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November 1, 1998ThoraxOpen Access

Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy

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Authors

ASAnita K. SimondsRoyal Brompton & Harefield NHS Foundation TrustFMFrancesco MuntoniUniversity of LondonSHS. HeatherBaton Rouge Clinic

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Implication

Cohort study demonstrates improved survival in hypercapnic Duchenne muscular dystrophy patients receiving nasal ventilation, indicating significant therapeutic benefit.

Key Points

  • To examine the effects of nocturnal nasal intermittent positive pressure ventilation on survival and blood gas levels in patients with Duchenne muscular dystrophy experiencing established ventilatory failure.
  • Nocturnal nasal intermittent positive pressure ventilation (NIPPV) was administered to a consecutive cohort of 23 patients with Duchenne muscular dystrophy presenting with diurnal and nocturnal hypercapnia.
  • Patients had a mean (SD) age of 20.3 (3.4) years and were monitored over a five-year follow-up period.
  • Survival rates following NIPPV were 85% (95% CI, 69 to 100) at 1 year and 73% (95% CI, 53 to 94) at 5 years.
  • Arterial blood gas tensions improved early and remained stable over five years, with mean (SD) PO2 rising from 7.6 (2.1) kPa to 10.8 (1.3) kPa and PCO2 decreasing from 10.3 (4.5) kPa to 6.1 (1.0) kPa.
  • Quality of life measurements using the SF-36 index indicated health perception and social functioning scores comparable to patients with nonprogressive disorders using NIPPV.

Cite This Study

Simonds et al. (1998) studied this question.

synapsesocial.com/papers/6a7f2659b5ccfbbe2d84ea28https://doi.org/10.1136/thx.53.11.949
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Also Consider

Synapse has enriched one closely related paper. Consider it for comparative context:

  1. 1A comparison of different modes of noninvasive ventilatory support: effects on ventilation and inspiratory muscle effort1994 · 61 citations