高雄激素血症
Hyperandrogenism is defined as the clinical manifestation of excess levels of androgens in women (1). The most common cause of hyperandrogenism in reproductive age women is polycystic ovarian syndrome (PCOS).
高雄激素血症表现为女性体内雄激素水平过高。育龄女性出现高雄激素血症最常见的原因是多囊卵巢综合征(1)。
Hyperandrogenism’s clinical manifestations are associated with different levels of testosterone and the origin of the over production of androgens differs. The two most common clinical presentations are:
1. hirsutism, defined as the male pattern distribution of excess hair, with conversion of vellus hair to terminal hair, and
2. virilization, defined as the masculinization of women with a marked increase in testosterone levels.
高雄激素血症的临床表现与睾酮水平有关，导致雄激素水平过高的原因也有所不同。高雄激素血症的两种临床表现是：
1. 多毛症，表现为多余毛发，分布类似男性，毫毛转化为终毛;
2. 男性化，表现为女性男性化，睾酮水平显著增加。
In addition to hirsutism, patients with hyperandrogenism typically present with clitoral enlargement, temporal balding and deepening of the voice.
除了多毛症外，高雄激素血症患者通常还有阴蒂增大、颞叶秃和声音变深等表现。
Hyperandrogenism is a clinical syndrome secondary to an increase of secretion of androgens. Androgen production results from the metabolism of cholesterol (2). Androgens are produced in the adrenal glands and ovaries, and each androgen has a different potency as well as individual characteristics.
高雄激素血症是继发于雄激素分泌增加的临床综合征。雄激素的产生源于胆固醇的代谢过程(2)，由肾上腺或卵巢分泌，每种雄激素有不同作用和特点。
Dehydroepiandrosterone (DHEA), a weak androgen, is produced mostly in the adrenal gland (90%). Its more stable and longer acting form is dehydroepiandrosterone sulfate (DHEAS), which is the most commonly measured adrenal androgen. Androstenodione is produced in the ovary (50%) and the adrenal gland (50%) and is also considered a weak androgen.
脱氢表雄酮 (DHEA) 是一种弱雄性激素，主要由肾上腺分泌(90%)。其更稳定且作用时间更长的形式是硫酸脱氢表雄酮 (DHEAS)，也是检查时最常测量的肾上腺雄激素。雄烯二酮由卵巢(50%)或肾上腺(50%)分泌，也被认为是一种弱雄性激素。
Most androgens circulate in their most effective clinical form of testosterone. The ovarian and adrenal androgen production accounts for about 50% of circulating androgens, whereas production elsewhere in the body accounts for the remaining 50%.
大多数雄激素以睾酮这种最有效的形式在体内循环。卵巢和肾上腺分泌的雄激素约占循环雄激素的 50%，而身体其他部位分泌的则占剩余的 50%。
Testosterone is distributed throughout the body, mostly bound to albumin and sex hormone building globulin (SHBG). This binding makes most of the circulating testosterone clinically inactive. Only between 1% and 3% of circulating testosterone travels unbound and is biologically active. For this reason, total circulating testosterone does not reflect the true amount of active hormone. Testosterone can be aromatized to estrone in adipose tissue while free testosterone is converted a more potent form, dihydrotestosterone (DHT), at the tissue level (hair follicles and within genital skin) via 5α-reductase (2). This conversion is responsible for constitutional effects of the hormone, including increase hair growth, acne, and the physical changes associated with virilization.
睾酮分布于全身，主要与白蛋白和性激素生成球蛋白(SHBG)结合。这种结合使大多数循环睾酮在临床上没有活性。只有1%至3%的循环睾酮未结合并具有生物活性。因此，总循环睾酮并不能反映活性激素的真实数量。睾酮可以在脂肪组织中芳香化为雌酮，而游离睾酮通过 5α-还原酶在组织水平(毛囊和生殖器皮肤内)转化为更有效的形式二氢睾酮(DHT)(2)。这种转化与激素对身体的作用相关，包括增加体毛生长、痤疮和与男性化相关的身体变化。
Adrenal androgen production is controlled by adrenocorticotropic hormone. With hyperandrogenism, enzyme deficiencies in the androgen production and metabolism pathway lead to precursor molecules such as DHEA and DHEAS accumulation, which can be measured to determine both the origin of the hyperandrogenism. DHEA can further be metabolized to androstenedione and testosterone.
肾上腺中雄激素的生成受到促肾上腺皮质激素的调控。对于高雄激素血症，雄激素产生和代谢途径中的酶缺乏导致前体分子如 DHEA 和 DHEAS 的积累，所以可以通过检测DHEA和DHEAS来确定导致高雄激素血症的原因。DHEA 可进一步代谢为雄烯二酮和睾酮。
Ovarian androgen production (mainly testosterone and some androstenedione) is stimulated by luteinizing hormone secretion from the pituitary gland. Extraglandular testosterone production occurs in adipocytes and depends on the magnitude of adrenal and ovarian androstenedione production.
卵巢中雄激素的生成(主要是睾酮和一些雄烯二酮)受到垂体分泌的促黄体激素的刺激。腺外睾酮生成于脂肪细胞中，取决于肾上腺和卵巢雄烯二酮的分泌数量。
References
参考文献
1.Azziz R. The evaluation and management of hirsutism. Obstet Gynecol. 2203;101:995-1007.
2. Hoffman BL, Schorge JO, Schaffer JI, et al., eds. Polycystic ovarian syndrome and hyperandrogenism. In：Hoffman BL, Schorge JO, Schaffer JI, et al., eds. Williams Gynecology, 2nd ed. New York: McGraw-Hill: 2012.
