Respiratory failure in Pompe disease: treatment with noninvasive ventilation

Neurology. 2005 Apr 26;64(8):1465-7. doi: 10.1212/01.WNL.0000158682.85052.C0.

Abstract

In this study, noninvasive ventilation (NIV) was prospectively applied to eight patients (35.8 +/- 11.4 years) with late-onset Pompe disease and respiratory failure apparent from severe restrictive lung disease, nocturnal hypoxemia (83 +/- 8%), and daytime hypercapnia (66.7 +/- 17.9 mm Hg). The impact of NIV on respiratory function was followed for 34 +/- 17 months. Despite further decrease of vital capacity and inspiratory muscle strength, NIV normalized oxygen saturation during sleep (96 +/- 1%), daytime carbon dioxide tensions (44.1 +/- 3.6 mm Hg), and symptoms.

Publication types

  • Clinical Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Dyspnea / etiology
  • Dyspnea / physiopathology
  • Dyspnea / therapy
  • Glycogen Storage Disease Type II / complications
  • Glycogen Storage Disease Type II / physiopathology
  • Glycogen Storage Disease Type II / therapy*
  • Humans
  • Hypercapnia / etiology
  • Hypercapnia / physiopathology
  • Hypercapnia / therapy
  • Hypoxia / etiology
  • Hypoxia / physiopathology
  • Hypoxia / therapy
  • Middle Aged
  • Muscle Weakness / etiology
  • Muscle Weakness / physiopathology
  • Muscle Weakness / therapy
  • Prospective Studies
  • Respiration, Artificial / trends*
  • Respiratory Insufficiency / etiology
  • Respiratory Insufficiency / physiopathology
  • Respiratory Insufficiency / therapy*
  • Respiratory Muscles / metabolism
  • Respiratory Muscles / physiopathology
  • Sleep Apnea Syndromes / etiology
  • Sleep Apnea Syndromes / physiopathology
  • Sleep Apnea Syndromes / therapy
  • Treatment Outcome
  • Vital Capacity / physiology