Puberty, Delayed

  • 文章类型: Journal Article
    Endo-EuropeanReferenceNetwork指南计划启动,包括16名内分泌学经验丰富的临床医生。儿科和成人以及2名患者代表。该指南得到了欧洲儿科内分泌学会的认可,欧洲内分泌学会和欧洲男科学院。目的是为先天性垂体或性腺激素缺乏症患者的临床评估和青春期诱导制定实践指南。进行了系统的文献检索,证据根据建议的分级进行分级,评估,开发和评估系统。如果证据不足或缺乏,然后结论是基于专家意见。该指南包括用雌激素或睾酮诱导青春期的建议。综述了有关在低促性腺激素性腺功能减退中用卵泡刺激激素和人绒毛膜促性腺激素诱导青春期的出版物。考虑了Klinefelter综合征或雄激素不敏感综合征患者的特定问题。专家小组建议,应由多学科小组照顾青春期诱导或性激素替代以维持青春期。患有已知疾病的儿童应从女孩的8岁和男孩的9岁开始随访。青春期诱导应个性化,但女孩应考虑11岁,男孩应考虑12岁。在患有性发育障碍或先天性垂体缺陷的个体中,青春期和生育问题的心理方面尤其重要。这些年轻人的转变凸显了多学科方法的重要性,讨论在这些慢性病的背景下出现的医学问题以及社会和心理问题。
    An Endo-European Reference Network guideline initiative was launched including 16 clinicians experienced in endocrinology, pediatric and adult and 2 patient representatives. The guideline was endorsed by the European Society for Pediatric Endocrinology, the European Society for Endocrinology and the European Academy of Andrology. The aim was to create practice guidelines for clinical assessment and puberty induction in individuals with congenital pituitary or gonadal hormone deficiency. A systematic literature search was conducted, and the evidence was graded according to the Grading of Recommendations, Assessment, Development and Evaluation system. If the evidence was insufficient or lacking, then the conclusions were based on expert opinion. The guideline includes recommendations for puberty induction with oestrogen or testosterone. Publications on the induction of puberty with follicle-stimulation hormone and human chorionic gonadotrophin in hypogonadotropic hypogonadism are reviewed. Specific issues in individuals with Klinefelter syndrome or androgen insensitivity syndrome are considered. The expert panel recommends that pubertal induction or sex hormone replacement to sustain puberty should be cared for by a multidisciplinary team. Children with a known condition should be followed from the age of 8 years for girls and 9 years for boys. Puberty induction should be individualised but considered at 11 years in girls and 12 years in boys. Psychological aspects of puberty and fertility issues are especially important to address in individuals with sex development disorders or congenital pituitary deficiencies. The transition of these young adults highlights the importance of a multidisciplinary approach, to discuss both medical issues and social and psychological issues that arise in the context of these chronic conditions.
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    文章类型: Journal Article
    Internationally and within Aotearoa, New Zealand, there has been a substantial increase in the demand for gender affirming healthcare over the past decade. It is likely that this level of referrals to health services will continue in the foreseeable future. The Guidelines for Gender Affirming Healthcare for Gender Diverse and Transgender Children, Young People and Adults in Aotearoa, New Zealand were developed following the recognition that the previous good practice guide required updating to be in step with current practice and international standards. This article presents a summary of the guideline focusing on puberty blockers, hormonal therapies, access to surgery and other gender affirming healthcare. We hope these guidelines will support the development and provision of services providing gender affirming healthcare around the country and provide helpful guidance to all health professionals involved in the care of trans people.
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  • 文章类型: Journal Article
    Although various expert and consensus meetings have been held, there remains uncertainty about the definition of idiopathic short stature (ISS) and its subgroups. In this short review, the hypothetical pathophysiology, diagnosis and subcategorization of ISS are discussed. ISS in childhood may be the result of a combination of variants of three groups of genes: (1) single genes (or combinations of a few single genes) of which mutations, deletions or duplications are associated with a relatively large negative effect on height; (2) genes of which relatively frequently occurring polymorphisms are associated with a small negative effect on height; and (3) genes associated with delayed maturation of the epiphyseal plate. Differentiation between ISS and short children born small for gestational age and those with dysmorphic syndromes, systemic diseases or growth hormone deficiency and resistance can be difficult. Subcategorization based on distance to target height and onset of puberty is arbitrary, but useful.
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