This Article Full Text Full Text (PDF) Submit a related Letter to the Editor Purchase Article View Shopping Cart Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Copyright Permission Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by New, M. I. Articles by O’Malley, B. Search for Related Content PubMed PubMed Citation Articles by New, M. I. Articles by O’Malley, B.

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.

This article has been cited by other articles:

				J. I. WEBSTER, M. MOAYERI, and E. M. STERNBERG Novel Repression of the Glucocorticoid Receptor by Anthrax Lethal Toxin Ann. N.Y. Acad. Sci., June 1, 2004; 1024(1): 9 - 23. [Abstract] [Full Text] [PDF]

				E. CHARMANDARI, T. KINO, and G. P. CHROUSOS Familial/Sporadic Glucocorticoid Resistance: Clinical Phenotype and Molecular Mechanisms Ann. N.Y. Acad. Sci., June 1, 2004; 1024(1): 168 - 181. [Abstract] [Full Text] [PDF]

				H. Chen, M. Hewison, B. Hu, and J. S. Adams Heterogeneous nuclear ribonucleoprotein (hnRNP) binding to hormone response elements: A cause of vitamin D resistance PNAS, May 13, 2003; 100(10): 6109 - 6114. [Abstract] [Full Text] [PDF]

				A. Vottero, T. Kino, H. Combe, P. Lecomte, and G. P. Chrousos A Novel, C-Terminal Dominant Negative Mutation of the GR Causes Familial Glucocorticoid Resistance through Abnormal Interactions with p160 Steroid Receptor Coactivators J. Clin. Endocrinol. Metab., June 1, 2002; 87(6): 2658 - 2667. [Abstract] [Full Text] [PDF]

				H. A. Molenda, A. L. Griffin, A. P. Auger, M. M. McCarthy, and M. J. Tetel Nuclear Receptor Coactivators Modulate Hormone-Dependent Gene Expression in Brain and Female Reproductive Behavior in Rats Endocrinology, February 1, 2002; 143(2): 436 - 444. [Abstract] [Full Text] [PDF]

				I. A. Hughes Minireview: Sex Differentiation Endocrinology, August 1, 2001; 142(8): 3281 - 3287. [Abstract] [Full Text] [PDF]

				I. A. Hughes A Novel Explanation for Resistance to Androgens N. Engl. J. Med., September 21, 2000; 343(12): 880 - 882. [Full Text]

				Editorial: A New "New" Syndrome in the New World: Is Multiple Postreceptor Steroid Hormone Resistance Due to a Coregulator Defect? J. Clin. Endocrinol. Metab., December 1, 1999; 84(12): 4450 - 4453. [Full Text]