Corticoresistant nephrotic syndrome associated with T-cell deficiency in two sisters.
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-mu receptor-bearing 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.
D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000235:genetics / Q000276:immunology; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000235:genetics; D007958:Leukocyte Count; D008231:Lymphopenia / Q000235:genetics; D009404:Nephrotic Syndrome / Q000188:drug therapy / Q000235:genetics* / Q000276:immunology; D011241:Prednisone / Q000627:therapeutic use; D012397:Rosette Formation; D013601:T-Lymphocytes / Q000276:immunology*
D011241:Prednisone