Vascular perfusion abnormalities in infants with spinal muscular atrophy

M Araujo, KJ Swoboda - The Journal of pediatrics, 2009 - Elsevier
M Araujo, KJ Swoboda
The Journal of pediatrics, 2009Elsevier
Spinal muscular atrophy (SMA) is an important cause of death in children and SMA type I,
also known as Werdnig-Hoffman disease, is the most severe form of this disease. We report
2 cases of infants with SMA I in whom a distal necrosis developed, a feature not previously
reported. Poor perfusion, autonomic dysfunction, and position-dependent factors may all
play a role in the development of this complication.
Spinal muscular atrophy (SMA) is an important cause of death in children and SMA type I, also known as Werdnig-Hoffman disease, is the most severe form of this disease. We report 2 cases of infants with SMA I in whom a distal necrosis developed, a feature not previously reported. Poor perfusion, autonomic dysfunction, and position-dependent factors may all play a role in the development of this complication.
Elsevier