A case of fibrodysplasia ossificans progressiva complicated by influenza pneumonia treated with long-term intensive care

進行性骨化性線維異形成症(fibrodysplasia ossificans progressiva:FOP)患者のインフルエンザ肺炎による急性呼吸不全に対する長期集中治療を経験した.患者は36歳女性で,7歳でFOPの診断を受けた.33歳時にカンジダ敗血症に起因する肺炎に罹患したが,集中治療室(ICU)での人工呼吸管理を受け,その後に軽快退院した.今回はインフルエンザA型ウイルス感染による発熱･嘔吐のため当院へ入院後,肺炎を併発し病棟で人工呼吸管理を開始したが,呼吸不全が急性増悪したために第18病日にICUへ入室した.入室後,血圧維持のためにノルアドレナリン投与を開始し,同日気管切開術を施行した.最終的に人工呼吸器からの離脱はできなかったが,胸部X線写真の肺炎像は著明に改善し,抗菌薬投与の終了後はP/F比が400以上で安定し,第105病日に長期療養型病院へ転院した.FOP患者で急性呼吸不全の治療を行う場合,呼吸管理の長期化を念頭に置くべきである.また必要に応じて気管切開や胸腔ドレナージなどの治療を,積極的かつ速やかに行うことが肝要である.

We provided long-term intensive care to a patient with fibrodysplasia ossificans progressiva(FOP)who had acute respiratory failure due to influenza pneumonia. The patient was a 36-year-old woman diagnosed with FOP at 7 years of age. When she was 33 years old, she experienced sepsis-induced pneumonia due to candidiasis, which was completely cured with artificial ventilation. Presently, she was admitted to our hospital for fever and vomiting caused by influenza A virus infection, which led to pneumonia. Artificial ventilation was started, and she was moved to the intensive care unit because of acute respiratory failure on the 18th day from the onset of disease. Administration of noradrenaline maintained the blood pressure, and she underwent tracheostomy on the same day. Therefore, she could not be weaned from artificial ventilation support

進行性骨化性線維異形成症

インフルエンザ肺炎

気管切開

長期人工呼吸管理

胸腔ドレナージ