特発性肺ヘモジデローシス5例の臨床像および病因論の考察  [in Japanese] Clinical Manifestations and Pathogenesis in 5 Cases of Idiopathic Pulmonary Hemosiderosis.  [in Japanese]

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Abstract

1983年10月から1993年11月までに5例の特発性肺ヘモジデローシスidiopathic pulmonary hemosiderosis (IPH) を経験した.男児1例, 女児4例であり, 発症時の年齢は11カ月から3歳であった.初発症状では全例に発熱および貧血を認めたが, 血疾を認めたのは1例のみで, 呼吸器症状を認めなかったのが1例みられた, 輸血を必要としたのは3例, ステロイド投与を行ったのは3例で, 輸血を必要とした3例は全例発症後6カ月以内に死亡し, 2例が現在生存中である.4例に種々の周産期異常を認め, 3例では新生児期に人工換気療法を施行していた.人工換気による肺損傷で肺胞上皮の変性をきたし, IPHの発症につながった可能性が示唆された.

We herein describe 5 children (1 boy, 4 girls) who were diagnosed with idiopathic pulmonary hemosiderosis (IPH) during these 10 years. The age of onset ranged from 11 months to 3 years. The first symptoms of all children were fever and anemia, however one child complained of hemoptysis. With the exception of one child, there were respiratory symptoms at the onset of IPH in all cases. Blood transfusions were needed in 3 children, all of whom died within 6 months after onset, and 2 others are alive. Four children had various perinatal abnormalities, and 3 of them received mechanical ventilation. Barotrauma due to mechanical ventilation may cause the alveolar epithelial alterations. We suggest that in our cases, these alterations may be one of the pathogenic mechanisms of IPH.

Journal

  • The Japanese Journal of Pediatric Hematology

    The Japanese Journal of Pediatric Hematology 8(6), 486-491, 1994

    THE JAPANESE SOCIETY OF PEDIATRIC HEMATOLOGY/ONCOLOGY

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