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[特纳综合征:问题概述、现状、护理建议及儿童、青少年和成年期监测方案]

[Turner syndrome: overview of problems, present status, proposals for care and a protocol for monitoring in childhood, adolescence and adulthood].

作者信息

Snajderová M, Heresová J, Mardesić T, Krenek M, Horejsí J, Kalvachová B, Popelová J, Mikesová E, Wohlová G, Feber J, Gerzová J, Hrobonová V, Teslík L, Zounarová M, Vejvalková S

机构信息

Klinika nukleární medicíny 2. LF UK a FNM, Praha.

出版信息

Cas Lek Cesk. 2001 Aug 30;140(17):533-7.

Abstract

BACKGROUND

Girls and adolescents with Turner syndrome (TS) usually receive intensive medical care in a multidisciplinary team, coordinated by paediatric endocrinologist. Majority of them are discharged from specialist clinics following the induction of puberty and attainment of final height. Patients with Turner syndrome have a reduced life expectancy, they are known to have multi-system impairments in addition to the short stature and to the absence of sexual development. Aim of this study is to propos a continuous follow-up by multidisciplinary team of physicians starting in childhood and following the discharge from the paediatric care.

METHODS AND RESULTS

This paper highlights the medical and psychosocial problems associated with Turner syndrome in childhood, adolescence and in adulthood. Analysis of these problems served as a background to management strategy.

CONCLUSIONS

Women with Turner syndrome are at risk of number of medical problems. Quality of their life and the life expectancy can be improved with increasing awareness to comorbities associated with Turner syndrome. Assisted reproduction technologies has recently offered a chance for pregnancy and delivery of a healthy child also to women with Turner syndrome. Therefore, long-term follow-up by multidisciplinary team of physicians knowledgeable about these medical problems is necessary. Introduction of a centralised system of systematic multidisciplinary approach to patients with Turner syndrome from childhood and adolescence to adulthood seems to be very important.

摘要

背景

患有特纳综合征(TS)的女孩和青少年通常在由儿科内分泌学家协调的多学科团队中接受强化医疗护理。她们中的大多数在青春期启动和达到最终身高后从专科诊所出院。特纳综合征患者的预期寿命缩短,除身材矮小和性发育缺失外,已知还存在多系统损害。本研究的目的是提议由多学科医生团队从儿童期开始并在儿科护理出院后进行持续随访。

方法与结果

本文强调了儿童期、青春期和成年期与特纳综合征相关的医学和心理社会问题。对这些问题的分析作为管理策略的背景。

结论

患有特纳综合征的女性面临多种医学问题的风险。随着对与特纳综合征相关的合并症认识的提高,她们的生活质量和预期寿命可以得到改善。辅助生殖技术最近也为患有特纳综合征的女性提供了怀孕和生育健康婴儿的机会。因此,由了解这些医学问题的多学科医生团队进行长期随访是必要的。从儿童期到青春期再到成年期,为特纳综合征患者引入集中化的系统多学科方法似乎非常重要。

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