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Resistance to Several Steroids in Two Sisters¹

Department of Pediatrics, New York-Presbyterian Hospital, New York Weill Cornell Center (M.I.N., S.N., S.C.-R., R.C.W., R.S.N.), New York, New York 10021; the Department of Medicine, Oregon Health Sciences University (D.D.B., D.L.L.), Portland, Oregon 97201; the Department of Pediatrics, McMaster University (J.V.), Hamilton, Ontario, Canada; and the Department of Cell Biology, Baylor College of Medicine (N.B., B.O.), Houston, Texas 77030

Address all correspondence and requests for reprints to: Maria I. New, M.D., Department of Pediatrics, Chief, Division of Pediatric Endocrinology, New York-Presbyterian Hospital, 525 East 68th Street, Room M-622, New York, New York 10021. E-mail: minew{at}mail.med.cornell.edu

A 14-yr-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, mineralocorticoids, and androgens, but no resistance to vitamin D or thyroid hormones. She lacked Cushingoid features despite significantly high cortisol levels. Menstruation was regular, and there was no clinical evidence of masculinization despite high serum androgen levels in the male range. The patient’s sister had similar clinical features. Partial resistance to exogenous glucocorticoid and mineralocorticoid administration was well demonstrated in both patients. It is proposed that these patients represent the first cases of partial resistance to multiple steroids, possibly due to a coactivator defect.

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