Familial Renal Tubular Dysgenesis: A Disorder Not Isolated to Proximal Convoluted Tubules

Abstract
The autopsy findings of a newborn with renal tubular dysgenesis, born to first cousins of Moslim Arob descent, are described. Hypocalvaria and hyperflexible joints were noted in addition to the renal lesion. A microdissection study demonstrated marked shortening of all the nephron segments from the glomeruli to the collecting tubules, rather than an isolated abnormality of the proximal convoluted tubules.