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Prof. Emilio Bucciarelli, Istituto di Anatomia e Istologia, Patologica Policlinico Monteluce, PO Box 54, I-06100 Perugia (Italy) Dear Sir, Congenital nephrotic syndrome of the Finnish type (CNS) is a rare recessively inherited disorder with invariably fatal outcome in infancy [1]. CNS was initially observed in Finland, where it remains most common; however, familial and sporadic cases have been described in many countries and in a variety of ethnic groups [2]. We observed a 4-month-old white male infant admitted for evaluation and treatment of nephrotic syndrome. He was the first child of healthy and not consanguineous parents of Italian ancestry. Gestation and delivery were uncomplicated. The placenta was regarded as ‘voluminous’ and birth weight of the patient was 4 kg. A cousin died at 18 months of age because of nephrotic syndrome sustained by mesangial proliferative glomeru-lonephritis. At 3 months of age the patient suffered from vomiting and eyelid edema. During the next 4 weeks he developed scrotal edema and was admitted to hospital. Physical examination revealed a male infant with anasarca and a poor general condition. Urinalysis showed heavy pro-teinuria (10 g/l). Total serum proteins were 4.16 g/l00 ml and albumin 1.90 g/l00 ml. Blood nitrogen was 10 mg/l00 ml and creatinine 0.55 mg/l00 ml, total lipids 1,190 mg/ 100 ml and cholesterol 292 mg/l00 ml. Electrolytes and C3 were normal. VDRL was nonreactive. Histologic examination of a surgical renal biopsy revealed numerous dilated tubules with microcystic appearance prevalently at the corticomedullary junction. Glomeruli showed a wide spectrum of appearance. The majority were normal in size with mild mesangial hypercellularity accompanied by increase of the matrix (fig. 1). In addition some glomeruli showed focal to global sclerosis (fig. 2) or extracapillary cell proliferation. Fetal glomeruli were scanty. Immunofluorescence studies of glomeruli revealed irregular deposits of C3 and Clq. Electron micros-