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- W2334604611 abstract "重症複合型免疫不全症(severe combined immunodeficiency, SCID)は原発性免疫不全症の最重症型で,救命のために造血幹細胞移植が必要であり,適切な診断と感染管理が重要となる。今回われわれはニューモシスチス肺炎を契機に診断されたSCIDの症例を経験したので報告する。症例は4ヶ月,女児。遷延する咳嗽・体重減少を認め受診した。SpO2 85%(空気呼吸下),胸部X線にて浸潤影を認めたが,発熱や血液検査にて炎症反応の上昇を認めなかった。肺炎による呼吸不全として抗菌薬治療を行うも改善なく,体重減少・リンパ球減少・胸腺欠損から原発性免疫不全症を疑い,T細胞・B細胞欠損(いずれも50 /μl以下)を認めSCIDの診断に至った。喀痰polymerase chain reaction(PCR)検査にてニューモシスチス肺炎と診断,ST合剤投与にて呼吸状態が改善し臍帯血移植施行,生着が得られ救命できた。非典型的な経過を辿り治療抵抗性の乳児期の重症感染症には原発性免疫不全症の可能性を考慮すべきである。" @default.
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- W2334604611 date "2013-01-01" @default.
- W2334604611 modified "2023-10-14" @default.
- W2334604611 title "A case of severe combined immunodeficiency presented with antibiotic treatment resistant pneumonia and respiratory failure" @default.
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- W2334604611 doi "https://doi.org/10.3918/jsicm.20.629" @default.
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