Casciaro Rosaria, Cresta Federico, Favilli Federica, Naselli Aldo, De Alessandri Alessandra, Minicucci Laura
IRCCS G. Gaslini Cystic Fibrosis Center, Genoa, Italy.
Pediatr Pulmonol. 2014 Feb;49(2):E10-2. doi: 10.1002/ppul.22778. Epub 2013 Feb 8.
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.
细菌呼吸道感染对囊性纤维化（CF）患者肺部疾病的发展和进展具有重要影响。病毒感染可能是CF患者临床状况急性恶化的触发因素。巨噬细胞活化综合征（MAS）是一种危及生命的风湿性疾病并发症，其特征为全血细胞减少、肝炎、高铁蛋白血症、凝血病和神经症状。该综合征被认为是由T淋巴细胞和充分分化的巨噬细胞的活化及不受控制的增殖引起的，导致广泛的噬血细胞作用和细胞因子过度产生。在此，我们报告一例患有CF的男孩发生大流行性H1N1流感引发的MAS的病例；通过高剂量泼尼松治疗获得了良好的临床反应。
