肥胖型PCOS患者的生物学特征和临床特征

多囊卵巢综合征（polycystic ovary syndrome,PCOS）是一种内分泌代谢紊乱综合征,临床表现高度异质性。肥胖是PCOS异质性临床表现之一,超过50%的PCOS患者超重或肥胖。肥胖型PCOS主要表现为高雄激素血症、中心型肥胖和糖脂代谢紊乱,非肥胖型PCOS主要表现为黄体生成激素（luteinizing hormone,LH）水平异常升高。尽管肥胖型和非肥胖型PCOS均存在内分泌代谢异常,然而肥胖可加重PCOS糖脂代谢紊乱;肥胖型PCOS还表现脂肪代谢的异常。综述肥胖型PCOS患者的临床特征、性激素水平、糖脂代谢特征,旨在为肥胖型和非肥胖型PCOS患者新的分型诊治提供参考。     

完全型雄激素不敏感综合征2例临床特点及处理

雄激素不敏感综合征(androgen insensitivity syndrome,AIS)又称为睾丸女性化综合征(testicular feminization syndrome,TFS),是一种X连锁遗传病,是男性假两性畸形中较常见的类型,可分为完全型AIS和不完全型AIS,其原因主要是雄激素受体(androgen receptor,AR)基因的突变导致其对雄激素产生抵抗和不应答。本文回顾南京医科大学附属妇产医院2例CAIS患者的临床资料及诊疗过程,以期能进一步提高对该病的认知及诊治水平。     

Social status constrains the stress response in the squirrel monkey.

The influence of dominance on the pituitary-adrenal and gonadal systems was evaluated in male squirrel monkeys. Basal and stress levels of plasma cortisol and testosterone were determined in eight male pairs across a 5-week period. The data indicated that squirrel monkeys have unusually high levels of steroid hormones in comparison to other species. Dominant males had higher levels of cortisol and testosterone and showed a smaller stress response than did subordinate males.     

Maintained aggressive behavior in gonadectomized male Siamese fighting fish (Betta splendens)

Adult male Betta splendens were gonadectomized and tested twice for aggressive behavior two weeks later. In a mutual viewing test environment, it was found that gonadectomized animals maintained their aggressive behavior on all six indices of aggression at a level equal to controls. Animals that showed some testis regeneration and sham operated fish were also equal to controls. The results are interpreted to mean that adult aggressive behavior in male B. splendens may not be under the control of gonadal hormones. Pituitary gonadotropins, interrenal gland, and critical period hypotheses are given as possible explanations of the maintained aggressiveness following castration.     

The inferior supernumerary renal arteries: A classification into three types

Among cases that had multiple renal arteries on one side, an inferior supernumerary renal artery was found in 24/270 cases (ca. 9%) on the right and in 19/270 cases (ca. 7%) on the left, together with the usual renal artery. We have noticed that there are correlations between their levels of origin from the aorta and their positional relation to the ureter and the inferior vena cava (IVC). An inferior supernumerary renal artery (InfRA) of lower origin passes in front of the IVC and behind the ureter. An InfRA of middle origin passes in front of both the IVC and the ureter. An InfRA of upper origin passes behind the IVC and in front of the ureter or renal pelvis. In addition there was a tendency for the lower origin type to have an ureteric branch, while the middle and upper origin types had a gonadal branch. These findings suggest that different derivations lead to the inferior supernumerary renal arteries.     

46,XY女性性发育异常临床分析

目的探讨完全型雄激素不敏感综合征(CAIS)和46,XY单纯性腺发育不全综合征(Swyer综合征)2种最常见46,XY女性性发育异常疾病(DSD)的临床特征、诊疗方式、鉴别诊断要点。方法选择2002—2020年,于中山大学孙逸仙纪念医院就诊的临床病例资料完整的41例46,XY DSD患者,包括20例CAIS(CAIS组)患者和21例Swyer综合征(Swyer组)患者。对该41例患者的临床表现、实验室检查结果、术中情况及组织病理学检查结果进行回顾性分析。本研究遵循的程序符合2013年新修订的《世界医学协会赫尔辛基宣言》要求。结果①41例46,XY DSD患者在出生及成长过程中的社会性别均为女性,就诊年龄为12~38岁。2组患者年龄比较,差异无统计学意义(P>0.05)。CAIS组患者身高、体重均高于或重于Swyer组,并且差异有统计学意义(P<0.05)。②2组患者血清卵泡刺激素(FSH)、睾酮、雌二醇、17α-羟孕酮(17α-OHP)水平比较,差异均有统计学意义(P<0.05);而2组血清促黄体激素(LH)、催乳素、脱氢表雄酮(DHEA)水平比较,则差异均无统计学意义(P>0.05)。CAIS组患者抗苗勒管激素(AMH)水平均>16 ng/mL,Swyer组均<0.03 ng/mL。③39例接受手术治疗患者中,35例行腹腔镜下性腺切除术,4例行开腹肿瘤细胞减灭术。④CAIS组患者性腺肿瘤发生率为35.0%(7/20),Swyer组为36.8%(7/19)。CAIS组患者性腺有自伞端向腹股沟管迁移趋势,其中4例性腺位于腹股沟管内,Swyer组患者性腺均位于盆腔内。⑤41位患者出院后,均继续以女性性别生活,已婚者性生活无障碍。结论CAIS与Swyer综合征患者临床表现相似,CAIS患者存在苗勒管遗迹,Swyer综合征患者存在子宫发育不良等变异,并且均有不同程度的性腺迁移趋势。AMH水平检测可作为区别CAIS与Swyer综合征患者的特异性指标。     

“It's Part of Me,Not All of Me”: Young Women's Experiences of Receiving a Diagnosis Related to Diverse Sex Development

Study ObjectiveTo understand young women's experiences of receiving a diagnosis related to diverse sex development.DesignA qualitative narrative analysis of interviews.SettingKarolinska University Hospital.ParticipantsNine women (aged 20-26 years) with complete androgen insensitivity syndrome, XY or XX gonadal dysgenesis.InterventionsSemistructured interviews.Main Outcome MeasuresA narrative approach was used to analyze the interviews. This involved identification of individual narratives of receiving the diagnosis, as well as identification of key issues that were common across interviews.ResultsThe analysis showed how participants' prediagnosis life experiences framed how medical information was perceived upon diagnosis. All participants had been informed about their condition before the study, but not all remembered the name of their diagnosis. Participants described positive characteristics of health professionals, such as being flexible and able to adapt to patients' individual needs. Clinicians' strategies, such as normalizing patients' experiences, were usually perceived as supportive, but were not always considered helpful. After the diagnosis, participants were worried about potential social, practical, and philosophical issues.ConclusionThis research highlighted the importance of clinicians taking an exploratory and individualized approach to the sensitive process of disclosing a diagnosis related to diverse sex development to young adults. There are various strategies health professionals can use that might help young people to develop their knowledge about their condition: (1) repeating information to help the patient remember; (2) using language that is not too medicalized; and (3) communicating in a way that is meaningfully connected to patients' everyday lives.     

性激素在老年男性骨质疏松发病及治疗中作用的研究进展

骨质疏松（osteoporosis，OP）与年龄增长密切相关，在老年人群中患病率显著高于中青年人群，严重危害老年人群的健康。性激素在男性骨骼代谢中发挥重要作用，其中，雌激素和雄激素都能够促进骨形成，对抗骨量丢失，而孕激素则可能对男性骨量的维持起到负性调节作用。老年男性体内雌激素、雄激素的缺乏与其骨密度降低、OP患病率增加都有着密切的关系，性激素及其受体调节剂为OP的治疗提供了新的思路。雌激素是目前女性绝经后骨质疏松（postmenopausal osteoporosis，PMOP）治疗的常用药物，并不被推荐用于治疗男性OP；雄激素替代治疗可使老年男性骨密度得到增加，但在前列腺和心血管安全性上存疑，因而并不推荐常规使用；选择性雌激素受体调节剂（selective estrogen receptor modulator，SERM）能提升血清睾酮水平，有助于增加腰椎和髋关节骨密度，降低骨折风险，但尚未被正式批准用于治疗男性OP，其疗效及安全性有待更多的临床试验来验证；选择性雄激素受体调节剂（selective androgen receptor modulator，SARM）应用于OP的相关研究尚处于起步阶段，目前仅在动物模型中被证实能够增加雄性大鼠的骨骼强度。本文就性激素在老年男性OP发病及治疗中的作用进行回顾和总结，以期为临床医师的工作提供一定参考。     

延经丸对于绝经后骨质疏松症治疗的探讨

绝经后骨质疏松症的诱发因素很多,主要有种族及遗传因素、绝经后雌激素降低、营养因素、运动因素、不良生活习惯等,但其中最为主要的是绝经后雌激素降低这个因素。我的导师牟慧琴教授根据多年临床实践经验,研究出了一组经验方—延经丸,不但能调节患者体内的内分泌激素和性激素,镇静催眠的作用,而且对于防治女性绝经后骨质疏松症效果十分显著。本文重点从基础理论、组方分析、预防与治疗等方面进行探讨。