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Comparative All-Cause Mortality Among a Large Population of Patients with Spinal Muscular Atrophy Versus Matched Controls.
- Source :
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Neurology and therapy [Neurol Ther] 2022 Mar; Vol. 11 (1), pp. 449-457. Date of Electronic Publication: 2021 Dec 22. - Publication Year :
- 2022
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Abstract
- Introduction: There is little information about survival of spinal muscular atrophy (SMA) patients into adulthood, in particular from population-based samples. We estimated and compared age-specific, all-cause mortality rates in patients with SMA and matched controls in a large, retrospective cohort study using electronic health records (EHRs) from the pre-treatment era.<br />Methods: The US Optum <superscript>®</superscript> de-identified EHR database contains EHRs for ~ 104 million persons (study period: January 1, 2007-December 22, 2016). SMA cases were identified by one or more International Classification of Diseases, Ninth/Tenth Edition codes for SMA. Controls with no SMA diagnosis code were matched 10:1 to SMA cases based on birth year, gender, and first diagnostic code date. For both groups, ≥ 1 month of observation and (if deceased) a valid date of death were required for inclusion. Age-specific mortality rates per person-year (PY) and hazard ratios were calculated.<br />Results: Five thousand one hundred seventy-nine SMA cases and 51,152 controls were analyzed. The overall hazard ratio comparing cases with controls was 1.76 (95% CI 1.63-1.90). In patients with SMA type III diagnostic codes only, the all-age mortality rate was 1059/100,000 PYs in cases and 603/100,000 PYs in controls. In older age groups (13-20, 21-30, 31-40, 41-50, 51-60, and > 60 years), age-specific mortality rates for cases consistently exceeded those of controls. Limitations of this study included the inability to confirm the SMA diagnosis or SMA type by genetic or clinical confirmation.<br />Conclusion: Patients with SMA of all ages, including adults and type III patients, had a higher all-cause mortality rate as compared to age-matched controls during the pre-treatment era.<br /> (© 2021. The Author(s).)
Details
- Language :
- English
- ISSN :
- 2193-8253
- Volume :
- 11
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Neurology and therapy
- Publication Type :
- Academic Journal
- Accession number :
- 34936050
- Full Text :
- https://doi.org/10.1007/s40120-021-00307-7