학술논문

Natural history of infantile-onset spinal muscular atrophy.
Document Type
article
Source
Annals of Neurology. 82(6)
Subject
Biomarkers
Child
Preschool
Cohort Studies
Female
Humans
Infant
Longitudinal Studies
Male
Prospective Studies
Spinal Muscular Atrophies of Childhood
Survival of Motor Neuron 1 Protein
Survival of Motor Neuron 2 Protein
Language
Abstract
OBJECTIVE: Infantile-onset spinal muscular atrophy (SMA) is the most common genetic cause of infant mortality, typically resulting in death preceding age 2. Clinical trials in this population require an understanding of disease progression and identification of meaningful biomarkers to hasten therapeutic development and predict outcomes. METHODS: A longitudinal, multicenter, prospective natural history study enrolled 26 SMA infants and 27 control infants aged