McCune-Albright Syndrome

Precocious Puberty.

 

     When the signs of puberty (development of breasts, testes, pubic and underarm hair, body odor, menstrual bleeding and increased growth rate) appear before the age of 8 years in a girl and 9 1/2 in a boy, it is termed 'precocious puberty'. In the most common form of precocious puberty, there is early activation of the regions in the brain which control the maturation of the ovaries in a girl and the testes in a boy. One brain center, the hypothalamus, secretes a substance called gonadotropin-releasing-hormone, or GnRH. This acts, in turn, on another part of the brain, the pituitary gland, to cause increased secretion of hormones called gonadotropins (LH and FSH) that travel through the bloodstream and act on the ovaries or testes to stimulate secretion of estrogen or testosterone. However, after studying many girls with MAS, it has been learned that most do not appear to have early activation of the hypothalamus and pituitary, because their levels of LH and FSH are usually low, or similar to those of prepubertal children. The precocious puberty in MAS girls is caused by estrogens which are secreted into the bloodstream by ovarian cysts, which enlarge and then decrease in size over periods of weeks to days.
Pam's Note: Although this sounds rather clinical and frightening, especially the part about cysts, I don't know that all girls with MAS have the cysts. Hannah was never diagnosed with any cysts; however, it's true that she rarely saw a doctor until all this started for us, because she was never sick, and I'm not much on going to the doctor to see "how you are growing". I'm not against that, you understand, I've just never been one to do that. So if Hannah indeed had the cysts causing this early puberty, it's obvious in her case that it was not serious enough for surgery. Every case will differ, so don't panic!
Frequently, menstrual bleeding and breast enlargement accompany the growth of a cyst. In fact, menstrual bleeding under 2 years of age has been the first symptom of MAS in 85% of patients. Although ovarian cysts and irregular menstrual bleeding may continue into adolescence and adulthood, many adult women with MAS are fertile and can bear normal children.

 

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