Abstract

CAMACHO PLASENCIA, Carlos Alberto et al. Werdnig-Hoffman disease. a long stay in a pediatric Intensive Care Unit. Rev haban cienc méd [online]. 2009, vol.8, n.1, pp. 0-0. ISSN 1729-519X.

The number of children that need long term mechanical ventilation is increasing worldwide. A female patient was reported with spinal muscular atrophy type 1 (Werdnig-Hoffman disease) and a stay at the Intensive Care Unit nearly to five years because she required chronic ventilatory support. A review of the illness was performed and the management strategies were analysed that have permitted an unusually prolonged survival with excellent physical and mental conditions.

Keywords : Werdnig-Hoffman disease; prolonged mechanical ventilation..