Bilateral renal dysplasia in three siblings: report of a survivor

Three siblings, born nonconsectively to an unrelated couple, have had bilateral dysplastic kidneys. The first child had associated skeletal anomalies, the second, ureteral atresia, and the third, no other anomalies. The occurrence of dysplastic kidneys in three siblings is compatible with an autosomal recessive inheritance pattern. In addition the variety of presenting syndromes in the children suggests that variable pathogenetic mechansims were responsible for the appearance of dysplastic kidneys. The third child is alive with kidneys that markedly decreased in size over the neonatal period but is growing well at 41 months of age indicating that the presence of bilateral dysplastic kidneys does not necessarily preclude a favorable prognosis.