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中間性
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中間性性象譜 |
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中間性的原因-
激素因素
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先天性腎上腺增生性肥大(Congenital
Adrenal Hyperplasia, CAH) |
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激素失衡
有關先天性腎上腺增生性肥大的三種主要的激素是：
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糖皮質激素(Cortisol)
(對抗心理壓力、損害和感染；協助控制血糖水準)；
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醛固酮(Aldosterone)
(為調節腎臟功能所需；協助重吸收鹽)；
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雄激素(Androgens),
其中一種是睾丸酮(為身體健康發育所需)。
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罹患先天性腎上腺增生性肥大者，上述三種激素的綜合平衡被打破：糖皮質激素和醛固酮分泌過少，而雄激素水準卻太高。這能夠導致許多非常嚴重的生理問題，其中像鹽分丟失等。同樣的道理：罹患先天性腎上腺增生性肥大的女孩可能具有“莫淩兩可”的外部性器官，通常諸如增大的陰蒂和融合的陰唇。
罹患先天性腎上腺增生性肥大的男孩可能有睾丸功能減弱，不能產生適量的精子，但是，與這種病患的女孩不同的是他可能沒有體格方面的問題。男女孩童患者可能在孩童起就會長出陰毛，甚至在學齡前他們的陰蒂或陰莖就可能會發育(這種狀況，可能會被誤診為性早熟——譯者注)。一般來說，病患孩童可能發育得非常快，但是，到了成年期，卻傾向於比平均身高為矮。先天性腎上腺增生性肥大有多種臨床類型，一些類型比其他的類型臨床症狀要輕。一些臨床類型在個體出生的當口就很明顯或容易被發現(“典型性”先天性腎上腺增生性肥大)，可是病情輕微的類型在青春期之前症狀表現得不明顯(“非典型性先天性腎上腺增生性肥大”或“遲發性先天性腎上腺增生性肥大”)。幸運的是，這兩種類型的病例都是能夠醫治的。大約在15000個新生兒中有一個這樣的病例發生(約0.007%的發病率)。
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腎上腺皮質 —糖皮質激素分泌量過少；—醛固酮分泌量過少；—過量的雄性激素。
Adrenal Cortex
- Lack of cortisol
- Lack of aldosterone
- Excessive androgens
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A:腎臟(白色)和腎上腺(橘紅色)。
B:增大的一側腎上腺縱切面。在此，功能障礙的皮質以橘紅色標示。
A: Kidneys (white) and adrenal glands (orange). B: Enlarged longitudinal section of one adrenal gland. The malfunctioning cortex is here colored orange.
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Intersexuality - The Intersexual Spectrum
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Causes of Intersexuality - Hormones
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Congenital Adrenal Hyperplasia
(CAH) |
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Hormonal Imbalance The three main hormones involved in CAH are:
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- Cortisol (works against stress, injury and infection; helps in controlling blood sugar levels),
- Aldosterone (needed for kidney functioning; helps in retaining salt), and
- Androgens, one of which is
testosterone (needed for healthy body development).
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In a person with CAH, the mixture of these hormones is wrong: The levels of both cortisol and aldosterone are too low, and the androgen level is too high. This can result in a number of physical problems, some of them, like salt loss, very severe. Also:
A girl born with CAH may have “ambiguous” external sex organs, such as an unusually large clitoris and fused vaginal lips. Boys with CAH may have poorly functioning testicles without proper sperm production, but otherwise may have no physical problems.
Both girls and boys with CAH may develop pubic hair in early childhood, and their clitoris or penis may grow even before they enter school. Generally speaking, children with CAH are likely to grow very fast, but, as adults, tend to remain shorter than average.
There are several forms of CAH, some less serious than others. Some forms are obvious or easily detected at birth (“classical CAH”), but a mild form of the condition does not become apparent until puberty (“non-classical CAH” or
“late onset CAH”). Fortunately, in either case the condition is treatable. It affects approximately 1 in 15,000 newborns (ca. 0,007%). |
