查詢結果分析
相關文獻
- Pediatric Wilson's Disease: Presentation and Management
- 一位威爾森氏症行肝臟移植後排斥復發病患之護理經驗
- Experience of Using FK 506 as a Rescue Therapy for Refractory Hepatic Allograft Rejection
- Emergent Orthotopic Liver Transplantation for Subfulminant Hepatic Failure: A Case Report
- 肝臟移植的演進--從全肝移植、縮小肝移植、活體肝移植到分割肝移植
- Steroids used to Desensitize Penicillamine Allergy in Wilson Disease
- 肝臟移植概論
- 肝臟移植概論
- A Useful Technique for Both Allogenic and Xenogenic Orthotopic Liver Transplantation in Rats
- 肝臟移植後出現左乳腫塊
頁籤選單縮合
題 名 | Pediatric Wilson's Disease: Presentation and Management=兒童威爾森氏症:其臨床表現及治療 |
---|---|
作 者 | On, Avi; Choi, Ha-joo; Heyman, Melvin B.; Vargas, Jorge; Ament, Marvin E.; | 書刊名 | 中華民國小兒科醫學會雜誌 |
卷 期 | 38:2 民86.03-04 |
頁 次 | 頁98-103+165 |
分類號 | 417.5926 |
關鍵詞 | 威爾森氏症; 無膽石性膽囊炎; 視覺模糊; 肝臟移植; Wilson's disease; Acalculous cholecysitis; Blurred vision; Liver transplantation; |
語 文 | 英文(English) |
中文摘要 | 本研究包括十一名十八歲以下威爾森氏症兒童 (男性四位,女性七位)。平均發病年齡為 11.2 ± 3.9 (SD) 歲,平均診斷之年齡為 13.3 ± 3.4 (SD)歲,所有病人在診斷時均有肝臟之表現:肝硬化 (6名)、慢性肝炎 (2名)、及猛暴性肝衰竭 (3名)。三名病人在診斷時無症狀,兩名病人出現未曾報告的臨床症狀:一位為視覺模糊,另一位為無膽石性膽囊炎。在診斷時,六位有Kayser Fleischer環,五位有溶血性貧血。三位猛暴性肝衰竭的病人均有溶血、低血清轉氨酵素及鹼性磷酸酵素,這些發現可能有助於在此種表現下快速診斷威爾森氏症。十位病人以青黴氨 (penicillamine) 治療。四位病人接受肝臟移植,其中兩位是猛暴性肝衰竭,一位病人在等待肝臟移植期間死亡,其他病人均存活且無症狀。在兒科病人中必須注意威爾森氏症的不同表現,以即時達到正確診斷並儘快開始做正確的治療。 |
英文摘要 | Eleven patients (4 males, 7 females) with Wilson's disease who presented before 18 years of age are described. The mean age onset of symptoms was 11.2 ± 3.9 (SD) years. The mean age at diagnosis was 13.3 ± 3.4 (SD) years. All patients had hepatic manifestations of the disease when diagnosed: cirrhosis 6 patients), chronic hepatitis (2) and fulminant hepatic failure (3). Three patients were asymptomatic at diagnosis. Two of the symptomatic patients presented with new undescribed manifestations: one with blurred vision and the other with acalculous cholecystitis. At diagnosis, 6 patients had Kayser Fleischer rings and 5 and hemolytic anemia. The three patients with fulminant hepatic failure had hemolysis with relatively low serum aminotransferase and alkaline phosphatase levels, possibly helpful findings for rapid diagnosis of Wilson's disease in such presentation. Ten patients were treated with penicillamine. Liver transplantation was performed in 4 patients, 2 of which presented with fulminant hepatic failure. One patient died while waiting for liver transplantation, the remainder of the patients live free of symptoms. It is important to be aware of the different manifestations of Wilson's disease in the pediatric population, in order to make appropriate evaluations in a timely manner to facilitate early diagnosis and appropriate treatment. |
本系統中英文摘要資訊取自各篇刊載內容。