Mortality associated with Down's syndrome in the USA from 1983 to 1997: a population-based study
Mortality associated with Down's syndrome in the USA from 1983 to 1997: a population-based study
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DOI：
10.1016/s0140-6736(02)08092-3
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发表时间：
2002-03-23
期刊：
影响因子：
168.9
通讯作者：
Friedman, JM
中科院分区：
文献类型：
--
作者：
Yang, QH;Rasmussen, SA;Friedman, JM
唐氏综合症是最常见的导致智力迟钝的原因，但关于唐氏综合症患者的死亡率和合并症的信息有限。方法：我们使用1983年至1997年美国死亡证明的数据，计算唐氏综合征患者常见医学疾病的死亡年龄中位数和标准化死亡率比值比（SMORs）。在17897例唐氏综合征患者中，平均死亡年龄从1983年的25岁增加到1997年的49岁，平均每年增加1.7岁（p < 0.0001）。患有唐氏综合症的黑人和其他种族的人的平均死亡年龄明显低于白人。正如预期的那样，诊断为唐氏综合症的死亡证明比没有报告唐氏综合症的死亡证明更有可能列出先天性心脏缺陷（SMOR 29.1, 95% CI 27.8-30.4）、痴呆（21.2,19.6-22.7）、甲状腺功能减退（20.3,18.5-22.3）或白血病（1.6,1.4-1.8）。相比之下，在唐氏综合症患者的死亡证明上，除白血病以外的恶性肿瘤所占比例不到预期的十分之一（0.07,0.06-0.08）。恶性肿瘤的低SMOR与唐氏综合症有关，在所有年龄，男女，以及除白血病和睾丸癌外的所有常见肿瘤类型。解释：确定造成记录的种族差异的因素可能有助于进一步改善唐氏综合症患者的生存率。减少暴露于导致癌症风险的环境因素，21号染色体上的肿瘤抑制基因，或者唐氏综合症细胞中较慢的复制速率或较高的细胞凋亡可能性，可能是唐氏综合症患者很少患癌症的原因。
Background Down's syndrome is the most frequently identified cause of mental retardation, but information about mortality and comorbidity in people with Down's syndrome is limited.Methods We used data from US death certificates from 1983 to 1997 to calculate median age at death and standardised mortality odds ratios (SMORs) for common medical disorders in people with Down's syndrome.Findings Of 17 897 people reported to have Down's syndrome, median age at death increased from 25 years in 1983 to 49 years in 1997, an average increase of 1.7 years per year studied (p < 0.0001). Median age at death was significantly lower in black people and people of other races than in white people with Down's syndrome. As expected, death certificates with a diagnosis of Down's syndrome were more likely to list congenital heart defects (SMOR 29.1, 95% CI 27.8-30.4), dementia (21.2, 19.6-22.7), hypothyroidism (20.3, 18.5-22.3), or leukaemia (1.6, 1.4-1.8) than were those that did not report Down's syndrome. By contrast, malignant neoplasms other than leukaemia were listed on death certificates of people with Down's syndrome less than one-tenth as often as expected (0.07, 0.06-0.08). A strikingly low SMOR for malignancy was associated with Down's syndrome at all ages, in both sexes, and for all common tumour types except leukaemia and testicular cancer.Interpretation Identification of factors responsible for the racial differences recorded could facilitate further improvement in survival of people with Down's syndrome. Reduced exposure to environmental factors that contribute to cancer risk, tumour-suppressor genes on chromosome 21, or a slower rate of replication or higher likelihood of apoptosis in Down's syndrome cells, could be possible reasons for paucity of cancer in people with Down's syndrome.