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題 名 | Alagille Syndrome: A Case Report=阿拉之症侯群:一病例報告 |
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作 者 | 劉武翹; 鄭慶明; 李紋瑜; 沈慶村; 王永成; 吳昭瑩; 孔慶惠; | 書刊名 | 中華放射線醫學雜誌 |
卷 期 | 23:2 1998.04[民87.04] |
頁 次 | 頁61-63 |
分類號 | 417.509 |
關鍵詞 | 肺動脈狹窄; 膽汁鬱滯; 磁振影像; Pulmonary arterial stenosis; Cholestasis; MRI; |
語 文 | 英文(English) |
中文摘要 | 阿拉之症侯群包含新生兒慢性膽汁鬱滯,肺動脈狹窄,蝴蝶狀椎體,特異臉形及眼睛病變。病理學上呈現肝內膽管數目減少為本症之特色。我們報告一名四歲大男孩,因持續黃疸入院。磁振影像顯示左側肺動脈週邊狹窄以及單一蝴蝶狀椎體,雙眼有胎生環,肝臟生檢呈肝內膽管數目減少為典型的阿拉之症侯群病例。 |
英文摘要 | Arteriohepatic dysplasia (Alagille syndrome) is a condition of chronic cholestasis in infancy associated with odd facies, pulmonic stenosis, butterfly vertebra and other abnormalities. The pathologic hallmark of arteriohepatic dysplasia is a paucity or absence of intrahepatic bile ducts. We present a 4-year-old boy with MRI study of heart showing peripheral stenosis of the left pulmonary artery and single butterfly vertebra. Ophthalmal examination discovered embryotoxon of both eyes. Scintigraphy revealed normal excretion of Tc-99m IDA into the intestine from the liver and liver biopsy exhibited paucity of intrahepatic bile ducts. |
本系統中英文摘要資訊取自各篇刊載內容。