• Childhood Kidney Diseases
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• v.15 no.1
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• pp.38-48
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• 2011

Purpose: Focal segmental glomerulosclerosis (FSGS) is the most common glomerulopathy causing pediatric renal failure. Since specific treatment targeting the etiology and pathophysiology of primary FSGS is yet elusive, the authors explored the pathophysiology of FSGS by transcriptome analysis of the disease using an animal model. Methods: FGS/kist strain, a mouse model of primary FSGS, and RFM/kist strain, as control and the parent strain of FGS/kist, were used. Kidney tissues were harvested and isolated renal cortex was used to extract mRNA, which was run on AB 1700 mouse microarray chip after reverse transcription to get the transcriptome profile. Results: Sixty two genes were differentially expressed in FGS/kist kidney tissue compared to the control. Those genes were related to cell cycle/cell death, immune reaction, and lipid metabolism/vasculopathy, and the key molecules of their networks were TNF, IL-6/4, IFN${\gamma}$, TP53, and PPAR${\gamma}$. Conclusion: This study confirmed that renal cell death, immune system activation with subsequent fibrosis, and lipid metabolism-related early vasculopathy were involved in the pathophysiology of FSGS. In addition, the relevance of methodology used in this study, namely transcriptome profiling, and Korean animal model of FGS/kist was validated. Further study would reveal novel pathophysiology of FSGS for new therapeutic targets.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.100-104
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• 2010

We report on two children with a high risk of recurrent focal segmental glomerulosclerosis (FSGS) after renal transplantation that could be effectively prevented by prophylactic administration of cyclosporine combined with preemptive plasmapheresis prior to renal transplantation.

• Clinical and Experimental Pediatrics
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• v.54 no.1
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• pp.36-39
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• 2011

Bartter syndrome (BS) is a clinically and genetically heterogeneous inherited renal tubular disorder characterized by renal salt wasting, hypokalemic metabolic alkalosis and normotensive hyperreninemic hyperaldosteronism. There have been several case reports of BS complicated by focal segmental glomerulosclerosis (FSGS). Here, we have reported the case of a BS patient who developed FSGS and subsequent end-stage renal disease (ESRD) and provided a brief literature review. The patient presented with classic BS at 3 months of age and developed proteinuria at 7 years. Renal biopsy performed at 11 years of age revealed a FSGS perihilar variant. Hemodialysis was initiated at 11 years of age, and kidney transplantation was performed at 16 years of age. The post-transplantation course has been uneventful for more than 3 years with complete disappearance of BS without the recurrence of FSGS. Genetic study revealed a homozygous p.Trp(TGG)610Stop(TGA) mutation in the CLCNKB gene. In summary, BS may be complicated by secondary FSGS due to the adaptive response to chronic salt-losing nephropathy, and FSGS may progress to ESRD in some patients. Renal transplantation in patients with BS and ESRD results in complete remission of BS.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.1-8
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• 2003

Purpose : Focal segmental glomerulosclerosis(FSGS) is known to have a poor prognosis and a poor response to treatment. We performed a combination therapy of plasmapheresis, steroid pulse and immunoglobulin in 4 patients with relapsed steroid dependent(SD) or steroid resistant(SR) FSGS after steroid pulse therapy. Materials and Methods : 8 cycles of plasmapheresis were performed in 4 patients with biopsy proven FSGS who had relapsed after steroid pulse therapy from March 1988 to July 2002. Clinical findings and treatment courses were reviewed retrospectively. Results : Among the 4 patients, there were 3 males and 1 female. After 8 cycles of plasmapheresis, clinical remissions were obtained. Two of the four patients had two relapses and received 2 more cycles of plasmapheresis which resulted in remissions. One of these patients had two further relapses and was treated with oral steroid resulting in clinical remission. Three patients have maintained normal serum creatinine level and glomerular filtration rates during the follow-up period of 10 years, and the other 1 patient for 5 months. Conclusion : A combination therapy of plasmapheresis, steroid pulse and immunoglobulin led to a complete remission in patients with FSGS who were SD or SR and was effective in maintaining normal renal function.

• 대한소아신장학회:학술대회논문집
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• 2007.04a
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• pp.7-8
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• 2007

• 대한소아신장학회:학술대회논문집
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• 2007.04a
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• pp.9-11
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• 2007

• Clinical and Experimental Pediatrics
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• v.60 no.3
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• pp.55-63
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• 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
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• v.3 no.1
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• pp.27-34
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• 1999

Purpose: This study was designed to investigate the changes in soluble interleukin-2 receptor (sIL-2R) level in sera and urines of children with primary nephrotic syndrome, eliminating the confounding effects of age, proteinuria, and steroid treatment. Methods: Soluble IL-2R was measured by ELISA in sera and urines from patients with minimal change nephrotic syndrome or focal segmental glomerulosclerosis as well as from healthy controls. The serum levels and urinary sIL-2R/creatinine ratios were compared between control group and the 12 patient groups divided by their ages (0-1, 2-4, over 5 years), and presence or absence of proteinuria and/or steroid treatment (PU+Tx-, PU+Tx+, PU-Tx+, PU-Tx-). Results: Though the differences were not statistically significant probably because of the small numbers, serum sIL-2R levels seemed to be higher in younger age groups both in patients and control group. Nephrotic children did not show higher serum levels than normal children. Among the patients, proteinuric condition seemed to raise and steroid treatment tended to suppress the serum sIL-2R levels. Urinary sIL-2R/creatinine ratios were higher in younger age groups, more significantly in patients (P<0.001). Proteinuria and steroid treatment affected the urinary sIL-2R/creatinine ratios by the same way as the serum sIL-2R levels. Serum sIL-2R levels and urinary sIL-2R/creatinine ratios were not different between groups of different histologic findings or steroid responsiveness (P>0.05). Conclusion: Serum sIL-2R levels and the urinary sIL-2R/creatinine ratios were higher in younger age, and they were not higher in nephrotic patients compared to control group. The patients in relapse showed higher levels, while the levels were suppressed with steroid treatment. In proteinuric state, urinary sIL-2R/creatinine ratios reflected serum sIL-2R levels.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.94-99
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• 2010

Obesity-related glomerulopathy (ORG) is a secondary form of focal and segmental glomerulosclerosis (FSGS) manifesting as proteinuria and progressive renal dysfunction that results from maladaptive glomerular response to increasing adiposity. Reports of ORG progressing to end stage renal diseases in rare in the pediatric population. We report a 9-year-old boy with obesity (body mass index $35\;kg/m^2$) who was diagnosed with ORG presenting with proteinuria. He was diagnosed with obesity-related glomerulopathy based on the laboratory, urinary, and kidney biopsy finding. In spite of treatment with angiotensin- converting enzyme (ACE) inhibitor and/or, angiotensin-receptor blocking agent, the degree or amount of proteinuria increased and renal function declined continuously. His BMI did not decrease and eventually progressed to chronic renal failure. Consequently, obese patients should be monitored for proteinuria, which may be the first manifestation of FSGS, a lesion that may be associated with serious renal sequelae.

• Journal of Yeungnam Medical Science
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• v.30 no.2
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• pp.149-151
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• 2013

Kimura's disease is an angiolymphoid-proliferative disorder that manifests with benign subcutaneous swelling predominantly in the head and the neck. Kidney involvement, including proteinuria, occurs in 12-16% of patients with the disease, and 60-78% of such cases is nephrotic syndrome. Reported etiologies of nephrotic syndrome in Kimura's disease include membranous glomerulonephritis, mesangial proliferative glomerulonephritis, minimal-change disease, focal segmental glomerulosclerosis, diffuse proliferative glomerulonephritis and immunoglobulin A (IgA) nephropathy. There have been only two case reports of IgA nephropathy in Kimura's disease, in 1998. In this report, we present a third case of IgA nephropathy associated with Kimura's disease.