ArticlesMortality associated with Down's syndrome in the USA from 1983 to 1997: a population-based study
Introduction
Down's syndrome, the most frequently identified cause of mental retardation, has a prevalence of about 1 in 800 livebirths and stillbirths.1 Survival is lower in people with Down's syndrome than in those without this disorder.2, 3, 4 The most frequent causes of death in people with Down's syndrome are congenital heart defects and respiratory infections.4, 5, 6, 7, 8 In the past 50 years, survival beyond the first year of life has improved strikingly for people with Down's syndrome, from below 50%9 to more than 90%.4 Most studies of death in people with this disorder have focused on survival in the first year of life, and almost all people included in these studies were white.
Satge and colleagues10 reviewed studies of cancers in people with Down's syndrome published before 1997 and concluded that malignant solid tumours seem to be under-represented compared with the general population. These investigators noted that cancer studies in Down's syndrome had limited numbers of participants and thus limited power to make general inferences. Hasle and colleagues11 did a cohort study of 2814 people with Down's syndrome and reported a high risk of leukaemia in affected children but a low risk of solid tumours in all age-groups.
We studied patterns of mortality and morbidity in over 17 800 people with Down's syndrome who died during a 15-year period. We assessed changes in age at death by racial group, most frequent diseases associated with death, and occurrence of major categories of malignant neoplasms.
Section snippets
Study protocol
We obtained data from multiple-cause mortality files (MCMFs) compiled by the US Centers for Disease Control and Prevention National Center for Health Statistics for 1983–97. MCMFs include: demographic information, International Classification of Disease ninth edition (ICD9) codes for underlying cause of death, and up to 20 disorders that are listed on death certificates.12 Death certificates record information only for livebirths; stillbirths are not included.13 MCMFs have used ICD9 codes since
Results
MCMFs for 1983–97 contain records for over 32 million deaths, 17 958 of which list Down's syndrome as a diagnosis. Of these, 61 records also listed the code for pregnancy termination and age 0 years, and these were excluded from the analysis. The remaining 17 897 records represented 5·6 deaths in people with Down's syndrome per 10 000 total deaths in the USA for the study period. Racial distribution in people with Down's syndrome was 87% white, 11% black, and 2% others. Of all US deaths during
Discussion
Our results show that median age at death of people with Down's syndrome rose strikingly in the USA from 1983 to 1997 but that substantial disparity exists between different racial groups. As expected, congenital heart defects and respiratory infections were the most frequently reported medical disorders on death certificates of people with Down's syndrome. Malignancies, except leukaemia and testicular cancer, were reported much less often than expected in people with Down's syndrome.
We used
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