Extreme hyperbilirubinemia: An indicator of morbidity and mortality in sickle cell disease

• 作者: Cesar Taborda Fuad El Rassi John Paul Haydek Morgan L McLemore Preeti A Reshamwala Rushikesh Shah Saurabh Chawla
• 来源期刊: 世界肝病学杂志:英文版(电子版)
• 学科: 医学
• 关键词: SICKLE CELL disease SICKLE CELL HEPATOPATHY Liver diseases EXTREME HYPERBILIRUBINEMIA MORTALITY
• 年，卷（期）: 2019,（3）
• 页码范围: 287-293
• 页数: 7页
• 分类号: R

摘要

BACKGROUND Sickle cell disease (SCD) is a disorder that results in increased hospitalizations and higher mortality. Advances in management have resulted in increases in life expectancy and led to increasing awareness of sickle cell hepatopathy (SCH). However, its impact in patients on the natural history and outcomes of SCD is not known. Our study aims to describe the prevalence of extreme hyperbilirubinemia (EH), one form of SCH, its effect on morbidity and mortality, and correlations between sickle cell genotype and SCH type. We hypothesize that EH is associated with higher morbidity and mortality. AIM To investigate the effects of EH on morbidity and mortality among patients with SCD. METHODS This retrospective cohort study was performed using a database of patients with SCD treated at Grady Memorial Hospital between May 2004 and January 2017. Patients with EH (defined as total bilirubin above 13.0 mg/dL) were identified. A control group was identified from the same database with patients with total serum bilirubin ≤ 5.0 mg/dL. Electronic medical records were used to extract demographic information, laboratory values, radiology results, current medications, need for transfusions and mortality data. Two samples T-test, chisquared test and Fisher's exact test were then used to compare the parameters between the two groups. RESULTS Out of the database, fifty-seven charts were found of patients with bilirubin > 13 mg/dL. Prevalence of severe SCH as defined by EH was 4.8%(57/1172). There were no demographic differences between patients with and without EH. Significant genotypic differences existed between the two groups, with hemoglobin SS SCD being much higher in the EH group (P < 0.001). Patients with severe EH had a significant elevations in alanine aminotransferase (157.0 ± 266.2 IU/L vs 19.8 ± 21.3 IU/L, P < 0.001), aspartate aminotransferase (256.5 ± 485.9 U/L vs 28.2 ± 14.7 U/L, P < 0.001) and alkaline phosphatase (218.0 ± 176.2 IU/L vs 85.9 ± 68.4 IU/L, P < 0.001). Patients with EH had significantly