Buxbaum James, Nguyen Nancy, Kulkarni Sujit, Palmer Suzanne, Rao Adupa, Selby Robert
Department of Medicine, Los Angeles County Hospital, Keck School of Medicine, University of Southern California, D & T Building Room B4H100, 1983 Marengo St., Los Angeles, CA, 90033-1370, USA,
Dig Dis Sci. 2015 Jun;60(6):1801-4. doi: 10.1007/s10620-015-3532-0. Epub 2015 Jan 29.
As the survival of cystic fibrosis patients improves due to better treatment of its pulmonary manifestations, the management of hepatobiliary complications becomes increasingly vital. While focal biliary cirrhosis is common, large duct manifestations are less frequently encountered.
We prospectively evaluated cases of large bile duct disease in a large adult cystic fibrosis practice at the Keck Hospital of the University of Southern California.
Over a 5-year period, six patients presented with cholangiectasia, hepatolithiasis, and strictures. Their clinical presentation and course closely resembled recurrent pyogenic cholangitis (RPC). Treatment of cholangitis and strictures was primarily by endoscopic retrograde cholangiopancreatography, but major hepatobiliary surgery following pulmonary optimization was required in 33 %.
In adult populations, CF-RPC may not be as unusual as previously reported and recognition allows optimal endoscopic, medical, and surgical management.
随着囊性纤维化患者因其肺部表现得到更好治疗而生存率提高，肝胆并发症的管理变得愈发重要。虽然局灶性胆汁性肝硬化很常见，但大导管表现较少见。
我们前瞻性评估了南加州大学凯克医院大型成人囊性纤维化诊疗中心的大胆管疾病病例。
在5年期间，6例患者出现胆管扩张、肝内胆管结石和狭窄。他们的临床表现和病程与复发性化脓性胆管炎（RPC）极为相似。胆管炎和狭窄的治疗主要通过内镜逆行胰胆管造影术，但33%的患者在肺部状况优化后需要进行大型肝胆手术。
在成人患者中，CF-RPC可能不像先前报道的那样罕见，认识到这一点有助于进行最佳的内镜、药物和手术管理。
