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| 신장이식 수술로 치험한 Oligomeganephronia 1예 |
| 권민중 , 김병길 , 박기일 , 임현이 , 최인준 |
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| Abstract |
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This is the first case report of oligomeganephronia in Korean literature. Oligomeganephronia is a congenital form of bilateral renal hypoplasia characterized histologically by reduction in number and hypertrophy of nephrons. Clinically, this condition is presented in early infancy with vomiting, polyuria, polydipsia, and dehydration. The problems are readily corrected, but there follows a course of slowly progressive renal failure, accompanied failure to thrive, short stature, and reanl osteodystrphy. But only a few patients are inci- dentally detected by urine screening without any early typical symptoms. An open renal biopsy specimen from a fourteen-year- old girl with proteinuria detected in an annual urine screening for school children was studied by light, electron and fluorescent microscopy. Light microscopic findings of renal biopsy showed significant hypertrophy of glomeruli and tubules, marked reduction in number of glomeruli when compared to the controls of the same age. Her symptoms were aggrevated after renal biopsy, and she finally ended up with end-stage renal disease. She underwent hemodialysis, and 6 months later renal transplantation along with bilateral nephrectomy was performed. Up to the present, the general condition of the patient has been relatively good. We report this case of oligomeganephronia with a review of the literatures. |
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