Pediatric Andrology PDF
Preview Pediatric Andrology
PEDIATRIC ANDROLOGY CLINICS IN ANDROLOGY E.S.E. HAFEZ, series editor VOLUME 7 I. J.e. Emperaire, A. Audebert. E.S.E. Hafez, eds., Homologous artificial insemination (AIH). 1980. ISBN 90-247-2269-1. 2. L.I. Lipshultz, J.N. Corriere Jr., E.S.E. Hafez, eds., Surgery of the male reproductive tract. 1980, ISBN 90-247-2315-9. 3. E.S.E. Hafez, ed., Descended and cryptorchid testis. 1980, ISBN 90-247-2299-3. 4. J. Bain, E.S.E. Hafez, eds., Diagnosis in andrology. 1980, ISBN 90-247-2365-5. 5. G.R. Cunningham, W.-B. Schill, E.S.E. Hafez, eds., Regulation of male fertility. 1980, ISBN 90-247-2373-6. 6. E.S.E. Hafez, E. Spring-Mills, eds., Prostatic carcinoma: biology and diagnosis. 1981, ISBN 90-247-2379-5. series ISBN 90-247-2333-7 PEDIATRIC ANDROLOGY edited by S.J. KOGAN Bronx, New York, U.S.A. and E.S.E. HAFEZ Detroit, Michigan, U.S.A . • 1981 MARTINUS NIJHOFF THE HAGUE/BOSTON/LONDON Distributors: for the United States and Canada Kluwer Boston, Inc. 190 Old Derby Street Hingham, MA 02043 USA for all other countries Kluwer Academic Publishers Group Distribution Center P.O. Box 322 3300 AH Dordrecht The Netherlands This volume is listed in the Library of Congress Cataloging in Publication Data ISBN -13: 978-94-010-3721-1 e-ISBN -13:978-94-010-3719-8 om: 10.1007/978-94-010-3719-8 Copyright © 1981 by Martinus NijhoffPublishers bv, The Hague. Softcover reprint of the hardcover 1st edition 1981 All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, mechanical, photocopying, recording, or otherwise, without the prior written permission of the publisher, Martinus NijholJ Publishers bv, P.O. Box 566, 2501 CN The Hague, The Netherlands. Very special thanks and appreciation are expressed to Mrs. E. Marash for her skill and patience in typing and collating this volume, and to Mrs. M. Scheffer, M. D'Alo, K. Maguire and L. Rust for their assistance. TABLE OF CONTENTS Contributors IX Foreword XI 1. Sex differentiation of male genitalia E.S.E. HAFEZ 2. The fetal hormonal environment and its effect on the morphogenesis of the genital system 9 P.J. SMAIL, F.I. REYES, J.S.D. WINTER, e. FAIMAN 3. Stromal influence on expression of morphological and functional characteristics of urogenital epithelia 21 G.R. CUNHA, H. FUJII, B.L. NEUBAUER, J.M. SHANNON, B.A. REESE 4. Biochemistry and biology of Mullerian Inhibiting Substance 37 P.K. DONOHOE, G.P. BUDZIK, D.A. SWANN 5. Control of Mullerian Inhibiting Substance 47 B.B. BERCU, Y MORIKAWA, P.K. DONOHOE 6. Ontogeny of 5 IX-reductase and the androgen receptor in the penis 53 J. RAJFER, P.e. NAMKUNG, P.H. PETRA 7. Studies on H-Y antigen: The genetic basis of abnormal gonadal differentiation 59 S.S. WACHTEL, M.l. NEW 8. Abnormal sexual development: A classification with emphasis on pathology and neoplastic conditions 71 W.R. WELCH, SJ. ROBBOY 9. Male pseudohermaphroditism due to abnormal testosterone biosynthesis and metabolism 87 P. SAENGER, L.S. LEVINE, M.l. NEW 10. The impact of androgens on the evolution of male gender identity 99 J. IMPERATO-McGINLEY, R.E. PETERSON, T. GAUTIER, E. STURLA II. Sexual differentiation of the brain 109 R.A. GORSKI, C.D. JACOBSON 12. Gender identity/role (G-I/R) in male hermaphroditism 135 E. HIGHAM 13. Pathogenesis of cryptorchidism 147 F. HADZISELIMOVIC VIII 14. Gonadotropin therapy for the undescended testis 163 J.R. BIERICH 15. Congenital anomalies of the vas deferens and epididymis 173 R.L. KROOVAND, A.D. PERLMUTTER 16. Leydig cells tumor and their distinction from testicular tumors associated with congenital adrenal hyperplasia 181 J. WACKSMAN 17. Lower urinary tract morphology in patients with hypospadias 187 L. GONZALEZ-SERVA, J.F. STECKER, JR., CJ. DEVINE, JR., C.E. HORTON 18. Micropenis: etiologic and management considerations 197 SJ. KOGAN Concluding remarks 209 Subject index 215 CONTRIBUTORS BERCU, B.B.: Neonatal and Pediatric Medicine Branch, National Institute of Health. 9000 Rockville Pike. Bethesda. MD 20205. USA BIERICH, J .R.: University-Kinderklinik. Rumelinstrasse 19-23, 0-7400 Tubingen. West Germany BUDZIK, G.P.: Departments of Surgery and Biological Chemistry. Harvard Medical School. Boston. MA 02114. USA CUNHA, G.R.: Department of Anatomy. University of Colorado, Health Sciences Center. 4200 East Ninth Avenue. Denver. CO 80262. USA DEVINE, CJ. JR.: Department of Urology. Eastern Virginia Medical School. 700 Botetourt Street. Post Office Box 1980, Norfolk, VA 23501, USA DONAHOE, P.K.: Pediatric Surgical Research Laboratories. Harvard Medical School. Boston. MA 02114. USA FAIMAN. c.: Department of Medicine, The University of Manitoba. Health Sciences Centre. General Centre Room G4. 700 William Avenue, Winnipeg, Manitoba R3E OZ3, Canada FUJII, H.: Department of Anatomy, University of Colorado, 4200 East Ninth Avenue, Denver. CO 80260. USA GAUTIER, T.: Universidad Nacional, Pedro Henriquez Urena. Department of Pediatrics. Santa Domingo. Dominican Republic GONZALEZ-SERVA, L.: Department of Urology. Eastern Virginia Medical School. 700 Botetourt Street. Norfolk, VA 23501. USA GORSKI, R.A.: Departments of Anatomy and Brain. Research Institute. University of California at Los Angeles. School of Medicine. Room AK 01, Los Angeles. USA HADZISELIMOVIC F.: Department of Pediatrics, Kinderspital Basel, Romergasse 8, 4005 Basel. Switzerland HAFEZ. E.S.E.: Department of Gynecology/Obstetrics, C.S. Mott Center for Human Growth and Development. Wayne State University School of Medicine. Detroit, MI 48201. USA HIGHAM E.: Department of Psychology. John Hopkins Hospital and University. Baltimore. MD 21205. USA HORTON, C.E.: Department of Plastic Surgery. Eastern Virginia Medical School. 700 Botetourt Street. Norfolk. VA 23501. USA IMPERATO-McGINLEY 1.: Department of Medicine/Division of Endocrinology. The New York Hospital. Cornell University Medical Center. 525 East 68th Street. New York. NY 10021. USA JACOBSON. C.D.: Department of Anatomy, University of California at Los Angeles, School of Medicine. The Center for Health Sciences 73-235, Los Angeles, CA 90024, USA KOGAN, SJ.: Department of Surgery. Division of Pediatric Urology, Albert Einstein College of Medicine. Bronx. NY 10461, USA KROOVAND. R.L.: Department of Pediatric Urology. Wayne State University School of Medicine. Children's Hospital of Michigan. 3901 Beaubien Boulevard. Detroit MI 48201, USA LEVINE. L.S.: Department of Pediatrics, Division of Pediatric Endocrinology. The New York Hospital. Cornell University Medical Center. 525 East 68th Street. Room N-236. New York. NY 10021. USA MORIKAWA. Y.: Department of Pediatric Surgery. Massachusetts General Hospital, 32 Fruit Street, Boston. MA 02114, USA NAMKUNG, P.c.: Department of Obstetrics-Gynecology, BB 638 University Hospital RH 20. University of Washington School of Medicine, Seattle, WA 98195, USA NEUBAUER. B.L.: Department of Anatomy. University of Colorado, Health Sciences Center. 4200 East Ninth Avenue, Denver. CO 80262, USA NEW, M.1.: Department of Pediatrics, Division of Pediatric Endocrinology, The New York Hospital. Cornell University Medical Center, 525 East 68th Street, New York, NY 10021, USA PERLMUTTER, A.D.: Department of Pediatric Urology, Wayne State University School of Medicine. Children's Hospital of Michigan, 3901 Beaubien Boulevard, Detroit. MI 48201. USA PETERSON, R.E.: Department of Medicine/Division of Endocrinology, The New York Hospital. Cornell University Medical Center, 525 East 68th Street. New York, NY 10021, USA PETRA, P.H.: Department of Obstetrics-Gynecology and Biochemistry and Laboratory Medicine, BB 632 University Hospital RH 20, University of Washington School of Medicine. Seattle. WA 98195. USA RAJFER, J.: Division of Urology, University of California at Los Angeles School of Medicine. Los Angeles, CA 90024, USA REESE, B.A.: Department of Anatomy. University of Colorado. 4200 East Ninth Avenue. Denver. CO 80262, USA REYES. F.I.: Department of Obstetrics/Gynecology. The University of Manitoba, Woman's Centre S021. 735 Notre Dame Avenue. Winnipeg, Manitoba R3E OL8, Canada ROBBOY, SJ.: Department of Pathology, Harvard Medical School. Boston, MA 02114. USA SAENGER, P.: Montefiore Hospital and Medical Center and Albert Einstein College of Medicine, Bronx. NY 10467. USA SHANNON, J.M.: Department of Anatomy. University of Colorado. Health Sciences Center. 4200 East Ninth Avenue, Denver. CO 80262. USA x SMAIL, P.J.: Department of Pediatrics, The University of Manitoba Health Sciences Centre, Children's Hospital, 685 Bannatyne Avenue, Winnipeg, Manitoba R3E OWl, Canada STECKER, l.F. lR.: Department of Urology, Eastern Virginia Medical School, 700 Botetourt Street, Norfolk, VA 2350 I, USA STURLA, E.: Department of Medicine/Division of Endocrinology, The New York Hospital, Cornell University Medical Center, 525 East 68th Street, New York, NY 10021, USA SWANN, D.A.: Department of Surgery, Massachusetts General HospitaL Shriners Burns Institute, Boston, MA 02114, USA WACKSMAN, l.: Department of Surgery, Division of Urology, Children's Hospital Medical Center, Elland and Bethesda Avenue, Cincinnati, OH 45229, USA WACHTEL, S.S.: Cornell University Graduate School of Medical Sciences, Cornell University, 1300 York Avenue at 69th Street, New York, NY 10021, USA WELCH, W.R.: Department of Pathology, Boston Hospital for Women and Harvard Medical School, Boston, MA 02114, USA WINTER, l.S.D.: Department of Pediatrics, Health Sciences Centre, Children's Hospital Room CN 103, 685 Bannatyne Avenue, Winnipeg, Manitoba R3E OWl, Canada FOREWORD The subject matter in this volume was derived from papers presented at the Pediatric Andrology session of the 1st Pan American Congress of Andrology held in Caracas, Venezuela in February 1979, as well as from selected additional manuscripts of interest in this field. Prior to this session, identification of a distinct discipline and specialty devoted to pediatric andrology had not occurred, nor had it been conceptualized. Pediatric andrology encompasses many different areas: disorders of sexual development, structural disease of the genital organs, normal and abnormal puberty, undescended testes, genital tumors, gonadal function and its relationship to growth, virilization, fertility and gender identity all represent areas of specific interest, to name but a few. Andrology itself is a unique field, bringing together clinicians and basic scientists with diverse back grounds. It should be noted that between one-half and three-quarters of the clinicians contributing to this issue conduct basic research as well. It has been this unique blend of basic research and clinical medicine which has done much to unravel many of these problems characteristic of this field. Why a separate discipline of pediatric andrology? Firstly, because many of the diseases first manifest in adulthood have their beginnings in childhood, or for that matter, at conception. Secondly, because children are not just miniature adults; their anatomy, physiology and mentality are different, as well as their medical problems, both andrologic and otherwise. The discipline of pediatric andrology represents an input from individuals who have a knowledge of the problems unique to children, and who are skilled in dealing with these problems. It is hoped that this volume will serve as a vehicle for communication between individuals who work in different specialty fields of interest, who all have a common interest in the problems unique to pediatric andrology, and that future volumes may be able to serve a similar function as well. SJ. KOGAN, M.D. Bronx, New York December 1980
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