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題名 | Diffuse Panbronchiolitis--A Case Report and Review of the Literature=瀰漫性廣泛性細支氣管炎--一病例報告 |
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作者 | 廖忠義; 吳清平; 彭萬誠; 陳群互; 于承平; 顏鴻欽; | 書刊名 | 胸腔醫學 |
卷期 | 16:3 2001.09[民90.09] |
頁次 | 頁197-201 |
分類號 | 415.426 |
關鍵詞 | 廣泛性細支氣管炎; 人類白血球抗原; 紅黴素; Diffuse panbronchiolitis; HLA haplotype; Erythromycin; |
語文 | 英文(English) |
中文摘要 | 瀰漫性廣泛性細支氣管炎(diffuse panbronchitis)是一種少見的慢性發炎性疾病,主要是侵犯呼吸性支氣管。此種疾病好發於遠東地區,並且與人類白血球抗原(HLA haplotype)有關。病人通常抱怨長時間咳嗽、多痰、活動性氣促,並且有長期性鼻竇炎病史。身體理學檢查常見肺部聽診兩側有嘯鳴聲和鑼音,肺功能檢查呈現阻塞型及限制型通氣障礙。胸部X光片可見兩側下肺葉瀰漫性小顆粒浸潤等。我們功成地治療一位43歲男性臺灣人,其臨床表徵、放射學檢查和病理組織切片皆和日本發表的病人相似。從文獻上記載此疾病之死亡率在過去極高,而低劑量的紅微素(400-600 mg/day)已被證實是有效且可改善存活率的治療。 |
英文摘要 | Diffuse panbronchiolitis (DPB) is an unusual form of bronchiolar disease that has been reported almost exclusively among Asians. Specific geographic preferences of the HLA haplotype, such as HLA Bw54 in Japanese, HLA A11 in Koreans, and HLA A11, Bw54 in Hong Kong and Singapore Chinese, have been linked to DPB. DPB is a distinctive sinobronchial syndrome with typical radiological and histologic features involving terminal and respiratory bronchioles. With appropriate erythromycin treatment, an improved overall survival rte of patients with DPB has been reported. In this report, we present a 43-year-old Taiwanese man with characteristic clinical, radiographic, and histiologic features of diffuse panbronchiolitis, resembling those described by others in the literature. HLA typing showed positive for HLA-A11, A31, and B35 for this particular patient. A mixed ventilatory and diffusion capacity impairment associated with hypoxemia, as well as radiological abnormalities, were significantly alleviated after OPD erythromycin treatment for four months. |
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