Cancer Risk in Myotonic Dystrophy Type I: Evidence of a Role for Disease Severity.

ABSTRACT:

Background

Myotonic dystrophy type 1 (DM1) is an inherited trinucleotide repeat disorder in which specific cancers have been implicated as part of the disease phenotype. This study aimed to assess whether cancer risk in DM1 patients is modified by disease severity.

Methods

Using the United Kingdom Clinical Practice Research Datalink (primary care electronic medical records), we identified a cohort of 927 DM1 and a matched cohort of 13 085 DM1-free individuals between January 1, 1988 and February 29, 2016. We used Cox regression models to calculate the hazard ratios (HRs) and 95% confidence intervals (CIs) of organ-specific cancer risks. Analyses were stratified by age at DM1 diagnosis as a surrogate for disease severity. Statistical tests were two-sided.

Results

Patie

SUBMITTER: Alsaggaf R 

PROVIDER: S-EPMC6286884 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature