Deeb Maya, Leung Kristel K, Ward Richard, Feld Jordan J, Kuo Kevin H M, Hirschfield Gideon M
Toronto Centre for Liver Disease, Toronto General Hospital, University Health Network, Toronto, Ontario, Canada.
Department of Medicine, The Autoimmune and Rare Liver Disease Programme, Division of Gastroenterology and Hepatology, Toronto General Hospital, Toronto, Ontario, Canada.
Hepatol Commun. 2025 Apr 30;9(5). doi: 10.1097/HC9.0000000000000712. eCollection 2025 May 1.
Sickle cell disease (SCD) is the most common hemoglobinopathy. We aimed to identify the prevalence of hepatobiliary injury and its association with mortality in SCD.
Patients with SCD followed at a dedicated clinic at our tertiary center were retrospectively evaluated with descriptive statistics. Correlations between hepatobiliary complications and SCD complications were expressed as ORs. To evaluate mortality predictors, log-rank testing was used for univariate analysis and Cox proportional hazards for multivariable analysis, with time-dependent covariates for biochemistry.
Between January 1990 and December 2020, 1009 patients with SCD were identified; 63.2% were HbSS. The median age at first clinic visit was 26.4 years (IQR: 18.9-37.1), 44.3% were male, and 62.6% were ever treated with hydroxyurea. The median follow-up was 4.8 years (IQR: 1.9-8.5); mortality was 8.9%. The most frequent hepatobiliary manifestations were cholelithiasis (n=431 [42.7%]) and iron overload (n=121; 12%). Chronic viral hepatitis was reported in only 18 patients. Twenty-nine patients (2.1%) had peak ALT> 2× upper limit of normal, 15 (2.3%) had peak ALP> 2× upper limit of normal, 97 (10.3%) had peak total bilirubin >103 μmol/L, (6.02 mg/dL), and 184 (18.2%) patients had elevated peak direct bilirubin. Hepatomegaly was reported in 37 patients (3.7%), while 24 patients (2.4%) were clinically cirrhotic. Five patients received a liver transplant. In an exploratory multivariate model, age (HR 1.08 [95% CI: 1.05-1.11]), ALT elevation (HR 1.52 [95% CI: 1.29-1.78]), and total bilirubin >103 μmol/L (HR 9.3 [95% CI: 3.95-21.9]) predicted mortality independently.
Hepatobiliary complications are common in patients with SCD and require vigilance for identification.
镰状细胞病（SCD）是最常见的血红蛋白病。我们旨在确定肝胆损伤的患病率及其与SCD死亡率的关联。
对在我们三级中心的专科门诊随访的SCD患者进行回顾性评估，并进行描述性统计分析。肝胆并发症与SCD并发症之间的相关性以比值比表示。为了评估死亡率预测因素，采用对数秩检验进行单变量分析，采用Cox比例风险模型进行多变量分析，并对生化指标采用时间依存协变量。
1990年1月至2020年12月期间，共确定1009例SCD患者；63.2%为HbSS型。首次门诊就诊的中位年龄为26.4岁（四分位间距：18.9 - 37.1岁），44.3%为男性，62.6%曾接受羟基脲治疗。中位随访时间为4.8年（四分位间距：1.9 - 8.5年）；死亡率为8.9%。最常见的肝胆表现为胆结石（n = 431例[42.7%]）和铁过载（n = 121例；12%）。仅18例患者报告有慢性病毒性肝炎。29例患者（2.1%）的谷丙转氨酶峰值>正常上限的2倍，15例患者（2.3%）的碱性磷酸酶峰值>正常上限的2倍，97例患者（10.3%）的总胆红素峰值>103 μmol/L（6.02 mg/dL），184例患者（18.2%）的直接胆红素峰值升高。37例患者（3.7%）报告有肝肿大，24例患者（2.4%）临床诊断为肝硬化。5例患者接受了肝移植。在探索性多变量模型中，年龄（风险比1.08 [95%置信区间：1.05 - 1.11]）、谷丙转氨酶升高（风险比1.52 [95%置信区间：1.29 - 1.78]）和总胆红素>103 μmol/L（风险比9.3 [95%置信区间：3.95 - 21.9]）可独立预测死亡率。
肝胆并发症在SCD患者中很常见，需要警惕识别。
