中枢性性早熟对儿童体格及性发育的影响

中枢性性早熟（CPP）是一种青春期发育异常,表现为第二性征提前、骨格成熟和体格提前发育,最终影响儿童的成年身高,甚至可能会产生如恐惧、不安等心理行为问题。目前国际上公认治疗最好的药物为促性腺激素释放激素类似物（GnRHa）,其主要目的是改善儿童的最终成年身高;但与此同时,其对患儿的生长发育也存在一些不良反应。该文就CPP及GnRHa治疗对儿童体格及性发育的影响作一综述,以引起临床医师对此疾病及其安全用药的关注。     

Delayed sexual development

Sexual maturation can occur at an early age, at the expected or average time, or late. In adolescents who mature late, osseous development may be delayed. A variety of causes for delayed growth and sexual development should be evaluated. Constitutional delay is a nonpathogenic variation within normal growth parameters. The Tanner criteria should be used in assessing sexual maturation and bone age evaluation for skeletal growth. These criteria are based on the fact that sexual growth follows predictable patterns. Children need reassurance about their developmental growth, and subsequent growth should be monitored carefully.     

男性单纯性肥胖患儿性发育的变化

目的 探讨男性单纯性肥胖患儿性发育的影响。方法 检测42例8-14岁男性单纯性肥胖儿童睾丸容积、阴茎横径长度、血清卵泡刺激素（FSH）、黄体生成素（LH）、雌二醇（E2）、睾酮（TTT）、泌乳素（PRL）、皮质醇（Cortisol）并与正常对照组比较。结果 患儿睾丸容积、阴茎大小及长度，血清FSH、LH、TTT均低于正常对照组，并与其肥胖程度呈负相关。PRL、E2、Cortisol均高于正常对照组，且与其肥胖程度呈正相关。结论 男性单纯性肥胖儿童具有性发育不良倾向。     

环境内分泌干扰物与儿童性分化、性发育异常

环境内分泌干扰物是普遍存在于环境中的一类外源性化学物质,这些物质可干扰体内天然激素的合成、释放、转运、与受体结合、代谢及清除等各个方面,干扰正常激素维持体内平衡和调节发育过程的作用.环境内分泌干扰物被证实是引起儿童性分化、性发育异常的重要致病因素.环境内分泌干扰物可扰乱下丘脑-垂体-性腺轴,干扰雄激素的生物合成、转运、...     

Kisspeptin在性发育中的作用

性发育启动始于促性腺激素释放激素(gonadotrophic hormone,GnRH)神经元脉冲发放GnRH的增加,导致卵泡刺激素和黄体生成素分泌增加,进而下丘脑-垂体-性腺(hypothalamic pituitarygonad,HPG)轴启动.HPG轴在不同生长发育阶段的神经内分泌表现不同,存在性别差异、季节差异等.多种激素可通过调节GnRH分泌,实现对HPG轴的调控作用.Kisspeptin是GnRH的强有力促泌剂,KISSl基因是性腺激素、代谢因素、光照及季节因素的作用靶点.该文对Kisspeptin在性发育中的作用作一综述.     

性发育障碍的诊断与鉴别程序

性发育障碍是成因复杂、临床表型多样的一类疾病。不同病因可有相同或相似的临床表现,同一病因发生时间不同或影响程度不同所致的临床表型差异很大,给诊断带来一定困难。早期确定诊断不仅可以明确患儿性别,还直接影响治疗策略和预后。文章结合近年来性发育和分化的分子生物学和遗传学研究进展,介绍性发育障碍新的分类标准、不同病因的临床特征以及诊断和鉴别程序。     

Children of homoparental families: psychological and sexual development

AIM: In the last few years some studies have been made about homosexual parenthood and especially if this interferes with the child's development, identity and future sexual tendency. These studies prove that the parents' homosexuality does not seem to compromise the child's psychosexual development and that the child's possible homosexuality does not depend on the parents' sexual behaviour but on different factors linked to the relationship; as a matter of fact there are no relevant differences in the occurrence of homosexuality in children born from homosexual and heterosexual parents. The present study investigated the psychological and sexual development in a group of children of homoparental family. METHODS: The sample observed included 37 children, 22 male and 15 female. The following psychodiagnostic tests were used for assessment: spontaneous drawing, draw-a-person test, family drawing, blacky pictures test. The developmental of the child's sexual identity was determined through the preferences and fantasies he/she showed while playing, in the relationship with his/her mates, in the way of dressing. We used interviews with the child and the parents. RESULTS: All the children showed a good adherence to their sexual role. The observations and interviews proved that the children's disorders are not linked to the parents' homosexuality. CONCLUSIONS: Being brought up by homosexual parents does not seem to compromise the child's future sexual tendency. However, in these children the sexual preference could change in adult life, being influenced by future situations and circumstances.     

Disorders of sexual differentiation and development. Psychological aspects

Children with abnormalities in sexual differentiation and development can have a smooth course of psychosocial development in spite of the significant risks and challenges they face. Chances for a positive emotional outcome are made more likely by the careful handling of these patients at the time of first presentation. Parents' unambiguous acceptance of the child's sex of rearing and early surgical intervention to normalize the child's external genital appearance are critical elements in a positive outcome. Further, the patterns of behavior documented in the materials reviewed in this article suggest difficulties of immaturity and social development rather than significant psychopathology. Parent-child interactions were repeatedly found to be central to the child's emotional well-being, underscoring the need to provide parents with adequate counsel and support. These patterns, however, represent findings across groups of patients and cannot predict the emotional, social, or academic functioning of any individual. Within all these clinical syndromes there is great individual variation in social, emotional, and physical presentation. Finally, rather than minimizing problems, physicians need to educate parents so they can be active advocates for their children in the educational and social arenas.     

Surgical and ethical challenges in disorders of sexual development

A resolution to the difficulties faced by parents, physicians, and pediatric patients in treating DSDs will only come with better communication and improved research methodologies. Advocacy groups and the Internet have allowed the intersex community to have a larger role in guiding the research and the ethical frameworks that are used in treating these disorders. These disorders are unusual and collaboration across medical centers should be the rule rather than the exception. When possible, treatments that are innovative or experimental should be subjected to rigorous research oversight [29,30]. Defined periods of family crisis in which counseling and education become important are at the time of diagnosis [30,31], at the time of any surgical procedure, and at the beginning of major developmental stages. Historically, children were often left uninformed until someone judged them old and mature enough to comprehend how they were different. These attempts to protect individual children from their condition may have left them vulnerable to a personal crisis at an age when sexual identity and identity with a peer group are important. Both the needs of the child and the adult the child will become should be considered in making treatment decisions for children and adolescents with DSDs. It is best to counsel parents and educate developing children in a way that parallels chronologic and conceptual growth. When possible, the child should be involved in an age-appropriate fashion in the decision-making process and accurate information about the child's history and body should be made available. In addition, parents and families need as much information as possible and support systems that will help them navigate these challenging situations.     

Growth and sexual development in children with meningomyleocoele

Forty-five children (25 girls; 20 boys) with meningomylcocoele (MMC) were assessed for growth, skeletal maturation and pubertal development. The spinal defects were operated on shortly after birth and all children required cerebral drainage for hydrocephalus.Standard deviation scores for height, sitting height, subischial leg length, head circumference, weight and bone age were compared with aged-matched data from a normal Swiss population. The children with MMC were shorter (height SDS boys -2.34±1.57; girls -2.01±1.57, mean±SD, P<0.0005), secondary to a decrease in trunk and lower limb length: Arm length was normal. The head circumference was increased, significantly in the girls (SDS+1.49±1.21, P<0.005). Weight was decreased in both sexes, this being significant in the boys (SDS -1.11±1.23, P<0.005); however, comparison of weight with height suggested that children with MMC were relatively obese. Bone age was significantly advance in both boys (SDS+1.07±2.13, P<0.025) and girls (SDS+1.36±1.77, P<0.0001). Secondary sex characteristics appeared early in both boys and girls and one girl and one boy presented with puberty advanced by several years. Cryptorchidism occurred in 25% of boys with MMC.Abbreviations MMC meningomyleocoele - H height - SH sitting height - AL arm length - W weight - SILH subischial leg height - BA bone age - SDS standard deviation scores