title: Macrophage activation syndrome induced by A/H1N1 influenza in cystic fibrosis.
abstract: Bacterial respiratory infections have an important impact on the development and progression of pulmonary disease in cystic fibrosis (CF). Viral infections are possible triggers of acute deterioration in the clinical status of CF patients. Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic disease characterized by pancytopenia, hepatitis, hyperferritinemia, coagulopathy, and neurologic symptoms. This syndrome is thought to be caused by the activation and uncontrolled proliferation of T lymphocytes and well-differentiated macrophages, leading to widespread hemophagocytosis and cytokine overproduction. Here, we report the case of a boy affected by CF who developed MAS triggered by pandemic H1N1 influenza; good clinical response was obtained through high dose prednisone treatment.
authors: IRCCS G. Gaslini Cystic Fibrosis Center, Genoa, Italy. rosariacasciaro@ospedale-gaslini.ge.it.
authors: Rosaria
authors: R
authors: Casciaro
authors: Federico
authors: F
authors: Cresta
authors: Federica
authors: F
authors: Favilli
authors: Aldo
authors: A
authors: Naselli
authors: Alessandra
authors: A
authors: De Alessandri
authors: Laura
authors: L
authors: Minicucci
mesh_terms: D002648:Child; D003550:Cystic Fibrosis / Q000150:complications*; D006801:Humans; D053118:Influenza A Virus, H1N1 Subtype / Q000302:isolation & purification; D007251:Influenza, Human / Q000150:complications* / Q000175:diagnosis; D055501:Macrophage Activation Syndrome / Q000175:diagnosis / Q000821:virology*; D008297:Male
keywords: H1N1; MAS; cystic fibrosis