Hereditary chronic nephritis (Alport's syndrome 1 or congenital hereditary hematuria2) is characterized by the familial association of nephropathy, nerve deafness, polyneuropathy, and ocular defects, particularly cataracts, spherophakia, and anterior lenticonus.3 Various combinations of these abnormalities may be present. Most affected males die with renal failure by the age of 30 years; females ordinarily have a mild form of the disease.4 The nephrotic syndrome rarely occurs.3
This paper describes a girl with severe hereditary chronic nephritis complicated by the nephrotic syndrome.
A 13 year-old Latin American girl was admitted to Ireland Army Hospital on May 12, 1965, because of edema of six weeks' duration. There had been a 7 kg increase in weight. History failed to disclose antecedent genitourinary disease or recent respiratory infections.On physical examination there was moderate edema involving the face and lower extremities. The blood pressure was 134/84 mm Hg. No evidence of cardiac decompensation