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Acute and chronic hepatobiliary manifestations of sickle cell disease: A review

World Journal of Gastrointestinal Pathophysiology 2017年第3期8卷 108-116页

作者： Rushikesh Shah Cesar Taborda Saurabh Chawla Division of Digestive Diseases Department of Internal Medicine Emory University School of Medicine Department on Medicine Emory University School of Medicine Grady Memorial Hospital

Sickle cell disease(SCD) is a common hemoglobinopathy which can affect multiple organ systems in the body. Within the digestive tract, the hepatobiliary system is most commonly affected in SCD. The manifestations range from benign hyperbilirubinemia to overt liver failure, with the spectrum of acute clinical presentations often referred to as "sickle cell hepatopathy". This is an umbrella term referring to liver dysfunction and hyperbilirubinemia due to intrahepatic sickling process during SCD crisis leading to ischemia, sequestration and cholestasis. In this review, we detail the pathophysiology, clinical presentation and biochemical features of various acute and chronic hepatobiliary manifestations of SCD and present and evaluate existing evidence with regards to management of this disease process. We also discuss recent advances and controversies such as the role of liver transplantation in sickle cell hepatopathy and highlight important questions in this field which would require further research. Our aim with this review is to help increase the understanding, aid in early diagnosis and improve management of this important disease process.

关键词： Sickle cell disease Hepatopathy Hepatobiliary Intrahepatic cholestasis hepatic sequestration Sickle cell hepatic crisis Sickle cell cholangiopathy Liver transplant Iron overload

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