PEDIATRICS Vol. 71 No. 1 January 1983, pp. 93-96
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Corticoresistant Nephrotic Syndrome Associated with T-Cell Deficiency in Two Sisters

René Tabin MD1, Jean-Pierre Guignard MD1, Emile Gautier MD1, Micheline Dubrit MD1, Michel Jeannet MD1, Jean Golaz MD1, and Lorenzo Moretta MD1

1 From the Department of Pediatrics, CHUV, Lausanne; Immunology Transplant Unit, University Hospital, Geneva; and Ludwig Institute for Cancer Research, Lausanne Branch, Epalinges, Switzerland

The coexistence in two sisters, born to related parents, of a corticoresistant nephrotic syndrome, lymphopenia, an immune deficit, short stature, and photophobia is described. The immune deficit is mainly cellular; studies of lymphocyte markers demonstrate a pronounced deficiency of T lymphocytes and Fc-µ receptorbearing cells. It is suggested that a thorough examination of number and function of T cells should be performed in patients with a familial corticoresistant nephrotic syndrome and recurrent infectious episodes before considering immunosuppressive treatment.

Key Words: familial nephrotic syndrome • familial glomerular sclerosis • focal segmental glomerular sclerosis • T-cell deficiency • corticoresistant nephrotic syndrome

Submitted on December 18, 1981
Accepted on March 29, 1982