Congenital Bilateral Oligonephronic Renal Hypoplasia with Hypertrophy of Nephrons (Oligoméganéphronie): Studies by Microdissection

Abstract

The accidental death of a 13-year-old child with the syndrome of congenital bilateral oligonephronic renal hypoplasia with hypertrophy of nephrons (oligoméganéphronie) provided an opportunity for morphologic study of the nephrons in the intermediate stage of this disease. Microdissection by the method of Oliver confirmed the existence of greatly hypertrophied glomeruli and proximal tubules, and permitted study of the abnormal configuration of the nephrons. Anatomic correlates of glomerulotubular balance in the reduced population of nephrons showed a markedly disproportionate enlargement of proximal tubules, relative to glomerular size. A brief description of the clinical and pathologic features of oligoméganéphronie is included in the report.