男性特发性低促性腺激素性性功能减退症

男性特发性低促性腺激素性性功能减退症是由于基因异常引起下丘脑分泌促性腺激素释放激素（GnRH）功能障碍导致睾丸功能低下的遗传性疾病，主要临床表现为第二性征发育不全，性功能低下和不育。诊断主要根据临床表现和血卵泡刺激素、黄体生成素和睾酮水平均明显降低，且除外体质性青春发育延迟和后天获得性低促性腺激素性性腺功能减退症。目前尚无根治措施，仅限于替代治疗。雄激素替代治疗可以促进第二性征的发育和维持性功能，使用促性腺激素或GnRH脉冲治疗可诱导精子生成，使部分患者实现生育愿望。早期诊断和恰当的治疗策略是治疗成功的关键。     

特发性低促性腺激素性性腺功能减退症诊治专家共识解读

特发性低促性腺激素性性腺功能减退症( 简称IHH),是下丘脑促性腺激素释放激素(GnRH) 分泌或作用障碍 导致的一种疾病,以青春不发育和配子生成障碍为主要临床表现。性激素替代治疗可促进第二性征发育。当患者 有生育需求时,促性腺激素或GnRH 泵治疗,有助于配子产生。2015 年中华内分泌学分会性腺学组专家,针对此病 制定了专家共识。文章针对共识中存在的一些争议问题进行探讨,这些问题包括疾病命名、基因诊断、儿童IHH 的 识别和治疗、生精治疗中的问题等,旨在增加内分泌医生对此疾病的认识,提高诊治水平。     

女性特发性低促性腺激素性性腺功能减退症的临床研究——附16例病例回顾

回顾性分析本院2004年至2011年12月收治的16例女性特发性低促性腺激素性性腺功能减退症(nIHH)患者的临床资料及随访记录.16例患者均具有正常女性染色体核型,粗测嗅觉正常;无第二性征发育,促性腺激素及性腺激素水平显著低于正常,100μg GnRH兴奋试验提示11例LH峰值低于正常水平(11/15),GnRH延长兴奋试验提示垂体性腺激素储备功能尚可(6/6);双腕和双膝关节正位X线片提示骨骺愈合延迟(9/9).垂体MRI提示1例右侧嗅球、嗅束缺失(1/16);妇科超声提示1例无子宫及附件,余15例幼稚型子宫.1例有严重骨质疏松.1例诊断垂体柄中断症.激素替代治疗随诊6例,最长随诊5年,子宫及附件发育及乳房发育均改善.由于治疗开始时间,患者依从性诸多因素,nIHH治疗效果差异较大.对于育龄期患者,停止激素替代治疗后无病程逆转.     

促性腺激素治疗男性低促性腺激素性性功能减退症的疗效评估

目的应用人绒毛膜促性腺激素(hCG)和绝经期妇女尿促性腺激素(HMG)治疗男性低促性腺激素性性腺功能减退症,评价其疗效.方法 64例低促性腺激素性性腺功能减退症患者中Kallmann综合征19例,特发性低促性腺激素型性腺功能低下41例,颅咽管瘤手术后性腺功能低下症4例.33例患者采用hCG 1500 IU肌肉注射,每周2次;31例患者采用hCG 1500 IU + HMG 75 IU联合肌肉注射,每周2次.疗程均6个月以上.结果治疗后所有患者体力改善,体质增强;42例患者出现胡须、阴毛和(或)腋毛.睾丸体积治疗前(3.08±2.44)ml, 治疗后(8.92±5.37)ml(P<0.01);血清卵泡刺激素、黄体生成素和睾酮水平有所提高(P<0.05);6/64患者出现遗精现象,2例有精子生成.以睾丸体积增大为判断疗效的标准,12例无效,52例有效,有效率达81.2%,hCG+HMG组效果明显好于hCG组.结论对男性低促性腺激素性性腺功能减退症患者,用hCG和HMG治疗能促进青春期第二性征发育,体力增加,外生殖器和睾丸进一步发育,并可望部分恢复睾丸产生雄激素和生成精子两项功能,明显优于以往单纯使用或者过早使用雄激素替代的治疗方案.     

2型糖尿病合并低促性腺激素性性腺功能减退研究进展

正低促性腺激素性性腺功能减退(HH),是由于促性腺激素释放激素(Gn RH)或促性腺激素分泌减少或缺乏所致的性腺功能减退,主要以低促性腺激素和低性激素分泌为特征。游离睾酮异常与卵泡刺激素(FSH)、促黄体生成素(LH)水平低下密切相关[1]。令人吃惊地是,男性2型糖尿病患者中HH的患病率竟然高达25%~40%[2]。因此,对2型糖尿病患者进行睾酮评估极为重要[3]。     

应用hpHMG进行促排卵治疗的临床观察

应用高纯度人绝经期尿促性腺激素(hpHMG)及普通尿源性人绝经期尿促性腺激素(HMG)联合人绒毛膜促性腺激素(HCG)治疗无排卵不孕患者97例,根据卵泡发育情况调整用量并抽取静脉血测定血激素.结果 两组患者的激素水平无明显差异,HMG组HCG注射日血清雌二醇水平明显低于hpHMG组,排卵后7 d的血清孕酮水平明显高于hpHMG组(P均＜0.05).认为hpHMG可获得与HMG相似的促排卵治疗效果及妊娠率,两种方案同样有效,可适于临床应用.     

低促性腺激素性性腺功能减退症合并1型糖尿病及桥本甲状腺炎1例报告并文献复习

<正>低促性腺激素性性腺功能减退症(HH)是一种由于促性腺激素释放激素或促性腺激素缺乏,导致性腺功能减退的遗传异质性疾病。先天性HH又称为特发性低促性腺激素性性腺功能减退症(IHH)。IHH合并嗅觉障碍者称为Kallmann综合征,无嗅觉障碍者称为n IHH[1]。在男性患者中常表现为小阴茎或小睾丸、阴毛和(或)腋毛缺如、少精或无精症、不育等[2]。目前有研究表明,低促性腺激素性性腺     

女性特发性低促性腺激素性性腺功能减退症合并骨密度异常2例报告并文献复习

正特发性低促性腺激素性性腺功能减退症(idiopathic hypogonadotropic hypogonadism,IHH)是由下丘脑病变导致的促性腺激素释放激素(GnRH)分泌障碍性疾病,表现为青春期无第二性征出现,生殖系统发育成熟受阻[1]。发病率在男性约1/10000,女性约1/50000,男∶女为5∶1。国内外文献报道女性IHH的病例甚少,女性患者大多因闭经和不孕症首诊,以往更多关注的是患者的性征发育、月经维持及生育状况,而因     

男性特发性低促性腺激素型性腺功能减退症治疗进展

男性特发性低促性腺激素型性腺功能减退症是由于先天性下丘脑功能障碍导致的一类以雄激素缺乏和不育为主要特点的疾病.此类患者在婴幼儿期、儿童期、青春期、成年期所需达到的治疗目的和相应的治疗措施各不相同.不同人生阶段的特发性低促性腺激素型性腺功能减退症患者应选择及时合理的治疗方案.     

胃复安试验诊断男性单纯促性腺激素低下性性腺功能减退症

对青春前期和青春早期男性促性腺激素低下性性腺功能减退(HH)和体质性青春期延迟(D)的患者作鉴别诊断是十分困难的。两者血及尿睾丸酮(T)和促性腺激素水平均低而无助于诊断。促性腺激素释放激素(GnRH)试验的价值也有限,因为约三分之一的HH 患者反应正常。D 及 HH 患者经促甲状腺素释放激素直接刺激,出现的血泌乳素(PRL)升高反应分别为正常和低下。胃复安(MET)是多巴胺受体拮抗剂,可     

The immunoneuroendocrine role of melatonin

Abstract: A tight, physiological link between the pineal gland and the immune system is emerging from a series of experimental studies. This link might reflect the evolutionary connection between self-recognition and reproduction. Pinealectomy or other experimental methods which inhibit melatonin synthesis and secretion induce a state of immunodepression which is counteracted by melatonin. In general, melatonin seems to have an immunoenhancing effect that is particularly apparent in immunodepressive states. The negative effect of acute stress or immunosuppressive pharmacological treatments on various immune parameters are counteracted by melatonin. It seems important to note that one of the main targets of melatonin is the thymus, i.e., the central organ of the immune system. The clinical use of melatonin as an immunotherapeutic agent seems promising in primary and secondary immunodeficiencies as well as in cancer immunotherapy. The immunoenhancing action of melatonin seems to be mediated by T-helper cell-derived opioid peptides as well as by lymphokines and, perhaps, by pituitary hormones. Melatonin-induced-immuno-opioids (MHO) and lymphokines imply the presence of specific binding sites or melatonin receptors on cells of the immune system. On the other hand, lymphokines such as -γ-interferon and interleukin-2 as well as thymic hormones can modulate the synthesis of melatonin in the pineal gland. The pineal gland might thus be viewed as the crux of a sophisticated immunoneuroendocrine network which functions as an unconscious, diffuse sensory organ.     

Response of rat pineal melatonin to calcium, magnesium, and lithium is circadian stage dependent

Abstract: Herein we documented the response of pineal melatonin production to electrolytes known to be effective on pineal function in view of a possible circadian stage dependence. We studied the release of melatonin by perifused rat pineal glands at 2 different circadian stages corresponding to the middle of the light and dark periods, i.e., respectively, 7 and 19 HALO (Hours After Light Onset, L:D = 12:12). The initial efflux rates were, as expected, much higher in the perifusates of glands removed from rats sacrificed during the dark phase than of those removed during the light phase. After 3 hr of perifusion, melatonin release reached similar levels which were found constant up to the 8th hr of perifusion, whatever the circadian stage. Perifusion of the glands with physiological concentrations for the rat of calcium (5.2 mmol/1) and magnesium (1.34 mmol/1) resulted in a stimulatory effect on the pineal glands removed from rats sacrificed in the middle of the dark period (19 HALO), whereas no effects were observed on the pineal glands removed from rats sacrificed during the light (7 HALO). Lithium (0.28 and 0.55 mmol/1) was ineffective on melatonin release in pineal glands removed 7 and 19 HALO. Our results show differences in the initial efflux rates of melatonin and in the response of perifused pineal glands to calcium and magnesium according to the circadian stage.     

Changes in connexin43, the gap junction protein of astrocytes, during development of the rat pineal gland

Abstract: The abundance of gap junctions between rat pineal astrocytes formed by connexin43 (Cx43) was studied during development. Levels and distribution of Cx43 were measured by immunoblotting and indirect immunofluorescence, respectively. The amount of Cx43 in cells located within the gland was low until about the 7th postnatal day and increased to adult values between the 14th and 21st days postpartum. Although astrocytes, recognized by their vimentin immunoreactivity, were scarce before birth, they were abundant by the 7th postnatal day suggesting that the low levels of Cx43 found at this age corresponded to a low expression of this protein. Localization of the immunoreactivity to Cx43 and vimentin showed a close correlation, indicating that mature or immature pineal astrocytes form gap junctions made of Cx43. Since Cx43 levels attained their adult values at about the time the innervation and the functional state of the gland reached maturity (2–3 weeks after birth), it is proposed that astrocyte gap junctions are involved in the function of the adult rat pineal gland.     

Conservative management of perforated duodenal diverticulum： A case report and review of the literature

Duodenal diverticula are a relatively common condition. They are asymptomatic, unless they become complicated, with perforation being the rarest but most severe complication. Surgical treatment is the most frequently performed approach. We report the case of a patient with a perforated duodenal diverticulum, which was diagnosed early and treated conservatively with antibiotics and percutaneous drainage of secondary retroperitoneal abscesses. We suggest this method could be an acceptable option for the management of similar cases, provided that the patient is in good general condition and without septic signs.     

Presence of immunoreactive growth hormone and prolactin in the ovine pineal gland

Abstract: The use of antisera raised against bovine growth hormone (GH) and ovine prolactin (PRL) enabled the detection of related immunoreactive (ir) sequences of proteins in ovine pineal tissue. The isolation of PRL-like ir-material was accomplished using a 0.25 M ammonium sulphate (pH 5.5) extraction followed by ethanol precipitation, whereas the resulting 2.0 M ammonium sulphate (pH 7.0) precipitate contained a GH-like immunoreactivity. Gel chromatography of the GH-like immunoreactivity (Sephadex G-100) indicated the presence of several GH-like fragments ranging in the M_r range of 7,000 to 55,000. Analyses of the PRL-like ir-material found in pineal tissue on HPLC using a TSK 545-DEAE column led to the resolution into a single peak of immunoreactivity. A single peak of activity was also observed following chromatofocusing and hydrophobic interaction chromatography of the ir-peak from the TSK 545-DEAE column. The PRL-like ir-material inhibited the binding of [¹²⁵I]ovine PRL-S14 to anti-ovine PRL antibodies without showing an affinity for binding to anti-rat PRL or anti-bovine GH antibodies. Scatchard analysis of the binding of pineal PRL-like ir-material and pituitary ovine PRL-S14 to liver membranes from day-20 pregnant rats revealed similar affinity constants (K_a of 4.7 ± 0.2 × 10⁹ M^-1). In addition, the replication of Nb 2 Node rat lymphoma cells was stimulated by pineal PRL-like ir-material, an effect known to be specific for lactogenic hormones. The pineal PRL-like immunoreactivity appeared on sodium dodecyl sulfate polyacrylamide gels as a single major band of M_r 24,000. The functional status of PRL-and GH-like ir-material in the ovine pineal remains to be determined, but evidence is presented that the overall protein synthesis rate of the rat pineal responded to circulating concentrations of PRL.     

Microbiology of human immunodeficiency virus anorectal disease

PURPOSE: Individuals who are seropositive for the human immunodeficiency virus are at high risk for opportunistic infection and anorectal disorders. Little prospective information is available regarding anorectal pathogens in these patients. METHODS: One hundred sixty-three HIV-seropositive patients presented to the colorectal clinic between 1989 and 1992. Forty-seven (29 percent) patients were thought to have an infectious process and were prospectively studied using a standardized multiculture protocol. RESULTS: Mean age was 33 (range, 19–59) years. All were male; high-risk behavior accounted for 87 percent of HIV transmissions. Presenting complaints included anorectal pain (79 percent), pus per anum (28 percent), and blood per anum (26 percent). Examination revealed perianal tenderness (60 percent), condyloma (38 percent), perianal ulcers (38 percent), and anal fissures (34 percent). Sixty-six sets of cultures were performed; 28 patients had one set, 15 had two sets, and 4 had three sets. Thirty-two of these 47 patients (68 percent) had positive cultures including herpes (50 percent), cytomegalovirus (25 percent),Neisseria gonorrhoeae (16 percent), chlamydia (16 percent), acidfast bacilli (2 percent), and others (9 percent). Six of 32 patients with positive cultures had more than one organism cultured. Sixteen (50 percent) patients with positive cultures were treated medically, 8 (25 percent) were treated surgically and 8 (25 percent) were treated with both modalities. Sixty-one procedures were performed on 17 patients for condylomata. Eighteen patients had 20 procedures for abscesses, 50 percent of whom had positive cultures for other than common bowel flora; all improved. Fourteen patients underwent 33 procedures for perianal fistulas.Mycobacterium fortuitum was cultured from one patient who required 13 procedures for abscesses and fistulas. Forty-five (96 percent) patients were followed for an average of 12.5 months ±2.9 SEM (range, 1–94 months). Symptoms were improved or resolved in 22 of 32 (69 percent) patients with positive cultures and in 11 of 13 (84 percent) with negative cultures. CONCLUSIONS: Specific pathogens may often be identified in human immunodeficiency virus-seropositive patients with anorectal disorders if aggressively sought. Although patients without specific pathogens identified may be expected to improve with planned empiric treatment, positive identification allows more directed therapy.