Resistance to multiple steroids in two sisters

A 14-year-old Native American girl from the Iroquois Nation was referred as a potential patient with the syndrome of Apparent Mineralocorticoid Excess . Instead, her evaluation revealed resistance to glucocorticoids, mineraloc orticoids, and androgens. She lacked Cushingoid features in spite of signif icantly high cortisol levels, Menstruation was regular and there was no cli nical evidence of masculinization despite high serum androgen levels in the male range. The patient's sister had similar clinical features. Partial re sistance to exogenous glucocorticoid and mineralocorticoid administration w as well demonstrated in both patients. It is proposed that these patients r epresent the first cases of partial resistance to multiple steroids, possib ly owing to a coactivator defect. (C) 2001 Elsevier Science Ltd. All rights reserved.