1、12l多毛症是指女性与其同年龄的女性相比表现多毛，累及受性激素影响的毛发。l主要表现在与同族同年龄女性相比，或其本人毛发与病前相比出现明显的改变。3l毛发的生长与分布存在性别、种族、家族差异。l所谓“多毛”，实际上并不是毛囊数量的增多，而是部分毛发生成速度加快，变粗、色泽深而已。4l毛囊：在胚胎早期就已形成，出生后不再有新的毛囊形成，覆盖全身皮肤，除手掌、脚掌。l呈周期性，分生长期、静止期和退化期5l毫毛(毳毛)：细柔、颜色淡的毛，生长期短而静止期长，覆盖于身体大部分皮肤。l终毛：粗而颜色深，生长期长而静止期短，如头发、眉毛、睫毛等。6l不受性激素的影响：头发、眼睫毛和眉毛等。l受男女两类性激
2、素的影响：腋毛、阴毛、四肢和下腹部的毛。l受男性激素的影响：胡须、耳、鼻、耻骨上三角和躯体的毛。成年男性随体内雄激素水平升高，上述部位的毛增粗变长而色深。7 血清浓度 血液中激素的来源(%)激 素 相对雄激素活性 (ng/ml)肾上腺 卵巢 周围组织转化睾丸酮 100 0.2-0.7 5-25 5-25 50-70双氢睾丸酮 250 0.05-0.3 -100雄烯二酮 10-20 0.5-2.5 30-45 45-60 10DHEA 5 1.3-9.8 80 20 -硫酸DHEA 微弱 400-3200 95 200ng/dl应疑有产生雄激素肿瘤，测定游离睾酮意义则更大。l尿17酮(17-ks
3、)升高。升高明显伴17羟类固醇增高，雄激素来自肾上腺可能性大。l血17羟孕酮升高，尿雌三醇增多应考虑CAH，中剂量地塞米松(3mg/日5天)抑制试验，17-ks下降可证实为CAH。21l血LH明显升高，或LH/FSH2伴卵巢增大应疑为多囊卵巢综合征。l肾上腺、卵巢的B超和CT检查，可发现增生或肿瘤。22differential diagnosisl多囊卵巢综合征(PCOD)：主要表现为多毛、肥胖、月经稀发或继发闭经、不育、卵巢增大，血LH升高，FSH相对不足。尿17-ks无升高。l柯兴氏综合征：除多毛，闭经外，有向心性肥胖，血皮质醇升高，尿17OH升高，尿游离皮质醇升高。l先天性肾上腺皮质增生
4、：多见21或11羟化酶缺陷，有外生殖器畸形，血17羟孕酮升高，尿孕三醇增多。迟发型CAH有时不易鉴别。23lTransvaginal ultrasound picture of a polycystic ovary.24lSection through a polycystic ovary.The ovary is enlarged and pearly white.The ovarian capsule is thickened.25lClinical evidence of hyperandrogenemia in PCOS-acne,oily skin and hirsutism.26l
5、A patient with an arrhenoblastoma with associated polycystic ovaries before and after treatment.Before treatment(a),the patient had marked facial hirsutism.In(b)the patient is shown successfully treated.The tumor was resected and ovulation ensued with clomiphene and human chorionic gonadotropin ther
6、apy.27lA patient with an arrhenoblastoma with associated polycystic ovaries before and after treatment.Before treatment(a),the patient had marked facial hirsutism.In(b)the patient is shown successfully treated.The tumor was resected and ovulation ensued with clomiphene and human chorionic gonadotrop
7、in therapy.28l去除病因l药物治疗l美容治疗29l肾上腺、卵巢肿瘤 手术切除l多囊卵巢 手术部 分切除l肥胖 减肥l药物性 停药30l口服避孕药或孕酮l糖皮质类固醇l对抗雄激素的药物l安体舒通31l口服避孕药含有雌激素及孕激素者，抑制LH及FSH的分泌，使依赖LH的卵巢雄激素生成减少。孕激素可使睾丸酮代谢率增快，雌激素还能刺激SHBG的生成。l副作用为水肿、体重增加、恶心及乳房压痛，高甘油三酯血症及糖耐量异常。32l由肾上腺雄激素分泌增多所引起的高雄激素血症(如CAH)，可用糖皮质类固醇治疗。多数患者的过多的雄激素是来自卵巢的，故用糖皮质类固醇疗效不佳。l地塞米松0.5-0.75m
8、g，每晚睡前口服。33l此药抑制LH，因而抑制卵巢雄激素的生成。此外，还阻断雄激素与毛囊上的受体相结合。此药治疗时雌激素分泌也见减少，因此可与雌激素合用。l环丙氯地孕酮2mg及乙炔雌二醇50ug，1次/日，月经第520天服用。lCyproterone 50-100mg/d。34l安体舒通有对抗雄激素的作用：在末梢组织与双氢睾丸酮竞争性地结合；抑制17羟化酶，使睾丸酮及雄烯二酮的生成减少；加速睾丸酮转化为雌二醇。l安体舒通25100mg，2次/日，于月经周期第422天服用，共6个周期。l副作用：高血钾、低血钠、血容量减少以及胃肠及神经系统症状。35l剃除终毛或使用脱毛剂，但不宜人工拔除，以免造成
9、创伤，引起感染。l内科治疗几个月后，新毛的生成显著减少，此时可采用电解法去除终毛。363738l激素测定：激素测定：血清血清 T、E2、P、PRL、FSH、LHl功能试验：兴奋试验：功能试验：兴奋试验：LHRH、克罗米克罗米芬、芬、h-CG 抑制试验：睾酮抑制试验：睾酮 l 染色体、精液、脱落细胞、基础体温染色体、精液、脱落细胞、基础体温l影像学：影像学：B超超 组织学组织学39l真性两性畸形l女性假两性畸形(CAH)l男性假两性畸形(17a-HO)4041 6岁性征，8岁月经l真性性早熟l假性性早熟 l完全性性早熟（体质性，特发性，肿瘤，甲减）l不完全性性早熟（肿瘤、酶异常）42l乳房早现
10、El阴毛早现 Cl男乳发育 E2/T43lFamilial gynecomastia(a)King Tutankhamun and(b)his brother Smenkhare are both depicted by contemporary artists as having gynecomastia.44l 女性18岁(16)l 男性20岁45l性腺机能低减:l低促性腺激素性性腺机能低减(Kallman)l高促性腺激素性性腺机能低减（Turner Klinefelter）46原发性闭经/继发性闭经l精神性闭经 l中枢神经性闭经（肿瘤，发育缺陷）l下丘脑性闭经（Kallmann）l垂体性
11、闭经（垂体瘤、空泡蝶鞍）l卵巢性闭经（早衰、PCOS、Turner）l子宫性闭经 47lTwo patients with Turners syndrome.Patient A has the characteristic dysmorphic features of Turners syndrome;patient B has minimal dysmorphic features.In some patients the only clinical features are short stature and pubertal delay.(Courtesy of Pharmacia Co
12、rporation.)484950lSeven-year-old boy with central precocious puberty secondary to neurofibromatosis with unaffected twin.(Courtesy of Pharmacia Corporation.)51lThe effect of testosterone cypionate therapy for 11 months in a 22-year-old man with hypogonadotropic hypogonadism.52lStandards for rating o
13、f pubertal changes in boys.(a)Genital changes.53lStandards for rating of pubertal changes in boys.(b)Pubic hair.(Reproduced from Marshal WA,Tanner JM.Variations in pattern of pubertal changes in boys.Arch Dis Child.1970;45:13-24.)54lStandards for rating of pubertal changes in girls.(a)Breast develop
14、ment.55lStandards for rating of pubertal changes in girls.(b)Pubic hair.(Reproduced from Marshal WA,Tanner JM.Variations in pattern of pubertal changes in girls.Arch Dis Child.1969;44:291-301.)56lRadiograph of a hand,used to assess skeletal maturity(bone age).The hand of a 6-year-old girl with preco
15、cious puberty is shown.Although the girl is tall for her chronological age,her bone age is advanced to that of a 10-year-old,which means that 83%of her growth has already taken place.Assessment of her bone age is important since it is used to predict her final height,which in this case will be short
16、.The normal ages at which the bone ossification centers develop are shown in brackets.57lA Praders orchidometer.Testicular volume(in milliliters)may be estimated by direct comparison with the ellipsoids.During the assessment of testicular volume,the epididymis should not be included.58lHormonal events in the ovarian cycle.FSH,follicle-stimulating hormone;LH,luteinizing hormone.59lEffect of ovarian steroids on the endometrium.FSH,follicle-stimulating hormone;LH,luteinizing hormone.60再 见
