Survival patterns and cancer determinants in families with myotonic dystrophy type 1.

ABSTRACT:

Background and purpose

Research indicates that patients with myotonic dystrophy type 1 (DM1) are at increased risk of cancer and early death. Family data may provide insights given DM1 phenotypic heterogeneity, the broad range of non-muscular manifestations and the usual delays in the diagnosis of DM1.

Method

Family history data were collected from 397 genetically and/or clinically confirmed DM1 patients (respondents) enrolled in the US or UK myotonic dystrophy registries. Standardized mortality ratios were calculated for DM1 first-degree relatives (parents, siblings and offspring) by their reported DM1 status (affected, unaffected or unknown). For cancer-related analyses, mixed effects logistic regression models were used to evaluate factors associated with cancer developme

SUBMITTER: Best AF 

PROVIDER: S-EPMC6283697 | biostudies-literature | 2019 Jan

REPOSITORIES: biostudies-literature