喉頭全摘の際発見された無カタラーゼ血液症の1症例

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  • A Case of Acatalasemia Detected during Total Laryngectomy

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In 1949, Takahara first reported acatalasemia, which was a constitutional abnormality characterized by a deficit of catalase in the blood. Acatalasemia patients inherited autosomal recessive genes and its incidence was high in children born of consanguineous marriage.<BR>The patient was a 71-year-old male who underwent total laryngectomy because of recurrence of laryngeal cancer. The operative area was prepared with Oxidol®, a widely used disinfectant in otolaryngological disease. This antiseptic treatment induced methemoglobinemia, and it took about 16 days to recover from the critical postoperative condition.<BR>In order to clarify the causative factor, a blood hydrogen peroxide mixing test, a skin scratch test, and gene analyses were performed, and catalase activity was measured. He was made the diagnosis of acatalasemia because of the significant decrease in catalase. The patient's family history was also studied and it was found that his parents were first cousins. A catalase activity test of his mother and three children was carried out, and the result showed that all of them had suffered from hypocatalasemia. His gene abnormality was considered as a Japanese-type.<BR>As in the present case, acatalasemia has been often induced accidentally by the use of hydrogen peroxide solution. The detection rate of acatalasemia was high in otolaryngology and oral surgery.<BR>It must be borne in mind that the risk of methemoglobinemia can be minimized by paying special attention to the treatment of patients with Oxidol®.

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