Table Of ContentPEDIATRIC 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
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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
