ArticleNoninvasive long-term ventilatory support for individuals with spinal muscular atrophy and functional bulbar musculature
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Cited by (32)
Standard of Care for Spinal Muscular Atrophy
2017, Spinal Muscular Atrophy: Disease Mechanisms and TherapyThe Respiratory System and Neuromuscular Diseases
2015, Murray and Nadel's Textbook of Respiratory Medicine: Volume 1,2, Sixth EditionClinical approach to the diagnostic evaluation of hereditary and acquired neuromuscular diseases
2012, Physical Medicine and Rehabilitation Clinics of North AmericaCitation Excerpt :Although no survival data exist for patients with SMA III, cases have been followed into the eighth decade without mechanical ventilation.52,66 Ventilatory failure due to neuromuscular restrictive lung disease is a rare event in SMA III, occurring only in adulthood.18,52 Zerres and Rudnik-Schoneborn66 have proposed further subtypes, including SMA IIIa (walks without support; age of onset before 3 years) and SMA IIIb (walks without support; age of onset 3–30 years).
Noninvasive positive pressure ventilation in the treatment of hypoventilation in children
2010, Sleep Medicine ClinicsCitation Excerpt :For SMA patients, NPPV can be provided as routine therapy or as a palliative tool. NPPV can be used to achieve adequate inspiratory chest wall expansion and air entry, and normalization of gas exchange.76,80–82 In a randomized controlled trial using mixed groups of patients with NMD with nocturnal hypoventilation and daytime normocapnia, noninvasive ventilation significantly improved nocturnal blood gas tensions.76
Diagnosis and Clinical Management of Spinal Muscular Atrophy
2008, Physical Medicine and Rehabilitation Clinics of North AmericaCitation Excerpt :In addition, SMA II patients showed more severe declines in maximal expiratory pressure versus maximal inspiratory pressure, suggesting relative diaphragmatic sparing [53,77–79]. Progressive restrictive lung disease in SMA III was shown to be mild and rarely necessitated the institution of ventilatory support [17,37,80–83]. Although no specific spirometry parameters for beginning ventilatory support have been established, it has been found that the institution of mechanical ventilation in SMA II was generally not required until FVC was about 20% of the predicted value [81,84].
Sleep-Disordered Breathing in Children
2005, Pediatric Pulmonology: The Requisites
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