唐氏综合症概述

M. Coleman
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引用次数: 3

摘要

在唐氏综合症历史上的这个时代,与唐氏综合症患者一起工作是一个具有挑战性和令人兴奋的时刻。刺激唐氏综合症患者中枢神经系统的新方法,加上对这些个体的新医疗护理方法,极大地改变了智力迟钝的主要形式之一的临床病程(图1)。唐氏综合症的发病率在1至412例活产婴儿(Harlap, 1974)和11,023例活产婴儿中有1例(Stark和Mantel, 1967)。大多数发病率数据集中在每688例活产中有1例,这是最好的流行病学调查之一的数据(Collman和Stoller, 1962)。据估计,大约9%的智障人士患有唐氏综合症(Johnston, Speed and Evans, 1976)。从历史上看,唐氏综合症的第一个时期始于1866年,持续了90多年。文献和艺术(Zellweger et al., 1977)都有证据表明,在Langdon-Down最初的临床描述之前,唐氏综合症患者就已经存在了;但从医学的角度来看,1866年以后才开始进行诊断,开始对病人进行分类。其他临床医生(Seguin 1866;邓肯(Duncan, 1866)也描述过病人,但早期的权威都同意兰登-唐恩对他所谓的“蒙古白痴”的描述(彭罗斯和史密斯,1966)(表1)。兰登-唐恩受到当代科学思想的影响,而达尔文的进化论工作占主导地位。他认为,如果疾病可以打破种族障碍,那么它也可以证明人类物种的统一性。因此,他把这些病人命名为“蒙古人”,因为他把内眦眼褶和东方眼褶混淆了。种族理论从未流行起来,但是这个术语
本文章由计算机程序翻译,如有差异,请以英文原文为准。
An Overview of Down's Syndrome
This era in the history of Down's Syndrome is a challenging and exciting time to be working with Down's syndrome patients. New approaches to stimulation of the central nervous system of Down's syndrome patients, combined with new approaches to medical care for these individuals, have dramatically changed the clinical course of one of the major forms of mental retardation (Fig. 1). The incidence of Down's syndrome varies between 1 and 412 live births (Harlap, 1974) and 1 in 11,023 live births (Stark and Mantel, 1967). Most incidence figures cluster around 1 in every 688 live births, which is the figure from one of the best epidemiologic surveys done (Collman and Stoller, 1962). It is estimated that approximately 9 percent of all retarded individuals have Down's syndrome (Johnston, Speed and Evans, 1976). Historically, the first period in the history of Down's syndrome began in 1866 and lasted for over 90 years. There is evidence in both literature and art (Zellweger et al., 1977) that Down's syndrome patients existed prior to their initial clinical description by Langdon-Down; but from a medical point of view, diagnoses started to be made and patients started to be classified after 1866. Other clinicians (Seguin 1866; Duncan, 1866) also have described patients, but the early authorities all agreed in crediting Langdon-Down with the description of what he called "mongolian idiocy" (Penrose and Smith, 1966) (Table 1). Langdon-Down was influenced by contemporary scientific thought, which was dominated by Darwin's work on evolution. He suggested that if disease could break down racial barriers it could also demonstrate the unity of the human species. Thus, he named the patients "mongol" because he confused the epicanthic eye fold with the Oriental fold. The ethnic theory never became popular, but the term
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