Müller G, Veyckemans F, Calier M, Van Obbergh L J, De Kock M, Sokal E M, Otte J B
Department of Anaesthesiology, Catholic University of Louvain Medical School, Cliniques Universitaires St Luc, Brussels, Belgium.
Can J Anaesth. 1995 Dec;42(12):1126-33. doi: 10.1007/BF03015100.
Progressive familial intrahepatic cholestasis (PFIC) or Byler's disease is one of the most common forms of intrahepatic cholestasis of metabolic and genetic origin. Affected children progress to terminal cirrhosis before adulthood and at present the only curative treatment of PFIC is orthotopic liver transplantation (OLT). We present a retrospective review of 40 general anaesthetics administered in our hospital to 22 patients with PFIC undergoing various procedures. The clinical features of PFIC and the anaesthetic implications of chronic cholestasis in children (malnutrition, cirrhosis, portal hypertension, chronic hypoxaemia) are reviewed.
进行性家族性肝内胆汁淤积症（PFIC）或比勒氏病是代谢和遗传起源的肝内胆汁淤积症最常见的形式之一。患病儿童在成年前会发展为终末期肝硬化，目前PFIC唯一的治愈性治疗方法是原位肝移植（OLT）。我们对我院为22例接受各种手术的PFIC患者实施的40例全身麻醉进行了回顾性研究。本文回顾了PFIC的临床特征以及儿童慢性胆汁淤积症的麻醉影响（营养不良、肝硬化、门静脉高压、慢性低氧血症）。
