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Liver Dysfunction in Sickle Cell Disease Takes Many Forms

Eight distinct patterns of liver injury identified, though considerable overlap exists

FRIDAY, Dec. 28 (HealthDay News) -- The patterns of liver disease encountered in patients with sickle cell disease are diverse but with considerable overlap, defying broad characterization, reports an article published in Clinical Gastroenterology and Hepatology in December.

Philip A. Berry, of King's College Hospital in London, U.K., and colleagues describe the clinical history and laboratory, radiographic and histologic findings in 38 patients (mean age 33 years) with hemoglobin sickle cell disease, SC or S-β thalassemia who were referred to a tertiary liver center for evaluation.

The researchers identified eight patterns of liver disease, including massive hepatocellular necrosis (5 percent), acute severe sequestration and cholestasis occurring with sepsis (18 percent), cirrhosis (18 percent), pattern of chronic, fluctuating sequestration without cholestasis (21 percent). Of 20 patients requiring emergency admission, eight patients died during the hospitalization, and three patients died during follow-up admissions.

"Although distinct patterns of liver dysfunction in sickle cell disease appear to exist, there are significant clinical heterogeneity and overlap in terms of presentation, investigation and natural history," write the authors. "However, the use of only one descriptive term (such as sickle hepatopathy) or even classification into mild versus severe or acute versus chronic hepatic dysfunction is not sufficient given the diversity observed in this cohort."

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