The effect of long term ventilatory support on hemodynamics in children with spinal muscle atrophy (SMA) type II

Markström, Agneta; Cohen, Gary; Katz‐Salamon, Miriam

doi:10.1016/j.sleep.2009.08.014

Cited by 10 publications

(6 citation statements)

References 28 publications

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“…For children who are greater than 5 years of age, forced vital capacity can be routinely monitored and non-invasive ventilatory support can be managed long-term. Nocturnal hypoventilation should also be treated with non-invasive ventilation [38]. …”

Section: Clinical Managementmentioning

confidence: 99%

Spinal Muscular Atrophy

2015

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Incidence The incidence of SMA is 1:11,000 live births [1]. Prevalence The prevalence of the carrier state is approximately 1 in 54 [1]. Severity The clinical severity of SMA correlates inversely with SMN2 gene copy number and varies from an extreme weakness and paraplegia of infancy to a mild proximal weakness of adulthood. Natural History The natural history of SMA is complex and variable. For this reason, clinical subgroups have been defined based upon best motor function attainment during development. Type 1 SMA infants never sit independently. Type 2 SMA children sit at some point during their childhood, but never walk independently. And Type 3 SMA children and adults are able to walk independently at some point in their childhood.

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Section: Clinical Managementmentioning

confidence: 99%

Spinal Muscular Atrophy

2015

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“…Proper use of bilevel positive airway pressure, with correct pressure adjustments and mask placement, has no significant side effects on Spinal Muscular Atrophies patient hemodynamics. 110 There should also be a low threshold for the use of antibiotics during acute illnesses in these patients, due to the risk of pneumonia. 15,108,109 Patients should be followed regularly by a pulmonologist experienced in caring for patients with neuromuscular diseases, with home visits by a home ventilation team if available.…”

Section: Care Of Patients With Spinal Muscular Atrophymentioning

confidence: 99%

Spinal Muscular Atrophies

Darras

2015

Pediatric Clinics of North America

143

107

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“…Survival was significantly affected by the increased use of ventilation (noninvasive, i.e., bilevel positive airway pressure, and invasive), mechanical cough assist devices, and gastrostomy feeding [15]. The proper use of bilevel positive airway pressure, with correct pressure adjustments and mask placement, produces no significant side effects on patient hemodynamics [130]. Patients with spinal muscular atrophy and respiratory muscle weakness also manifest a weak cough, which impairs their ability to clear the airway of secretions.…”

Section: Pulmonarymentioning

confidence: 99%