title: Survival of a child with spinal muscular atrophy and acute respiratory distress syndrome.
abstract: Spinal muscular atrophy (SMA), a lower motor neuron anterior horn cell disease, causes significant respiratory morbidity and mortality in children. Acute respiratory distress syndrome (ARDS) accounts for 1-4% of all Pediatric Intensive Care Unit (PICU) admissions. Management outcomes for ARDS in patients with SMA have not been described. We present the case of a 5-year-old boy with Type II SMA and ARDS requiring invasive mechanical ventilation. He improved with meticulous management of mechanical ventilation, airway clearance, fluid/nutrition, and sedation/analgesia. He was successfully extubated after 14 days of invasive mechanical ventilation and discharged home after a 20 day hospitalization.
authors: Department of Pediatrics, University of Wisconsin Hospital and Clinics, American Family Children's Hospital, Madison, Wisconsin.
authors: Anthony
authors: A
authors: Wampole
authors: Department of Pediatrics, University of Wisconsin School of Medicine and Public Health, American Family Children's Hospital, Madison, Wisconsin.
authors: Mary
authors: M
authors: Schroth
authors: Department of Pediatrics, University of Wisconsin School of Medicine and Public Health, American Family Children's Hospital, Madison, Wisconsin.
authors: Juan
authors: J
authors: Boriosi
mesh_terms: D035641:Chest Wall Oscillation; D002675:Child, Preschool; D004750:Enteral Nutrition; D006801:Humans; D006993:Hypnotics and Sedatives / Q000627:therapeutic use; D007442:Intubation, Intratracheal; D008297:Male; D009134:Muscular Atrophy, Spinal / Q000628:therapy*; D012121:Respiration, Artificial; D012128:Respiratory Distress Syndrome / Q000628:therapy*
chemical_list: D006993:Hypnotics and Sedatives
keywords: Acute Respiratory Distress Syndrome (ARDS); acute lung injury (ALI); mechanical ventilation; spinal muscular atrophy