Vascular Perfusion Abnormalities in Infants With Spinal Muscular Atrophy

J Pediatr. 2009 Aug;155(2):292-4. doi: 10.1016/j.jpeds.2009.01.071.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Anti-Infective Agents, Local / therapeutic use
  • Anticoagulants / therapeutic use
  • Aspirin / therapeutic use
  • Diosmin / therapeutic use
  • Female
  • Fingers / blood supply*
  • Fingers / pathology*
  • Heparin / therapeutic use
  • Humans
  • Infant
  • Necrosis / drug therapy
  • Necrosis / etiology
  • Pentoxifylline / therapeutic use
  • Platelet Aggregation Inhibitors / therapeutic use
  • Spinal Muscular Atrophies of Childhood / complications*
  • Toes / blood supply*
  • Toes / pathology*
  • Vasodilator Agents / therapeutic use

Substances

  • Anti-Infective Agents, Local
  • Anticoagulants
  • Platelet Aggregation Inhibitors
  • Vasodilator Agents
  • Diosmin
  • Heparin
  • Aspirin
  • Pentoxifylline