CONGENITAL NEPHROSIS WITH GONADAL DYSGENESIS IN TWINS

Non-Finnish type congenital nephrosis is uncommon and of heterogen ous etiology. We report congenital nephrosis occurring in monozy gotic twin boys. Family history is otherwise negative for renal disease. The parents are not of Finnish origin and there is no consanguinity. The pregnancy was unremarkable and the placenta not enlarged as in the Finnish variety.At 10 weeks of age both boys presented with diarrhea. The first twin died suddenly in acute renal failure in another hospital; the second was transferred to our care. A diagnosis of congenital nephrosis was made on admission. Rapid deterioration in renal and respiratory function ensued and the infant died at 12 weeks of age. Post-mortem examination revealed microcystic renal disease with glomeruli showing variable and segmental mesangial hypercellularity and sclerosis. The microscopic appearances were most consistent with diffuse glomerular mesangial as described by Habib et al. In addition, gonadal dysgenesis was also seen. The outer zone of the testes resembled ovarian stroma with a few immature tubular structures as well as a polyovular follicular appearance. This case represents a rare example of congenital nephrosis associated with abnormal gonadogenesis perhaps best explained by a common insult in embryological development. This association may be representative of the nephropathies previously documented with gonadal dysgenesis and may represent a distinct category of congenital nephrosis.