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February 1969

Familial Nephritis Associated With the Nephrotic Syndrome: In a Family With Severe Involvement in Females

Author Affiliations

New York
From the departments of pediatrics (Drs. Albert and Leeming) and pathology (Division of Developmental Pathology) (Dr. Wigger), Columbia University College of Physicians and Surgeons, and Babies Hospital, Columbia-Presbyterian Medical Center, New York. Doctor Albert is now with the Pediatric Service, Walter Reed General Hospital, Washington, DC.

Am J Dis Child. 1969;117(2):153-155. doi:10.1001/archpedi.1969.02100030155006

THE hereditary features of renal disease have engendered considerable interest within the past few years.1,2 Foremost among the hereditary nephropathies has been the syndrome of familial nephritis.2 In the majority of the kindred with this disorder, affected males initially develop gross or microscopic hematuria followed by progressive nephritis which results in death in early adult life. In contrast, affected females manifest various urinary abnormalities but do not usually show deterioration in renal function and have a fairly normal life span. Nerve deafness is a common associated abnormality in some families, while ocular abnormalities, particularly cataracts and spherophakia,3 have been described in others. The ocular abnormalities affect both sexes with equal severity and may or may not be associated with renal disease in any given individual.

The present paper describes an unusual family in which three out of nine siblings two of whom are female, had chronic nephritis.