Table 2.

Distribution of observed and expected deaths among myotonic dystrophy patients by causes of death

DeathsStandardized mortality ratio
Observed (O), n (%)Expected (E),* nO/E95% CI
* Matched general population of the same age; based on 1987–1990 Quebec life tables (Bureau de la Statistique du Québec).
† International Classification of Diseases, 9th revision, clinical modification.
‡ Prostatic adenocarcinoma (2), digestive system cancer (2), thymoma (1), pleural mesothelioma (1), cerebral astrocytoma (1), acute myeloblastic leukemia (1).
Diseases of the respiratory system (460–519)32 (42.6)0.656.638.7–78.0
Acute respiratory failure11
Diseases of the circulatory system (390–459)15 (20.0)–7.7
Congestive heart failure5
Acute myocardial infarction4
Pulmonary embolism4
Acute cerebrovascular disease1
Ruptured thoracic aneurysm1
Sudden death (798)8 (10.7)0.0Undetermined
Neoplasms (140–239)8 (10.7)32.51.1–4.6
Others9 (12.0)
Infectious disease2
Unknown3 (4.0)
Total75 (100.0)–9.1