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Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients
Open accessAbstract
The present study aimed to assess the impact of diurnal mouthpiece intermittent positive pressure ventilation (MIPPV) as the extension of the nasal intermittent positive pressure ventilation (NIPPV) in Duchenne muscular dystrophy (DMD). In total, 42 DMD patients aged 15–33 yrs, normocapnic at night with NIPPV and receiving MIPPV since end-diurnal hypercapnia, were studied. Transcutaneous CO 2 tension (Pt,CO 2) was prospectively monitored at the end of the day, before and after MIPPV initiation. Vital capacity (VC), breathing pattern and maximal inspiratory strength were measured. Patients were asked to score the presence (1 point) or absence (0 point) of seven respiratory-linked symptoms before and after MIPPV establishment. Survival rates reached 88, 77, 58 and 51% after 1, 3, 5 and 7 yrs, respectively. The mean survival rate was 31 yrs. VC stabilised during 5 yrs with MIPPV. Symptom scores significantly decreased and Pt,CO 2normalised during the day (8.17±2.22 to 5.78±0.73 kPa). No accident and minor side-effects were observed in this 184 cumulated patient-yrs study. In conclusion, daytime mouthpiece ventilation is safe, prolongs survival and stabilises vital capacity in Duchenne muscular dystrophy patients. It is recommended on the condition that patients are equipped with a self-supporting harness.
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Cited by
Hull, J., Aniapravan, R., Chan, E., Chatwin, M., Forton, J., Gallagher, J., Gibson, N., Gordon, J., Hughes, I., McCulloch, R., et al. (2012) · Thorax · doi:10.1136/thoraxjnl-2012-201964 · Cited by 394
Yiu, E. M., & Kornberg, A. J. (2015) · Journal of Paediatrics and Child Health · doi:10.1111/jpc.12868 · Cited by 335
The growing role of noninvasive ventilation in patients requiring prolonged mechanical ventilation
Hess, D. R. (2012) · Respiratory Care · doi:10.4187/respcare.01692 · Cited by 59
Long-Term Ventilation in Neuromuscular Patients: Review of Concerns, Beliefs, and Ethical Dilemmas
Crimi, C., Pierucci, P., Carlucci, A., Cortegiani, A., & Gregoretti, C. (2019) · Respiration · doi:10.1159/000495941 · Cited by 46
The right interface for the right patient in noninvasive ventilation: a systematic review
Pierucci, P., Portacci, A., Carpagnano, G. E., Banfi, P., Crimi, C., Misseri, G., & Gregoretti, C. (2022) · Expert Review of Respiratory Medicine · doi:10.1080/17476348.2022.2121706 · Cited by 40
Metadata
- Authors
- Toussaint, M., Steens, M., Wasteels, G., & Soudon, P.
- Venue
- European Respiratory Journal
- Publisher
- European Respiratory Society (ERS)
- Published
- 2006-07-26
- Volume
- 28
- Issue
- 3
- Pages
- 549-555
- ISSN
- 0903-1936 (ISSN-L)
- 1399-3003
- OpenAlex
- W2164295406
- Cited by
- 181
- References
- 0
- FWCI
- 6.71
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Toussaint, M., Steens, M., Wasteels, G., & Soudon, P. (2006). Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients. European Respiratory Journal. https://doi.org/10.1183/09031936.06.00004906
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