深頸部膿瘍を認めた慢性肉芽腫症の1幼児例 Chronic Granulomatous Disease Accompanying a Deep Cervical Abscess in a Young Child

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慢性肉芽腫症(chronic granulomatous disease,CGDと略す)は食細胞の殺菌作用の主体である活性酸素の産生に障害をもつ食細胞機能不全症である.細胞内殺菌機構を障害されるため,乳幼児期よりカタラーゼ陽性,H2O2非産生性の細菌(ブドウ球菌,Gram陰性桿菌,結核菌.真菌など)による反復性,遷延性の感染症を引き起こす原発性免疫不全症候群の一つである.難治性疾患であり感染症に対する一般的治療以外に,骨髄移植や遺伝子治療等が試みられてはいるが,いまだ治療法は確立していない.本例は難治性深頸部腫瘍のため免疫不全を疑われた1歳7ヵ月の幼児で,各種の免疫能検査にて異常を示さず,NBT還元試験においてもCGDと診断できなかった症例であった.確定診断のために過酸化水素産生能試験を行い,同時に家系の検索を行った.その結果,患児は常染色体劣性遺伝でチトクロームb陽性のCGDと考えられた.頸部外切開による下レナージ手術を中心とした治療を行い,感染を制御することができた.

We report the diagnosis and otorhinological treatment of a rare case of cervical abscess in association with chronic granulomatous disease (CGD). A 19-month-old boy was admitted to our hospital's Department of Pediatrics with fever and a swollen neck. Antibiotic treatment (FMOX PIPC and PAPM) was ineffective, so the patient was referred to our department for otorhinological treatment. A deep cervical abscess was drained using paracentesis in the Outpatient Department, but the condition did not improve. Surgery to drain the cavity was therefore performed. The wound was irrigated with oxydol, and the surgery was a success.<br>Immunodeficiency was suspected, because of the antibiotic treatment's failure, Further examination clarified that the disease was CGD, but this diagnosis was difficult to establish. The result of the NBT test was 120%. However, some examination demonstrated that active oxygen production was impaired. The production of cytochrome b in this patient was confirmed using flow cytometric analysis. Both parents were confirmed to be carriers upon examination. The patient was therefore diagnosed as having CGD as a result of an autosomal recessive inheritance.

収録刊行物

  • 日本耳鼻咽喉科學會會報

    日本耳鼻咽喉科學會會報 103(3), 219-222, 2000-03-20

    The Oto-Rhino-Laryngological Society of Japan, Inc.

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各種コード

  • NII論文ID(NAID)
    10008574635
  • NII書誌ID(NCID)
    AN00191551
  • 本文言語コード
    JPN
  • 資料種別
    ART
  • ISSN
    00306622
  • データ提供元
    CJP書誌  J-STAGE 
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