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Journal of the Korean Society of Pediatric Nephrology 2009;13(2): 161-169. doi: https://doi.org/10.3339/jkspn.2009.13.2.161
1세 이하의 소아에서 발병한 신증후군의 임상적 고찰
조성희, 이주훈, 조영미, 박영서, 정해일
1울산대학교 의과대학 서울아산병원 소아과학교실
2울산대학교 의과대학 서울아산병원 소아과학교실
3울산대학교 의과대학 서울아산병원 병리학교실
4울산대학교 의과대학 서울아산병원 소아과학교실
5서울대학교 어린이병원 소아청소년과
Clinical Evaluation of Nephrotic Syndrome Manifesting in the First Year of Life
Sung-Hee Cho, Joo-Hoon Lee, Young-Mi Cho, Young-Seo Park, Hae-Il Cheong
1Department of Pediatrics, Asan Medical Center University of Ulsan College of Medicine
2Department of Pediatrics, Asan Medical Center University of Ulsan College of Medicine
3Department of Pathology, Asan Medical Center University of Ulsan College of Medicine
4Department of Pediatrics, Asan Medical Center University of Ulsan College of Medicine
5Department of Pediatrics, Seoul National University Children's Hospital Kidney Research Institute
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ABSTRACT
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.
Key words: Congenital nephrotic syndrome | Infantile nephrotic syndrome
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