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February 1979

An Unusual Variation of Familial Nephrosis

Author Affiliations

Children's Hospital of Philadelphia 34th Street and Civic Center Boulevard Philadelphia, PA 19104

Am J Dis Child. 1979;133(2):216-217. doi:10.1001/archpedi.1979.02130020108022

This is a report of an unusual occurrence of cases of nephrotic syndrome in a family in which a mother, her brother, and her child had the steroidsensitive relapsing form of the disease. The nephrotic syndrome in children is usually idiopathic and rarely familial. Its peak incidence is in children ages 1 to 6 years. It generally follows a relapsing course, but is steroid sensitive and demonstrates minimal glomerular changes on pathologic study. It is very unusual to see a familial pattern to the disease, except in the rare autosomal recessive syndrome or in association with certain systemic metabolic disorders of which the nephrotic syndrome is only one component.

Report of Cases.—Case 1.—Nephrosis developed in the index patient at age 8 years. Her clinical course and laboratory values were consistent with the diagnosis of idiopathic nephrotic syndrome of childhood. She has always been steroid sensitive. A needle biopsy