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27 Cards in this Set

  • Front
  • Back
What is the definition of primary amenorrhea?
never had menstruation
What is secondary amenorrhea?
when an established menstruation has stopped for a certain period of time
What is the karyotype of a person with Turner's Syndrome?
45,x (Monosomy X)
What is gonadal dysgenesis?
• generally refers to a condition where gonadal development is abnormal

• often only presenting streaks of connective tissue
What are characteristics of Turner's Syndrome?
• ovaries fail to develop

• estrogen deficiency » leads to sexual infantilism

• has increased levels of LH and FSH (due to negative feedback to the pituitary)
What are physical characteristics of Turner's Syndrome?
• coarctation of the aorta
• cubitus valgus (elbows that are turned out)
• epicanthal folds
• fish-like mouth
• low set ears
• most patients are short (between 48-58 inches)
• neonatal lymph edema
• shield-like chest w/ widely spaced nipples
• short, webbed neck
What methods are used to investigate for Turner's Syndrome?
• Karyotype (most common; analyzes the chromosomal composition of the indivdual)

• Pelvic ultrasound (would see no ovaries OR a strip)

• Gonadotropin levels (would be elevated)
What is the treatment of Turner's Syndrome?
• no cure, but can minimize symptoms

• growth hormone (will improve growth)

• estrogen replacement therapy (to promote the development of secondary sex characteristics)

• surgical removal of streak gonads (to reduce risk of ovarian cancer)
Can a patient with Turner's Syndrome become pregnant?
Yes. Patients with Turner's Syndrome can use reproductive technolgies and carry a donor egg. Uterus is still capable of carry a pregnancy
What is the karyotype of patients with Testicular Feminization Syndrome?
46, XY
What are characteristics of patients with Testicular Feminization Syndrome?
• patients are genetic males
• they have normal female external genitalia
• have resistance of target tissues to action of androgen (target cells do not respond to testosterone)
What prevents patients with Testicular Feminization Syndrome from developing fallopian tubes, a uterus, and an upper vagina?
fetal testes produce mullerian duct inhibiting factor which prevents the development of internal female reproductive organs
What is the treatment/management of patients with Testicular Feminization Syndrome?
• surgical removal of the testes (to prevent chances of malignancy)

• lifelong estrogen therapy
What are other causes of primary amenorrhea?
• Delayed Menarche
• Hypogonadotropic hypogonadism
• Isolated gonadotropin deficiency
• Panhypopituitarism
• Resistant Ovary Syndrome
What is the most common cause of secondary amenorrhea?
What are characteristics of Polycystic Ovary Syndrome?
• female hyperandrogenism
• increased release of LH relative to FSH, resulting in an increased production of androgens (from Currents)
• results in anovulation, multiple cysts, and ovarian theca cell hyperplasia

* Santana said LH would be low; Current says increased release of LH (pg 765)
Polycystic ovary syndrome is seen most commonly in what age group?
• seen most common post-puberty and women between 20-30 years of age

• associated with a family history of PCOS
What are clinical features of polycystic ovary syndrome?
• hirsutism
• infertility
• obesity
• oligomenorrhea (decreased menstruation)
What tests would you do when diagnosing polycystic ovary syndrome?
• serum LH and/or testosterone concentrations

• ultrasound (would see cysts)
How do you manage a patient with polycystic ovary syndrome?
• estrogen and cyproterone acetate therapy (for Hirsutism)

• menstrual disturbance often responds to combined oral contraceptive pill

• oral clomiphene citrate or parenteral gonadotrophins (to treat anovulation)
What percentage of women with PCOS, who present with anovulatory infertility, will respond to ovulation induction?
What is the cause of Male Hypogonadotrophic Hypogonadism?
deficiency in FSH and LH
What other abnormalities should be investigated for patient with Hypogonadotrophic Hypogonadism?
• the deficiency in FSH and LH may be isolated or associated with other pituitary hormonal abnormalities

• patients must evaluated for signs of:
• Cushings syndrome
• Adrenal Insufficiency
• GH excess or deficiency
• Thyroid hormone excess or deficiency
What are possible causes of testicular failure?
• Cancer chemotherapy
• Irradiation
• Viral infection (mumps)
What are clinical features of hypogonadotrophic hypogonadism?
Depends on age of onset of androgen deficiency

• Cryptorchism
• Decreased libido
• Hypospadias
• Impaired sexual function
• Late closure of epiphyses
• Microphallus
• Scant body and facial hair
What test should be done when investigating for hypogonadotrophic hypogonadism?
• full pituitary function tests
• serum testosterone, LH, & FHS measurements
• pituitary CT or MRI
How do you manage a patient with hypogonadotrophic hypogonadism?
• androgen replacement therapy

• gonadotrophins (LH & FSH) are required to induce spermatogenesis