Journal of the Korean Pediatric Society 1981;24(6):535-548.
Published online June 15, 1981.
Clinical Observation in Children with Relapsing Minimal Change Nephrotic Syndrome.
Hae II Cheong, Kwang Wook Ko
Department of Pediatrics, College of Medicine, Seoul National University, Korea.
再發을 反復한 Minimal Change 腎症候群 患兒에 對한 臨床的 考察
丁海日, 高光昱
서울大學校 醫科大學 小兒科學敎室
Abstract
The clinical findings and follow-up data of 37 children with relapsing minimal change nephrotic syndrome were analyzed retrospectively. Among total 37 cases, 31 were male, and 6 were female. Male to female ratio was 5.2:1. All cases were admitted to the Pediatric Ward of Seoul National University Hospital more than one time, and percutaneous kidney needle biopsy which was performed during admission revealed minimal change lesion.(Kidney biopsy was done in cases with unfavorable response to initial conventional steroid therapy and/or with rather frequent relapses.) Mean age at onset was 6 5/12 years, and mean period of follow-up, 3 8/12 years. All of the patients were divided into "Initial Responder"(36 cases, 97.3%) and "Initial Nonresponder"1 case, 2.7%) according to response to steroid therapy and mode of relapse. And, the former group was subdivided into "No Relapser"(1 case, 2.7%, in this case, though no relapse was happened, persistent residual proteinuria after initial steroid therapy led to renal biopsy), "Infrequent Relapser"(18 cases, 48.6%), "Frequent Relapser"(13 cases, 35.1%), and "Subsequent Nonresponder"(4 cases, 10.8%), and the latter group, into "Late Responder"(no case) and "Continuing Nonresponder"(1case, 2.7%). In all cases, as initial symptoms, preceding URI history was detected in 32.4%, and hypertension, hematuria, and azotemia were noted in 13.5%, 10.8%, and 13.5%, respectively. Past medical history of allergy and increased serum IgE level which were suggestive of the parts of evidences for immunopathogenesis of minimal change nephrotic syndrome were found in 29.7% and 35.3%, respectively. Measuring serum levels of other immunoglobulins, IgG had a tendency of decrease, IgM, increase, IgA, variable, and IgD, normal. Among precipitating factors of relapses, URI was found most frequently(15.9%), and in 76.5% of episodes, no precipitating factor was found. Several different findings between in "Infrequent Relapser"(and in "Frequent Relapser"were observed as follows. As for age at onset, in "Infrequent Relapser" the distribution of age was diffusely scattered between 2 to 13 years, while in "Frequent Relapser" its distribution showed a decreasing tendency after initial peak incidence at 3~4 years of age. In "Frequent Relapser"(first relapse was occurred within 3 months after onset in 84.6% of cases, and within 6 months, in all, And in "Infrequent Relapser" first relapse was happened within 3 months after onset in 27.8%, within 6 months in 55.6%, and within 12 months in 94.4% of cases. In most patients of both patient groups, diuresis was initiated within 1 week after the beginning of initial steroid therapy, and loss of proteinuria was achieved within 2 weeks, which were sonewhat faster in "Infrequent Relapser"and 8 cases of "Frequent Relapser" who were followed up more than 3 years, about a half of "Infrequent Relapser" and about three fourths of "Frequent Relapser" were in active disease process with relapse(s) in the third year of follow-up. In 1 case of "Frequent Relapser" and 1 case of "Subsequent Nonresponder", cytoxan therapy was tried with good response.In most cases, several side effects due to long-term steroid therapy were discovered, but all were mild and reversible except 1 case of cataract. Through total period of follow-up in all cases, mortality was absent.


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