University of Florida College of Medicine, Gainesville, FL, USA.
Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Clin Res Hepatol Gastroenterol. 2022 Nov;46(9):102015. doi: 10.1016/j.clinre.2022.102015. Epub 2022 Sep 5.
Vanishing bile duct syndrome (VBDS) is a serious cholestatic liver disease that can be a complication of drug-induced liver injury (DILI). While journals have published case reports of this condition, large studies on a cohort of these patients are lacking. We aimed to compile published case reports and case series of patients with VBDS and DILI to describe the clinical and laboratory characteristics of the disease and identify factors associated with good and poor outcomes.
We included case reports and case series of VBDS secondary only to DILI. We extracted demographic, clinical, laboratory, treatment, and exposure data from each case report and categorized cases by outcome, good versus poor. We defined poor outcomes as cases with severe long-term complications or death. We analyzed risk factors for poor outcomes using logistic regression.
We identified a total of 59 eligible cases. Of those, 39 (59%) were female, the median age was 36 (IQR:12-58), and 18 (31%) were pediatric cases (≤18 years). The most common offending drug class was antibiotics, especially beta-lactams. Patients with increased total bilirubin (OR=4.69; 95% CI=1.55-15.49; p = 0.008), increased direct bilirubin (OR=6.50; 95% CI=1.34-48.91; p = 0.034), lower liver synthetic activity (OR=0.11; 95% CI=0.02-0.55; p = 0.013), and older age (OR=3.31; 95% CI=1.15-10.04; p = 0.029) were more likely to develop poor outcomes.
In patients with VBDS and DILI, antibiotics were the most common offending agents. Higher total and direct bilirubin levels were associated with poor outcomes.
消失胆管综合征（VBDS）是一种严重的胆汁淤积性肝病，可能是药物性肝损伤（DILI）的并发症。虽然期刊已经发表了关于这种情况的病例报告，但缺乏对这些患者队列的大型研究。我们旨在汇编已发表的 VBDS 和 DILI 患者的病例报告和病例系列，以描述该疾病的临床和实验室特征，并确定与良好和不良结局相关的因素。
我们纳入了仅由 DILI 引起的 VBDS 的病例报告和病例系列。我们从每个病例报告中提取人口统计学、临床、实验室、治疗和暴露数据，并根据结局（良好与不良）对病例进行分类。我们将严重的长期并发症或死亡定义为不良结局。我们使用逻辑回归分析不良结局的危险因素。
我们共确定了 59 例符合条件的病例。其中，39 例（59%）为女性，中位年龄为 36（IQR：12-58）岁，18 例（31%）为儿科病例（≤18 岁）。最常见的致病药物类别是抗生素，尤其是β-内酰胺类。总胆红素升高（OR=4.69；95%CI=1.55-15.49；p=0.008）、直接胆红素升高（OR=6.50；95%CI=1.34-48.91；p=0.034）、肝脏合成活性降低（OR=0.11；95%CI=0.02-0.55；p=0.013）和年龄较大（OR=3.31；95%CI=1.15-10.04；p=0.029）的患者更有可能出现不良结局。
在 VBDS 和 DILI 患者中，抗生素是最常见的致病药物。较高的总胆红素和直接胆红素水平与不良结局相关。
