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Amenorrhea (Hypergonadotropic Hypogonadism Increased LH/FSH Low Estrogen…
Amenorrhea
Hypergonadotropic Hypogonadism
Increased LH/FSH
Low Estrogen
Primary Defect in Ovary
Pure (complete) gonadal dysgenesis)
46 XX
Phenotypic female
Streak gonad, nl external and internal genetalia
Normal Height (2 Shox genes)
Arms 5cm>height
Tanner 1-2 Breast
Tanner 2-3 Pubic Hair
46 XY
Phenotypic female
Streak gonad (may have taste remnant--could lead to CA)
Female external and internal genetalia
Tanner 1 Breast
Tanner 3 Pubic
Normal Height
Arms 5cm> height
17 Hydroxylase Deficiency (46 XX or 46 XY)
Phenotypic female
Hypertension
Decreased RAAS
Hypokalemia
Metabolic Alkalosis
Increased Progesterone
Low 17 Hydroxyprogesterone
No high Aldo because ATII decreases it
Tall stature
Arms 5cm>height (no estrogen to close growth plates)
Tanner 1: breast
Tanner 1 Pubic
No adrenal sex hormones either
Adrenal DOC has mineral activity
Corticosterone has cortisol activity
Increased progesterone
46XX have ovaries, uterus, vagina, FT.
46 XY have testes:
-AMF: Don't have uterus or FT,
-No Test: no epidymus, Vas Deferens.
-Shallow vagina, clit, labia (no DHT)
Turner syndrome (XO)
*Most common cause
Shield chest, webbed neck, bicusp valve, coarc of aorta, nl external and internal genitalia
Short Stature due to 1 SHOX gene
Arms 5cm > height
Tanner 1 Breast
Tanner 3 Pubic
*could be normal if mosaic
Ovarian Insensitivity Syndrome (Savage)
:warning: Nl LH
Lack nl FSH receptors in ovarian follicles or have post-receptor defect
Normal height
Arms 5cm>height ?
Tanner breast immature depending on receptor block
Pubic hair is normal unless LH receptors are also affected
Hypogonadotropic Hypogonadism
Low FSH/LH
Low Estrogen
Primary Fxn Hypothalamic Amenorrhea
Due to stress, weight loss, severe exercise, low caloric intake
Usually cause of secondary amenorrhea
CRH, gherkin and leptin prevent GnRH pulse generator
Serum FSH nl
LH low-nl--> chronic an ovulation and chronic hypoestrogenism
Lack of cycle changes in circulation estrogen and prog abolishes endometrial cyclicality. Stuck in early proliferating phase
Sufficient ovarian androgen and estrogen production for Tanner stage 4 pubic hair and breast development in most cases
Constitutional Delay of Puberty
Strong genetic component
Delayed growth spurt
Delayed reactivation of GnRH pulse generator
Prepubertal levels of FSH , LH, Estrogen are present
Short but achieve nl height
Tanner 1-2 breast
Tanner 2-3 Pubic
Isolated hypogonadotropic Hypogonadism
Kallmans Sydrome
(could have renal genesis, deafness, cleft palate, syndactily)
Yes or no Anosmia
Child-like internal and external genital organs
Arm span 5cm>height
Breast Tanner 1
Tanner 2-3 pubic hair (adrenal production)
Absent axillary hair
Craniopharyngioma
Eugonadrotropic hypogonadism
Nl sex hormones
46 XX Mullerian Agenesis
Nl ovulation
Nl development
Nl external genetalia
Vag opening <2cm in depth
Nl height
Tanner stage 4-5 breast
Tanner stage 5 pubic
Complete androgen insensitivity syndrome (x linked) 46 XY
Functioning testes but no functioning AR during prenatal and postnatal life
Maternal hCG causes testes to produce Te and AMH.
No uterus, FT, or Distal vag.
Wolf never develops --> no epidy, VD, SV
Female external genetalia
At puberty--Hight LH and high Testosterone which aromatizes to estrogen (nl for woman)
Nl height
Nl epiphyseal closure
Tanner 4
breast
but not 5 because no prog
Tanner 1 pubic hair or terminal hair anywhere
Genital outflow tract obstruction
Imperf hymen, transvers vag septum, labial fusion
Normal breast and pubic hair
Cryptomenorrhea, cyclic pain, difficulty urinating every 25-35 days
5 reductase def (autosomal recessive)
Some newborns have female ext genetalia with clitoromegaly
blind vag pouch
no uterus, FT, nl testes in inguinal region/abd
**Virilization at puberty for male pattern terminal hair growth
Nl Lh, FSH, nl-elevated test, DHT low
No breast
development - test inhibits FSH and LH
Tanner 4-5 pubic hair