Talk:Book - Human fetal endocrines (1980)

From Embryology




. 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


Institute for the Care of Mother and Child,


5 J a=} Prey


a r . 1 ei i beeen eee



for the United States and Canada Kluwer Boston, Inc.

190 Old Derby Street Hingham, MA 02043


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


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) .


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. .


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


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


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 .


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 .


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 .







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.


Book Review Table

Book Review by F. De Prins 
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