多毛症课件.ppt

1,多 毛 症Hirsutism,2,定 义 definition,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。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。,3,概 述 summarize,毛发的生长与分布存在性别、种族、家族差异。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。,4,毛发的生长,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。呈周期性，分生长期、静止期和退化期,5,毛发的分布,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。终毛：粗而颜色深，生长期长而静止期短，如头发、眉毛、睫毛等。,6,毛发分类（按性激素的影响）：,不受性激素的影响：头发、眼睫毛和眉毛等。受男女两类性激素的影响：腋毛、阴毛、四肢和下腹部的毛。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,女性血循环中的雄激素,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 5 -,8,雄性激素的来源,有正常月经的女性产生的雄激素有睾酮、雄烯二酮和去氢表雄酮。睾酮约20%来自卵巢，30%来自肾上腺，50%来自外围组织的雄烯二酮的转化。而雄烯二酮50%来自卵巢，50%来自肾上腺。去氢表雄酮则大部分来自肾上腺。,9,雄性激素的代谢,皮肤含有多种酶，使血中的雄激素前体转化为雄激素，引起生物效应，又能将雄激素分解结合，从尿中排出。多毛妇女皮肤比正常妇女皮肤能利用更多的激素前体，将其转化为具有生物活性的雄激素，如睾酮和双氢睾酮。 特发性多毛和多囊卵巢综合征者体内雄激素的生产率与睾酮的代谢率均高于正常妇女，其靶器官表现的对雄激素的活性也明显升高。,10,多毛症病因分类,1、特发性多毛症 2、多囊卵巢综合症3、卵巢男性化肿瘤 4、迟发性先天性肾上腺增生5、肾上腺增生或肿瘤 柯兴氏综合征6、药物因素 7、绝经后多毛8、肥胖,11,多毛症的病理生理,正常女性血中主要的雄激素是睾丸酮 Testosterone。对雄激素敏感的组织，如毛囊，睾丸酮先由 5-还原酶转化为双氢睾丸酮。 reductase dihydro,12,雄激素生成过多,轻、中度多毛症女性，几乎都有雄激素水平的增高或5-还原酶活力的增强。现已很少被诊为特发性多毛症。雄激素来自卵巢和肾上腺，以卵巢为主。约15%患者血17羟孕酮增高，提示成年晚发CAH。,13,血清结合蛋白及雄激素在血清中的运转,在正常女性，65%的血清睾酮与SHBG结合，游离睾酮仅占1-2%。相反，血清雄烯二酮85%与ALB结合，10%与SHBG结合。多毛妇女的SHBG多降低，总睾酮轻度增高，游离睾酮即明显增高。测定游离睾酮更好地反映雄激素的活性。大多数轻、中度多毛症妇女血游离睾酮的比例增高。,14,雄激素在皮肤毛囊的代谢,多毛妇女5-还原酶活性增强，使毛囊中睾酮转化为双氢睾酮增多。因此，许多被认为是特发性多毛症者血清睾酮正常，而葡萄糖醛酸雄烷二醇水平增高，说明这些患者的5-还原酶活性增强。,15,临 床 表 现 Clinical situation,毛发分布异常男性化 virilization,16,毛发分布异常abnormity of distributing,正常女性上唇、鬓角、乳晕周围、腹部及四肢可见终毛。但需与原毛发分布相比，与同龄、同族女性比较。华人正常女性前胸、耻骨上三角、大腿内侧、腰骶部、背上部、耳、鼻处很少有粗毛。女性多毛者还可有男性秃顶，颞侧脱发。,17,18,男性化表现,肌肉发达，声音变粗，阴蒂增大， 乳房萎缩，闭经等。应注意分泌雄激素的肿瘤，发生于卵巢或肾上腺。,19,Enlarged clitoris in a patient with an androgen-secreting tumor.,20,实验室检查Laboratory examination,血睾酮升高。正常女性20-80ng/dl，200ng/dl应疑有产生雄激素肿瘤，测定游离睾酮意义则更大。尿17酮(17-ks)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。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。,21,实验室检查,血LH明显升高，或LH/FSH2伴卵巢增大应疑为多囊卵巢综合征 。肾上腺、卵巢的B超和CT检查，可发现增生或肿瘤。,22,鉴别诊断differential diagnosis,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。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。先天性肾上腺皮质增生：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。,23,Transvaginal ultrasound picture of a polycystic ovary.,24,Section through a polycystic ovary. The ovary is enlarged and pearly white. The ovarian capsule is thickened.,25,Clinical evidence of hyperandrogenemia in PCOS - acne, oily skin and hirsutism.,26,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 therapy.,27,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 therapy.,28,多毛症的治疗,去除病因药物治疗美容治疗,29,去除病因,肾上腺、卵巢肿瘤 手术切除多囊卵巢 手术部 分切除肥胖 减肥药物性 停药,30,药物治疗：,口服避孕药或孕酮糖皮质类固醇对抗雄激素的药物安体舒通,31,口服避孕药或孕酮：,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。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。,32,糖皮质类固醇：,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。地塞米松0.5-0.75mg，每晚睡前口服。,33,对抗雄激素的药物：,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。环丙氯地孕酮2mg及乙炔雌二醇50ug， 1次/日，月经第520天服用。Cyproterone 50-100mg/d。,34,安体舒通：,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。安体舒通25100mg，2次/日，于月经周期第422天服用，共6个周期。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。,35,美容治疗,剃除终毛或使用脱毛剂，但不宜人工拔除，以免造成创伤，引起感染。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。,36,性腺疾病概述,37,38,性腺疾病常见实验室检查,激素测定： 血清 T、E2、P、PRL、FSH、LH功能试验：兴奋试验：LHRH、克罗米芬、h-CG 抑制试验：睾酮 染色体、精液、脱落细胞、基础体温影像学： B超 组织学,39,性 分 化 异 常,真性两性畸形女性假两性畸形 (CAH)男性假两性畸形 (17a-HO),40,性 发 育 异 常,41,性 早 熟,6岁性征，8岁月经真性性早熟假性性早熟 完全性性早熟（体质性，特发性，肿瘤，甲减）不完全性性早熟（肿瘤、酶异常）,42,青春期发育变型,乳房早现 E阴毛早现 C男乳发育 E2/T,43,Familial gynecomastia (a) King Tutankhamun and (b) his brother Smenkhare are both depicted by contemporary artists as having gynecomastia.,44,青春期发育障碍,女性18岁(16) 男性20岁,45,性腺机能异常,性腺机能低减:低促性腺激素性性腺机能低减 (Kallman)高促性腺激素性性腺机能低减 （Turner Klinefelter）,46,闭 经,原发性闭经 / 继发性闭经精神性闭经 中枢神经性闭经 （肿瘤，发育缺陷）下丘脑性闭经 （Kallmann）垂体性闭经 （垂体瘤、空泡蝶鞍）卵巢性闭经（早衰、PCOS、Turner）子宫性闭经,47,Two 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 Corporation.),48,49,50,Seven-year-old boy with central precocious puberty secondary to neurofibromatosis with unaffected twin. (Courtesy of Pharmacia Corporation.),51,The effect of testosterone cypionate therapy for 11 months in a 22-year-old man with hypogonadotropic hypogonadism.,52,Standards for rating of pubertal changes in boys. (a) Genital changes.,53,Standards 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.),54,Standards for rating of pubertal changes in girls. (a) Breast development.,55,Standards 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.),56,Radiograph of a hand, used to assess skeletal maturity (bone age). The hand of a 6-year-old girl with precocious 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. The normal ages at which the bone ossification centers develop are shown in brackets.,57,A 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.,58,Hormonal events in the ovarian cycle. FSH, follicle-stimulating hormone; LH, luteinizing hormone.,59,Effect of ovarian steroids on the endometrium. FSH, follicle-stimulating hormone; LH, luteinizing hormone.,60,再 见,