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  Hypopituitary hypogonadism
 
Authors: Shehzad Basaria, MD, Adrian Dobs Last modified: January 3, 2002
DIAGNOSTIC CRITERIA

  EPIDEMIOLOGY

  • Rare congenital syndromes either with isolated central hypogonadism or associated with defects in the sense of smell (Kallmann's syndrome) or with mental retardation and obesity (Prader-Willi syndrom
  • More frequently, hypopituitary hypogonadism is acquired secondary to pathological processes in the pituitary or hypothalamus.

  RISK FACTORS

  • Neoplastic, vascular and inflammatory diseases of the pituitary or the hypothalamus
  • Brain irradiation
  • HIV infection
  • Aging
  • Drugs that increase prolactin, such as several psychtropic medications (especially antipsychotic) and metoclopramide
  • Renal or hepatic failure
  • Infiltrative diseases like hemochromatosis, sarcoidosis and histiocytosis.
  • Heroin, marjuana

  SIGNS AND SYMPTOMS

  • Reduced libido and or impotence
  • Fatigue
  • Reduced bone mineral density and increased risk of fractures
  • Decrease in body hair.
  • Decrease muscle mass and strength.
  • Visual field defects in patients with pituitary macroadenoma.
  • Signa and symptoms of other pituitary hormone deficiency (adrenal in sufficiency, hypothyroidism).

  DIFFERENTIAL DIAGNOSIS

  • Primary (testicular damage) vs secondary (hypothalamic or pituitary damage) hypogonadism.
  • Hyperprolactinemia.
  • Obesity can result in low testosterone and low or inappropriately normal gonadotropins mimicking central hypogonadism. Usually free testosterone is normal in those circumstances.

  LABORATORY FINDINGS

  • Low serum testosterone with low or inappropriately normal serum FSH and LH
  • Low cortisol with low or inappropriately normal ACTH in central adrenal insufficiency.
  • Low T4 with low or inappropriately normal TSH in central hypothyroidism.
  • Elevated prolactin in prolactinoma.
 
TREATMENT
TESTOSTERONE VIA GEL, PATCHES OR INJECTIONS
  • If fertility is desired, HCG intramuscular injections three times/week are needed, often for several months, to reach fertile sperm count
  • Testosterone therapy is sufficient for those men who don't desire fertility. Androgel and patches need to be applied once a day. Patches have a relatively high incidence of skin reactions (redness an
IF HIGH PROLACTIN IS PRESENT, SPECIFIC PHARMACOLOGICAL TREATMENT SHOULD BE INSTITUTED
  • Treatment with bromocriptine or cabergoline is effective in lowering prolactin levels by shrinking prolactinoma. Cabergoline is better tolerated since it does not have side effects of orthostasis and
  • Once hyperprolactinemia is corrected, testosterone levels return to normal.

more...

 
IMPORTANT POINTS/RECOMMENDATIONS
  • Periodic monitoring of serum PSA for men above 50 is recommended
  • Hematocrit levels should be monitored to prevent polycythemia. Polycythemia is more common with intramuscular injections.
 
REFERENCES
  1. Hardelin JP, Petit C ;  A molecular approach to the pathophysiology of the X chromosome-linked Kallmann's syndrome. ;  Baillieres Clin Endocrinol Metab. 1995;9(3):489-507

    Comment:
    A review of Kalmann's syndrome, the most prevalent form of congenital hypogonadotropic hypogonadism

  2. Layman LC ;  Genetics of human hypogonadotropic hypogonadism. ;  Am J Med Genet. 1999 29;89(4):240-8

    Comment:
    Comprehensive review of genetic causes of hypogonadotropic hypogonadism

  3. Siemons LJ, Tyson JE, Tolis G. ;  Hypogonadotropic hypogonadism in hemochromatosis: Recovery of reproductive function after iron depletion. ;  J Clin Endocrinol Metab 1987; 65:585.

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