Congenital Adrenal Hyperplasia
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Volume 26
Edited by F. Gross, Heidelberg' M. M. Grumbach, San Francisco A. Labhart, Zurich . M. B. Lipsett, Bethesda T. Mann, Cambridge' L. T. Samuels (t), Salt Lake City J. Zander, Munchen
M. I. New
L. S. Levine
Congenital Adrenal Hyperplasia With 35 Figures and 6 Tables
Springer-Verlag Berlin Heidelberg New York Tokyo 1984
Maria I. New, M. D. Lenore S. Levine, M.D. Division of Pediatric Endocrinology Department of Pediatrics The New York Hospital-Cornell Medical Center 525 East 68th Street New York, NY 10021jU.S.A.
ISBN-13 :978-3-642-820 13-7 DOl: 10.1007/978-3-642-82011-3
e- ISBN -13 :978-3-642-82011-3
Library of Congress Cataloging in Publication Data New. Maria L Congenital adrenal hyperplasia. (Monographs on endocrinology; v. 26) Includes bibliographical references and index. 1. Adrenogenital syndrome. 2. Pediatric endocrinology. L Levine, L. S. (Lenore S.), 1933~ . II. Title. III. Series. RJ420.A3N48 1984 618.92'45 84-1234 ISBN-13 :978-3-642-82013-7 (US.) This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically those of translation, reprinting, fe-use of illustrations, broadcasting, reproduction by photocopying machine or similar means, and storage in data banks. Under ยง54 of the German Copyright Law where copies are made for other than private use. a fee is payable to 'Verwertungsgesellschaft Wort'. Munich. Springer-Verlag Berlin. Heidelberg 1984 Softcover reprint of the hardcover 1st edition 1984
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The use of registered names, trademarks, etc. in this publication does not imply. even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use.
Product Liability: The publisher can give no guarantee for information about drug dosage and application thereof contained in this book. In every individual case the respective user must check its accuracy by consulting other pharmaceutical literature.
2127/3020-543210
Acknowledgements
Much of the work described in this monograph was supported by NIH grants HD 00072, HD 15084 and HL 17749, and by a grant (RR 47) from the General Clinical Research Centers Program of the Division of Research Resources, NIH. This manuscript could not have been completed without the tireless efforts and extremely knowledgeable assistance of Mrs. Vita Amendolagine and Ms. Robin Grimm.
Contents
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
IX
Steroidogenesis and Enzymatic Conversions of Adrenal Steroid Hormones. . ... ... . . ...... . ..... . . . . .. . .. .. .. .... ......... A. Steroidogenesis............................................ I. Mineralocorticoids (17-Deoxy Pathway). . . . . . . . . . . . . . . . . . II. Glucocorticoids (17-Hydroxy Pathway) .................. III. Sex Steroids. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . B. Mechanism of Adrenal Steroid Regulation . . . . . . . . . . . . . .
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