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Symptoms, clinical and physiological findings motivating home mechanical ventilation in patients with neuromuscular diseases

Laub, Michael LU ; Berg, Sören LU and Midgren, Bengt LU (2006) In Journal of Rehabilitation Medicine 38(4). p.250-254
Abstract
Objective: To clarify the relationship between symptoms, clinical signs and physiological abnormalities that were motivating the initiation of home mechanical ventilation in patients suffering from neuromuscular diseases. Methods: From The Swedish Home Mechanical Ventilation Register we identified 352 patients with neuromuscular diseases and we looked at circumstances ( acute vs elective) and clinical motives for starting ventilatory support. Results: Home mechanical ventilation was commenced electively in 268 patients ( 76%) and among these daytime sleepiness was the most common motive, being reported in 56% of the patients. In the 24 children with spinal muscular atrophy, however, 96% started ventilation electively and cough... (More)
Objective: To clarify the relationship between symptoms, clinical signs and physiological abnormalities that were motivating the initiation of home mechanical ventilation in patients suffering from neuromuscular diseases. Methods: From The Swedish Home Mechanical Ventilation Register we identified 352 patients with neuromuscular diseases and we looked at circumstances ( acute vs elective) and clinical motives for starting ventilatory support. Results: Home mechanical ventilation was commenced electively in 268 patients ( 76%) and among these daytime sleepiness was the most common motive, being reported in 56% of the patients. In the 24 children with spinal muscular atrophy, however, 96% started ventilation electively and cough insufficiency was the most common motive. The patients were moderately hypercapnic ( PaCO2: 7.0 kPa, SD 1.3). None of the clinical motives were related to the PaCO2 level. Average PaO2 was above 8 kPa in all groups, but lowest in the patients with post-polio and dystrophia myotonica. Mean vital capacity was close to 40% of predicted, but significantly lower in the Duchenne patients ( 26% of predicted). Conclusion: Daytime sleepiness was the most common clinical symptom motivating home mechanical ventilation in this group of patients with chronic hypercapnic respiratory insufficiency secondary to neuro/myopathies. Respiratory function testing is therefore suggested to be included in the diagnostic work up of daytime sleepiness in these patients. (Less)
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author
; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
invasive ventilation, respiratory failure, non-invasive ventilation, symptoms and signs
in
Journal of Rehabilitation Medicine
volume
38
issue
4
pages
250 - 254
publisher
Taylor & Francis
external identifiers
  • pmid:16801208
  • wos:000238610100008
  • scopus:33745531992
ISSN
1651-2081
DOI
10.1080/16501970600721033
language
English
LU publication?
yes
id
86f2f7e4-2afd-4684-a807-83cbdbbeca21 (old id 404844)
date added to LUP
2016-04-01 16:50:49
date last changed
2021-10-10 03:56:58
@article{86f2f7e4-2afd-4684-a807-83cbdbbeca21,
  abstract     = {Objective: To clarify the relationship between symptoms, clinical signs and physiological abnormalities that were motivating the initiation of home mechanical ventilation in patients suffering from neuromuscular diseases. Methods: From The Swedish Home Mechanical Ventilation Register we identified 352 patients with neuromuscular diseases and we looked at circumstances ( acute vs elective) and clinical motives for starting ventilatory support. Results: Home mechanical ventilation was commenced electively in 268 patients ( 76%) and among these daytime sleepiness was the most common motive, being reported in 56% of the patients. In the 24 children with spinal muscular atrophy, however, 96% started ventilation electively and cough insufficiency was the most common motive. The patients were moderately hypercapnic ( PaCO2: 7.0 kPa, SD 1.3). None of the clinical motives were related to the PaCO2 level. Average PaO2 was above 8 kPa in all groups, but lowest in the patients with post-polio and dystrophia myotonica. Mean vital capacity was close to 40% of predicted, but significantly lower in the Duchenne patients ( 26% of predicted). Conclusion: Daytime sleepiness was the most common clinical symptom motivating home mechanical ventilation in this group of patients with chronic hypercapnic respiratory insufficiency secondary to neuro/myopathies. Respiratory function testing is therefore suggested to be included in the diagnostic work up of daytime sleepiness in these patients.},
  author       = {Laub, Michael and Berg, Sören and Midgren, Bengt},
  issn         = {1651-2081},
  language     = {eng},
  number       = {4},
  pages        = {250--254},
  publisher    = {Taylor & Francis},
  series       = {Journal of Rehabilitation Medicine},
  title        = {Symptoms, clinical and physiological findings motivating home mechanical ventilation in patients with neuromuscular diseases},
  url          = {http://dx.doi.org/10.1080/16501970600721033},
  doi          = {10.1080/16501970600721033},
  volume       = {38},
  year         = {2006},
}