A prospective study of the association between sickle cell disease and hepatobiliary effects in Bahrain.

ABSTRACT

BACKGROUND: Sickle cell disease is a genetic disorder that leads to abnormally high levels of hemoglobin sickling in erythrocytes. Patients suffer debilitating and severe complications that affect multiple organs, though mainly the liver, gallbladder, spleen, bones, and kidneys. It has a significant impact on morbidity and mortality rates and is associated with substantial health care costs. METHODS: For this study, the researchers prospectively reviewed the charts of 154 sickle cell disease patients who had been treated in Salmaniya Medical Complex hospital in Bahrain between September 2013 and December 2014. RESULTS: The results obtained showed that 95% of patients had sickle cell hemoglobin type. Of the sample patients, 80% exhibited bone pain, with all patients exhibiting bone crisis. Biochemical tests revealed reduced hemoglobin concentration, elevated bilirubin, compromised liver function, and lower white blood cell counts. The incidence of hepatitis B and C was very low, at 0% and 9%, respectively. There were a large number of hospital admissions, with 11 days as an average length of stay. The most common ultrasound findings in this study were hepatomegaly, hepatosplenomegaly, cholelithiasis, gallbladder sludge, and splenomegaly. CONCLUSION: Sickle cell disease had profound negative effects on multiple organs, with a particularly large number of complications related to the hepatobiliary system.