46, XY complete gonadal dysgenesis (Swyer syndrome): Report of two different ...Apollo Hospitals
In 1955, Swyer first described two phenotypic women with
gonadal dysgenesis without the stigma of Turner syndrome
(46 XY pure gonadal dysgenesis, now known as Swyer syndrome). The chance of tumor development in Swyer syndrome is 20e30%. The most common tumor described is bilateral gonadoblastoma, but also seen are dysgerminoma and even embryonal carcinoma. Five percent of dysgerminomas are discovered in patients who are phenotypically females with abnormal gonads and 46 XY karyotye.
In this case report, we aimed to present a case with pure
gonadal dysgenesis who presented with complaints of primary amenorrhea and was detected to have bilateral dysgenetic gonads, 46, XY karyotype, as a rare cause of male pseudo-hermaphroditism.
46, XY complete gonadal dysgenesis (Swyer syndrome): Report of two different ...Apollo Hospitals
In 1955, Swyer first described two phenotypic women with
gonadal dysgenesis without the stigma of Turner syndrome
(46 XY pure gonadal dysgenesis, now known as Swyer syndrome). The chance of tumor development in Swyer syndrome is 20e30%. The most common tumor described is bilateral gonadoblastoma, but also seen are dysgerminoma and even embryonal carcinoma. Five percent of dysgerminomas are discovered in patients who are phenotypically females with abnormal gonads and 46 XY karyotye.
In this case report, we aimed to present a case with pure
gonadal dysgenesis who presented with complaints of primary amenorrhea and was detected to have bilateral dysgenetic gonads, 46, XY karyotype, as a rare cause of male pseudo-hermaphroditism.
USMLE REPRODUCTIVE 06 Development of female genital system.pdfAHMED ASHOUR
The development of the female genital system is a complex process involving the differentiation of structures that eventually form the reproductive and associated organs.
Understanding the embryonic development of the female genital system is crucial for surgeons, obstetricians, and gynecologists, especially in the context of congenital anomalies or surgical interventions.
Sexual differentiation in men and women with special attention to: Gonads, Mullerian structures, Wolffian ducts and urogenital sinus.
Examples of gonadal dysgenesis
Disorders of Sexual differentiation phenotype, chromosomal background, biological background, mechanism of hormonal disruption and endocrinological mechanisms leading to: Swyer syndrome, Androgen insensitivity syndrome and masculinization of female fetus in congenital adrenal hyperplasia. Describe three uterine anomalies resulting from variation in the fusion of Muller’s tubercles.
Describe the anatomical situation in a patient with Mayer-Rokitanski-Kuster
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Recomendações da OMS sobre cuidados maternos e neonatais para uma experiência pós-natal positiva.
Em consonância com os ODS – Objetivos do Desenvolvimento Sustentável e a Estratégia Global para a Saúde das Mulheres, Crianças e Adolescentes, e aplicando uma abordagem baseada nos direitos humanos, os esforços de cuidados pós-natais devem expandir-se para além da cobertura e da simples sobrevivência, de modo a incluir cuidados de qualidade.
Estas diretrizes visam melhorar a qualidade dos cuidados pós-natais essenciais e de rotina prestados às mulheres e aos recém-nascidos, com o objetivo final de melhorar a saúde e o bem-estar materno e neonatal.
Uma “experiência pós-natal positiva” é um resultado importante para todas as mulheres que dão à luz e para os seus recém-nascidos, estabelecendo as bases para a melhoria da saúde e do bem-estar a curto e longo prazo. Uma experiência pós-natal positiva é definida como aquela em que as mulheres, pessoas que gestam, os recém-nascidos, os casais, os pais, os cuidadores e as famílias recebem informação consistente, garantia e apoio de profissionais de saúde motivados; e onde um sistema de saúde flexível e com recursos reconheça as necessidades das mulheres e dos bebês e respeite o seu contexto cultural.
Estas diretrizes consolidadas apresentam algumas recomendações novas e já bem fundamentadas sobre cuidados pós-natais de rotina para mulheres e neonatos que recebem cuidados no pós-parto em unidades de saúde ou na comunidade, independentemente dos recursos disponíveis.
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O estabelecimento da amamentação e o manejo das principais intercorrências é contemplada.
Recomendamos muito.
Vamos discutir essas recomendações no nosso curso de pós-graduação em Aleitamento no Instituto Ciclos.
Esta publicação só está disponível em inglês até o momento.
Prof. Marcus Renato de Carvalho
www.agostodourado.com
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Basavarajeeyam is an important text for ayurvedic physician belonging to andhra pradehs. It is a popular compendium in various parts of our country as well as in andhra pradesh. The content of the text was presented in sanskrit and telugu language (Bilingual). One of the most famous book in ayurvedic pharmaceutics and therapeutics. This book contains 25 chapters called as prakaranas. Many rasaoushadis were explained, pioneer of dhatu druti, nadi pareeksha, mutra pareeksha etc. Belongs to the period of 15-16 century. New diseases like upadamsha, phiranga rogas are explained.
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2. • Genetic sex determined at fertilization .
• Gender become apparent in normal fetus by the 12th week
of development .
• By 6th week of life the following structure start to develop
either side of midline :-
• Genital ridge ( induced by primordial germ cell from yolk
sac.
• Mesonephric ( walffian duct ) lateral to genital ridge .
• Paramesonephric duct ( müllerian ) duct lateral to
mesonephric duct .
• By 6th week " Y " OR " X " chromosome produce their effect
leading to differention of gonads into either testis or
ovaries.
3.
4.
5.
6. Testis Produce :-
1)Müllerian inhibiting factor ( MIF ) that inhibits
development of müllerian system .
2)Testosterone that cause development of
(WALFFIAN SYSTEM ).
3)In absence of " Y " chromosome there is lack
of MIF and walffian system regress .
7. • In female fetus the mesonephric duct regress ,
the paramesonephric duct go on to develop into
:-
• Fallopian tube ( upper & middle part of the duct
• Uterus develop from intermediate horizontal
part of the two müllerian duct which fuses
together in the midline .
• Cervix and upper vagina develop from the
caudal part of two müllerian ducts .
8. • Development of lower 1/3 of the vagina the caudal
parts of the two müllerian duct come in contact with
urogenital sinus and produce elevation called müllerian
tubercle , the epithelium overlying the tubercle
proliferate to form 2 small masses called sino-vaginal
bulbs .
• Growth of sino-vaginal bulbs leads to formation of
solid cord called vaginal plate .
• Conalizaton of vaginal plate communicates above with
the caudal part of müllerian ducts and below with
urogenital sinus to forms the lower 1/3 of vagina
9. • Hymen is formed at the point of the origin of
sino-vaginal bulbs (at the junction of vagina
and the urogenital sinus ).
• Development of the external genitalia .
• Clitoris develops from genital tubercle .
• Labia majora develop from the genital
swellings .
• Labia minora lie medial to labia majora
and develop from the genital folds .
10. • The ovary develops from 3 sources :-
1- Coelomic epithelium it consist of mesothelial cells
which lines the coelomic ( peritoneal cavity ) .
These cells cover the genital ridge and forms primitive sex
cords , these sex cords break into separate clusters .
To form primordial follicles .
2- Primordial germ ( sex ) cells : these cell are endodermal
in origin and arise from yolk sac .
They migrate along the hindgut and its mesentery to
reach genital ridge they give rise to oogonia then primary
oocytes and become arrested until puberty .
11. 3- Intermediate mesoderm : it forms the connective
tissue of the ovary .
• The ovary is attached by (gubernaculum ) which
is fibromuscular band of mesodermal origin
which travers the inquinal canal to get attached
to labia majora.
• The gubernaculum is necessary for the descent of
the ovary in the pelvis .
• The gubernaculum forms the ovarian ligament
and the round ligament of the uterus .
12. Conginital Anomalies of Female
Genital System
1- The Ovary :-
* Gonadal agenesis the primordial germ cells
fail to migrate to genital ridge , the ovary is
completely absent , the ovary is represented by
fibrous tissue ( streak gonadal as in turner's
syndrome ).
* Hermaphroditism :-
• True hermaphroditism gonads and external
genitilia of both sexes are present .
• Pseudohermaphroditism gonads of one sex
and external genitilia of other sex .
13. 2- The Uterus :-
• Uterine aplasia or müllerian agenesis " Mayer –
Rokitansky – Kauster – Hauser Syndrome ".
• Uterine hypoplasia .
• Unicornate ulerus uterus with one horn only
with uterine tube , the other horn may present
but rudimentary.
• Uterus didelphys " double uterus " due to
complete failure of fusion of 2 müllerian ducts ,
each duct forms aseperate uterus with its own
vagina
14. • Bicornuate uterus incomplete fusion of 2
müllerian ducts.
• Bicornate uterus with double cervix (uterus bicornis
bicolis) the uterus has 2 horns double cervix and
single vagina .
• Bicornuate uterus with single cervix ( uterus bicornis
unicollis) the uterus has 2 horns but with single
cervix and vagina .
• Septate uterus :- the uterine cavity divided by septum
into 2 compartment after fusion of the two duct , there
is failure of resorption of midline septum ( it differs
from bicornuate uterus in having a normal fundus ).
15.
16. Clinical significance of uterine
anomalies
• Müllerian agenesis ( absent uterus ) primary
amenorrhea and infertility .
• Septate uterus recurrant pregnancy loss 65%
• Unicornuate uterus 50% pregnancy loss .
• Bicornuate uterus and didelphys 40% loss .
• Cervical cerculage improve pregnancy out come in
unicornuate and bicornuate uterus .
• Septate uterus corrected by hystroscopic septoplasty (
septum resection).
• Rudimentary horn ectopic pregnancy .
• Fetal malpresentation
17. 3- The Vagina :-
• Atresia of the vagina due to failure of the vaginal
plate canalization .
• Imperforate hymen due to the vaginal
communication between the vaginal lumen and
urogenital sinus ( crypto menorrhea ) .
• Double vagina due to failure of fusion of coudal
of the 2 müllerian ducts
• Vaginal septum either longitudinal or
transverse .