title: Pulmonary function in spinal muscular atrophy.
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.
authors.affiliation: Department of Neurology, University of Cincinnati Medical Center, OH 45267-0525.
authors.forename: F J
authors.initials: FJ
authors.lastname: Samaha
authors.forename: C R
authors.initials: CR
authors.lastname: Buncher
authors.forename: B S
authors.initials: BS
authors.lastname: Russman
authors.forename: M L
authors.initials: ML
authors.lastname: White
authors.forename: S T
authors.initials: ST
authors.lastname: Iannaccone
authors.forename: L
authors.initials: L
authors.lastname: Barker
authors.forename: K
authors.initials: K
authors.lastname: Burhans
authors.forename: C
authors.initials: C
authors.lastname: Smith
authors.forename: B
authors.initials: B
authors.lastname: Perkins
authors.forename: L
authors.initials: L
authors.lastname: Zimmerman
mesh_terms: D000293:Adolescent; D000367:Age Factors; D001827:Body Height; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D011446:Prospective Studies; D011596:Psychomotor Disorders / Q000175:diagnosis / Q000209:etiology; D012129:Respiratory Function Tests; D014897:Spinal Muscular Atrophies of Childhood / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D015996:Survival Rate