Last Day Flashcards
Follistatin
Testicular Peptide hormones
-inhibits activins and inhibing
Activins
Testicular Peptide hormones
- produced in sertoli cells
- stimulate FSH beta-subunit production
Inhibins
Testicular Peptide hormones
- produced in seminiferous tubules and sertoli cells
- suppresses FSH secretion
- injury to seminiferous leads to elevated FSH
Primary Hypogonadism
-failure of testes
Secondary Hypogonadism
-non-testicular condition including hypothalamic and pituitary diseases
Hypothalamic disorders
-low GnRH leads to failed LH and FSH production
Pituitary disorders
-low LH and/or FSH production leads to failed testosterone production and/or spermatogenesis
Gonadal Disorders
-failure of testosterone production from Leydig cells and/or spermatogenesis, no feedback leads to elevated LH and FSH
Post-Gonadal Disorders
-defects in testosterone receptor function
Kallman’s Syndrome
Hypothalamic Disease - inappropriately ‘normal’ LH and FSH with low testosterone
-anosmia
Prader-Willi Syndrome
Hypothalamic Disease - inappropriately ‘normal’ LH and FSH with low testosterone
-obesity, hyperphagia, hypotonia, micropenis, small hands & feet
Lawrence-Moon Syndrome
Hypothalamic Disease - inappropriately ‘normal’ LH and FSH with low testosterone
-retinitis pigmentosa polydactyly
Fertile Eunuch Syndrome
Pituitary Disease - nappropriately ‘normal’ LH and FSH with low testosterone
-LH deficiency
Pre-pubertal Gonadal Failure
- small testes, phallus, & prostate
- delayed puberty
- scant pubic and axillary hair
- disproportionately long arms & legs (delayed epiphyseal closure)
- Reduced male musculature
- Gyneocomastia
- Persistently high-pitched voice
Post-pubertal Gonadal Failure
- progressive decrease in muscle mass
- loss of libido
- impotence
- oligospermia or azoospermia
- occasionally, menopausal-type hot flushes (with acute onset of hypogonadism)
- poor ability to concentrate
Evaluation of hypogonadal patient
History: sexual function, family, fertility status
Physical Exam: arm span to height, axillary/pubic hair, phallus and testes
Lab: Test. FSH, LH, Prolactin, Karyotyping
Provacative Testing: GnRH stimulation, Clomiphene stimulation, hCG stimulation
Pituitary MRI
Isolated FSH Deficiency
Pituitary Disease - nappropriately ‘normal’ LH and FSH with low testosterone
Hyperprolactinemia
Pituitary Disease - nappropriately ‘normal’ LH and FSH with low testosterone
-inhibited GnRH release and libido
Hemochromatosis
Pituitary Disease - nappropriately ‘normal’ LH and FSH with low testosterone
-loss of LH and FSH (also effects testes directly and can cause a primary hypogonadism)
Testicular Diseases
High LH and FSH due to absent feedback of testosterone
- bilateral anorchia (vanishing testes syndrome)
- chyptorchidism
- sertoli cell only syndrome
- myotonic dystrophy
- gonadotoxins
- chemo
- radiation
- orchitis (mumps)
- systemic illness
- hemachromatosis
Testosterone
- levels vary from hour to hour
- normally highest levels in the early morning hours
- circulating bound to sex hormone-binding globulin (SHBG) and albumin
- only ~2% of total hormone is free for biological availability
- normal total test. can be seen in bypogonadal patients with increased SHBG in whom the available test. is truly low
- SHBG increase about 1% per year with aging
- equilibrium dialysis measures of free test. are most accurate
What can cause low SHBG?
hypothyroidism, acromegaly and obesity
Gonadotropins
- LH and FSH are both released in pulsatile fashion
- LH has a shorter plasma half-life than FSH and single low measures may be misleading
- biologic activity is affected by post-translational glycosylation and 2-site radioimmunometric assays yeild results with correlate well with biologic activity
Prolactin
- unusual in that it is tonically secreted and requires dopaminergic signaling from the hypothalamus to down-regulate its release
- directly down-regulates release of FSH and LH
- directly decreases libido independent of testosterone levels
Semen Analysis
- primary test to assess fertility potential of male
- should be collected after 2-5 days of abstinence and evaluate within 2 hours
- volume should range from 1.5-6mL
Fertile Sperm Sample
- motility of more than 50%
- sperm count that exceeds 20 million/mL
Klinefelter’s Syndrome
- chromosomal disorders
- XXY
- tall, gynecomastia, eunuchoid habitus, MR common
XYY
- chromosomal disorders
- oligo/azospermia
Noonan’s Syndrome
- chromosomal disorders
- XO
- phenotypically similar to Turners patients but male
XX Male Syndrome
- chromosomal disorders
- normal height, no MR, azospermia
Male primary sex organ?
-testes
Testes
1) located in scrotum (temp. regulating system)
2) need temp <37C for optimal spermatogenesis
3) blood supply via pampinoform pelxis (countercurrent heat exchanger)
4) testicular movement: cremaster muscle
5) scrotal movement and sweating
Secondary Sex Organs
1) genital tract
2) accessary glands
Genital tract
epididymis, vas deferens, ejaculatory duct, urethra (and penis)
Accessary glands
- bulbourethral glands
- seminal vesicles (60% of seminal volume, contains prostaglandins, fructose, cirtic acid)
- prostate (20% seminal volume)
Permanence/reversibility of pubertal changes
- (by dec. gonadotropin or androgen secretion)
- spermatogeness, size, secretions, content (fructose, citrate) or sex accessor glands