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Acute and chronic hepatobiliary manifestations of sickle cell disease: A review 预览
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作者 Rushikesh Shah Cesar Taborda Saurabh Chawla 《世界胃肠病理生理学杂志:英文版(电子版)》 2017年第3期108-116,共9页
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 rang... 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. 展开更多
关键词 镰刀房间疾病 HEPATOPATHY HEPATOBILIARY Intrahepatic cholestasis 隐遁 镰刀房间危机 镰刀房间 cholangiopathy 移植 铁超载
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