The recommendations address treatment of both adult and pediatric SCD. ASH formed a committee of medical experts, researchers and patients to review evidence and form recommendations on SCD. In 2016, the American Society of Hematology (ASH) initiated an effort to develop clinical practice guidelines on Sickle Cell Disease (SCD). All rights reserved.ASH Clinical Practice Guidelines on Sickle Cell Disease © 2020 Indian National Association for Study of the Liver. Finally, various management options including exchange transfusion, liver transplantation, and hydroxyurea in hepatic sequestration syndromes will be discussed in brief.ĪASLD, American Association for the Study of Liver Diseases ACLF, Acute on chronic liver failure ALF, Acute liver failure ALT, Alanine transaminase AST, Aspartate transaminase FFP, Fresh frozen plasma GIT, Gastrointestinal tract HAV, Hepatitis A virus HBV, Hepatitis B virus HCV, Hepatitis C virus HEV, Hepatitis E virus HIC, Hepatic iron content HbS, Sickle hemoglobin HbSS, Sickle cell disease homozygous INR, International normalized ratio PT, Prothrombin time RUQ, Right upper quadrant SC, Scheduled caste SCD, Sickle cell disease SCIC, Sickle cell intrahepatic cholestasis ST, Scheduled tribe TJLB, Transjugular liver biopsy UDCA, Ursodeoxycholic acid cholelithiasis intrahepatic cholestasis sickle cell hepatopathy sickle cholangiopathy sickle hepatic crisis. The purpose of this review is to provide insights into epidemiology of sickle cell disease in India and pathogenesis and classification of hepatobiliary involvement in sickle cell disease. Hydroxyurea, the antitumor agent, which is popularly used to prevent life-threatening complications such as acute chest syndrome or stroke in these patients, has been used only sparingly in hepatic sequestrations. Liver transplantation may also be beneficial in end-stage liver disease.
Similarly, there is paucity of the literature regarding the end point of exchange transfusion in this scenario. However, evidence for their benefits, optimal indications, and threshold to start exchange transfusion is limited. However, rarely these episodes may present with coagulopathy and encephalopathy like acute liver failure, which are life-threatening, requiring exchange transfusions or even liver transplantation. The sequestration syndromes are usually episodic and self-limiting requiring conservative management such as antibiotics and intravenous fluids or packed red cell transfusions. The clinical spectrum of disease ranges from ischemic injury due to sickling of red blood cells in hepatic sinusoids, pigment gall stones, and acute/chronic sequestration syndromes.
Liver disease may result from viral hepatitis and iron overload due to multiple transfusions of blood products or due to disease activity causing varying changes in vasculature. Hepatic involvement in sickle cell disease is not uncommon. The disease is not uncommon in India in terms of prevalence, India is second only to Sub-Saharan Africa where sickle cell disease is most prevalent. Sickle hepatopathy is an umbrella term describing various pattern of liver injury seen in patients with sickle cell disease.
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