Research Article
Assessment of Respiratory Function and Need for Noninvasive Ventilation in a Cohort of Patients with Myotonic Dystrophy Type 1 Followed at One Single Expert Center
Table 3
Clinical and demographic comparison between cohorts according to NIV indication.
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All values are presented as median and interquartile range, except where otherwise indicated. ,§,° significance is indicated after Bonferroni correction. CTG expansion size was classified according to the scale of Tsilfidis and colleagues (Tsilfidis C, Mackenzie AE, Mettler G, et al. Correlation between CTG trinucleotide repeat length and frequency of severe congenital myotonic dystrophy. Nat Genet 1992; 1 : 192–5): E0, 38–79; E1, 80–499; E2, 500–999; E3, 1000–1499; E4, >1500. NIV, noninvasive ventilation; MIRS, Muscular Impairment Rating Scale; BMI, body mass index; PM, pacemaker; ICD, implantable cardiac defibrillator. |