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.

Carola R Ferrari AggradiElisa FalcierAndrea LizioAlice PirolaJacopo CasiraghiAlice ZanoliniElena CarraroLuca MauroFabrizio RaoElisabetta RomaAntonino IannelloElisa De MattiaAndrea BarpSara LuponeValentina GattiCristina ItalianoValeria Ada Sansone

Published in: Canadian respiratory journal (2022)

We provide additional data on the natural history of respiratory function decline and treatment adherence in a relatively large cohort of well-characterized patients with DM1. A high proportion (28%) was lost to follow-up. A minority (11%) required NIV, and only 25% were treatment adherent, irrespective of specific demographics and respiratory features. Our results also confirm previous findings, showing that age, disease duration, and higher BMIs are predisposing factors for respiratory impairment.

Keyphrases

respiratory tract
type diabetes
adipose tissue
early onset
electronic health record
machine learning
insulin resistance
big data
metabolic syndrome
mechanical ventilation
respiratory failure