Nephrotic syndrome under 2 years of age

Ko, Jae Sung; Ahn, Kang Mo; Park, Hye Won; Ha, Il Soo; Cheong, Hae Il; Choi, Yong; Ko, Kwang Wook

Abstract

There had been total 20 patients with early onset(4 months~2 years) primary nephrotic syndrome in the Department 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.