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Masters Thesis | Hematology | India | Volume 12 Issue 11, November 2023 | Rating: 5.8 / 10
A Study on Acute & Chronic Hepatobiliary Manifestations of Sickle Cell Syndromes - Clinical, Biochemical, and Ultrasonographic Abnormalities
Kavyasree Sunil | Tilottama Parate [5] | Ramesh Parate [2]
Abstract: Background: Sickle cell disease (SCD) encompasses a group of hemoglobinopathies characterized by a single amino acid substitution in the beta - globin chain. Hepatobiliary complications are frequent among sickle cell disease patients. Sickle cell disease has been extensively studied. However, data about hepatobiliary abnormalities among the adult age group are limited. Our study aims to identify the pattern of hepatobiliary manifestations in terms of presentation, clinical features, routinely available laboratory indices and abdominal ultrasonography and to asses the extent of morbidity and mortality among patients of sickle cell syndrome due to hepatobiliary abnormalities. Methods: a cross sectional observational study was performed among 100 diagnosed cases of sickle cell syndrome, from January 2021 to october 2022. Subjects of both sexes above 18 years were enrolled. Thorough history taking, full clinical examination, hematological and biochemical parameters assessment, and abdominal ultrasonographic studies were performed in all patients. Results: Hepatobiliary involvement was found in 60% of patients. Abdominal pain was the most common symptom. Pallor was the most common sign, followed by icterus. Cholelithiasis was the most common (40%) complication followed by biliary sludge (14%). Tender hepatomegaly was found in almost all patients of acute hepatic crisis. Acute hepatic crisis & acute hepatic sequestration were the most common causes of mortality. Conclusions: Clinical spectrum of sickle cell syndrome ranges from mild liver function test abnormalities in asymptomatic patients to significant hepatobiliary abnormalities with marked hyperbilirubinemia. Acute hepatic crisis & acute hepatic sequestration were the most common acute hepatobiliary manifestations encountered during the study and they were the frequent causes of mortality, acute hepatic sequestration even though it is rare, it is life threatening & associated with high mortality. Acute hepatobiliary complications were not found in patients of sickle cell AS pattern.
Keywords: sickle cell syndrome, hepatobiliary
Edition: Volume 12 Issue 11, November 2023,
Pages: 1769 - 1773