穴位埋线治疗更年期综合征及其对性腺激素、β-内啡肽的影响

目的:探索穴位埋线治疗更年期综合征的临床疗效及其机制.方法:将60例患者随机分为埋线组和西药组,每组30例.埋线组选取肾俞、子宫、三阴交进行穴位埋线治疗,西药组每天口服己烯雌酚片,观察比较两组的临床疗效及其对患者血清促卵泡生成激素(FSH)、促黄体生成激素(LH)、雌二醇(E_2)及β-内啡肽的影响.结果:埋线组总有效率为93.3%,西药组总有效率为70.0%,埋线组优于西药组(P<0.05);埋线组β-内啡肽较治疗前明显上升;同时FSH降低,E_2水平升高,与西药组比较差异均有统计学意义(P<0.05,P<0.01).结论:穴位埋线治疗更年期综合征疗效显著,可明显改善患者卵巢功能,对内源性阿片肽类递质具有良性调整作用.     

宫、腹腔镜联合诊治性腺发育不全6例报告

目的：探讨宫、腹腔镜联合诊治性腺发育不全的作用及性腺发育不全的镜下特点。方法：对6例性腺发育不全患者进行宫、腹腔镜检查及腹腔镜下切除的性腺病例资料进行分析。结果：6例均为女性外阴,含有Y染色体,性激素低,5例骨龄明显低于同龄,3例为xo/xy性腺发育不全,3例为xy单纯性性腺发育不全,性腺均位于腹腔内,5例宫腔镜下见有宫颈,2例合并腹股沟斜疝,1例因B超未见子宫外院诊为CAIS,1例xo/xy性腺发育不全者性腺合并性腺母细胞瘤（16.67%）。结论：宫、腹腔镜是鉴别性腺发育不全抑或性激素及功能异常较好的诊断手段,且腹腔镜下观察清晰,在镜下切除性腺组织简单易行,可防止性腺发生病变。     

Extreme virilization in patients with congenital adrenal hyperplasia fails to induce descent of the ovary

The commonly held hypothesis that androgens cause testicular descent in the male [9] predicts that excess endogeous androgens in the female fetus might cause descent of the ovary. To re-evaluate this prediction from a clinical perspective, a retrospective review was made of genotypic females with congenital adrenal hyperplasia and severe virilization. Patients with maximal virilization were included, since this is the group with the earlies and highest production of adrenal androgens during fetal development. Records were reviewed of five children in whom the external genitalia were completely or almost completely masculinized, who had been regarded as males with undescended testes at birth. The position of the ovaries was determined where possible from operative or pathological reports. In four patients the ovaries had been identified at operation, and were found to be in their normal position. In none were the ovaries in or adjacent to the inguinal canal. In the most recent patient, who presented in 1979, laparotomy was not performed because of the belief that the female internal genitalia were normal. In addition, review of the literature revealed two other similar female children with maximal virilization but documented normal ovarian position. The significance of these well-known findings has been ignored in recent studies of testicular descent. Failure of endogenous androgens to affect ovarian position supports the view that initiation of gonadal descent is independent of androgens. Since Müllerian inhibiting substance is the other recognizable hormone in the fetal testis apart from testosterone, it is suggested that it may be responsible for initiating gonadal descent.     

Functional cerebral asymmetries during the menstrual cycle: a cross-sectional and longitudinal analysis

This study aims at answering two basic questions regarding the mechanisms with which hormones modulate functional cerebral asymmetries. Which steroids or gonadotropins fluctuating during the menstrual cycle affect perceptual asymmetries? Can these effects be demonstrated in a cross-sectional (follicular and midluteal cycle phases analyzed) and a longitudinal design, in which the continuous hormone and asymmetry fluctuations were measured over a time course of 6 weeks? To answer these questions, 12 spontaneously cycling right-handed women participated in an experiment in which their levels of progesterone, estradiol, testosterone, LH, and FSH were assessed every 3 days by blood-sample based radioimmunoassays (RIAs). At the same points in time their asymmetries were analyzed with visual half-field (VHF) techniques using a lexical decision, a figure recognition, and a face discrimination task. Both cross-sectional and longitudinal analyzes showed that an increase of progesterone is related to a reduction in asymmetries in a figure recognition task by increasing the performance of the left-hemisphere which is less specialized for this task. Cross-sectionally, estradiol was shown to have significant relationships to the accuracy and the response speed of both hemispheres. However, since these effects were in the same direction, asymmetry was not affected. This was not the case in the longitudinal design, where estradiol affected the asymmetry in the lexical decision and the figural comparison task. Overall, these data show that hormonal fluctuations within the menstrual cycle have important impacts on functional cerebral asymmetries. The effect of progesterone was highly reliable and could be shown in both analysis schemes. By contrast, estradiol mainly, but not exclusively, affected both hemispheres in the same direction.     

Hypothalamic-pituitary-gonadal function in adolescent females with beta-thalassemia major.

OBJECTIVE: To evaluate the function of the hypothalamic-pituitary-gonadal axis in adolescent female patients with beta-thalassemia major. MATERIALS AND METHODS: A prospective study of the function of the hypothalamic-pituitary-gonadal axis function of 31 beta-thalassemia major females aged between 13 and 22 years and in 12 control females aged between 12 and 22 years. All were treated at Princess Rahma Teaching Hospital, Irbid, Northern Jordan between April 2001 and April 2003. RESULTS: Of the 31 beta-thalassemia major females, 13 (41.9%) had delayed puberty. Hypothalamic-pituitary-ovarian axis dysfunction was found in 15 patients (48.4%). Twelve patients (38.7%) had hypogonadotropic hypogonadism and 5 (16.1%) had ovarian failure. High levels of serum ferritin were significantly higher in patients with delayed puberty. CONCLUSION: Pituitary and ovarian dysfunction are common problems in beta-thalassemia major patients. The main possible cause is iron overload. This stresses the need for intensive and regular use of chelation therapy to prevent damage to the hypothalamic-pituitary-ovarian axis.     

79例XY性腺发育异常患者性腺肿瘤发生情况分析

目的 探讨表型为女性、染色体含有Y或Y成分的性腺发育异常患者的性腺肿瘤发生情况.方法 1989年10月至2005年12月间于北京协和医院妇产科因性腺发育异常手术切除双侧性腺的患者共79例,所有患者均取外周血进行淋巴细胞染色体核型分析、Y基因性决定区检测以及相关激素和酶检测,影像学检查了解性腺位置.行经腹或腹腔镜下性腺切除术,所有手术标本均行病理检查.结果 79例患者中,雄激素不敏感综合征患者41例,其中发生精原细胞瘤1例、支持细胞腺瘤2例,占7%;170r羟化酶缺乏症患者14例,其中发生支持细胞腺瘤1例,占7%;XY单纯性腺发育不全患者4例,其中发生性腺母细胞瘤及无性细胞瘤1例,占1/4;XO/XY性腺发育不全患者16例,其中发生精原细胞瘤及性腺母细胞瘤1例,占6%;睾丸退化患者4例中无肿瘤发生.发生肿瘤的性腺多位于盆腔内,发生肿瘤的年龄集中于15～23岁.结论 表型为女性、染色体含有Y或Y成分的性腺发育异常患者易发生性腺母细胞瘤和生殖细胞肿瘤,一旦确诊宜及早切除双侧性腺.     

17α羟化酶缺乏症13例临床分析

目的 探讨染色体核型为46，XY的17α羟化酶缺乏症的诊断及治疗．方法 分析1985年1月至2002年10月北京协和医院妇产科收治的13例染色体核型为46，XY的17α羟化酶缺乏症病例．结果 高血压、低血钾及原发闭经，性激素水平低下，无第二性征发育为该病的临床表现，性腺病理结果是发育不良的睾丸．对1例患者采用PCR和DNA测序进行了SRY基因的分析，结果未发现缺失和突变。经糖皮质激素治疗，满意的控制血钾及血压后，手术切除双侧性腺，维持女性社会性别．术后继续糖皮质激素及雌激素的治疗。结论 正确及时的诊断治疗17α羟化酶缺乏症，维持女性的社会性别，术后继续激素治疗，可获得满意的疗效。     

XY sex-reversed campomelia

A phenotypic female with campomelic syndrome and XY gonadal dysgenesis is described. A minor structural chromosome abnormality was detected in the mother.     

Gonadal dysgenesis and somatic stigmata in patients with 45,X/46,Xr(X) ring chromosome

Two cases of gonadal dysgenesis and stigmata of Turner's syndrome with ring chromosome X are described. Their features support the idea that ring chromosome X should be considered as a deletion in the genetic sense, affecting both the gonadal and statural determinants. Without knowing the cytogenetic findings, these patients are usually labeled as having Turner's syndrome. Furthermore, endocrine data and histologic examination of the gonads are indistinguishable from those of individuals with 45,X or 46XX gonadal dysgenesis.     

Alterations of prenatal morphine exposure in mu-opioid receptor density in hypothalamic nuclei associated with sexual behavior

Our previous work demonstrated that prenatal morphine exposure twice daily during gestational days 11-18 differentially alters male and female sexual behavior. One possible explanation may be that prenatal morphine exposure alters the sexual behavior via alterations of mu-opioid receptors in brain regions involved in reproductive function and behavior, including the ventromedial nucleus of the hypothalamus (VMH), arcuate nucleus (ARC), and medial preoptic area (mPOA). In experiment 1, mu-opioid receptor density was analyzed in three groups of adult male rats: gonadally intact, gonadectomized (GNX), and GNX and testosterone 17beta-propionate-treated (TP). In experiment 2, mu-opioid receptor density was analyzed in four groups of adult female rats: ovariectomized (OVX), OVX and estradiol benzoate-treated (EB), OVX and progesterone-treated (P), and OVX and EB- and P-treated (EB+P). Experiment 1 demonstrated that prenatal morphine exposure lowered the mu-opioid receptor density in the mPOA of adult, gonadally intact and in TP males, while this difference was not apparent in GNX male rats. Experiment 2 demonstrated that prenatal morphine exposure increased mu-opioid receptor density in OVX females, while decreasing it in EB females in the VMH. When compared to our previous sexual behavior data, the present results demonstrate that at least some changes in sexual behavior of adult male and female rats prenatally exposed to morphine may be related to alterations in mu-opioid receptors in brain regions controlling sexual behavior.