• Childhood Kidney Diseases
• /
• v.2 no.1
• /
• pp.73-76
• /
• 1998

Behcet syndrome is a multisystem disease complex of unknown cause. It is usually manifested by aphthous oral and genital ulcers, uveitis and skin eruption. Less frequently, CNS involvement, colitis, large vessel vasculitis, and myocarditis occur. Recently, several studies have reported renal manifestations of amyloidosis and focal necrotizing glomerulonephritis in Behcet syndrome. We describe a patient with Behcet syndrome who experienced nephrotic syndrome with focal effacement of epithelial foot process in glomeruli. A brief review of literature ensues.

• Childhood Kidney Diseases
• /
• v.9 no.2
• /
• pp.245-250
• /
• 2005

The syndrome of reversible posterior leukoencephalopathy syndrome(RPLS) is characterized clinically by acute neurologic signs such as headache, vomiting, confusion, seizures, and visual abnormalities. Radiologically, abnormalities consistent with reversible white matter edema in the occipital and parietal lobes are characteristic. RPLS has often been associated with various systemic disorders, such as hypertensive encefhalopathy, eclampsia, and the use of intravenous or intrathecal immunosuppressive drugs. We report a case of RPLS that occurred after intravenous steroid pulse therapy and treatment with oral cyclophosphamide in a child with nephrotic syndrome, and we emphasize the importance of early recognition of RPLS in the treatment of nephrotic syndrome and appropriate management tn prevent Permanent neurologic disability. (J Korean Soc Pediatr Nephrol 2005;9:245-250)

• Journal of Yeungnam Medical Science
• /
• v.4 no.2
• /
• pp.179-184
• /
• 1987

Renal vein thrombosis is usually a complication of multiple underlying renal disease rather than primary process. High incidence of renal vein thrombosis in patients with nephrotic syndrome, which suggest the nephrotic syndrome play a paramount role in the genesis of renal vein thrombosis or thromboembolic phenomena. But these are likely to relationship of "egg and chicken", and then we cannot determine what is primary of these. Recently authors experienced a case that was questioned renal vein thrombosis with nephrotic-syndrome clinically, laboratory and preliminary radiologically, and this case is confirmed by selective left renal venography. Here we report a case of renal vein thrombosis with nephrotic syndrome which successfully managed with oral anticoagulants and reviewed literatures.

• Childhood Kidney Diseases
• /
• v.7 no.1
• /
• pp.91-95
• /
• 2003

The nephrotic syndrome is characterized by generalized edema, hypoproteinemia(<2 g/dL), proteinuria(>40 $mg/m^2/hr$), and hypercholesterolemia(>200 mg/dL). It is reported that hypoalbuminemia, which is one of the four diagnostic criteria of the nephrotic syndrome, is associated with gallbladder wall thickening. An explanation for the thickened wall in hypoalbuminemic states is the accumulation of fluid in the subserosal layer of the gallbladder wall which contains the most areolar tissue. This report describes a patient who was initially diagnosed with the nephrotic syndrome at the age of 4 and subsequently developed acute acalculous cholecystitis at the age of 5.8 with an albumin level of 1.3 g/dL. The patient responded to fluid therapy, nasogastric suction, and broad spectrum antibiotics.

• Childhood Kidney Diseases
• /
• v.23 no.2
• /
• pp.86-92
• /
• 2019

Steroid-resistant nephrotic syndrome (SRNS) has long been a challenge for clinicians due to its poor responsiveness to immunosuppressants, and rapid progression to end-stage renal disease. Identifying a monogenic cause for SRNS may lead to a better understanding of podocyte structure and function in the glomerular filtration barrier. This review focuses on genes associated with slit diaphragm, actin cytoskeleton, transcription factors, nucleus, glomerular basement membrane, mitochondria, and other proteins that affect podocyte biology.

• The Journal of Korean Obstetrics and Gynecology
• /
• v.34 no.4
• /
• pp.1-11
• /
• 2021

Objectives: Nephrotic syndrome is a kidney disorder, which is characterized by proteinuria, edema (swelling), and hyperlipidemia. Maydis Stigma (Corn silk) has been widely used in Asia as a traditional medicine and is known to have a diuretic effect and is used for the treatment of edema and indigestion. Methods: The aim of this study is to investigate the improvement effect of Maydis Stigma in treating nephrotic syndrome induced by puromycin aminonucleoside. Sprague-Dawley rats were intravenously injected with 75 mg/kg/day puromycin aminonucleoside, then treated with either Losartan or 200 mg/kg/day Maydis Stigma for seven days. Results: Maydis Stigma significantly decreased ascites and proteinuria level. Plasma levels of blood urea nitrogen (BUN) and plasma creatinine reduced significantly by Maydis Stigma. In addition, treatment with Maydis Stigma attenuated histological damage. Treatment with Maydis Stigma also restored podocin expression and reduced inflammation markers such as intracellular adhesion molecules (ICAM-1), monocyte chemotactic protein-1 (MCP-1), tumor necrosis factor alpha (TNF-α) and high-mobility group box-1 (HMGB1). Conclusions: Maydis Stigma ameliorates kidney injury in nephrotic syndrome rat models. Maydis Stigma exerts a renoprotective effect owing to its anti-inflammatory effects and reductions of ascites and proteinuria. Thus, these results indicate that Maydis Stigma is likely to be a promising agent in the treatment of nephrotic syndrome.

• Clinical and Experimental Pediatrics
• /
• v.60 no.3
• /
• pp.55-63
• /
• 2017

Advances in podocytology and genetic techniques have expanded our understanding of the pathogenesis of hereditary steroid-resistant nephrotic syndrome (SRNS). In the past 20 years, over 45 genetic mutations have been identified in patients with hereditary SRNS. Genetic mutations on structural and functional molecules in podocytes can lead to serious injury in the podocytes themselves and in adjacent structures, causing sclerotic lesions such as focal segmental glomerulosclerosis or diffuse mesangial sclerosis. This paper provides an update on the current knowledge of podocyte genes involved in the development of hereditary nephrotic syndrome and, thereby, reviews genotype-phenotype correlations to propose an approach for appropriate mutational screening based on clinical aspects.

• Childhood Kidney Diseases
• /
• v.9 no.1
• /
• pp.91-96
• /
• 2005

Cyclosporin A-induced central neurotoxicity has been rarely reported in patients with nephrotic syndrome. We report a pediatric patient who developed acute leukoencephalopathy diagnosed by MRI during CsA therapy for nephrotic syndrome.

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

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