Cah 3 beta hydroxysteroid dehydrogenase deficiency

Much of our modern understanding and treatment of CAH comes from research conducted at Johns Hopkins Medical School in Baltimore in the middle of the 20th century. Lawson Wilkins , "founder" of pediatric endocrinology , worked out the apparently paradoxical pathophysiology: that hyperplasia and overproduction of adrenal androgens resulted from impaired capacity for making cortisol. He reported use of adrenal cortical extracts to treat children with CAH in 1950. Genital reconstructive surgery was also pioneered at Hopkins. After application of karyotyping to CAH and other intersex disorders in the 1950s, John Money , JL Hampson, and JG Hampson persuaded both the scientific community and the public that sex assignment should not be based on any single biological criterion, and gender identity was largely learned and has no simple relationship with chromosomes or hormones. See Intersex for a fuller history, including recent controversies over reconstructive surgery.

Assigning a sex of rearing to a child never requires surgery. Genital or gonadal surgeries on intersex children too young to declare their gender identity carry the risk of surgically assigning the wrong sex. Depending on the condition, this risk can be between as high as 40 percent—meaning that many children will grow up to reject the sex that has been irreversibly surgically assigned to them. This means that for conditions where it is not possible to predict gender identity outcomes with confidence, doctors are conducting sex assignment surgeries based on guesswork.

Signs of hyperestrogenism may include heightened levels of one or more of the estrogen sex hormones (usually estradiol and/or estrone ), lowered levels of follicle-stimulating hormone and/or luteinizing hormone (due to suppression of the hypothalamic–pituitary–gonadal axis by estrogen), and lowered levels of androgens such as testosterone (generally only relevant to males). [1] Symptoms of the condition in women may consist of menstrual irregularities , amenorrhea , abnormal vaginal bleeding , and enlargement of the uterus and breasts . [1] [2] It may also present as isosexual precocity in children [1] [2] and as hypogonadism , gynecomastia , feminization , impotence , and loss of libido in males. [3] If left untreated, hyperestrogenism may increase the risk of estrogen-sensitive cancers such as breast cancer later in life.

Cah 3 beta hydroxysteroid dehydrogenase deficiency

cah 3 beta hydroxysteroid dehydrogenase deficiency

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