Scoliosis and lung function in spinal muscular atrophy
β Scribed by D. Robinson; C. S. B. Galasko; C. Delaney; J. B. Williamson; J. L. Barrie
- Publisher
- Springer
- Year
- 1995
- Tongue
- English
- Weight
- 630 KB
- Volume
- 4
- Category
- Article
- ISSN
- 0940-6719
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β¦ Synopsis
The notes and radiographs of 43 patients with a confirmed diagnosis of spinal muscular atrophy were reviewed. A significant inverse linear relationship between the severity of scoliosis and the percentage of predicted vital capacity and peak flow was found. The patients who stood had a significantly better lung function than patients who were confined to a wheelchair, and their scoliosis deteriorated significantly more slowly. Sixteen patients underwent surgical spinal stabilisation, 4 with Harrington instrumentation and 12 with segmental spinal instrumentation, at an average age of 12 years and 11 months. The average curve correction achieved was 40%. The decline in lung function seen pre-operatively was not only reversed, but a significant improvement was found at final follow-up.
π SIMILAR VOLUMES
Evidence of impaired cell-mediated immunity in children with spinal muscular atrophy was obtained using lymphocyte transformation with PHA (20 children) and skin tests with tuberculin and DNCB (35 children). Laryngological examination of 16 children demonstrated hypoplasia of lymphatic tissue in Wal