• Childhood Kidney Diseases
• /
• v.23 no.1
• /
• pp.7-21
• /
• 2019

Nephrotic syndrome (NS) is a common chronic glomerular disease in children characterized by significant proteinuria with resulting hypoalbuminemia, edema, and hyperlipidemia. Renal biopsy findings of diffuse foot processes effacement on electron microscopy and minimal change disease, focal segmental glomerulosclerosis (FSGS), or diffuse mesangial proliferation on light microscopy. It has been speculated that circulating permeability factors would be implicated in the pathogenesis of NS because they have been reportedly detected in the sera of patients and in experimental models of induced proteinuria. Moreover, a substantial portion of the patients with primary FSGS recurrence shortly after transplantation. This report reviews the current knowledge regarding the role of circulating permeability factors in the pathogenesis of proteinuria in NS and suggests future targeted therapeutic approaches for NS.

• Childhood Kidney Diseases
• /
• v.26 no.1
• /
• pp.25-30
• /
• 2022

Dyslipidemia in nephrotic syndrome (NS) is often characterized by marked increases in the levels of total cholesterol, triglycerides, low-density lipoprotein cholesterol, and other lipoproteins, such as very low-density lipoprotein, intermediate-density lipoprotein, and lipoprotein(a). It has been suggested that impaired catabolism of lipoproteins and cholesterol is mainly due to decreased lipoprotein lipase and hepatic lipase activity, and increased biosynthesis of lipoproteins in the liver. The management strategies for dyslipidemia in patients with NS consist of lifestyle modification, lipid-lowering agents represented by statins, second-line agents such as fibrates and bile acid sequestrants, and lipid apheresis. Compared with dyslipidemia in adult NS patients, whose risks of atherosclerotic disease and progressive renal injury are considered high, clinical data on dyslipidemia in pediatric NS patients are limited. Therefore, it is necessary to pay more attention to the evaluation and management of dyslipidemia in pediatric patients with NS in clinical practice.

• Child Health Nursing Research
• /
• v.3 no.1
• /
• pp.30-41
• /
• 1997

This is descripitive study conducted to identify educational needs of mothers of nephrotic syndrome patients. The study subjects were composed of 74 mothers of nephrotic syndrome patients whose children were hospitalized in 2 Pediatric wards of University Hospital in Seoul and 1 in Pusan from June to september in 1996. A questionaire for this study was item Kikert type 5 point scale, developed on the basis of previous literature and researcher's clinical experience and the reliability of the used instruments was α=.97. t-test, and ANOVA were used to determine the effect of general characteristics of subjects on their educational needs. Pearson correlation was done to measure relations between general characterictics of subjects and their educational needs and Stepwise Multiple Regression was done to test a variable affecting educational needs. The results were as follows. 1. Mean score of the educational needs of the subjects was 137.06(Maximum 176) . The educational needs of home care was the highest score, but the question numbers are smaller than other categories. So, the educational need of the diagnosis and treatment was regarded as the highest in contents. 2. The number of subject's children, except for patient revealed significant negative correlation to educational need. 3. The number of subject's children, except for patient(R²=.215289 P=.0006)and the age of patient (R²=.23770 P=.0001) were emerged as important variables affecting the degree of mothers' educational need. Suggestion are as follows on the basis of these results. 1. It is proposed that nurses use these results of the study activly for the educational program for Nephrotic Syndrome patients and their mothers. 2. It is identified that the educational needs of the mothers of nephrotic syndrom is high. So, it is suggested to identify and analysis the degree of the nurse's educational performance perceived by mother. 3. It is suggested to make a comparative study of the degree of nurse's understanding of the importance on educational items with the instruments of this study.

• Childhood Kidney Diseases
• /
• v.10 no.2
• /
• pp.162-173
• /
• 2006

Purpose : To report the decreasing indicence of HBV(Hepatitis B virus)-associated membranous nephropathy in children after HBV vaccination and to elucidate the clinical course and treatment strategies of IMN(Idiopathic membranous nephropathy). Methods : We retrospectively reviewed the clinico-pathological findings of HBV-MN and IMN patients who underwent a renal biopsy from 1986 to 2005. We compared the HBV-MN and the IMN groups and the remission and the non-remission groups of patients with IMN. Results : Among 24 cases of MN patients, HBV-MN comprised 6 cases(25%) and IMN 18 cases(75%). Clinical masnifestations were nephrotic syndrome(3 cases, 50%), nephritic syndrome(1 case, 16.7%), asymptomatic(2 cases, 33.4%) in the HBV-MN group, asymptomatic(10 cases, 55.5%), nephrotic syndrome(5 cases, 27.8%), and gross hematuria(3 cases, 16.7%) in the IMN groups. From 1996 to 2000, there were 2 cases(28%) of HBV-MN and 5 cases(72%) of IMN. After 2001 all 10 cases were IMN. In the HBV-MN group, 4 cases(66.7%) received interferon and 1 cases received methylprednisolone pulse therapy. In the IMN group, 16 cases(88.9%) received methylprednisolone, 8 cases(44.4%) were in complete remission, 2 cases(11.1%) were in partial remission, 2 cases(11.1%) were in chronic renal failure, and 5 cases(27.8%) were lost to follow-up with sustained proteinuria, 1 case(5.6%) continued to have frequent relapse of nephrotic syndrome without renal insufficiency. In the comparison between remission and non-remission groups, nephrotic range proteinuria and hypertension were more significantly common in the non-remission group(P<0.05). Conclusion : With HBV vaccination, HBV-MN has decreased markedly. IMN is a rare glomerular disease in children. Because the prognosis for patients with nephrotic range proteinuria is poor this group needs more aggressive treatment.

• Childhood Kidney Diseases
• /
• v.16 no.1
• /
• pp.46-50
• /
• 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
• /
• v.18 no.2
• /
• pp.123-127
• /
• 2014

Erythema multiforme (EM) is an acute mucocutaneous disorder involving the skin, mouth, eyes, and genital organs. It is classified into EM minor and EM major according to the involvement of the mucosal membrane. Stevens-Johnson syndrome (SJS) and toxic epidermal necrosis (TEN) belong to EM major. Compared to EM minor, SJS presents with more severe and progressive symptoms, and has a higher mortality rate. Corticosteroids are used in the treatment of EM. We report three cases of EM (two cases of EM minor and one case of SJS) that developed during treatment with oral corticosteroid (deflazacort; $Calcort^{(R)}$) in children with nephrotic syndrome.

• Tuberculosis and Respiratory Diseases
• /
• v.52 no.6
• /
• pp.633-639
• /
• 2002

Paraneoplastic nephrotic syndrome can be diagnosed from its clinical and immunological features. The development of several types of glomerular injury in patients with cancer have been recognized, and are considered as paraneoplastic syndrome. Most prominent are the occurrence of membranous glomerulonephritis in patients with carcinomas. We report a case of a 60-year-old-man with small cell lung cancer presenting as nephrotic syndrome. A renal biopsy revealed membranous glomerulonephritis. Six lots of chemotherapy were administered, which led to a complete tumor response with total resolution of the nephrotic syndrome following treatment.

• Journal of Yeungnam Medical Science
• /
• v.9 no.2
• /
• pp.427-435
• /
• 1992

We have experienced a case of infantile nephrotic syndrome confirmed by renal biopsy in a 13-month-old female patient who showed growth and develop mental retardation and persistent proteinuria. She revealed mild eyelid edema, joint laxity, delayed speech development and adrenal cortical calcification on the radiologic study. Renal biopsy showed microcystic tubular change, micro-glomeruli and marked mesangial proliferation.

• Childhood Kidney Diseases
• /
• v.2 no.2
• /
• pp.183-186
• /
• 1998

Steroid Resistant Nephrotic Syndrome(SRNS) in children has poor prognosis and no effective therapy. In 1994, Ravi Elhence have reported that IV cyclophosphamide therapy was effective against SRNS of children. So, we evaluated the efficacy of IV cyclophosphamide in 3 children with biopsy proven steroid-resistant MCNS. And the result was the rapeutic failure. In conclusion, IV cyclophosphamide therapy wass not effective against SRNS of children.

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