16歳で死亡に至った進行性肺気腫女児例 : 診断と治療上の問題点 A Case Report of a Girl with Progressive Pulmonary Emphysema who Died at the Age of 16 years : Problems with Diagnosis and Treatment

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肺気腫, 閉塞性細気管支炎 (以下BO) は, 小児では頻度が低く, 病因, 病態も様々である。今回, 労作時呼吸困難を主訴に来院, 臨床経過, 胸部CT像, 肺血流シンチグラムより臨床的にBOと診断した初診時9歳女児例を経験した。薬物, 酸素, 理学療法に加えQuality of Life向上のため各機関と連携し包括的治療を進めたが, 繰り返す急性増悪で進行性に肺機能は低下, 14歳時に脳死肺移植を申請待機中となった。そして16歳時, カンジダ・緑膿菌肺炎を機に急性増悪し, 集中治療を行うも永眠された。病理解剖で著明な肺気腫を認めたが細気管支レベルのBOに典型的な病理組織像は認めず, 病理診断は肺気腫であった。その理由として, 病理検査時期や採取部位の問題, ステロイド治療の影響を考えた。また本症例の病態に新生児期の胎便吸引症候群による末梢気道病変も考慮した。両肺にわたる進行性の小児肺気腫疾患の整理と病態の解明が待たれる。

Pulmonary emphysema and bronchiolitis obliterans (BO) are rare during childhood, and there are various etiologies and pathological findings.A 9-year-old girl who was brought to our department with a chief complaint of exertional dyspnea was clinically diagnosed with BO based on clinical course, chest CT and pulmonary perfusion scintigraphy.In addition to medication, oxygen and physical therapies, multidisciplinary care was provided by collaboration of different departments to improve her quality of life.However, repeated exacerbations progressively worsened her pulmonary function, and at the age of 14 years, she was placed on the waiting list for brain-dead donor lung transplantation.At the age of 16 years, her condition rapidly deteriorated due to pneumonia caused. by Candida and Pseudomonas, and despite intensive care, she died.Autopsy showed marked pulmonary emphysema, but no typical histopathological findings associated with BO at the bronchiolar level, and she was accordingly diagnosed with pulmonary emphysema.<BR>To the reason we raise the timing of pathological study, the site of tissue sampling and the effects of steroid therapy.We also consider the peripheral airway lesion caused by meconium aspiration syndrome of newborn.<BR>In the future, it will be necessary to clarify the pathology of childhood-onset progressive bilateral pulmonary emphysema.

収録刊行物

  • 日本小児呼吸器疾患学会雑誌 = Japanese journal of pediatric pulmonology  

    日本小児呼吸器疾患学会雑誌 = Japanese journal of pediatric pulmonology 17(1), 37-47, 2006-06-30 

    Japanese Society of Pediatric Pulmonology

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各種コード

  • NII論文ID(NAID)
    10018262594
  • NII書誌ID(NCID)
    AN10297832
  • 本文言語コード
    JPN
  • 資料種別
    NOT
  • ISSN
    09183876
  • データ提供元
    CJP書誌  J-STAGE 
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