السبت، 11 مارس 2017

Embryology of Female genital tract, Malformations, intersexuality and Puberty:


Embryology of Female genital tract, Malformations, intersexuality and Puberty:


In patient with bicornuate uterus when getting pregnant can get all these complication, EXCEPT:
Polyhydramnios
Abortion
Preterm labor
Abnormal fetal lie
Retained placenta

In Turner's syndrome:
A chromosomal structure of 45 XY is characteristic
Secondary amenorrhea is usual
Ovaries are streak
The ovaries are multicystic
Phenotypically are male

Sexual differentiation
Development of male genitalia depends on the presence of functioning testes and responsive end organs
Due to the absence of testes XX fetus exposed to androgens in uteri will NOT be musculinized
The development of the female genital requires presence of the ovary
45 XO fetus will have normal ovaries
The development of the testes does not require presence of the Y chromosome

Turner syndrome: Which is true?
Genetically is 46 X O
Has testis in inguinal area
Usually presents with primary amenorrhea.
Has low I.Q
Usually tall

Androgen insensitivity syndrome: Which is true?
Genotype is 46 XX
Phenotype they are female but with ill-developed breast
Usually have secondary amenorrhea
They have testes that should be kept to produce hormones
They have no uterus


The karyotype of patient with Androgen insensitivity Syndrome is
46XX
46XY
47XXY
45XO
45 XY

In Turner syndrome the following are usually present EXCEPT:
the ovary are usually well developed.
The nipple are widely spaced
The girls are of short stature
Has web neck
The condition can be diagnosed by chromosomal

Regarding Androgen insensitivity syndrome all true EXCEPT:
The chromosomal sex is 46XX.
Scant or no pubic and axillary hair.
No uterus
Normal female external genetailia
Breast are usually well develop

Which is not true about Turner's syndrome:
Short stature
Buccal smear is chromatin positive
The majority are 45XO
Very low urinary estrogen titer
High pituitary gonadotropin titer.

Androgen Insensitivity Syndrome:
The characteristic features include normal uterus and breast development, and ambiguous genitalia.
The Karyotyping is 46XX.
They have normal female testosterone level.
Gonadectomy must be performed after puberty because of the increased risk of malignancy.
Estrogen replacement therapy is not indicated because they have enough estrogens to produce breast development.

While evaluating a 30-year-old woman for infertility, you diagnosed a bicornuate uterus. You explain that additional testing is necessary because of the woman's increased risk of congenital anomalies in which system?
Skeletal.
Hematopoietic.
Urinary.


Central nervous.
Tracheoesophageal.

Development stage:
Testosterone is secreted by Sertoli cells.
Anti-müllerian hormone is secreted by Leydig cells.
Anti-müllerian hormone is responsible for involution of normal müllerian system.

Bicornuate uterus can cause all of the following EXCEPT:
Abortions
Abnormal fetal lie.
Infertility.
Retained placenta.
Congenital anomalies of the baby.

In Turner’s syndrome patients, all of the following are true EXCEPT:
The streak ovaries should be removed surgically due to 25% tendency to be malignant.
Are usually less than 5 feet tall.
Have raised FSH levels.
Have female internal genitalia.
Have normal but infertile external genitalia.

The adenxea Uteri include all of the following EXCEPT:
Ovary.
Fallopian tubes.
Uterus.
Broad ligament.
Round ligament.

In the development of external genitalia:
Genital tubercles from the labia minora.
Genital smoothing from the labia majora.
Genital fold from scrotum in male.
Chlydrotestone is essential for muscularity of external genitalia.
Müllerian system develops to external genitalia.

Congenital uterine malformations causes all of the following EXCEPT:
Spontaneous abortions.
Premature labor.
Pregnancy induced hypertension.
Abnormal fetal lie.
May Obstruct labor.


Ovarian Dysgenesis is associated with the elevation of which of the following hormones.
Pituitary Gonadotropins.
Estradiol.
Estriol.
Pregnandiol.
Progesterone.

Confirmation of the diagnosis of Turner syndrome is best done by:
Gyn PV examination.
Pregnantriol estimation.
Hysterosalpingography (HSG)
Chromosomal analysis (Karyotyping).
Estimation of hypophyseal gonadotropins.

In cases of androgen insensitivity syndrome the following findings are true EXCEPT:
The chromosomal sex is 46 XX
Scant or no pubic or axillary hair.
No uterus.
Normal female external genitalia.
Breast are usually well developed.

In testicular feminization syndrome:
There are usually normal testes.
Kalman's syndrome is a recognized cause.
Breast development is usually lacking.
There's usually very low testosterone level.
The karyotype is 46 XX.

In Turner's syndrome, the following are usually observed EXCEPT:
The ovaries are usually well developed.
The nipples are widely spaced.
The girl is of short stature.
Has a webbed neck.
The condition can be diagnosed by chromosomal analysis.

Regarding the Development of internal genital organs:
The uterus, fallopian tubes, cervix & upper vagina develop from the mesophrenic duct.
The testes secretes testosterone which cause regression of the müllerian ducts in the male fetus.
The vagina is formed by the Urogenital sinus.


Müllerian agenesis (Mayer- Rokittanky - Kuster - Huser syndromes) is characterized by the absence of the uterus & upper vagina with normal female external genetailia & 46 XX.
Bicornuate uterus & uterus didelphus result from vertical fusion defects of the müllerian ducts.

Regarding puberty, all of the following are true EXCEPT:
It is the transitional period of development during which an individual matures from childhood to sexual & reproductive maturity.
Breast budding is the 1ST visible sign of puberty.
The maximum growth velocity occurs at 12 years.
The age of menarche has decreased over the last 3-4 decades due to improved nutrition, general health & lifestyle changes.
Thelarche marks the attainment of reproductive maturity.

Turner's syndrome is associated with :
Absent uterus
Normal breast development
Primary amenorrhea
Hirsutism
Normal height

Anomalies or the uterus can be associated with the following EXCEPT:
Urinary tract abnormalities.
Recurrent pregnancies loss.
Preterm labor.
Müllerian tract anomalies.
F.    Polycystic ovary.

First endocrine influence in 2ry sexual characters:
Secretion of delta-4 Androstenedione from the adrenal gland
Pineal body maturation
Pituitary maturation
Hypothalamic down regulation.

Follicular growth (in non-ovulating follicle) is usually followed by:
Ovulation.
Cyst formation.
Atresia.
Arrest.
Regression.


The normal sequence of pubertal changes in the female is:
Thelarche, Maximal growth velocity, menarche.
Maximal growth velocity, Thelarche, menarche.
Thelarche, menarche, maximal growth velocity.
Menarche, maximal growth velocity, Thelarche.
Menarche, body weight, Thelarche.

The barr body is:
The condensed nonfunctioning X chromosome.
The darkest, widest band found on chromosomes.
On extra lobe on the female polymorpholnuclear leukocytes.
Found only in females.
The largest chromosome in the female genotype.

The most common cause of precocious puberty is :
Idiopathic.
Gonadoblastoma.
Albright syndrome.
Abnormal skull development.
Granulosa cell tumor.

The normal sequence of puberty is :
Thelarche , adrenarche, growth, menarche.
Menarche, adrenarche, thelarche ,growth.
Growth. thelarche, adrenarche, menarche
Adrenarche , thelarche, growth, menarche
Thelarche , menarche, adrenarche, growth.

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