Primary Hypogonadism
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Primary Hypogonadism
[edit] Klinefelter syndrome
This chromosomal abnormality in males, where there are two or more X chromosomes and one Y chromosome (47,XXY), is found with a frequency of 1 in 800 live male births. These males grow tall and have disproportionally long arms and legs. They get into puberty at the normal age, but facial hair growth is very light.
They have learning difficulties and their small testicles do not produce sperm. However, there is a wide variation and some males with Klinefelter syndrome have normal intelligence. Often this syndrome is only detected in infertility clinics. As testosterone production in the gonads is missing or extremely low, and there are high FSH levels in the blood because of the missing negative inhibition on the hypothalamus.
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[edit] Bilateral anorchia
The other name for this condition is "vanishing testes syndrome". It is thought that during the course of embryonic life the testicular tissue was present, but later during the pregnancy it was resorbed.
The scrotum is empty, but contrary to cryptorchidism where the testicles can be found in the inguinal canal, with bilateral anorchia the testicles are missing. Injection of human chorionic gonadotropin does not lead to a rise in testosterone hormone, because there is no testicular tissue in the body. With cryptorchidism this test leads to a marked testosterone level increase.
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