2016-08-012024-05-13https://scholars.lib.ntu.edu.tw/handle/123456789/645875摘要：造成新生兒膽汁滯留性黃疸的疾病中，膽道閉鎖是最嚴重的疾病，也是兒童肝臟移植原因中最常見者，更是最常引起兒童死亡的肝病。無論是否及時接受葛西氏手術，至少40%存活者還是發生膽道性肝臟纖維硬化，進而演變成肝門脈高壓合併食道靜脈瘤出血致死。肝臟穿刺仍是評估肝臟纖維硬化程度的黃金標準，但有一定風險，同時肝內各分節存在局部纖維硬化程度的差異性，並且要以小塊切片發現能否推論整體肝臟實質變化不無疑慮。困難案例，甚至得間隔時日多次穿刺，增加危險。數篇包含本研究團隊在內的研究顯示，不具侵襲性的水分子擴散影像的肝臟表面擴散係數在罹患膽道閉鎖肝纖維化患者會降低。更進階的擴散峰度影像與體素內不同調水分子運動影像檢查，則不只可以評估擴散係數更可呈現微血管灌流。在此前瞻研究，我們希望在臨床常規的磁振造影檢查增加擴散峰度影像與體素內不同調水分子運動影像檢查約延長10 分鐘，企圖研究膽道閉鎖病患肝臟纖維硬化臨床分期與擴散權重磁振造影所得肝臟平均擴散係數、慢擴散係數、快擴散係數、峰度值、灌注比例及快擴散係數與灌注比例乘積等相關指標之間的相關性，找出不具侵襲性的水分子擴散影像指標與臨床指標，預測和追蹤膽道閉鎖患者術前術後肝臟纖維硬化進展，達到輔助診斷及減少肝臟穿刺的目的。<br> Abstract: Jaundice is not uncommon during the neonatal period. Biliary atresia (BA) is one of the most commonetiologies of neonatal cholestatic jaundice and requires surgical intervention in time. Liver transplantation inchildren is most commonly performed in victims with BA to avoid mortality from hepatic failure. Incidenceof BA is around 2-3/10,000 in Taiwan, much higher than that of caucasians, about 0.4~0.8/10,000. Even afterreceiving Kasai operation, no less than 40% of long-term BA survivors suffer from progressive biliary,hepatic fibrosis, leading to portal hypertension, esophageal variceal rupture, ascites and hypersplenismthough the underlying mechanism is still not clear.To assess the severity of hepatic fibrosis, percutaneous liver biopsy remains the golden standard butassociated untoward events, though uncommon, are inevitable. It is also questionable whether the smallbiopsy sample with regional difference can be representative of the whole liver changes. In BA patients withquestionable biopsy results, another percutaneous liver sampling will be repeated some days after the firstsampling. According to the results of previous MR diffusion-weighted image (DWI) reports, including thoseof our team, hepatic apparent diffusion coefficients decrease in BA children with hepatic fibrosis.Non-invasive diffusion kurtosis (DK) and intravoxel incoherent motion images (IVIM) of magneticresonance imaging disclosing both microscopic perfusion and water diffusion are more sophisticated andinformative DWI techniques.. In this prospective study, we hope to get DK and IVIM images during routine MR examinations, only toincrease the examination time by 10 minutes. Our purpose is to correlate Child classifications and Mayo riskscores for primary biliary cirrhosis with mean diffusivities, slow diffusion coefficients, fast diffusioncoefficients, kurtosis, perfusion fraction and the products of a fast diffusion coefficient and perfusionfraction … etc. The non-invasive IVIM and DK related indices may help to predict and follow theprogression of biliary hepatic fibrosis in BA patients before and after operation without potential risksaccompanying liver biopsies.膽道閉鎖膽道性肝硬化非侵襲性影像指標擴散峰度影像體素內不同調水分子運動影像磁振造影