This is the first case report of oligomeganephronia in Korean literature. Cligomeganephronia is a congenital form of bilateral renal hypoplasia characterized histologically by reduction in 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 slowlv progresive renal failure, accompanied failure to thrive, short stature,
and
reanl
osteodystrphy. But only a few patients are incidentally 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-stagte renal disease. She
underwent hemodialysis, and 6 months lat4r 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 oligmoegtanephronia with a
review
of the literatures.
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