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KMID : 0367419790200100030
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Journal of Korean Pediatric Society
1979 Volume.20 No. 10 p.30 ~ p.35
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A Case of Congenital Nephrotic Syndrome
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황향옥/Hwang, Hyang Ok
이경숙/신미자/강석철/김기홍/Lee, Kyong Sook/Shin, Mi Ja/Kang, Suk Chul/Kim, Ki Hong
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Abstract
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Nephrotic syndrome occurs infrequently prior to the second year of life. When nephrotic syndrome does develop during the first year, the course differs from that of older children with nephrotic syndrome, being characterized by an extremely poor prognosis and an almost complete refractoriness to therapy. Despite its low incidence congenital nephritic syndrome is important, not only because of the severity and the disorde itself but also because the occurrenze of nephrotic syndrome in this age group raises question regarding the etiology of *he disease.
We experienced one case of congenital nephrotic syndrome which was confirmed by autopsy. The patient was born as a premature infant with body weight 1, 400gm and 37 weeks of gestational age, to a toxemic mother, gravida 3 and parity 3 in Obsteric L,~partment of Seoul Red Cross Hospital. The patient had an uncomplicated nursery staying even though routine weekly urinalysis did show up various degree of preteinuria and micrescopic hematuria without any obvious edema till the age of 50 days with body weight 2, 400gm on the discharge. He was lost to be followed at Out Patient Department until] the age of 4 months when he was brought to admission because of respiratory distress in generalized ed^matous state. He died at 7 months of age foiloing progressive down-hill course, despite treatment with prednisolone and cyc;ophosphamide for 2 months. At autopsy, almost all of the glomeruli (99%) were sclerotic with occassional crescent formation and tubules shcwed microcystic dilatations. It is considered that this case was the first one which was presented on literature in Korea.
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