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Journal of the Korean Pediatric Society 1993;36(10):1395-1401.
Published online October 15, 1993.
Nephrotic Syndrome under 2 Years of Age
Jae Sung Ko, Kang Mo Ahn, Hye Won Park, Il Soo Ha, Hae Il Cheong, Yong Choi, Kwang Wook Ko
Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
조기(2세 미만)에 발병한 신증후군
고재성, 안강모, 박혜원, 하일수, 정해일, 최용, 고광욱
서울대학교 의과대학 소아과학교실
Abstract
There had been total 20 patients with early onset (4 months~2 years) primary nephrotic syndrome in the Deparment of Pediatrics, Seoul National University Children's Hospital, during the period from March 1987 to February 1993. We analysed clinical courses, response to treatment, pathological findings and prognosis of the patients And the results were as follows; 1) The initial responders to steroid treatment were 10(50%), of whom 3 became late nonresponders. Of the 10 initial nonresponders, 8 revealed continuing nonresponsiveness. 2) Incidence of hypertension was significantly higher in the intial nonresponders than in the initial responders. 3) Renal biopsies were performed in 9 initial nonresponders and 2 late nonresponders. And the results were 6 with minimal change lesion, 4 with focal segmental glomerulosclerosis and 1 with mesangiocapillary glomerulonephritis. 4) Six continuing or late nonresponders received methylprednisolone pulse therapy or cyclophosphamide. And partial remission was induced in 4 of them. 5) The overall 3 year maintenance rate of normal renal function was 81%(100% in the initial responders and 64% in the initial nonresponders). In conclusion, the initial steroid responsiveness and prognosis were poorer in patients with early onset nephrotic syndrome than in patients with usual childhood nephrotic syndrome.
Key Words: Nephrotic syndrome, Early onset


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