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

Vascular perfusion abnormalities in infants with spinal muscular atrophy.

Author information

1
Federal University of Rio de Janeiro, Pediatrics, Rio de Janeiro, Brazil. alexprufer@ufrj.br

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.

PMID:
19619755
PMCID:
PMC3250227
DOI:
10.1016/j.jpeds.2009.01.071
[Indexed for MEDLINE]
Free PMC Article

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