Pulmonary management of spinal muscular atrophy
Authors:
T. Doušová 1; J. Plešková 2; A. Chmelařová 2
Authors place of work:
Pediatrická klinika 2. LF UK a FN Motol
1; Klinika rehabilitace a tělovýchovného lékařství 2. LF UK a FN Motol
2
Published in the journal:
Cesk Slov Neurol N 2020; 83/116(Supplementum 2): 35-40
doi:
https://doi.org/10.48095/cccsnn20202S35
Summary
Spinal muscular atrophy (SMA) has an impact on respiratory complications that are dependent on the severity of the loss of muscle function. Individuals with SMA type I or II typically develop respiratory muscle weakness, with progressive respiratory failure in most severe forms. Ineffective cough is a result of inspiratory and more expiratory muscle weakness and contributes to repeated respiratory infections. In some patients, gradual progression results in sleep-disordered breathing. Over the last decade, there has been increasing evidence of improvements in the natural history of all the SMA types. Even in the most severe form, there has been an increase insurvival and quality of life as a result of a more proactive approach, following the introduction of non-invasive ventilation and cough assist devices. The approval of the first drug for SMA in December 2016 and promising early results from other clinical trials have changed the perspective of physicians and families dramatically, especially in SMA type I. The mainstay of care is amultidisciplinary team, where a pneumologist and a respiratory physiotherapist play an important role. The variability in clinical care between medical centers has resulted in Consensus Statement for Standards of Care. Here we present a modified version of these standards.
Zdroje
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Štítky
Paediatric neurology Neurosurgery NeurologyČlánok vyšiel v časopise
Czech and Slovak Neurology and Neurosurgery
2020 Číslo Supplementum 2
- Memantine Eases Daily Life for Patients and Caregivers
- Metamizole at a Glance and in Practice – Effective Non-Opioid Analgesic for All Ages
- Advances in the Treatment of Myasthenia Gravis on the Horizon
- Metamizole vs. Tramadol in Postoperative Analgesia
Najčítanejšie v tomto čísle
- Clinical manifestations of spinal muscular atrophy in adult patients
- Genetics of spinal muscular atrophy
- Therapy of spinal muscular atrophy
- Rehabilitation in spinal muscular atrophy