MICROCEPHALY AND CONGENITAL NEPHROTIC SYNDROME OWING TO DIFFUSE MESANGIAL SCLEROSIS - AN AUTOSOMAL RECESSIVE SYNDROME

Three sibs born to consanguineous parents had congenital nephrotic syn drome, microcephaly, and psychomotor retardation. Pathology of the kid neys showed diffuse mesangial sclerosis with deposits of IgG and C3 in the mesangium and glomerular basement membranes. All three children d ied before the age of 3 years. Of 19 published cases of children with the association of congenital nephrotic syndrome and microcephaly, onl y four had histological evidence of diffuse mesangial sclerosis, and t wo of their sibs probably had the same disease. The association of nep hrotic syndrome owing to congenital diffuse mesangial sclerosis, micro cephaly, and mental retardation appears to be a distinct syndrome with an autosomal recessive mode of inheritance.