Background
Methods
Design
Identification of individuals in datasets
Analysis
Results
Overall mortality and sociodemographic characteristics
Individuals with intellectual disability (n = 15,289) n (%) | Matched control population (n = 76,445) n (%) | |||
---|---|---|---|---|
Sub-sample of individuals with DS (n = 942) n (%) | Sub-sample of individuals with ID (DS excluded) (n = 14,347) n (%) | All individuals with ID (n = 15,289) n (%) | ||
Sex | ||||
Men | 486 (51.6) | 7844 (54.7) | 8330 (54.5) | 41,650 (54.5) |
Women | 456 (48.4) | 6503 (45.3) | 6959 (45.5) | 34,795 (45.5) |
Age group in 2002 | ||||
42–44 year | 88 (9.3) | 1720 (12) | 1808 (11.8) | 9040 (11.8) |
45–54 year | 440 (46.7) | 6044 (42.1) | 6484 (42.4) | 32,420 (42.4) |
55–64 year | 360 (38.2) | 4182 (29.2) | 4542 (29.7) | 22,710 (29.7) |
65–74 year | 50 (5.3) | 1755 (12.2) | 1805 (11.8) | 9025 (11.8) |
75–79 year | 1 (0.1) | 401 (2.8) | 402 (2.6) | 2010 (2.6) |
80+ year | 3 (0.3) | 245 (1.7) | 248 (1.6) | 1240 (1.6) |
Mean age at death (standard deviation)* | 63.5 (5.4) | 72.1 (9.4) | 70.7 (9.5) | 76.2 (10.3) |
Average years of follow-up (standard deviation)* | 9.4 (4.0) | 12.7 (2.7) | 12.5 (2.9) | 13.5 (1.7) |
Number of deaths during 2002–2015 (%) | 761 (80.8) | 3977 (27.7) | 4738 (31.0) | 8364 (10.9) |
Total follow-up period in person-year* | 8848 | 181,940 | 190,788 | 1,032,245 |
Mortality rate during 2002–2015 (per 100,000 people)* | 8600 | 2186 | 2483 | 810 |
Deaths among individuals with intellectual disability (ID) (n = 3147) | Deaths among matched control population | |||
---|---|---|---|---|
Sub-sample of individuals with DS (n = 761) n (%) | Sub-sample of individuals with ID (DS excluded) (n = 3977) n (%) | All individuals with ID (n = 4738) n (%) | Total (n = 8364) n (%) | |
Sex | ||||
Men | 385 (50.6) | 2227 (56.0) | 2612 (55.1) | 4877 (58.3) |
Women | 376 (49.4) | 1750 (44.0) | 2126 (44.9) | 3487 (41.7) |
Age group in 2002 | ||||
42–44 year | 18 (2.4) | 36 (0.9) | 54 (1.1) | 51 (0.6) |
45–54 year | 344 (45.2) | 852 (21.4) | 1196 (25.2) | 1172 (14.0) |
55–64 year | 346 (45.5) | 1446 (36.4) | 1792 (37.8) | 2450 (29.3) |
65–74 year | 49 (6.4) | 1067 (26.8) | 1116 (23.6) | 2501 (29.9) |
75–79 year | 1 (0.1) | 339 (8.5) | 340 (7.2) | 1160 (13.9) |
80+ year | 3 (0.4) | 237 (6.0) | 240 (5.1) | 1030 (12.3) |
Marital status | ||||
Married | 9 (1.2) | 195 (5.0) | 204 (4.4) | 4284 (51.6) |
Not married | 732 (98.8) | 3743 (95.1) | 4475 (95.6) | 4025 (48.4) |
Country of birth | ||||
Swedish-born | 749 (98.4) | 3857 (97.0) | 4606 (97.2) | 7479 (89.4) |
Foreign-born | 12 (1.6) | 120 (3.0) | 132 (2.8) | 885 (10.6) |
Cause-specific mortality proportions and rates
Causes of death | Deaths among individuals with intellectual disability (ID) (n = 4738) | Deaths among matched control population (n = 8364) | Adjusted cause-specific mortality rate ratio (95% CI)* | ||
---|---|---|---|---|---|
Number of deaths n (%) | Cause-Specific Mortality Rate (per 100,000 people) | Number of deaths n (%) | Cause-Specific Mortality Rate (per 100,000 people) | ||
Ch. I – Certain infectious and parasitic diseases, A00-B99 | 181 (3.8) | 95 | 170 (2.0) | 16 | 5.32 (4.32–6.56) |
Ch. II – Neoplasms, C00-D48 | 710 (15.0) | 372 | 2749 (32.9) | 266 | 1.29 (1.19–1.40) |
Ch. III – Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism, D50-D89 | 25 (0.5) | 13 | 21 (0.3) | 2 | 5.95 (3.33–10.6) |
Ch. IV – Endocrine, nutritional and metabolic diseases, E00-E90 | 174 (3.7) | 91 | 228 (2.7) | 22 | 3.82 (3.13–4.65) |
Ch. V – Mental and behavioural disorders, F00-F99 | 224 (4.7) | 117 | 342 (4.1) | 33 | 3.27 (2.77–3.88) |
Ch. VI – Diseases of the nervous system, G00-G99 | 296 (6.3) | 155 | 325 (3.9) | 31 | 4.55 (3.89–5.33) |
Ch. IX – Diseases of the circulatory system, I00-I99 | 1601 (33.8) | 839 | 2959 (35.4) | 287 | 2.71 (2.55–2.87) |
Ch. X – Diseases of the respiratory system, J00-J99 | 807 (17.0) | 423 | 530 (6.3) | 51 | 7.61 (6.82–8.49) |
Ch. XI – Diseases of the digestive system, K00-K93 | 205 (4.3) | 107 | 318 (3.8) | 31 | 3.22 (2.70–3.84) |
Ch. XII – Diseases of the skin and subcutaneous tissue, L00-L99 | 5 (0.1) | 3 | 14 (0.2) | 1 | 1.79 (0.64–4.96) |
Ch. XIII – Diseases of the musculoskeletal system and connective tissue, M00-M99 | 30 (0.6) | 16 | 40 (0.5) | 4 | 3.75 (2.34–6.02) |
Ch. XIV – Diseases of the genitourinary system, N00-N99 | 105 (2.2) | 55 | 99 (1.2) | 10 | 5.30 (4.03–6.98) |
Ch. XVI - Certain conditions originating in the perinatal period, P00-P96 | 9 (0.2) | 5 | 0 (0) | 0 | NA |
Ch. XVII – Congenital malformations, deformations and chromosomal abnormalities, Q00-Q99 | 49 (1.0) | 26 | 5 (0.1) | 0.5 | 49.0 (19.5–123) |
Ch. XVIII – Symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified, R00-R99 | 166 (3.5) | 87 | 208 (2.5) | 20 | 3.99 (3.25–4.89) |
Ch. XIX – Injury, poisoning and certain other consequences of external causes, S00-T98 | 6 (0.1) | 3 | 0 (0) | 0 | NA |
Ch. XX – External causes of morbidity and mortality, V01-Y98 | 144 (3.0) | 75 | 356 (4.3) | 34 | 2.02 (1.67–2.45) |
Causes of death | Number of deaths n (%) | Cause-Specific Mortality Rate (per 1000 people) |
---|---|---|
Ch. X – Diseases of the respiratory system, J00-J99 | 282 (37.1) | 3187 |
Ch. IX – Diseases of the circulatory system, I00-I99 | 197 (25.9) | 2227 |
Ch. V – Mental and behavioural disorders, F00-F99 | 81 (10.7) | 916 |
Ch. VI – Diseases of the nervous system, G00-G99 | 59 (7.8) | 669 |
Ch. I – Certain infectious and parasitic diseases, A00-B99 | 33 (4.3) | 373 |
Ch. XVIII – Symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified, R00-R99 | 28 (3.7) | 317 |
Ch. XI – Diseases of the digestive system, K00-K93 | 16 (2.1) | 181 |
Ch. II – Neoplasms, C00-D48 | 15 (2) | 170 |
Ch. IV – Endocrine, nutritional and metabolic diseases, E00-E90 | 14 (1.8) | 158 |
Ch. XIV – Diseases of the genitourinary system, N00-N99 | 10 (1.3) | 113 |
Ch. XVII – Congenital malformations, deformations and chromosomal abnormalities, Q00-Q99 | 9 (1.2) | 102 |
Ch. XX – External causes of morbidity and mortality, V01-Y98 | 8 (1.1) | 90 |
Ch. III – Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism, D50-D89 | 3 (0.4) | 34 |
Ch. XIX – Injury, poisoning and certain other consequences of external causes, S00-T98 | 3 (0.4) | 34 |
Ch. XIII – Diseases of the musculoskeletal system and connective tissue, M00-M99 | 2 (0.3) | 23 |
Ch. XII – Diseases of the skin and subcutaneous tissue, L00-L99 | 0 (0) | NA |
Ch. XVI - Certain conditions originating in the perinatal period, P00-P96 | 0 (0) | NA |
ICD categories | Underlying causes of death among individuals with DS (n = 761) | Underlying causes of death among individuals with ID (DS excluded) (n = 3977) | Underlying causes of death among matched samples from the control population (n = 8364) |
---|---|---|---|
Ch. IX – Diseases of the circulatory system (I00-I99) |
n = 122 |
n = 1368 |
n = 2959 |
I50 Heart failure (25.4%) | I21 Acute myocardial infarction (23.3%) | I21 Acute myocardial infarction (24.1%) | |
I21 Acute myocardial infarction (14.8%) | I25 Chronic ischaemic heart disease (16.6%) | I25 Chronic ischaemic heart disease (21.3%) | |
I25 Chronic ischaemic heart disease (12.3%) | I50 Heart failure (11.8%) | I50 Heart failure (7.7%) | |
Ch. X – Diseases of the respiratory system (J00-J99) |
n = 51 |
n = 468 |
n = 530 |
J69 Pneumonitis due to solids and liquids (31.4%) | J18 Pneumonia, organism unspecified (50.0%) | J44 Other chronic obstructive pulmonary disease (48.9%) | |
J18 Pneumonia, organism unspecified (19.6%) | J44 Other chronic obstructive pulmonary disease (19.9%) | J18 Pneumonia, organism unspecified (24.5%) | |
J45 Asthma (7.8%) | J69 Pneumonitis due to solids and liquids (10.0%) | J84 Other interstitial pulmonary diseases (8.7%) | |
J20 Acute bronchitis (7.8%) | |||
J98 Other respiratory disorders (7.8%) | |||
Ch. II – Neoplasms in men (C00-D48) |
n = 9 |
n = 381 |
n = 1555 |
D37 Neoplasm of uncertain or unknown behaviour of oral cavity and digestive organs (33.3%) | C18 Malignant neoplasm of colon (12.9%) | C34 Malignant neoplasm of bronchus and lung (18.3%) | |
C25 Malignant neoplasm of pancreas (11.1%) | C61 Malignant neoplasm of prostate (10.5%) | C61 Malignant neoplasm of prostate (16.5%) | |
C67 Malignant neoplasm of bladder (11.1%) | C34 Malignant neoplasm of bronchus and lung (7.1%) | C25 Malignant neoplasm of pancreas (6.8%) | |
C16 Malignant neoplasm of stomach (11.1%) | |||
C90 Multiple myeloma and malignant plasma cell neoplasms (11.1%) | |||
C76 Malignant neoplasm of other and ill-defined sites (11.1%) | |||
C14 Malignant neoplasm of other and ill-defined sites in the lip, oral cavity and pharynx (11.1%) | |||
Ch. II – Neoplasms in women (C00-D48) |
n = 6 |
n = 313 |
n = 1194 |
C67 Malignant neoplasm of bladder (16.7%) | C50 Malignant neoplasm of breast (13.4%) | C34 Malignant neoplasm of bronchus and lung (19.9%) | |
C24 Malignant neoplasm of other and unspecified parts of biliary tract (16.7%) | C18 Malignant neoplasm of colon (9.3%) | C50 Malignant neoplasm of breast (13.0%) | |
C26 Malignant neoplasm of other and ill-defined digestive organs (16.7%) | C34 Malignant neoplasm of bronchus and lung (8.6%) | C25 Malignant neoplasm of pancreas (8.0%) | |
C22 Malignant neoplasm of liver and intrahepatic bile ducts (16.7%) | |||
C57 Malignant neoplasm of other and unspecified female genital organs (16.7%) | |||
C76 Malignant neoplasm of other and ill-defined sites (16.7%) | |||
Ch. VI – Diseases of the nervous system (G00-G99) |
n = 35 |
n = 218 |
n = 325 |
G30 Alzheimer disease (54.3%) | G40 Epilepsy (21.6%) | G30 Alzheimer disease (44.0%) | |
G40 Epilepsy (37.1%) | G80 Cerebral palsy (19.3%) | G20 Parkinson disease (15.7%) | |
G31 Other degenerative diseases of nervous system, not elsewhere classified (5.7%) | G30 Alzheimer disease (10.1%) | G12 Spinal muscular atrophy and related syndromes (12.0%) |
Mortality risk observed in groups
All individuals with intellectual disability vs. their control population (15,289 vs. 76,445 individuals) | Only individuals with Down’s syndrome vs. their control population (942 vs. 4710 individuals) | |
---|---|---|
Hazard Ratio (95% CI) | Hazard Ratio (95% CI) | |
All deaths | 4.14 (3.98–4.31) | 25.4 (21.3–30.4) |
Circulatory diseases-related deaths | 4.20 (3.92–4.50) | 25.0 (17.7–35.4) |
Neoplasm-related deaths | 1.78 (1.63–1.94) | 1.22 (0.70–2.16) |
Respiratory diseases-related deaths | 12.5 (10.9–14.2) | 170 (84.6–345) |
Nervous diseases-related deaths | 6.68 (5.58–7.99) | 41.2 (18.8–90.3) |