Williams S G, Evanson J E, Barrett N, Hodson M E, Boultbee J E, Westaby D
Department of Gastroenterology, Charing Cross Hospital, London, UK.
J Hepatol. 1995 May;22(5):513-21. doi: 10.1016/0168-8278(95)80444-7.
Advances in the management of the pulmonary complications of cystic fibrosis may result in an increasing prevalence of patients with chronic liver disease which may, therefore, become more important in the long-term management of cystic fibrosis patients. However, no simple and reliable test is available for the diagnosis of liver disease in cystic fibrosis. In particular percutaneous liver biopsy is highly inaccurate and potentially dangerous. Imaging techniques, including real-time ultrasound scanning, have been used to evaluate the hepato-biliary system in cystic fibrosis and may represent the best available techniques for documenting hepatic involvement. The purposes of this study were to construct an ultrasound scoring system using three cardinal features of hepatic ultrasound in cystic fibrosis: coarseness of the parenchyma, nodularity of the liver edge and increased periportal echogenicity, to enable the accurate, early diagnosis of liver involvement in cystic fibrosis. The scoring system was validated by correlating the results against ultrasound markers of portal hypertension, clinical and haematological data. The scoring system proved to be reproducible and to correlate well with the markers of hepatic disease detailed above. The results also suggest that the scoring system may allow the identification of patients with pre-cirrhotic chronic liver disease and so may prove of value in selecting a sub-group of patients more likely to respond to therapy.
囊性纤维化肺部并发症管理方面的进展可能导致慢性肝病患者的患病率上升，因此，这在囊性纤维化患者的长期管理中可能变得更加重要。然而，目前尚无简单可靠的检测方法用于诊断囊性纤维化患者的肝病。特别是经皮肝活检准确性极低且有潜在危险。包括实时超声扫描在内的成像技术已被用于评估囊性纤维化患者的肝胆系统，可能是记录肝脏受累情况的最佳可用技术。本研究的目的是利用囊性纤维化肝脏超声的三个主要特征构建一个超声评分系统：实质回声粗糙、肝边缘结节状以及门静脉周围回声增强，以便准确、早期诊断囊性纤维化患者的肝脏受累情况。通过将结果与门静脉高压的超声标志物、临床和血液学数据相关联，对该评分系统进行了验证。该评分系统被证明具有可重复性，且与上述肝病标志物相关性良好。结果还表明，该评分系统可能有助于识别肝硬化前慢性肝病患者，因此在选择更可能对治疗有反应的患者亚组方面可能具有价值。
