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中间性
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中间性性象谱
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中间性的原因-
激素因素
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先天性肾上腺增生性肥大(Congenital
Adrenal Hyperplasia, CAH)
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激素失衡
有关先天性肾上腺增生性肥大的三种主要的激素是：
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- 糖皮质激素(Cortisol)
(对抗心理压力、损害和感染；协助控制血糖水平)；
- 醛固酮(Aldosterone)
(为调节肾脏功能所需；协助重吸收盐)；
- 雄激素(Androgens),
其中一种是睾丸酮(为身体健康发育所需)。
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罹患先天性肾上腺增生性肥大者，上述三种激素的综合平衡被打破：糖皮质激素和醛固酮分泌过少，而雄激素水平却太高。这能够导致许多非常严重的生理问题，其中像盐分丢失等。同样的道理：罹患先天性肾上腺增生性肥大的女孩可能具有“莫凌两可”的外部性器官，通常诸如增大的阴蒂和融合的阴唇。
罹患先天性肾上腺增生性肥大的男孩可能有睾丸功能减弱，不能产生适量的精子，但是，与这种病患的女孩不同的是他可能没有体格方面的问题。男女孩童患者可能在孩童起就会长出阴毛，甚至在学龄前他们的阴蒂或阴茎就可能会发育(这种状况，可能会被误诊为性早熟——译者注)。一般来说，病患孩童可能发育得非常快，但是，到了成年期，却倾向于比平均身高为矮。先天性肾上腺增生性肥大有多种临床类型，一些类型比其他的类型临床症状要轻。一些临床类型在个体出生的当口就很明显或容易被发现(“典型性”先天性肾上腺增生性肥大)，可是病情轻微的类型在青春期之前症状表现得不明显(“非典型性先天性肾上腺增生性肥大”或“迟发性先天性肾上腺增生性肥大”)。幸运的是，这两种类型的病例都是能够医治的。大约在15000个新生儿中有一个这样的病例发生(约0.007%的发病率)。
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肾上腺皮质 —糖皮质激素分泌量过少；—醛固酮分泌量过少；—过量的雄性激素。
Adrenal Cortex
- Lack of cortisol
- Lack of aldosterone
- Excessive androgens
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A:肾脏(白色)和肾上腺(橘红色)。
B:增大的一侧肾上腺纵切面。在此，功能障碍的皮质以橘红色标示。
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%). |
