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Male Gonadal Disorders
Terms in this set (40)
Endocrine function - Leydig cells of the interstitial compartment produce testosterone. 2% is free (unbound) and is biologically active.
Exocrine function - Sertoli cells produce products to nurture germ cell growth. They are sometimes called 'nurse cells'.
Normal semen analysis results?
Normal men produce more than 20 million sperm/ml of ejaculate, or >40 million sperm per ejaculate.
50% of the sperm should be motile.
25% of the sperm should be moving forward rapidly.
More than 50% should have normal morphology.
Must demonstrate two normal samples.
-non descent of the testicle
urethral opening on the inferior side of the penis
urethral opening on the superior side of the penis
Male hypogonadism =
-a decrease in either of two major functions in the testes:
1) sperm production, or
2) testosterone production
-a result of?
1) disease of the testes (primary hypogonadism),
2) disease of the pituitary or hypothalamus (secondary hypogonadism).
Lab results of primary hypogonadism?
serum testosterone and/or sperm count are subnormal, while the serum LH and/or FSH concentrations are ABOVE normal.
Lab results of secondary hypogonadism?
serum testosterone and/or the sperm count are subnormal, and the serum LH and/or FSH concentrations are normal or reduced.
Three hypogonadal states?
Primary, or HYPERgonadotropic hypogonadism
Secondary, or HYPOgonadotropic hypogonadism
Androgen resistance - typically seen in intersex states, such as those associated with congenital adrenal hyperplasia. Problem is caused by defective androgen action, primarily due to defective receptor activity.
Primary, or HYPERgonadotropic hypogonadism
-damage to the Leydig cells impairs testosterone production and/or damages the seminiferous tubules, with resulting oligospermia or azoospermia and elevated LH and FSH, in a setting of low testosterone.
Infertility due to primary hypogonadism does not respond to hormonal therapy.
Causes of Primary (HYPERgonadotropic) hypogonadism?
Klinefelters Syndrome =
The most common genetic reason for azoospermia (1 in 500 males). Seminiferous tubule dysgenesis.
The classic traid is small, firm testes; gynecomastia; and azoospermia. (Though only about 30% have the gynecomastia.)
Can present with delayed maturation, increased height, decreased intelligence, obesity, and diabetes, and is often only diagnosed at puberty when there is inadequate sexual development.
20X higher risk of breast CA than in normal males.
The low testosterone levels and elevated LH and FSH, are accompanied by an increase in estradiol.
Diagnosis can be made by karyotype analysis of blood lymphocytes.
Will respond to testosterone replacement with development of secondary sex characteristics: pubic hair, testicular enlargement, muscle growth, etc.
Autosomal dominant mutation on chromosome 12 -->
- short stature, shortened 4th metacarpals
- hyperelastic skin, ptosis, low-set ears
- Right-sided heart abnormalities (particularly pulmonic stenosis, atrial septal defect, and hypertrophic cardiomyopathy).
- often also cryptorchidism, mental retardation, and chest deformity
-auto. dominant disorder that leads to muscle atrophy.
- usually accompanied by hypogonadism which is recognized
only at adulthood
- characteristically, have frontal balding, mental retardation, cataracts, diabetes, primary hypoparathyroidism, cranial hyperostosis.
All have FSH elevated, 50% with low LH and low testosterone
Vanishing testes syndrome - testes resorbed before birth, but were present during first trimester, enabling development of normal-appearing external genitalia
- patients appear similar to those with bilateral cryptorchism, but these patients have no testes at all.
FSH, LH elevated, testosterone low.
Secondary (Hypogonadotropic) Hypogonadism:
-Disorders which include hypothalamic and pituitary deficiencies due to genetic or other causes.
-Impotency and/or infertility are frequent problems.
-FSH and LH are normal or low (unlike in primary hypogonadism, in which they are high) and testosterone is low.
Causes of Secondary (Hypogonadotropic) Hypogonadism?
Congenital Hypogonaditropic Syndromes
Isolated LH Deficiency
Isolated FSH Deficiency
X-linked inherited hypothalamic disorder --> ansence of GnRH
-anosmia (loss of sense of smell) due to agenesis of the olfactory lobes and hypogonadism 2° deficiency of hypothalamic GnRH (gonadotropin- releasing hormone) are seen.
- microphallus and cryptochism or severely atrophic testes
- color blindness, deafness, cleft palate
- low testosterone, low LH, and low FSH
Fertility and virility are restored by giving hormone replacement
Prader Willi Syndrome:
Problem with Chromosome 15
Features: genetic obesity, retardation, small hands/feet, short stature, hypogonadism.
Involves several chromosomes.
Features:mental retardaton, retinitis pigmentosa, polydactyly, cardiomyopathy, and hypogonadism.
-Sickle cell can cause pituitary and testicular micro-infarcts; results in low testosterone and variable LH and FSH levels.
- Hemochromatosis causes iron deposition in the liver, testes, and pituitary and is associated with testicular dysfunction in 80% of cases
Isolated LH Deficiency:
In this very rare condition, LH is sufficient to promote enough testosterone for spermatogenesis, but not eneough testosterone for normal virilization.
1. normal sized testes
3. normal FSH, low-normal
LH and testosterone
Isolated FSH Deficiency:
In this extremely rare condition, the pituitary is not producing enough FSH. LH production is normal.
1. oligospermia or
2. normal virilization
3. normal LH and
testosterone, low FSH
Elevated prolactin from a pituitary adenoma can cause low FSH, LH and testosterone
Symptoms are infertility, impotence, and loss of libido.
This can occur with increased conversion of androgen to estrogen in extraglandular tissue, such as in hepatic cirrhosis and excessive obesity. Some testicular tumors form estrogen. Exogenous estrogen exposure from beef and pesticides may be a factor.
Excess androgens can suppress pituitary gonadotropin secretion and lead to secondary testicular failure. Anabolic steroids can do this, as can congenital adrenal hyperplasia, which produces large amounts of androgenic steroids.
95% of testicular tumors are _______________________, the other 5 % are ________________
Primary germ cell tumors
sec cord stromal tumors (affecting the leydig/sertoli cells)
Testicular cancer presentation?
painless testicular mass, or as a testicular swelling with vague discomfort, suggesting a mild epididymitis, though there will be no response to antibiotics
Staging germ cell tumors?
Stage I = limited to the testis, epididymis, or spermatic cord.
Stage II = limited to retroperitoneal (regional) lymph nodes
Stage III = outside the retroperitoneum, involving subdiaphragmatic nodes or the viscera
Germ Cell Tumor Markers:
beta - hCG is the most commonly elevated tumor marker in testicular cancer, but tumor histology determines which particular tumors produce hCG.
-Alpha fetoprotein (AFP)
-Lactate dehydrogenase (LDH) a
Two categories of germ cell tumors?
seminomas (less aggressive) and nonseminomatous germ cell tumors (more aggressive, metastasize early to retroperitoneal nodes and the lungs)
Four histologies of NON-seminomatous GCT's?
endodermal sinus tumor
Outcomes for testicular cancer?
-quite responsive to chemo!
Stage I and II seminomas have > 90% cure
Stage I and II non-seminomas have > 95% cure
NON germ cell tumors?
Leydig Cell tumors
Sertoli Cell Tumors
Rx with orchiectomy (sometimes bilateral)
-estrogen like compounds
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