Testicular failure

Arbizu, T., Santamaria, J., Gomez, J. M. et al. A family with adult spinal and bulbar muscular atrophy, X-linked inheritance and associated testicular failure./. Neurol. Sci. 59 371-382,1983. 

Testicular failure may occur before puberty and present as delayed puberty and the eunuchoid phenotype, or after puberty, with the development of infertility, impotence, or decreased libido in otherwise fully virilized males. The source of hypogonadism can be testicular, as occurs in primary hypogonadism, or it may result from abnormalities of the hypothalamic-pituitary axis, as in secondary hypogonadism. 

G. Other considerations Recombinant follitropin alfa has been designated an orphan product for the induction of spermatogenesis in men with primary and secondary hypogonadotropic hypogonadism in whom the cause of infertility is not due to primary testicular failure. 

Replacement Therapy. Testosterone and other androgens are administered as replacement therapy when the endogenous production of testosterone is impaired. Such conditions include removal of the testes (orchiectomy), various intrinsic forms of testicular failure (cryptorchidism, orchitis), and problems in the endocrine regulation of testosterone production, such as lack of LH production and other forms of hypogonadism.32,144... 

The prime indication is testicular failure which may be primary or secondary (due to lack of pituitary gonadotrophins). In either case replacement with androgens is often necessary. 

It has been reported that -50% of infertility problems are male in origin. However, male infertility often goes undetected, because low sperm count or abnormal sperm motility, combmed with normal female reproductive function, merely results in delayed conception. Most infertile men (80%) will not have a definable cause and are said to have so-called idiopathic impaired sperm function. In about 20% of cases, a cause can be determined. A study at Monash University Infertility Clinic in Australia indicated that primary testicular failure accounted for 11% of male patients seen 8.5% had genital tract obstruction, 0.5% had hypothalamic-pituitary disease, and less than 1% had coital disorders or abnormal sperm. A fist of the most common male infertility factors is given in Box 53-4. 

Exogenous androgens Thyroid disorders Adrenal hyperplasia Testicular failure... 

Other endocrine causes of infertility include exogenous androgens, thyroid disorders, adrenal hyperplasia, and testicular failure. Gynecomastia or obesity in the infertile male may signify elevated concentrations of estrogen and possibly testicular feminization syndrome (see earlier section on male reproductive abnormalities). ... 

Li M, Fogarty J, Whitney KD, Stone P. Repeated testicular infarction in a patient with sickle cell disease A possible mechanism for testicular failure. Urology 2003 62 551. 

Primary testicular failure. Where both the interstitial cells and tubules are damaged. FSH and LH will be elevated and testosterone reduced. Where tubular function only is impaired. FSH is selectively increased and androgen levels may be normal. 

Primary testicular failure Hypogonadotrophic hypogonadism Hyperprolactinaemia... 

Men with infertility also can benefit from therapy with follitropin alfa if their infertility is related to hypothalamic or pituitary hormonal dysfunction and not primary testicular failure, because it... 

Recently a new copper transport defect was described by Buchmann et al. [16] in a patient with symptoms of a demyelinating neuropathy, chronic intestinal pseudo-obstruction, osteoporosis, testicular failure, retinal degeneration and a cardiomyopathy with a tortuous aorta. In this patient, copper could not be incorporated into ceruloplasmin. The symptoms started at age 10 and the patient died at 21 years. These findings were clearly different from Wilson s and Menkes diseases. 

High serum FSH levels suggest ovarian failure in females or primary testicular failure in males. Low FSH levels suggest hypothalamic or pituitary dysfunction. 

A test of testicular function based on the fact that human chorionic gonadotrophin (HCG) resembles LH in its actions and stimulates the Leydig cells of the testis to secrete testosterone. It consists of injecting HCG and then measuring plasma testosterone in samples collected over several days. Patients with primary testicular failure fail to respond. 

In males, a high plasma LH level indicates primary testicular failure. 

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