TY - JOUR
T1 - Nonclassical 3β-hydroxysteroid dehydrogenase deficiency
T2 - A review of our experience with 25 female patients
AU - Schram, P.
AU - Zerah, M.
AU - Mani, P.
AU - Jewelewicz, R.
AU - Jaffe, S.
AU - New, M. I.
N1 - Funding Information:
Received July 29, 1991; revised and accepted March 4, 1992. * Supported by United States Public Health Service, National Institutes of Health, Bethesda, Maryland, grant awards HD00072 and RR 06020. Support was also received from the United Jewish Appeal: Federation of Jewish Philantrophies of New York, Inc; and the Reichman Family Fund (Olympia and York [US] Holdings Co., New York New York) t Presented in part at the 72nd Annual Meeting of The Endocrine Society, Atlanta, Georgia, June 20 to 23, 1990. t Department of Pediatrics, Division of Pediatric Endocrinology, The New York Hospital-Cornell Medical Center. § Reprint requests: Michele Zerah, M.D., Division of Pediatric Endocrinology, The New Yark Hospital-Cornell Medical Center, 525 East 68th Street, New York, New York 1002l. II Department of Obstetrics and Gynecology, Columbia University College of Physicians and Surgeons.
PY - 1992
Y1 - 1992
N2 - Objective: To report 15 new menarchial women affected with nonclassical 3β-hydroxysteroid dehydrogenase deficiency (nonclassical 3β-HSD) and evaluation of glucocorticoid therapy in treated patients. Design: Diagnosis of these new patients using a standard adrenocorticotropin test. Effects of glucocorticoid therapy on clinical hormonal and sonographic features of each patient are appreciated for periods varying between 4 months and 7 1/2 years. Setting: All at The New York Hospital-Cornell Medical Center. The Pediatric Endocrinology Ambulatory Service; the Children's Clinical Research Center Core Laboratories; and the Department of Radiology. Patients, Participants: Fifteen menarchial women (14 to 30 years of age) newly diagnosed and 10 women previously diagnosed were evaluated for symptoms of hyperandrogenism and/or irregular menses. Main Outcome Measure(s): Positive effect of glucocorticoid therapy on signs and symptoms, hormonal levels, and ovarian imaging. Results: Polycystic ovarian syndrome is noted in approximately half the cases. Glucocorticoid treatment > 3 months duration results in a reversal of symptoms in most cases.
AB - Objective: To report 15 new menarchial women affected with nonclassical 3β-hydroxysteroid dehydrogenase deficiency (nonclassical 3β-HSD) and evaluation of glucocorticoid therapy in treated patients. Design: Diagnosis of these new patients using a standard adrenocorticotropin test. Effects of glucocorticoid therapy on clinical hormonal and sonographic features of each patient are appreciated for periods varying between 4 months and 7 1/2 years. Setting: All at The New York Hospital-Cornell Medical Center. The Pediatric Endocrinology Ambulatory Service; the Children's Clinical Research Center Core Laboratories; and the Department of Radiology. Patients, Participants: Fifteen menarchial women (14 to 30 years of age) newly diagnosed and 10 women previously diagnosed were evaluated for symptoms of hyperandrogenism and/or irregular menses. Main Outcome Measure(s): Positive effect of glucocorticoid therapy on signs and symptoms, hormonal levels, and ovarian imaging. Results: Polycystic ovarian syndrome is noted in approximately half the cases. Glucocorticoid treatment > 3 months duration results in a reversal of symptoms in most cases.
KW - Adrenal steroid 3β-hydroxysteroid dehydrogenase deficiency
KW - glucocorticoid therapy
KW - polycystic ovarian syndrome
UR - http://www.scopus.com/inward/record.url?scp=0026649663&partnerID=8YFLogxK
U2 - 10.1016/s0015-0282(16)55149-8
DO - 10.1016/s0015-0282(16)55149-8
M3 - Article
C2 - 1623993
AN - SCOPUS:0026649663
SN - 0015-0282
VL - 58
SP - 129
EP - 136
JO - Fertility and Sterility
JF - Fertility and Sterility
IS - 1
ER -