Pulmonary function in spinal muscular atrophy

F. J. Samaha, C. R. Buncher, B. S. Russman, M. L. White, S. T. Iannaccone, L. Barker, K. Burhans, C. Smith, B. Perkins, L. Zimmerman

Research output: Contribution to journalArticle

38 Citations (Scopus)

Abstract

We present the first prospective study on pulmonary function in spinal muscular atrophy patients. Seventy-seven spinal muscular atrophy patients, ages 5 to 18 years, from three centers, were studied with regard to forced vital capacity, using height as a predictor. Patients were categorized into four motor function categories. The highest-functioning group had normal or near-normal values, and those who sat with support had the lowest values. Those with intermediate function had intermediate values. Forced vital capacity was studied longitudinally in 40 spinal muscular atrophy patients for 1.1 to 4.4 years. Eighty-eight percent of patients grew in height, but only 35% showed an increase in height-adjusted forced vital capacity percent. In those patients with the least function, 100% lost height-adjusted forced vital capacity over time. In those patients with the highest function, 57% lost height-adjusted forced vital capacity. In addition, the basic forced vital capacity, not correlated to height, decreased in 43% of cases. These pulmonary function alterations appear to be important determinants for function and survival in spinal muscular atrophy patients.

Original languageEnglish (US)
Pages (from-to)326-329
Number of pages4
JournalJournal of Child Neurology
Volume9
Issue number3
StatePublished - 1994

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Spinal Muscular Atrophy
Vital Capacity
Lung
Reference Values
Prospective Studies
Survival

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health

Cite this

Samaha, F. J., Buncher, C. R., Russman, B. S., White, M. L., Iannaccone, S. T., Barker, L., ... Zimmerman, L. (1994). Pulmonary function in spinal muscular atrophy. Journal of Child Neurology, 9(3), 326-329.

Pulmonary function in spinal muscular atrophy. / Samaha, F. J.; Buncher, C. R.; Russman, B. S.; White, M. L.; Iannaccone, S. T.; Barker, L.; Burhans, K.; Smith, C.; Perkins, B.; Zimmerman, L.

In: Journal of Child Neurology, Vol. 9, No. 3, 1994, p. 326-329.

Research output: Contribution to journalArticle

Samaha, FJ, Buncher, CR, Russman, BS, White, ML, Iannaccone, ST, Barker, L, Burhans, K, Smith, C, Perkins, B & Zimmerman, L 1994, 'Pulmonary function in spinal muscular atrophy', Journal of Child Neurology, vol. 9, no. 3, pp. 326-329.
Samaha FJ, Buncher CR, Russman BS, White ML, Iannaccone ST, Barker L et al. Pulmonary function in spinal muscular atrophy. Journal of Child Neurology. 1994;9(3):326-329.
Samaha, F. J. ; Buncher, C. R. ; Russman, B. S. ; White, M. L. ; Iannaccone, S. T. ; Barker, L. ; Burhans, K. ; Smith, C. ; Perkins, B. ; Zimmerman, L. / Pulmonary function in spinal muscular atrophy. In: Journal of Child Neurology. 1994 ; Vol. 9, No. 3. pp. 326-329.
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