低促性腺激素性性腺功能减退Hypogonadal hormone hypogonadal function转载2008-02-24

低促性腺激素性性腺功能减退的概述 An overview of hypogonadal gonadal hypotropy

低促性腺激素性性腺功能减退(HH)是指，下丘脑和垂体前叶异常，导致的性腺不能产生性激素或完全的生殖细胞。临床上男性表现为无精、少精和弱精;女性表现为原发闭经。部分患者伴有嗅觉丧失或躯体畸形。

Hypogonadal hormone hypogonadal function(HH) refers to abnormal hypothalamus and pituitary anterior lobe, resulting in gonads that can not produce sex hormones or complete germ cells. Clinically, men show no fine, little fine and weak fine; Women show primary amenorrhea. Some patients were accompanied by olfactory loss or body deformity.

低促性腺激素性性腺功能减退的分类 Classification of hypogonadal gonadal hypotropy

1、下丘脑性 1, hypothalamus

下丘脑异常导致促性腺激素释放激素(GnRH)、卵泡刺激素(FSH)黄体生成素(LH)低下或缺如;

Hypothalamus abnormalities lead to low or absent gonadotropin release hormone(GnRH) and follicular stimulating hormone(Huangtishengchengsu);

2、垂体前叶性 2, pituitary anterior leaf sex

由于垂体前叶异常导致FSH和LH降低或缺如。

Due to abnormal pituitary anterior lobe, FSH and LH are reduced or absent.

下丘脑低促性腺激素性性腺功能减退发病机制

The pathogenesis of hypothalamus hypogonadal gonadal hypotropy

下丘脑异常最多的原因是卡曼氏综合征(Kallmann syndrone)发病机制是：正常胚胎发育时，促性腺激素释放激素神经原，由鼻腔上皮转移至下丘脑，而卡曼氏患者由于基因突变，在胚胎发育中未能完成这一转移，下丘脑没有这种神经元，因而不能分泌促性腺激素释放激素，进而促性腺激素分泌减低，继发性腺机能低下。临床上低促性腺激素性腺功能减退、嗅觉丧失或低下。偶尔出现单侧肾缺如、腭裂等躯体畸形。

The reason for the most abnormal hypothalamus is that the pathogenesis of Kallmann syndrome is that during normal embryonic development, gonadotropin releases hormone neurons from the nasal epithelium to the hypothalamus, and Carman's patients are due to gene mutations. Failure to complete this transfer in embryonic development, The hypothalamus does not have such neurons and therefore can not secrete gonadotropin releasing hormones, which in turn reduces gonadotropin secretion and has a secondary hypogonadal function. Hypogonadal gonadal hypoplasia, olfactory loss or hypoplasia. Occasionally, unilateral renal defects such as cleft palate and other body deformities occur.

其次是部分侏儒症。 Followed by some dwarfism.

垂体前叶性的发病机制是垂体瘤或垂体放射过渡导致分泌FSH和LH的细胞减少或缺如，继发多见。

The pathogenesis of pituitary profoliation is that pituitary tumors or pituitary radiation transitions cause the secretion of FSH and LH cells to be reduced or absent, and secondary development is common.

低促性腺激素性性腺功能减退的诊断和鉴别诊断

Diagnosis and differential diagnosis of hypogonadal gonadal hypotropy

临床上见到原因不明的少精弱精无精子或原发闭经，经电化学发光法检测发现FSH、 LH、T、E均明显低于正常就可确定为低促性腺激素性性腺功能减退症，但是尚须用电化学发光法做垂体兴奋试验区别病变是在下丘脑还是垂体前叶。

Clinically, the absence of spermatozoa or primary amenorrhoea with unknown cause was found to be significantly lower than normal in FSH, LH, T, and E. Hypogonadal gonadal function reduction. However, electrochemiluminescence is still required to do pituitary stimulation test to distinguish whether the lesion is in the hypothalamus or the anterior pituitary lobe.

安太特色治疗 Antai special treatment

北京安太医院批量实践证明，在去除已知原发病后(如切除垂体肿瘤)，可采用宫腹腔镜检查，雌、雄激素，促性腺激素和促性腺激素释放激素治疗。95%以上的患者可以达到：①诱导青春期，恢复患者的第二性征;②恢复及维持正常性功能;③诱导或恢复生育能力。

Beijing Antai Hospital batch practice proved that after removing the known primary disease(such as removing pituitary tumors), uterine laparoscopy, female, androgen, gonadotropin and gonadotropin release hormone treatment can be used. Patients with 95 or more can achieve: 1 Induction of puberty, recovery of the patient's secondary sexual characteristics; 2 Restoration and maintenance of normal sexual functions; 3 Induction or recovery of fertility.

1、促性腺激素释放激素治疗：对于病变位于下丘脑的患者应采用GnRH治疗，GnRH对于垂体病变者无效。由于正常生理状态下GnRH是以脉冲方式释放的，约每70～90分钟一个脉冲，因此，在进行GnRH治疗时也需采用电子泵皮下或静脉脉冲式给予，每90分钟一个脉冲(每个脉冲含GnRH 1～2Ug)，25ng/kg。

1, gonadotropin release hormone treatment: For patients with lesions located in the hypothalamus should be treated with GnRH, GnRH is not effective for pituitary lesions. Since GnRH is released in a pulse mode under normal physiological conditions, about one pulse every 70 to 90 minutes, it is also necessary to use an electronic pump subcutaneous or venous pulse to give GnRH treatment. One pulse per 90 minutes(each pulse contains GnRH 1 to 2Ug), 25 ng/kg.

(1)男性患者治疗一周后血清促性腺激素和睾酮水平上升，治疗2～3个月后无精症患者精液中可出现精子，少精症和弱精症患者精液质量改善。因此，应在治疗3个月后检查精液，若精液质量恢复，可将精液冷冻以备人工授精等。

(1) The level of serum gonadotropin and testosterone increased after one week of treatment in male patients. Sperm can appear in spermatozoa after 2 to 3 months of treatment. Semen quality in patients with spermatozoa and spermatozoa is improved. Therefore, semen should be examined after 3 months of treatment. If the quality of semen is restored, semen can be frozen for artificial insemination.

(2)女性患者治疗后会有排卵和月经来潮，一般在半年内都能怀孕，我院已经有一些成功的例子。

(2) Female patients will have ovulation and menstrual cramps after treatment. Generally, they can become pregnant within six months. There have been some successful examples in our hospital.

2、促性腺激素治疗：

2, gonadotropin therapy:

(1)男性患者常用睾酮及LH诱导青春期的启动及第二性征的出现，而对于精子生成过程的诱导则需用FSH，但若为了使睾丸产生成熟精子，还需加用睾酮和LH。目前尚无市售的LH制剂，故常用长效的hCG来替代LH。对于青春期延缓的患者常从小剂量雄、激素替代治疗开始，以诱导其青春期的起始。此时若应用促性腺激素可以使精子生成过程的起始和第二性征的发育同时发生。因此，对于精子生成过程而言，FSH是必须的;而对于青春期的诱导LH或hCG必不可少。

(1) In male patients, testosterone and LH are commonly used to induce the initiation of puberty and the emergence of secondary sexual characteristics. For the induction of spermatogenesis, FSH is used, but testosterone and LH are also needed in order to produce mature sperm in the testis. There is currently no commercially available LH formulation, so long-acting hCG is often used instead of LH. For patients with delayed puberty, they often begin with dose male and hormone replacement therapy to induce the onset of puberty. At this point, the application of gonadotropin can cause the initiation of spermatogenesis and the development of secondary sexual characteristics to occur simultaneously. Therefore, for the spermatogenesis process, FSH is necessary; It is indispensable for inducing LH or hCG in adolescence.

对于低促性腺激素性性腺功能减退症伴有青春期迟缓表现的年轻患者建议使用FSH和hCG联合治疗。常用方法为首先应用hCG 2000IU肌肉注射，每周三次，共6周，以使血清睾酮恢复至正常水平;6周hCG治疗结束时，加用FSH 37.5Iu(与hCG合用)，每周三次。对于有生育要求的患者，应检查精液，以便了解其生育能力。由于促同时还应检查其配偶，增加受孕机会，缩短治疗时间。一般联合治疗3～6个月后才可能受孕。若联合治疗3个月后患者睾丸体积或受孕情况未改善，FSH和hCG的量可加倍。若患者对联合治疗反应差，尤其是初诊时睾丸体积小，无青春期表现的患者，联合治疗可持续1-2年。合并隐睾的患者，即使联合治疗后睾丸可下降，其生育能力恢复的可能性也很小。

Combined FSH and hCG treatment is recommended for young patients with hypogonadal hypogonadotropy accompanied by adolescent retardation. The commonly used method is to first apply hCG 2000 IU intramuscular injection three times a week for a total of 6 weeks to restore serum testosterone to normal levels; At the end of 6 weeks of hCG treatment, add FSH 37.5 Iu(shared with hCG) three times a week. For patients with fertility requirements, semen should be examined in order to understand their fertility. It is also necessary to check the spouse, increase the chances of conception and shorten the time for treatment. It is generally possible to conceive after 3 to 6 months of joint treatment. If the testicle volume or fertility of the patient does not improve after 3 months of combined treatment, the amount of FSH and hCG can be doubled. If the patient has a poor response to combined treatment, especially in patients with small testicle size and no adolescent performance at the time of initial diagnosis, combined treatment can last 1-2 years. In patients with cryptorchidism, even if the testicle can be reduced after combined treatment, the possibility of recovery of fertility is also very small.

对于较轻的性腺功能减退症的患者或成人后患低促性腺激素性性腺功能减退症的患者，单纯应用hCG即可达到雄激素化和恢复生育能力的目的。其hCG的用法是2000Iu，每周三次肌肉注射。

For patients with lighter hypogonadal dysfunction or patients with hypogonadal hypogonadotropy after adulthood, the use of hCG alone can achieve the purpose of androgenization and restoration of fertility. Its hCG usage is 2000 Iu, intramuscular injection three times a week.

(2)女性患者 (2) Female patients

每天用FHS类药物10天后开始阴道B超检测排卵，待一个卵泡达到18mm以上时，给HCG5000iu，34—36小时同房和人工受精即可能怀孕，未孕者会按时来月经，在月经5天再开始下个周期，周而复始，一般在半年内都能怀孕。FHS类药物的种类和用量要根据年龄、体重和治疗目的而定，安太的经验是可以从较大剂量开始，逐渐减量找到个性化剂量。

Every day, after 10 days of using FHS drugs, vaginal B ultrasound tests ovulation. When a follicle reaches 18mm or more, HCG5000 IU is given, 34-36 hours with the same room and artificial insemination may be pregnant, and those who are not pregnant will menstruate on time. In the 5 days of menstruation, the next cycle begins again and again, and generally becomes pregnant within six months. The type and amount of FHS drugs are determined according to age, weight, and therapeutic purpose. The experience of Anta is that it can start with a larger dose and gradually reduce the amount to find a personalized dose.

3、雌、雄激素治疗：只能改善患者的性功能和有助于诱导其青春期的出现，不能恢复其生育能力，因而只有当患者没有生育要求时，才可采用雌、雄激素治疗低促性腺激素性性腺功能减退症。与促性腺激素治疗相比，雄激素治疗的最大优点是价格便宜。

3, female, androgen treatment: can only improve the patient's sexual function and help induce the emergence of puberty, can not restore their fertility, so only when the patient does not have fertility requirements, can use female, androgen treatment of hypogonadotropin sex adenosis. Compared with gonadotropin therapy, the biggest advantage of androgen therapy is that it is cheap.

雌雄激素治疗要联合促进钙吸收和固钙药物应用，临床上多采用密钙息和罗盖全，用法用量要依据患者具体情况而定。

Female androgen therapy should be combined to promote calcium absorption and calcium fixation drug applications. Clinically, dense calcium and Luogai are used. The amount of usage should be determined according to the specific conditions of the patient.

4、宫腹腔镜检查

4, laparoscopic examination

宫腹腔镜检查和手术目的是去除那些合并症和盆腔宫腔疾病，如果有生育要求应该先进行宫腹腔镜检查，以缩短治疗周期。

The purpose of uterine laparoscopy and surgery is to remove those complications and pelvic cavity diseases. If there is a reproductive requirement, uterine laparoscopy should be performed first to shorten the treatment cycle.