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==Contents== | |||
HUMAN FETAL ENDOCRINES | |||
DEVELOPMENTS IN OBSTETRICS | |||
AND GYNECOLOGY | |||
VOLUME 1 | |||
— | |||
. J. E. Jirasek, Human fetal endocrines, 1980. ISBN 90-247-2325-6. | |||
. P.M. Motta, E. 8. E. Hafez, eds., Biology of the ovary, 1980. ISBN 90-247-2316-7. | |||
. J. Horsky, J. Presl, eds., Ovarian function and its disorders, 1980. ISBN | |||
90-247-2326-4. . | |||
4. D. W. Richardson, D. Joyce, E. M. Symonds, eds., Frozen human semen, 1980. | |||
ISBN 90-247-2370-1. | |||
5. E. 8S. E. Hafez, W. A. A. van Os, eds., Medicated intrauterine devices, 1980. | |||
ISBN 90-247-2371-X. | | |||
Oo bo | |||
Series ISBN 90— 247— 2334—5 © | |||
Human Fetal Endocrines | |||
JAN E. JIRASEK, M. D., D. Se. | |||
Institute for the Care of Mother and Child, | |||
Prague | |||
5 | |||
J | |||
a=} Prey | |||
WES | |||
l | |||
a | |||
r | |||
. | |||
1 ei | |||
i beeen eee | |||
1980 | |||
SPRINGER-SCIENCE+BUSINESS MEDIA, B.V. | |||
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 | |||
ISBN 978-94-009-8194-2 | |||
DOI 10.1007/978-94-009-8192-8 | |||
for Hungary, Albania, | |||
Bulgaria, China, | |||
Cuba, Czechoslovakia, | |||
German Democratic Republic, | |||
Democratic People’s Republic | |||
of Korea, Mongolia, Poland, | |||
Rumania, Soviet Union, | |||
Democratic Republic of Vietnam, | |||
and Yugoslavia | |||
AVICENUM, | |||
Czechoslovak Medical Press, | |||
Prague | |||
ISBN 978-94-009-8192-8 (eBook) | |||
Copyright © by Springer Science+Business Media Dordrecht 1980 | |||
Urspriinglich erschienen bei Martinus Nijhoff, The Hague, Netherlands 1980 | |||
Softcover reprint of the hardcover Ist edition 1980 | |||
This book is published with an arrangement of Avicenum, Czechoslovak Medical Press, Prague | |||
All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, | |||
or transmitted in any form by any means, mechanical, photocopying, recording, or otherwise, without | |||
the the prior written permission of the publisher, Springer-Science+Business Media, B.V. | |||
To my wife Véra | |||
in appreciation of all her support. | |||
CONTENTS | |||
CHAPTER 1 | |||
General ideas. | |||
Hormones. | |||
Action of hormones. | |||
Feed-back mechanisms . | |||
CHAPTER 2 oe . toe | |||
Basic Survey of Human Prenatal Development. | |||
Embryonal period . Loe ee wee | |||
Unicellular stage (48 hours) . | |||
Blastomeric stage (days 3 and 4) | |||
Blastodermic stage (days 4—6) | |||
Bilaminar embryo (days 6— 14) | |||
Trilaminar embryo (days 15—20) | |||
Early somite embryos (days 20—30) .. . | |||
Stage of limb development (days 28—53) . | |||
Late embryonal stage (days 52—60) | |||
Fetal period (weeks 9—26) ..... | |||
Perinatal period (weeks 27—40) . | |||
CHAPTER 3 | |||
The placenta . . | |||
Trophoblast of the © blastocyst a and | trophoblastic s shell . | |||
Decidua . Le | |||
Chorion wo | |||
Early chorion . | |||
The late chorion . . | |||
Structure of the placenta . | |||
Placental trophoblast | |||
Cytotrophoblast . | |||
Syncytiotrophoblast Se | |||
Fibrinoid . . Se ee | |||
Uteroplacental circulation Se ee | |||
The placental membranes . | |||
Extraplacental chorion . | |||
Extraplacental amnion . | |||
Survey of placental functions | |||
Endocrinology of the placenta . | |||
Human chorionic gonadotropin (hOG) | |||
Human chorionic somatomammotropin (hC8) . | |||
Human chorionic thyrotropin (hCT) | |||
Chorionic TRH .. . | |||
Human chorionic corticotropin (hCC) . . | |||
Steroid hormone synthesis by the trophoblast (placenta) | |||
Trophoblastic (placental) progesterone-forming compartment | |||
Placental 178-estradiol-forming compartment . | |||
Placental estriol-forming compartment . | |||
Compartmentalization of steroid production within the fetoplacental unit . | |||
The fetoplacental unit . | |||
Phylogenesis of the placenta . | |||
Pathology of the chorion and placenta | |||
Avascular chorionic villi . | |||
Vil | |||
Choriodeciduitis and chorioamnitis . | |||
Primary intervillous thrombosis . | |||
Chorionic stromal fibrosis . | |||
Placental infarcts . | |||
Placentitis secondary to infections . | |||
Listerial placentitis | |||
Tuberculous placentitis . | |||
Syphilitic placentitis . | |||
Toxoplasmic placentitis . . . | |||
Placental pathology in metabolic diseases . | |||
Placental tumors . | |||
Placental chorioangioma | |||
Trophoblastic disease | |||
Trophoblastic infiltration and deportation | |||
Placental steroid sulphatase deficiency | |||
Placental insufficiency | |||
The fetus, fetal membranes and parturition. . | |||
CHAPTER 4 | |||
Fetal adrenal . | |||
The adrenal cortex . | |||
The adrenal medulla . . | |||
Prenatal growth of the adrenal | |||
Accessory adrenals . . | |||
Phylogenesis . | |||
Steroidogenesis in the fetal adrenal | |||
The effects of aldosterone . | |||
The effect of glucocorticoids . . | |||
Compartmentation of the steroid synthesis i in . the ‘adrenal . | |||
Adrenal androgens and congenital adrenal hyperplasia | |||
Clinical forms of congenital adrenal hyperplasia | |||
Congenital adrenal hypoplasia . . | |||
Hereditary adrenocortical atrophy and diffuse cerebral sclerosis | |||
Hormones of the adrenal medulla | |||
Congenital neuroblastoma | |||
CHAPTER 5 | |||
Testes, Ovaries and the Genital System | |||
Primordial germ cells . Le | |||
The indifferent gonad (genital ridge) | |||
Development of the testis . . . | |||
Embryonal testis. | |||
Fetal testis | |||
Development of ‘the ovary . | |||
Early fetal ovary | |||
Late fetal ovary . | |||
Perinatal ovary | |||
Prenatal and perinatal degeneration of the oogonia and oocytes and atresia of follicles . | |||
Development of the genital ducts . | |||
Mesonephric (Wolffian) duct . | |||
Paramesonephric (Mullerian) duct | |||
Urogenital sinus . , | |||
Masculinization of the genital ducts | |||
Regression of the paramesonephric duct. . | |||
Development of epididymis, vas deferens and male accessory ‘glands | |||
Feminization of the genital ducts . | |||
Regression of the epigenital and paragenital parts of the mesonephros | |||
Development of the external genitalia . | |||
Masculinization of the external genitalia | |||
Feminization of the external genitalia. | |||
Genetic control and sex-differentiation . | |||
Control of gonadal development . . | |||
Control of paramesonephric duct regression . | |||
Androgens of the fetal testes: testosterone synthesis | |||
Vill | |||
Fetal testosterone effects . | |||
Steroid production by the fetal and ‘perinatal Ov vary | |||
Phylogenesis of sex-determination . | |||
Malformations affecting genital organs . | |||
Syndromes related to a complete bilateral gonadal developmental failure . | |||
Pure gonadal dysgenesis (including X Y-gonadal dysgenesis, Swyer’s syndrome) | |||
True agonadism (agonadia) Se ee ee | |||
Anorehism (syndrome of “‘vanishing”’’ testes) | |||
Turner’s syndrome and its variants | |||
Gonadal dysgenesis: complete form | |||
Gonadal dysgenesis: incomplete form . | |||
Pseudo-Turner syndrome (Noonan syndrome) . . | |||
Ovarian dysgenesis and early ovarian failure associated with X- chromosome abnormalities | |||
in patients with a normal stature | |||
46,X, i (Xp-)females . . | |||
Male pseudohermaphroditism (m. Pp. ) | |||
Mixed gonadal dysgenesis . . | |||
Bilateral testicular dysgenesis . re | |||
Unilateral testicular dysgenesis (‘‘uterus in males’’) | |||
Dysgenetic gonadal tumors (gonadoblastomas) | |||
Testosterone deficiencies syndromes | |||
Eunuchoid male pseudohermaphroditism . | |||
Male pseudohermaphroditism with pseudovaginal hypospadia . | |||
Testosterone insensitivity syndromes . | |||
Complete testicular feminization (t. fm.) | |||
Malformations of genital ducts | |||
Mesonephric duct aplasia . | |||
Rokitanski-Kistner syndrome . | |||
Vaginal atresia Loe | |||
Rudimentary uterus . | |||
Malformations of the external genitalia . | |||
Hypospadias . . | |||
Androgen- independent (teratogenic) malformations of ‘the external genitalia . | |||
Cryptorchism . . | |||
Female pseudohermaphroditism . | |||
True hermaphroditism | |||
Klinefelter’s syndrome . | |||
Multi X Syndromes in Males | |||
Trisomy X in females. | |||
Trisomy 9 Syndrome . | |||
Trisomy 13 Syndrome | |||
Cornelia de Lange Syndrome . . | |||
Fetal Face Syndrome (Robinow Syndrome) . | |||
Hypertelorism-hypospadias Syndrome (Opitz) . | |||
Hypospadias-Dysphagia Syndrome (G-syndrome) | |||
Leopard Syndrome . . | |||
Meckel Syndrome (Dysencephalia splanchnocystica) . | |||
Multiple Pterygium Syndrome (Bonnevie-Ullrych Syndrome) | |||
Myotonic Dystrophy (Steinert Syndrome) . . | |||
Potter Syndrome (oligohydramnion syndrome) . | |||
Prader-Willi Syndrome (Hypotonia- Hypometia- Hypogonadisin Obesity Syndrome) | |||
Russel-Silver Syndrome . | |||
Smith-Lemli- Opitz- Syndrome | |||
CHAPTER6... | |||
The Hypophysis | |||
Cells of the adenohypophysis | |||
The neurohypophysis | |||
The hypophyseal hormones | |||
Growth hormone (GH) | |||
Prolactin (PRL)... . | |||
Adrenocorticotropin (ACTH) | |||
Melanotropin (MSH) . | |||
Thyrotropin (TSH) | |||
Hypophyseal gonadotropins, FSH and LH | |||
Vasopressin (AVP) . | |||
Cerebral neuroendocrine complex and hypothalamic feedback systems. | |||
Vasopressin and oxytocin . | |||
Vasopressin feedback system . . | |||
Somatostatin and STH feedback system | |||
Prolactin feedback system. . . | |||
CRF and adrenocorticotropin feedback system | |||
TRF and TSH feedback system . . | |||
LH RF and FSH, LH feedback systems | |||
Steroid sensitive areas of the brain . | |||
Phylogenesis of the hypophysis | |||
Pathology . . | |||
Hypophyseal aplasia . | |||
Pituitary dwarfism . | |||
Anencephaly . . . | |||
Doubling of the hypophysis . . | |||
Leprechaunism (Donohue Syndrome) . | |||
CHAPTER 7 | |||
The epiphysis . | |||
CHAPTER 8 | |||
Pharyngeal derivatives. | |||
The thyroid | |||
Histogenesis . | |||
Thyroidal hormones . . | |||
Thyroid function in the human fetus . | |||
Placenta and fetal thyroidal function . | |||
Calcitonin Soe ee ee | |||
Phylogenesis | |||
Pathology . | |||
Thyroid duct remnants c or cysts | |||
Thyroid aplasia and dysgenesis | |||
Congenital goiter and hypothyroidism | |||
Thyrotropin insensitivity . . . | |||
Isolated TSH deficiency . | |||
Syndrome of thyroid aplasia, cutis verticis gyrate and mental retardation. | |||
Pendred syndrome . Boe ee ee | |||
Sipple syndrome . | |||
CHAPTER 9 | |||
The parathyhroid gland Le | |||
Cells of the parathyroid (PTH cells) | |||
Parathormone ere) Lo | |||
Phylogenesis . . | |||
Calcium homeostasis in pregnancy . | |||
Calcium and cellular functions . | |||
Magnesium | |||
Phosphates . | |||
Citrate . | |||
Pathology . | |||
Osteodysplasia cystica generalisata | |||
Absence of parathyroids and thymus . | |||
Hypoperathyroidism . Lo | |||
Errors in parathormone synthesis | |||
Albright’s hereditary osteodystrophy . | |||
CHAPTER 10 | |||
Enteroendocrine cells and pancreatic islets. | |||
The pancreas . | |||
Development . | |||
Cells of the pancreatic islets . | |||
B-cells and insulin formation | |||
A-cells and glucagon . | |||
D-cells . . . | |||
Pancreatic islets phylogeny . | |||
Insulin and its metabolism | |||
Glucagon and its metabolism Le | |||
Glycemia in pregnancy and diabetes . . | |||
Modification in lipid metabolism in pregnancy . | |||
Maternal diabetes and the diabetic ‘etopathy | |||
Diabetic fetopathy . Le . . | |||
Transient neonatal diabetes mellitus . | |||
Exomphalos- macroglossia- -gigantisms syndrome | |||
Pancreatic agenesis | |||
Neonatal hypoglycemia, hyperinsulinism and absence of pancreatic alpha- cells . | |||
CHAPTER 11 . | |||
Amnioniec fluid as a hormonal compartment. | |||
Hormones | |||
Estrogens . | |||
Progesterone | |||
Pregnanediol . . | |||
Dehydroepiandrosterone (DHA) and 16- hydrohy: DHA . | |||
Testosterone and dihydrotestosterone . . | |||
Pregnanetriol | |||
Cortisol | |||
hCG | |||
hos. | |||
FSH . | |||
LH. . | |||
TSH . | |||
Prolactin . | |||
ACTH | |||
hGH . | |||
Oxytocin . | |||
Epinephrine and norepinephrin | |||
Insulin . oe | |||
List of abbreviations | |||
References | |||
Subject index . | |||
XI | |||
200 | |||
200 | |||
202 | |||
204 | |||
241 | |||
PREFACE | |||
The study of prenatal development provides many clues for understanding the | |||
physiology as well as the pathogeny of malformations and many diseases. I became | |||
interested in the analysis of human development as a young medical student more | |||
than 30 years ago, and I have stayed in this field all my life. In my studies, I always | |||
tried to compare the events of different disciplines such as genetics, anatomy, biochemistry and physiology. I learned that the development of a structure is, under | |||
normal circumstances, strictly determined and that the development of structures | |||
always precedes their proper function. There are no changes in function without | |||
changes in structure. The life of every cell is genetically preprogrammed and the | |||
program may be modified by complicated interactions with environment. Recent | |||
progress in our knowledge is basicly related to technology. However, using all the | |||
tools of today’s technology, we are still unable to understand the basic normal | |||
development. | |||
After almost thirty years of work, I am trying to present a subjective review of the | |||
development of the human endocrine glands. I am presenting an image emerging | |||
from my experience. I personally studied several hundred human embryos using | |||
mostly anatomical, histochemical and some biochemical techniques. I found much | |||
additional information in the literature. However, there is so much different information available today that, using the limited brain capacity, I did not try to register | |||
all observations and discoveries, but I selected some which seemed to me to be the | |||
most important. The following books were the valuable sources of my information: | |||
In genetics and congenital anomalies: | |||
McKusick V. A.: Mendelian inheritance in man; catalogs of autosomal dominat, | |||
autosomal recessive and X-linked phenotypes. Baltimore, Johns Hopkins Press, | |||
1975. | |||
Bergsma D. (Ed.) Birth defects, atlas and compendium. The Nationa] Foundation | |||
March of Dimes, Baltimore, Williams and Wilkins Co., 1973. | |||
In embryology: | |||
Keibel F. and Mall. F. P. (Eds.) Manual of human embryology. Philadelphia | |||
London, J. B. Lippincott Co., Vol. I, 1910, Vol. II, 1912. | |||
Streeter G. L.: Developmental horizons in human embryos. Contrib. Embryol. | |||
Carnegie Inst., Washington, 1951. | |||
O’Rahilly R.: Developmental stages in human embryos, including a survey of the | |||
Carnegie Collection. Carnegie Inst., Washington, 1973. | |||
Willis R. A.: The borderland of embryology and pathology. London, Butterworth, | |||
1958. | |||
In comparative anatomy and phylogenesis: | |||
Kent G. C. Ji.:| Comparative anatomy of the vertebrates. St. Louis, C. V. Mosby | |||
Co. 1969. | |||
Romer A. S. Parson T. S.: The vertebrate body. Philadelphia, London, Toronto, | |||
W. B. Saunders Co., 1977. | |||
In biochemistry: | |||
Harper H. A.: Review of physiological chemistry. 15th ed., Los Altos, California, | |||
Lange Med. Pub., 1975. | |||
These references will generally not be mentioned in the text. | |||
The first version of Human Fetal Endocrines appeared in Czech in 1977. Since then, | |||
much new information has emerged. I completely revised and rewrite the manuscript | |||
for the English edition during my stay in Minneapolis as Visiting Professor in the | |||
Department of Obstetrics and Gynecology, University of Minnesota Medical School. | |||
Iam deeply grateful to Dr. K. A. Prem for his kind invitation to join the Department of Obstetrics and Gynecology, University of Minnesota. I also wish to express | |||
many thanks to Mrs. Blanka Faltinova, Miss Donna Lidstad and Mrs. Rachel | |||
Roverud for their linguistic and secretarial help. | |||
XIV | |||
==Book Review Table== | |||
{| class="wikitable mw-collapsible mw-collapsed" | {| class="wikitable mw-collapsible mw-collapsed" | ||
! Book Review by F. De Prins | ! Book Review by F. De Prins |
Latest revision as of 13:01, 31 July 2019
Contents
HUMAN FETAL ENDOCRINES DEVELOPMENTS IN OBSTETRICS AND GYNECOLOGY
VOLUME 1
—
. J. E. Jirasek, Human fetal endocrines, 1980. ISBN 90-247-2325-6.
. P.M. Motta, E. 8. E. Hafez, eds., Biology of the ovary, 1980. ISBN 90-247-2316-7.
. J. Horsky, J. Presl, eds., Ovarian function and its disorders, 1980. ISBN 90-247-2326-4. .
4. D. W. Richardson, D. Joyce, E. M. Symonds, eds., Frozen human semen, 1980.
ISBN 90-247-2370-1.
5. E. 8S. E. Hafez, W. A. A. van Os, eds., Medicated intrauterine devices, 1980.
ISBN 90-247-2371-X. |
Oo bo
Series ISBN 90— 247— 2334—5 © Human Fetal Endocrines
JAN E. JIRASEK, M. D., D. Se.
Institute for the Care of Mother and Child,
Prague
5 J a=} Prey
WES l
a r . 1 ei i beeen eee
1980
SPRINGER-SCIENCE+BUSINESS MEDIA, B.V. 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
ISBN 978-94-009-8194-2 DOI 10.1007/978-94-009-8192-8
for Hungary, Albania,
Bulgaria, China,
Cuba, Czechoslovakia,
German Democratic Republic, Democratic People’s Republic
of Korea, Mongolia, Poland, Rumania, Soviet Union, Democratic Republic of Vietnam, and Yugoslavia
AVICENUM, Czechoslovak Medical Press, Prague
ISBN 978-94-009-8192-8 (eBook)
Copyright © by Springer Science+Business Media Dordrecht 1980 Urspriinglich erschienen bei Martinus Nijhoff, The Hague, Netherlands 1980 Softcover reprint of the hardcover Ist edition 1980
This book is published with an arrangement of Avicenum, Czechoslovak Medical Press, Prague
All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted in any form by any means, mechanical, photocopying, recording, or otherwise, without
the the prior written permission of the publisher, Springer-Science+Business Media, B.V. To my wife Véra
in appreciation of all her support. CONTENTS
CHAPTER 1
General ideas. Hormones.
Action of hormones. Feed-back mechanisms .
CHAPTER 2 oe . toe Basic Survey of Human Prenatal Development. Embryonal period . Loe ee wee Unicellular stage (48 hours) . Blastomeric stage (days 3 and 4) Blastodermic stage (days 4—6) Bilaminar embryo (days 6— 14) Trilaminar embryo (days 15—20) Early somite embryos (days 20—30) .. . Stage of limb development (days 28—53) . Late embryonal stage (days 52—60) Fetal period (weeks 9—26) ..... Perinatal period (weeks 27—40) .
CHAPTER 3
The placenta . .
Trophoblast of the © blastocyst a and | trophoblastic s shell .
Decidua . Le
Chorion wo Early chorion .
The late chorion . . Structure of the placenta .
Placental trophoblast
Cytotrophoblast .
Syncytiotrophoblast Se
Fibrinoid . . Se ee Uteroplacental circulation Se ee The placental membranes .
Extraplacental chorion .
Extraplacental amnion . Survey of placental functions Endocrinology of the placenta .
Human chorionic gonadotropin (hOG)
Human chorionic somatomammotropin (hC8) .
Human chorionic thyrotropin (hCT)
Chorionic TRH .. .
Human chorionic corticotropin (hCC) . .
Steroid hormone synthesis by the trophoblast (placenta) Trophoblastic (placental) progesterone-forming compartment Placental 178-estradiol-forming compartment . Placental estriol-forming compartment .
Compartmentalization of steroid production within the fetoplacental unit .
The fetoplacental unit .
Phylogenesis of the placenta .
Pathology of the chorion and placenta Avascular chorionic villi .
Vil Choriodeciduitis and chorioamnitis . Primary intervillous thrombosis . Chorionic stromal fibrosis . Placental infarcts . Placentitis secondary to infections . Listerial placentitis Tuberculous placentitis . Syphilitic placentitis . Toxoplasmic placentitis . . . Placental pathology in metabolic diseases . Placental tumors .
Placental chorioangioma
Trophoblastic disease
Trophoblastic infiltration and deportation Placental steroid sulphatase deficiency Placental insufficiency
The fetus, fetal membranes and parturition. .
CHAPTER 4
Fetal adrenal .
The adrenal cortex .
The adrenal medulla . .
Prenatal growth of the adrenal
Accessory adrenals . .
Phylogenesis .
Steroidogenesis in the fetal adrenal
The effects of aldosterone .
The effect of glucocorticoids . .
Compartmentation of the steroid synthesis i in . the ‘adrenal . Adrenal androgens and congenital adrenal hyperplasia Clinical forms of congenital adrenal hyperplasia Congenital adrenal hypoplasia . .
Hereditary adrenocortical atrophy and diffuse cerebral sclerosis Hormones of the adrenal medulla
Congenital neuroblastoma
CHAPTER 5
Testes, Ovaries and the Genital System Primordial germ cells . Le The indifferent gonad (genital ridge) Development of the testis . . . Embryonal testis.
Fetal testis
Development of ‘the ovary .
Early fetal ovary
Late fetal ovary .
Perinatal ovary
Prenatal and perinatal degeneration of the oogonia and oocytes and atresia of follicles .
Development of the genital ducts . Mesonephric (Wolffian) duct .
Paramesonephric (Mullerian) duct
Urogenital sinus . ,
Masculinization of the genital ducts
Regression of the paramesonephric duct. .
Development of epididymis, vas deferens and male accessory ‘glands Feminization of the genital ducts .
Regression of the epigenital and paragenital parts of the mesonephros Development of the external genitalia . Masculinization of the external genitalia
Feminization of the external genitalia.
Genetic control and sex-differentiation .
Control of gonadal development . .
Control of paramesonephric duct regression .
Androgens of the fetal testes: testosterone synthesis
Vill Fetal testosterone effects .
Steroid production by the fetal and ‘perinatal Ov vary
Phylogenesis of sex-determination .
Malformations affecting genital organs .
Syndromes related to a complete bilateral gonadal developmental failure . Pure gonadal dysgenesis (including X Y-gonadal dysgenesis, Swyer’s syndrome) True agonadism (agonadia) Se ee ee Anorehism (syndrome of “‘vanishing”’’ testes)
Turner’s syndrome and its variants Gonadal dysgenesis: complete form Gonadal dysgenesis: incomplete form .
Pseudo-Turner syndrome (Noonan syndrome) . .
Ovarian dysgenesis and early ovarian failure associated with X- chromosome abnormalities
in patients with a normal stature 46,X, i (Xp-)females . .
Male pseudohermaphroditism (m. Pp. )
Mixed gonadal dysgenesis . .
Bilateral testicular dysgenesis . re Unilateral testicular dysgenesis (‘‘uterus in males’’) Dysgenetic gonadal tumors (gonadoblastomas)
Testosterone deficiencies syndromes
Eunuchoid male pseudohermaphroditism .
Male pseudohermaphroditism with pseudovaginal hypospadia .
Testosterone insensitivity syndromes .
Complete testicular feminization (t. fm.)
Malformations of genital ducts
Mesonephric duct aplasia . Rokitanski-Kistner syndrome . Vaginal atresia Loe
Rudimentary uterus .
Malformations of the external genitalia .
Hypospadias . . Androgen- independent (teratogenic) malformations of ‘the external genitalia .
Cryptorchism . .
Female pseudohermaphroditism .
True hermaphroditism
Klinefelter’s syndrome .
Multi X Syndromes in Males
Trisomy X in females.
Trisomy 9 Syndrome .
Trisomy 13 Syndrome
Cornelia de Lange Syndrome . .
Fetal Face Syndrome (Robinow Syndrome) .
Hypertelorism-hypospadias Syndrome (Opitz) .
Hypospadias-Dysphagia Syndrome (G-syndrome)
Leopard Syndrome . .
Meckel Syndrome (Dysencephalia splanchnocystica) .
Multiple Pterygium Syndrome (Bonnevie-Ullrych Syndrome)
Myotonic Dystrophy (Steinert Syndrome) . .
Potter Syndrome (oligohydramnion syndrome) .
Prader-Willi Syndrome (Hypotonia- Hypometia- Hypogonadisin Obesity Syndrome)
Russel-Silver Syndrome .
Smith-Lemli- Opitz- Syndrome
CHAPTER6...
The Hypophysis
Cells of the adenohypophysis
The neurohypophysis
The hypophyseal hormones Growth hormone (GH) Prolactin (PRL)... . Adrenocorticotropin (ACTH) Melanotropin (MSH) . Thyrotropin (TSH)
Hypophyseal gonadotropins, FSH and LH
Vasopressin (AVP) .
Cerebral neuroendocrine complex and hypothalamic feedback systems.
Vasopressin and oxytocin .
Vasopressin feedback system . .
Somatostatin and STH feedback system
Prolactin feedback system. . .
CRF and adrenocorticotropin feedback system
TRF and TSH feedback system . .
LH RF and FSH, LH feedback systems Steroid sensitive areas of the brain . Phylogenesis of the hypophysis Pathology . .
Hypophyseal aplasia .
Pituitary dwarfism .
Anencephaly . . .
Doubling of the hypophysis . .
Leprechaunism (Donohue Syndrome) .
CHAPTER 7 The epiphysis .
CHAPTER 8
Pharyngeal derivatives. The thyroid Histogenesis . Thyroidal hormones . . Thyroid function in the human fetus . Placenta and fetal thyroidal function . Calcitonin Soe ee ee Phylogenesis Pathology . Thyroid duct remnants c or cysts Thyroid aplasia and dysgenesis Congenital goiter and hypothyroidism Thyrotropin insensitivity . . . Isolated TSH deficiency . Syndrome of thyroid aplasia, cutis verticis gyrate and mental retardation. Pendred syndrome . Boe ee ee Sipple syndrome .
CHAPTER 9
The parathyhroid gland Le Cells of the parathyroid (PTH cells) Parathormone ere) Lo Phylogenesis . . Calcium homeostasis in pregnancy . Calcium and cellular functions . Magnesium Phosphates . Citrate . Pathology . Osteodysplasia cystica generalisata Absence of parathyroids and thymus . Hypoperathyroidism . Lo Errors in parathormone synthesis Albright’s hereditary osteodystrophy . CHAPTER 10
Enteroendocrine cells and pancreatic islets. The pancreas .
Development .
Cells of the pancreatic islets .
B-cells and insulin formation
A-cells and glucagon .
D-cells . . .
Pancreatic islets phylogeny .
Insulin and its metabolism
Glucagon and its metabolism Le Glycemia in pregnancy and diabetes . . Modification in lipid metabolism in pregnancy . Maternal diabetes and the diabetic ‘etopathy Diabetic fetopathy . Le . . Transient neonatal diabetes mellitus . Exomphalos- macroglossia- -gigantisms syndrome Pancreatic agenesis
Neonatal hypoglycemia, hyperinsulinism and absence of pancreatic alpha- cells .
CHAPTER 11 . Amnioniec fluid as a hormonal compartment. Hormones Estrogens . Progesterone Pregnanediol . . Dehydroepiandrosterone (DHA) and 16- hydrohy: DHA . Testosterone and dihydrotestosterone . . Pregnanetriol Cortisol hCG hos. FSH . LH. . TSH . Prolactin . ACTH hGH . Oxytocin . Epinephrine and norepinephrin Insulin . oe
List of abbreviations References
Subject index .
XI
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241 PREFACE
The study of prenatal development provides many clues for understanding the physiology as well as the pathogeny of malformations and many diseases. I became interested in the analysis of human development as a young medical student more than 30 years ago, and I have stayed in this field all my life. In my studies, I always tried to compare the events of different disciplines such as genetics, anatomy, biochemistry and physiology. I learned that the development of a structure is, under normal circumstances, strictly determined and that the development of structures always precedes their proper function. There are no changes in function without changes in structure. The life of every cell is genetically preprogrammed and the program may be modified by complicated interactions with environment. Recent progress in our knowledge is basicly related to technology. However, using all the tools of today’s technology, we are still unable to understand the basic normal development.
After almost thirty years of work, I am trying to present a subjective review of the development of the human endocrine glands. I am presenting an image emerging from my experience. I personally studied several hundred human embryos using mostly anatomical, histochemical and some biochemical techniques. I found much additional information in the literature. However, there is so much different information available today that, using the limited brain capacity, I did not try to register all observations and discoveries, but I selected some which seemed to me to be the most important. The following books were the valuable sources of my information:
In genetics and congenital anomalies:
McKusick V. A.: Mendelian inheritance in man; catalogs of autosomal dominat, autosomal recessive and X-linked phenotypes. Baltimore, Johns Hopkins Press, 1975.
Bergsma D. (Ed.) Birth defects, atlas and compendium. The Nationa] Foundation March of Dimes, Baltimore, Williams and Wilkins Co., 1973.
In embryology:
Keibel F. and Mall. F. P. (Eds.) Manual of human embryology. Philadelphia London, J. B. Lippincott Co., Vol. I, 1910, Vol. II, 1912.
Streeter G. L.: Developmental horizons in human embryos. Contrib. Embryol. Carnegie Inst., Washington, 1951.
O’Rahilly R.: Developmental stages in human embryos, including a survey of the Carnegie Collection. Carnegie Inst., Washington, 1973.
Willis R. A.: The borderland of embryology and pathology. London, Butterworth, 1958.
In comparative anatomy and phylogenesis:
Kent G. C. Ji.:| Comparative anatomy of the vertebrates. St. Louis, C. V. Mosby Co. 1969.
Romer A. S. Parson T. S.: The vertebrate body. Philadelphia, London, Toronto, W. B. Saunders Co., 1977.
In biochemistry:
Harper H. A.: Review of physiological chemistry. 15th ed., Los Altos, California, Lange Med. Pub., 1975.
These references will generally not be mentioned in the text.
The first version of Human Fetal Endocrines appeared in Czech in 1977. Since then, much new information has emerged. I completely revised and rewrite the manuscript for the English edition during my stay in Minneapolis as Visiting Professor in the Department of Obstetrics and Gynecology, University of Minnesota Medical School.
Iam deeply grateful to Dr. K. A. Prem for his kind invitation to join the Department of Obstetrics and Gynecology, University of Minnesota. I also wish to express many thanks to Mrs. Blanka Faltinova, Miss Donna Lidstad and Mrs. Rachel Roverud for their linguistic and secretarial help.
XIV
Book Review Table
Book Review by F. De Prins |
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European Journal of Obstetrics & Gynecology and Reproductive Biology
Human Fetal Endocrines J.E. Jirasek Martinus Nijhoff Publishers BV, The Hague, 1980 Dfl. 82.00 (224 pp.) ‘Human fetal endocrines’ by Jan E. Jirasek concerns the embryonic development, the histology, the pathologic anatomy and the function of the endocrine glands during fetal life. It is interesting to find in one book current knowledge on items so diverse as placental HCG secretion and fetal parathyroid regulation of calcium levels in plasma. However, this book is disfigured by many shortcomings. It looks as if there were a competition between the number of grammatical mistakes and the number of misprints. The lay-out of the. book is very poor and old fashioned. The table of contents refers to some 60 items on one page and covers 5 pages of very small print. Figures and photographs are included without mentioning the origin. As for the contents, the title of this book misleads the potential reader. This is not a textbook on fetal endocrinology, it is a textbook on embryonic and fetal morphol- ogy. Eighty percent of the work is devoted to embryology, phylogenesis, histology, pathologic anatomy and comparative anatomy. There is only one figure of the chemical structure of a hormone but there are almost 100 pictures of histologic sections of fetal organs. Over 100 pages deal with the production of steroid hormones but the steroid molecule structure will be looked for in vain in this book. The histologic subdivision of molar disease, covering 4 pages, has nothing to do with fetal endocrinology. Moreover, it is no longer of clinical value and it has long been replaced by the follow-up of HCG f-subunit concentrations in plasma both as a criterion for prognosis and as a guide for therapy. The hypothesis on the onset of labor by the distension of the amnion by accumulation of amniotic fluid may be original but it does not explain the earlier onset of labor in cases of intrauterine growth retardation where the amount of amniotic fluid usually is reduced. Neither does it explain the onset of labor in cases of Potter’s syndrome or in cases of urethral stenosis. The chapter on the fetal endocrine pancreas is not up-to-date. De-cells are not considered to be heterogenous anymore. The presence of gastrin producing cells has been questioned. The author claims that epinephrine and norepinephrine stimulate insulin secretion although in the next paragraph he claims epinephrine and norepinephrine inhibit insulin secretion. The positive correlation between amniotic insulin levels and fetal age or fetal weight, mentioned by the author, has been disproved in more recent work. It is not clear for whom this book is written. It is too specialized for medical students, it is of no use for medical practitioners and it does not offer anything new for scientific workers. F. De Prins, Leuven |