• Childhood Kidney Diseases
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• v.5 no.2
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• pp.87-99
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• 2001

Purpose: We studied to find out apo-E genotype polymorphism in minimal change nephrotic syndrome(MCNS) and IgA nephropathy(IgAN) and to determine the relationship between apo-E genotype and clinical course of MCNS. Materials and Method: 43 MCNS patients and 15 IgAN patients were examined for apo-E polymorphism. 50 healthy blood donors were examined for apo-E genotype as control. Genomic DNA was prepared front peripheral blood leukocytes according to standard procedures. Results: As compared with control group, e4 allele frequency was significantly increased in MCNS (p<0.01). However, in IgAN e2 allele frequency, however, was 2.6 times higher than normal control (P<0.01). The frequency of e4 allele of frequent relapser group was 4.6 times higher than normal control and was 2 times higher than infrequent relapser group. Conclusion: We think that apo-E typing might be one of the parameters, which should be considered to predict the course of MCNS in children. MCNS with risky HLA profile and E4/4 genotype could indicate the need for a longer steroid administration. And apo-E genotype needs to be considered for the evaluation of therapeutic responses to other drugs. (J, Korean Soc Pediatr Nephrol 2001 ; 5 : 87-99)

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.145-152
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• 1999

Purpose: The prevalence of childhood renal diseases including the nephrotic syndrome in Korea has not been well established. In priority to this point, we analyzed ninety-six nephrotic children of the annual incidence, the ratio of them to all inpatients and estimated incidence of childhood nephrotic syndrome under 15 years of age in Taejon, Korea. Also we classified them by clinical and histopathological findings. Methods: we analyzed the admission and outpatient records retrospectively between June 1986 and December 1998. For the estimation of incidence, we were assisted with three other general hospitals in Taejon Results: The mean annual number and the ratio to inpatients were $7.4{\pm}2.1,\;0.3{\pm}0.11%$ respectively. The latter showed a tendency to decrease during the recent three years. The estimated annual incidences of childhood nephrotic syndrome in Taejon were 5.6 in 1988, 5.5 in 1993 and 4.8 in 1998 per 100,000 for children aged up to 15 years. The mean age of our patients was $6.8{\pm}3.5$ tears, 1-7 years of age was 55 cases(57.3%), and male to female ratio was 3.6:1. By the clinicopathologic classification, 89 cases(92.7%) were classified as primary nephrotic syndrome, and 7 cases(7.3%) as secondary nephrotic syndrome. Among the primary nephrotic syndrome, there was 79.8% of minimal change nephrotic syndrome, focal segmental glomerulosclerosis 11.2%, mesangial proliferation 4.5%, membranoproliferative glomerulonephritis 3.4%, and membranous nephropathy 1.1%. $Henoch-Sch\"{o}nlein$ nephritis was the most frequent of 3 cases in the secondary nephrotic syndrome. Respones to steroid therapy of 71 cases minimal change were classified as non-relapse 22.5%, non-frequent relapse 49.3%, frequent relapse 18.3% and steroid-dependence 9.9%. Conclusion: The estimated incidence of childhood nephrotic syndrome was about 5 per 100,000 for children aged up to 15 years and it showed little changes during 10 years in Taejon, Korea. Our results of clinicopathologic study was little difference from the results reported in other literatures.

• Clinical and Experimental Pediatrics
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• v.52 no.11
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• pp.1260-1266
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• 2009

Purpose : Idiopathic nephrotic syndrome (NS) can be clinically classified as steroid-sensitive and steroid-resistant. The detailed mechanism of glucocorticoid action in NS is currently unknown. Methods : In this study, we investigated 3 known single nucleotide polymorphisms (SNPs) (ER22/23EK, N363S, and BclI) of the glucocorticoid receptor gene (the NR3C1 gene) in 190 children with NS using polymerase chain reaction-restriction fragment length polymorphism and analyzed the correlation between the genotypes and clinicopathologic features of the patients. Results : Eighty patients (42.1%) were initial steroid nonresponders, of which 31 (16.3% of the total) developed end-stage renal disease during follow-up. Renal biopsy findings of 133 patients were available, of which 36 (31.9%) showed minimal changes in NS and 77 (68.1%) had focal segmental glomerulosclerosis. The distribution of the BclI genotypes was comparable between the patient and control groups, and the G allele frequencies in both the groups were almost the same. The ER22/23EK and N363S genotypes were homogenous as ER/ER and NN, respectively, in all the patients and in 100 control subjects. The BclI genotype showed no correlation with the NS onset age, initial steroid responsiveness, renal pathologic findings, or progression to end-stage renal disease. Conclusion : These data suggested that the ER22/23EK, N363S, and BclI SNPs in the NR3C1 gene do not affect the development of NS, initial steroid responsiveness, renal pathologic lesion, and progression to end-stage renal disease in Korean children with NS.

• 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.5 no.2
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• pp.117-124
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• 2001

Purpose : Since Mendoza(1990)'s report that long term methylprednisolone pulse therapy by Mendoza protocol (MP therapy) is a good treatment option in focal segmental glomerulosclerosis(FSGS), there have been reports of the effects of this therapy in steroid-resistant nephrotic syndrome. However, no studies have been performed on the effects of MP therapy in steroid- dependent nephrotic syndrome and secondary nephrotic syndrome. In this study, we investigated the effects of long term MP therapy in primary and secondary nephrotic syndrome in which previous treatment options were not effective. Methods : We chose 10 children who were diagnosed with steroid-dependent minimal change nephrotic syndrome(SD-MCNS), who had shown frequent relapse during the immunocompromised or cytotoxic therapy Period, and 6 children with FSGS and 5 children with secondary nephrotic syndrome children, who had shown no response during the previous therapy period. We treated these patients according to Mendoza protocol involving infusions of high doses of methylprednisolone, often in combination with oral cyclophosphamide for 82 weeks. Results : In all the 10 children with SD-MCNS, complete remission was visible on average of $18{\pm}9$ days after MP therapy was started. However, all these children relapsed during or after MP therapy. In these children, the mean relapse rate prior to MP therapy was $2.1{\pm}1.0$ relpases/year, which was reduced to $1.4{\pm}0.9$ relapses/year during MP therapy(P>0.05) and rose to $2.7{\pm}1.0$ relapse/year after MP therapy. Of the 6 children with FSGS, 4 children($67\%$) showed complete remission, of whom 3 children($50\%$) remained in the remission status during the follow up period, $1.2{\pm}0.7$ years, after the end of MP therapy. 2 children($33\%$) showed no response. All of the 5 children with secondary nephrotic syndrome showed remission and remained in the remissiom status during the follow up period, $1.7{\pm}0.6$ years The only side effect of MP therapy was transient hypertension in 10 children of ail subjects during the intravenous infusion of methylprednisolone. Conclusion : We conclude that although long term MP therapy is not effective in the treatment of SD-MCNS, it is an effective therapy against intractable FSGS and secondary nephrotic syndrome. (J Korean Soc Pediatr Nephrol 2001 ; 5 : 117-24)

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.161-169
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• 2009

Purpose : This study was performed to report the diagnosis and treatment of nephrotic syndrome manifesting in the first year of life. Methods : We retrospectively reviewed the clinical data with chart review in 7 patients who were diagnosed as nephrotic syndrome manifesting in the first year of life from 1996 to 2007. Results : Three patients had congenital nephrotic syndrome, the other 4 patients had infantile nephrotic syndrome. Their ages ranged from birth to 11 months and male to female ratio was 1 to 6. Renal biopsies were done in 6 patients. One patient had Finnish type congenital nephrotic syndrome, 2 patients had diffuse mesangial sclerosis, 2 patients had focal segmental glomerulosclerosis and 1 patient had minimal change disease. Genetic analyses of NPHS2, PLCE1, and WT1 were done in 4 patients and 2 of them had WT1 mutation. Among 3 patients with congenital nephrotic syndrome, 1 patient was diagnosed as congenital nephrotic syndrome of Finnish type and the other 2 patients were diagnosed as Denys-Drash syndrome. All of the patients with congenital nephrotic syndrome died due to sepsis. Among 4 patients with infantile nephrotic syndrome, 2 patients died and 1 had remission, another patient progressed to end stage renal disease. Conclusion : Most of nephrotic syndrome manifesting in the first year was hereditary renal disease. Patients with nephrotic syndrome manifesting in the 3 month of life had poorer prognosis and needed more aggressive management including early dialysis and renal transplantation might be considered compared with infantile nephrotic syndrome. Further genotype-phenotype correlation studies are needed.

• Childhood Kidney Diseases
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• v.18 no.1
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• pp.47-50
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• 2014

Infection is the most important cause of death in children with nephrotic syndrome. Influenza viral infections can be fatal for these children, given the annual outbreak of this virus, with the mortality rate being similar to that of respiratory syncytial virus in healthy children. Pneumonia is recognized as the most important complication of influenza infections, as it is associated with high death rates. However, the influenza vaccine, as well as antiviral agents, can be used for prevention and treatment. Therefore, aggressive management with influenza vaccination and antiviral agents will lower the overall mortality rate in children with nephrotic syndrome. Here we report the case of a 7-year-old boy with nephrotic syndrome and influenza A virus (H1N1) pneumonia.

• Journal of Veterinary Clinics
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• v.20 no.4
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• pp.431-436
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• 2003

The purpose of this study is to evaluate urine protein-to-creatinine ratio as a parameter for early detection of nephrotic syndrome and as a parameter for monitoring effectiveness in early course of treatment. Nine healthy dogs were sensitized by intravenous injection with 1 $\mu$g of endotoxin and 5 mg of native bovine serum albumin. After 1 week, 120 mg of cationized bovine serum albumin was injected intravenously 5 times a week. Among nine dogs, five dogs were confirmed as having developed glomerulonephritis and nephrotic syndrome by increase of urine protein-to-creatinine ratio(>1.0), hypoalbuminemia (<1.5 g/dl), hypercholesterolemia (> 240 mg/dl) and azotemia (BUN>40 mg/dl). During the induction of glomerulonephritis and the progression to nephrotic syndrome, the increase of urine protein-to-creatinine ratio was firstly detected. 1 to 4 weeks later, hypoalbuminemia, hypercholesterolemia, and azotemia were detected. Prednisolone (2.2 mg/kg, bid) was administered orally to the dogs with induced nephrotic syndrome. In early stage of treatment, the increase of serum albumin and decrease of serum cholesterol were detected. 1 to 4 weeks later, decrease of urine protein-to-creatinine ratio was detected. It was concluded that urine protein-to-creatinine ratio is a useful parameter for early detection of nephrotic syndrome, and serum albumin and cholesterol are useful parameters for the monitoring in early course of treatment in nephrotic syndrome.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.197-203
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• 2003

The Galloway-Mowat syndrome, a rare inherited disorder, is characterized by congenital microcephaly with various neurological abnormalities and early onset of nephrotic syndrome with unresponsiveness to treatment, progressive deterioration in renal function and death in early lifetime. In this report, we describe a girl with microcephaly, seizures. and psychomotor retardation who developed nephrotic syndrome at 17 months of age.

• Childhood Kidney Diseases
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• v.8 no.1
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• pp.26-32
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• 2004

Purpose : Hypercoagulability is present in patients with nephrotic syndrome. Plasminogen activator inhibitor type 1(PAI-1) is a major inhibitor of plasminogen activators. PAI-1 inactivates both tissue plasminogen activator(tPA) and urokinase plasminogen activator(uPA) by rapid formation of inactive 1:1 stoichiometric complexes. Recently some studies showed that the enhanced PAI-1 expression may be involved in the intraglomerular fibrinogen/fibrinrelated antigen deposition seen in nephrotic syndrome. Methods : PAI-1 gene promoter -844(G/A) polymorphism was evaluated in 146 children with minimal change nephrotic syndrome(MCNS) and 230 control subjects. The patients with MCNS were subdivided into 85 infrequent-relapser(IR) group and 61 frequent relapser(FR) group. PCR of PAI-1 gene promoter region including -844(G/A) and RFLP using the restriction enzyme Xhol were performed for each DNA samples extracted from the groups. Results : The distribution of PAI-1 genotype in the control group was G/G 81(32.5%), A/A 42(16.9%), and G/A 126(50.6%). The distribution of PAI-1 genotypes in the IR group of MCNS was G/G 29(34.1%), A/A 15(17.7%), and G/A 41(48.2%). The distribution of PAI-1 genotype in the FR group of MCNS was G/G 17(27.9%), A/A 18(29.5%), and G/A 26(42.6%). There was a significantly increased frequency of A/A genotype(P=0.0251) in the FR group of MCNS. Conclusion : Our results indicate that the PAI-1 gene promoter A/A genotype may be associated with the FR in MCNS.