childhood Kidney Diseases
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• v.13 no.1
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• pp.84-91
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• 2009

We experienced a female neonate with congenital nephrotic syndrome (CNS) associated with congenital diaphragmatic hernia (CDH). Because of the rare combination of two conditions, we report this case with literature review. CDH was found immediately after birth and emergency operation was done for hernia repair. But on the next day, generalized edema and oliguria(0.59 mL/kg/hour) was found and her blood chemistry showed hypoalbuminemia (1.6 g/dL), increased BUN (27.7 mg/dL) and serum creatinine( 1.8 mg/dL) along with heavy proteinuria (4+). We started albumin infusion with a bolus of intravenous furosemide. We suspected the neonate had congenital nephrotic syndrome and her 24hr urine protein was 1,816 mg/day. In spite of immunosuppressive therapy, the nephrotic syndrome and renal failure progressed. We started peritoneal dialysis on the day of life 22 but it was not satisfactory. She was complicated by intracranial hemorrhage and multi-organ failure and expired at 34 days of age. Kidney necropsy was performed which showed diffuse mesangial sclerosis (DMS). Her chromosome study revealed 46, XX and her gene study revealed a heterozygous missense mutation, Arg366His, in Wilms tumor suppressor gene (WT1). This case deserves attention on account of the 4th case of CNS with CDH revealing the Arg366His mutation in the WT1 gene and G the 1st case of early onset renal failure without male pseudohermaphroditism and Wilms tumor with CNS, CDH and the Arg366His mutation in the WT1 gene. So, this report gives support to the hypothesis that Arg366His mutation in the WT1 gene can result in CNS and CDH.