Survival among people with Down syndrome: a nationwide population-based study in Denmark

Zhu, Jin Liang; Hasle, Henrik; Correa, Adolfo; Schendel, Diana; Friedman, Jan M.; Olsen, Jørn; Rasmussen, Sonja A.

doi:10.1038/gim.2012.93

Cited by 74 publications

(77 citation statements)

References 31 publications

(36 reference statements)

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“…20,21 Mortality among people with Down syndrome has been more widely studied. [22][23][24][25] The findings from these investigations are consistent with the high risk of death we found among people with Down syndrome. For example, researchers in a large Danish population study concluded that people with standard trisomy 21 had a mortality hazard ratio of 8.9, 24 and a smaller American study showed that community-residing adults with Down syndrome were almost 4 times as likely to die as adults with other types of intellectual disability.…”

Section: Contextsupporting

confidence: 80%

“…[22][23][24][25] The findings from these investigations are consistent with the high risk of death we found among people with Down syndrome. For example, researchers in a large Danish population study concluded that people with standard trisomy 21 had a mortality hazard ratio of 8.9, 24 and a smaller American study showed that community-residing adults with Down syndrome were almost 4 times as likely to die as adults with other types of intellectual disability. 25 The UK confidential inquiry mentioned earlier reported a high proportion of deaths amenable to health care intervention, but the study researchers were able to compare this proportion only with the national average; they were unable to quantify absolute and relative risks.…”

Section: Contextsupporting

confidence: 80%

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Mortality Among Adults With Intellectual Disability in England: Comparisons With the General Population

Hosking¹,

Carey²,

Shah³

et al. 2016

Am J Public Health

191

174

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Objectives. To describe mortality among adults with intellectual disability in England in comparison with the general population.Methods. We conducted a cohort study from 2009 to 2013 using data from 343 general practices. Adults with intellectual disability (n = 16 666; 656 deaths) were compared with age-, gender-, and practice-matched controls (n = 113 562; 1358 deaths).Results. Adults with intellectual disability had higher mortality rates than controls (hazard ratio [HR] = 3.6; 95% confidence interval [CI] = 3.3, 3.9). This risk remained high after adjustment for comorbidity, smoking, and deprivation (HR = 3.1; 95% CI = 2.7, 3.4); it was even higher among adults with intellectual disability and Down syndrome or epilepsy. A total of 37.0% of all deaths among adults with intellectual disability were classified as being amenable to health care intervention, compared with 22.5% in the general population (HR = 5.9; 95% CI = 5.1, 6.8).Conclusions. Mortality among adults with intellectual disability is markedly elevated in comparison with the general population, with more than a third of deaths potentially amenable to health care interventions. This mortality disparity suggests the need to improve access to, and quality of, health care among people with intellectual disability. (Am J Public Health.

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Section: Contextsupporting

confidence: 80%

Section: Contextsupporting

confidence: 80%

Mortality Among Adults With Intellectual Disability in England: Comparisons With the General Population

Hosking¹,

Carey²,

Shah³

et al. 2016

Am J Public Health

191

174

View full text Add to dashboard Cite

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“…Mortality in people with Down syndrome has been widely studied. 125,[127][128][129][130] A large Danish study 129 of 3530 persons with Down syndrome found a HR of 8.94 for standard trisomy 21 versus the general population for mortality between 1968 and 2009, which compares closely with the HR of 9.21 (see Table 23) that we found before any adjustment for comorbidity. A smaller American study 130 of 169 adults with Down syndrome residing in the community found an adjusted risk of death almost four times as high (3.77) as that for other adults with ID without Down syndrome.…”

Section: Mortalitysupporting

confidence: 75%

An evaluation of the effectiveness of annual health checks and quality of health care for adults with intellectual disability: an observational study using a primary care database

Carey

Hosking

Harris

et al. 2017

Health Serv Deliv Res

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Background People with intellectual disability (ID) have poorer health than the general population; however, there is a lack of comprehensive national data describing their health-care needs and utilisation. Annual health checks for adults with ID have been incentivised through primary care since 2009, but only half of those eligible for such a health check receive one. It is unclear what impact health checks have had on important health outcomes, such as emergency hospitalisation. Objectives To evaluate whether or not annual health checks for adults with ID have reduced emergency hospitalisation, and to describe health, health care and mortality for adults with ID. Design A retrospective matched cohort study using primary care data linked to national hospital admissions and mortality data sets. Setting A total of 451 English general practices contributing data to Clinical Practice Research Datalink (CPRD). Participants A total of 21,859 adults with ID compared with 152,846 age-, gender- and practice-matched controls without ID registered during 2009–13. Interventions None. Main outcome measures Emergency hospital admissions. Other outcomes – preventable admissions for ambulatory care sensitive conditions, and mortality. Data sources CPRD, Hospital Episodes Statistics and Office for National Statistics. Results Compared with the general population, adults with ID had higher levels of recorded comorbidity and were more likely to consult in primary care. However, they were less likely to have long doctor consultations, and had lower continuity of care. They had higher mortality rates [hazard ratio (HR) 3.6, 95% confidence interval (CI) 3.3 to 3.9], with 37.0% of deaths classified as being amenable to health-care intervention (HR 5.9, 95% CI 5.1 to 6.8). They were more likely to have emergency hospital admissions [incidence rate ratio (IRR) 2.82, 95% CI 2.66 to 2.98], with 33.7% deemed preventable compared with 17.3% in controls (IRR 5.62, 95% CI 5.14 to 6.13). Health checks for adults with ID had no effect on overall emergency admissions compared with controls (IRR 0.96, 95% CI 0.87 to 1.07), although there was a relative reduction in emergency admissions for ambulatory care-sensitive conditions (IRR 0.82, 95% CI 0.69 to 0.99). Practices with high health check participation also showed a relative fall in preventable emergency admissions for their patients with ID, compared with practices with minimal participation (IRR 0.73, 95% CI 0.57 to 0.95). There were large variations in the health check-related content that was recorded on electronic records. Limitations Patients with milder ID not known to health services were not identified. We could not comment on the quality of health checks. Conclusions Compared with the general population, adults with ID have more chronic diseases and greater primary and secondary care utilisation. With more than one-third of deaths potentially amenable to health-care interventions, improvements in access to, and quality of, health care are required. In primary care, better continuity of care and longer appointment times are important examples that we identified. Although annual health checks can also improve access, not every eligible adult with ID receives one, and health check content varies by practice. Health checks had no impact on overall emergency admissions, but they appeared influential in reducing preventable emergency admissions. Future work No formal cost-effectiveness analysis of annual health checks was performed, but this could be attempted in relation to our estimates of a reduction in preventable emergency admissions. Funding The National Institute for Health Research Health Services and Delivery Research programme.

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“…3 The survival of individuals with Down syndrome has increased in recent decades (owing mainly to improved management of congenital heart defects), resulting in large numbers of adults with Down syndrome. 4 There is a need for studies of health issues in this growing population. Previous studies have suggested a lower incidence of solid tumors in adults with Down syndrome, but the results have been limited by the inclusion of a relatively small proportion of older individuals.…”

Section: Introductionmentioning

confidence: 99%

Low risk of solid tumors in persons with Down syndrome

Hasle

Friedman

Olsen

et al. 2016

Genetics in Medicine

Self Cite

132

125

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Purpose: The aim of this study was to investigate cancer incidence in a large cohort of persons with Down syndrome.Methods: Down syndrome was identified from the Danish Cytogenetic Register. Cancer occurrence was identified by linkage to the Danish Cancer Registry. Standardized incidence ratios (SIRs) and 95% confidence intervals (CIs) were calculated based on observed and expected numbers from rates for all Danish residents. The cohort consisted of 3,530 persons with Down syndrome contributing 89,570 person-years at risk.Results: Acute leukemia risk was highest from 1-4 years of age and remained elevated until age 30. The overall risk of solid tumors was decreased (SIR 0.45; 95% CI 0.34-0.59), especially in persons 50 years or older (SIR 0.27; 95% CI 0.16-0.43). We found a significantly lower risk of lung cancer (SIR 0.10; 95% CI 0.00-0.56), breast cancer (SIR 0.16; 95% CI 0.03-0.47), and cervical cancer (SIR 0.0; 95% CI 0.00-0.77). Testicular cancer was the only solid tumor with an increased SIR (2.9; 95% CI 1.6-4.8). Conclusions:The risk of all major groups of solid tumors was decreased, except testicular cancer. Altered screening strategies should be considered for persons with Down syndrome. This unusual pattern of cancer occurrence may help understanding carcinogenesis in the general population.

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Survival among people with Down syndrome: a nationwide population-based study in Denmark

Cited by 74 publications

References 31 publications

Mortality Among Adults With Intellectual Disability in England: Comparisons With the General Population

Mortality Among Adults With Intellectual Disability in England: Comparisons With the General Population

An evaluation of the effectiveness of annual health checks and quality of health care for adults with intellectual disability: an observational study using a primary care database

Low risk of solid tumors in persons with Down syndrome

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