RENAL TUBULAR DYSGENESIS WITH MICROCEPHALY

Renal tubular dysgenesis, a rare, lethal, autosomal recessive disorder , is characterized by short and poorly differentiated proximal convolu ted tubules associated with oligohydramnios, Potter sequence. and neon atal death due to respiratory failure. Abnormalities of the skull may occur in some cases. We report an infant born of a consanguineous unio n who also had microcephaly, among other features, A history of oligoh ydramnios with or without skull abnormalities and a lethal outcome wit hout obvious explanation should occasion renal histological study aime d at precise diagnosis and genetic counselling.