A Case of Secondary FSGS due to Chronic Chloride DiarrheaA Case of Secondary FSGS due to Chronic Chloride Diarrhea
- Other Titles
- A Case of Secondary FSGS due to Chronic Chloride Diarrhea
- Authors
- 김병관; 이현순; 임형은; 정해일; 유기환
- Issue Date
- 2016
- Publisher
- 대한소아신장학회
- Keywords
- Congenital chloride diarrhea; Renal complication; Focal segmental glomerulosclerosis; Renal dysplasia
- Citation
- Childhood Kidney Diseases, v.20, no.2, pp.83 - 87
- Indexed
- KCI
- Journal Title
- Childhood Kidney Diseases
- Volume
- 20
- Number
- 2
- Start Page
- 83
- End Page
- 87
- URI
- https://scholar.korea.ac.kr/handle/2021.sw.korea/91113
- ISSN
- 2384-0242
- Abstract
- Congenital chloride diarrhea (CLD) is a rare autosomal recessive disease that is dif ficult to diagnose. CLD requires early treatment to correct electrolyte imbalance and alkalosis and to prevent severe dehydration. Renal injury is clearly associated with defective electrolyte balance induced by CLD, particularly during the first months or years of life. A 7-year-old boy was diagnosed with CLD following detec tion of a homozygous mutation (c.2063-1G>T) in SLC26A3 at 6 months of age.
During treatment with electrolyte supplements, mild proteinuria was detected at 8 months of age, and is still present. Renal biopsy showed the presence of focal renal dysplasia, with metaplastic cartilage and mononuclear cell infiltration, calcification, and fibrosis in the interstitium. Up to two-thirds of the glomeruli exhibited global obsolescence, mostly aggregated in the dysplastic area. In nondysplastic areas, the glomeruli were markedly increased in size and severely hyper cellular, with increased mesangial matrix, and displayed segmental sclerosis. The marked glomerular hypertrophy with focal segmental glomerulosclerosis sug gested a compensatory reaction to the severe nephron loss or glomerular obsole scence associated with renal dysplasia, with superimposed by CLD aggravating the tubulointerstitial damage.
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Collections - College of Medicine > Department of Medical Science > 1. Journal Articles
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