Vascular perfusion abnormalities in infants with spinal muscular atrophy.
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.
Araujo
Alexandra prufer de Queiroz Campos
Ap
Federal University of Rio de Janeiro, Pediatrics, Rio de Janeiro, Brazil. alexprufer@ufrj.br
Araujo
Mario
M
Swoboda
Kathryn J
KJ
D000891:Anti-Infective Agents, Local / Q000627:therapeutic use; D000925:Anticoagulants / Q000627:therapeutic use; D001241:Aspirin / Q000627:therapeutic use; D004145:Diosmin / Q000627:therapeutic use; D005260:Female; D005385:Fingers / Q000098:blood supply* / Q000473:pathology*; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D009336:Necrosis / Q000188:drug therapy / Q000209:etiology; D010431:Pentoxifylline / Q000627:therapeutic use; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D014897:Spinal Muscular Atrophies of Childhood / Q000150:complications*; D014034:Toes / Q000098:blood supply* / Q000473:pathology*; D014665:Vasodilator Agents / Q000627:therapeutic use
D000891:Anti-Infective Agents, Local; D000925:Anticoagulants; D010975:Platelet Aggregation Inhibitors; D014665:Vasodilator Agents; D004145:Diosmin; D006493:Heparin; D001241:Aspirin; D010431:Pentoxifylline