Acute and chronic hepatobiliary manifestations of sickle cell disease: A review.
| Autor: | Shah R; Rushikesh Shah, Saurabh Chawla, Division of Digestive Diseases, Department of Internal Medicine, Emory University School of Medicine, Atlanta, GA 30322, United States., Taborda C; Rushikesh Shah, Saurabh Chawla, Division of Digestive Diseases, Department of Internal Medicine, Emory University School of Medicine, Atlanta, GA 30322, United States., Chawla S; Rushikesh Shah, Saurabh Chawla, Division of Digestive Diseases, Department of Internal Medicine, Emory University School of Medicine, Atlanta, GA 30322, United States. |
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| Jazyk: | angličtina |
| Zdroj: | World journal of gastrointestinal pathophysiology [World J Gastrointest Pathophysiol] 2017 Aug 15; Vol. 8 (3), pp. 108-116. |
| DOI: | 10.4291/wjgp.v8.i3.108 |
| Abstrakt: | 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. Competing Interests: Conflict-of-interest statement: This review is contributed by all authors as a voluntary work. Authors have no commercial, financial, political, religious or any other conflicts of interest for contributing to this review. |
| Databáze: | MEDLINE |
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