Correlation of Pulmonary Function to a Novel Radiographic Parameter of Collapsing Parasol Deformity in Spinal Muscular Atrophy

2018 ◽  
Vol 6 (6) ◽  
pp. 809-810
Author(s):  
Mitchell Johnson ◽  
Suzanne Ho ◽  
Didja Hilmara ◽  
Nirupa Galagedera ◽  
Robert Campbell ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Radiographic Parameter

The effect of scoliosis surgery on pulmonary function in spinal muscular atrophy type II patients

2016 ◽  
Vol 26 (6) ◽  
pp. 1721-1731 ◽  
Author(s):  
Shih-Hsiang Chou ◽  
Gau-Tyan Lin ◽  
Po-Chih Shen ◽  
Yi-Jing Lue ◽  
Cheng-Chang Lu ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Scoliosis Surgery ◽  
Type Ii ◽  
Spinal Muscular Atrophy Type

Pulmonary function and scoliosis in children with spinal muscular atrophy types II and III

2003 ◽  
Vol 39 (9) ◽  
pp. 673-676 ◽  
Author(s):  
SY Chng ◽  
YQ Wong ◽  
JH Hui ◽  
HK Wong ◽  
HT Ong ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Spinal Muscular Atrophy ◽  
Muscular Atrophy

Impact of scoliosis surgery on pulmonary function in patients with muscular dystrophies and spinal muscular atrophy

2020 ◽  
Vol 55 (4) ◽  
pp. 1037-1042
Author(s):  
Harold J. Farber ◽  
William A. Phillips ◽  
Kenneth L. Kocab ◽  
Darrell S. Hanson ◽  
John A. Heydemann ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Muscular Dystrophies ◽  
Scoliosis Surgery

Pulmonary Function in Spinal Muscular Atrophy

1994 ◽  
Vol 9 (3) ◽  
pp. 326-329 ◽  
Author(s):  
F.J. Samaha ◽  
C.R. Buncher ◽  
B.S. Russman ◽  
M.L. White ◽  
S.T. Iannaccone ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Spinal Muscular Atrophy ◽  
Muscular Atrophy

Improvement of Pulmonary Function Measured by Patient-reported Outcomes in Patients With Spinal Muscular Atrophy After Growth-friendly Instrumentation

2020 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Hiroko Matsumoto ◽  
John Mueller ◽  
Matthew Konigsberg ◽  
Jacob Ball ◽  
Tricia St. Hilaire ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Spinal Muscular Atrophy ◽  
Patient Reported Outcomes ◽  
Muscular Atrophy ◽  
Patient Reported

scholarly journals Air stacking: effects on pulmonary function in patients with spinal muscular atrophy and in patients with congenital muscular dystrophy,

2014 ◽  
Vol 40 (5) ◽  
pp. 528-534 ◽  
Author(s):  
Tanyse Bahia Carvalho Marques ◽  
Juliana de Carvalho Neves ◽  
Leslie Andrews Portes ◽  
João Marcos Salge ◽  
Edmar Zanoteli ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Pulmonary Function ◽  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Pulmonary Function Tests ◽  
Congenital Muscular Dystrophy ◽  
Spinal Deformities ◽  
Respiratory Complications ◽  
Function Tests ◽  
Before And After

OBJECTIVE: Respiratory complications are the main causes of morbidity and mortality in patients with neuromuscular disease (NMD). The objectives of this study were to determine the effects that routine daily home air-stacking maneuvers have on pulmonary function in patients with spinal muscular atrophy (SMA) and in patients with congenital muscular dystrophy (CMD), as well as to identify associations between spinal deformities and the effects of the maneuvers. METHODS: Eighteen NMD patients (ten with CMD and eight with SMA) were submitted to routine daily air-stacking maneuvers at home with manual resuscitators for four to six months, undergoing pulmonary function tests before and after that period. The pulmonary function tests included measurements of FVC; PEF; maximum insufflation capacity (MIC); and assisted and unassisted peak cough flow (APCF and UPCF, respectively) with insufflations. RESULTS: After the use of home air-stacking maneuvers, there were improvements in the APCF and UPCF. In the patients without scoliosis, there was also a significant increase in FVC. When comparing patients with and without scoliosis, the increases in APCF and UPCF were more pronounced in those without scoliosis. CONCLUSIONS: Routine daily air-stacking maneuvers with a manual resuscitator appear to increase UPCF and APCF in patients with NMD, especially in those without scoliosis.

scholarly journals The impact of scoliosis surgery on pulmonary function in spinal muscular atrophy: a systematic review

2021 ◽  
Author(s):  
Abduljabber Alhammoud ◽  
Yahya Othman ◽  
Ron El-Hawary ◽  
William G. Mackenzie ◽  
Jason J. Howard
Keyword(s):  
Pulmonary Function ◽  
Spinal Muscular Atrophy ◽  
Primary Outcome ◽  
Muscular Atrophy ◽  
Scoliosis Surgery ◽  
Inclusion Criteria ◽  
Rate Of Decline ◽  
The Impact ◽  
Function Testing

AbstractScoliosis often occurs coincident with pulmonary function deterioration in spinal muscular atrophy but a causal relationship has not yet been reliably established. A systematic literature review was performed, with pulmonary function testing being the primary outcome pre- and post-scoliosis surgery. Levels of evidence were determined and GRADE recommendations made. Ninety studies were identified with only 14 meeting inclusion criteria. Four studies were level III and the rest were level IV evidence. The average age at surgical intervention was 11.8 years (follow-up 6.1 years). Post-operative pulmonary function progressively declined for the majority of studies. Otherwise, pulmonary function: improved (two studies), were unchanged (two studies), had a decreased rate of decline (three studies), declined initially then returned to baseline (two studies). Respiratory and spine-based complications were common. Given the available evidence, the following GRADE C recommendations were made: (1) surgery is most often associated with decreases in pulmonary function; (2) the impact of surgery on pulmonary function is variable, but does not improve over pre-operative baseline; (3) surgery may result in a decreased rate of decline in pulmonary function post-operatively. Given this lack of evidence-based support, the risk–benefit balance should be taken into consideration when contemplating scoliosis surgery.

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