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Pediatr Pulmonol. 2014 Feb;49(2):E10-2. doi: 10.1002/ppul.22778. Epub 2013 Feb 8.

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

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  • 1IRCCS G. Gaslini Cystic Fibrosis Center, Genoa, Italy.


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.

© 2013 Wiley Periodicals, Inc.


H1N1; MAS; cystic fibrosis

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