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Ped. Pulm.: Macrophage activation syndrome induced by A/H1N1 influenza in cystic fibrosis

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  • Ped. Pulm.: Macrophage activation syndrome induced by A/H1N1 influenza in cystic fibrosis

    Pediatric Pulmonology

    Rosaria Casciaro MD*,
    Federico Cresta MD,
    Federica Favilli MD,
    Aldo Naselli MD,
    Alessandra De Alessandri MD,
    Laura Minicucci MD

    Article first published online: 8 FEB 2013

    DOI: 10.1002/ppul.22778


    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. Pediatr Pulmonol. ? 2013 Wiley Periodicals, Inc.

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