Congenital Nephrotic Syndrome With Interstitial Nephritis

THE CONGENITAL nephrotic syndrome is probably a heterologous group from which microcystic disease and membranous glomerulonephritis have been separated as histologic entities. This syndrome has also been reported in association with cytomegalic inclusion disease,¹ renal vein thrombosis,² and congenital syphilis.³ A heredofamilial form is suggested by a number of reports of this syndrome in two or more siblings.^1,4-9 Pyelonephritis in association with the nephrotic syndrome is not well established, particularly in the early phases of the disease, and is not recognized as an etiologic factor.^10,11 This report describes the association of chronic interstitial nephritis with the nephrotic syndrome in an infant with abnormal external genitalia.

Report of a Case  A male infant, weighing 8 lb 7 oz (3,832 gm), was born following a 41-week uncomplicated gestation. His 18-year-old, gravida 1, para 1, mother was in good health and had no known infection or renal disease