Hepatic Dry Copper Weight in Pediatric Autoimmune Liver Disease.
Hepatic Dry Copper Weight in Pediatric Autoimmune Liver Disease.
复制标题
儿童自身免疫性肝病中的肝干铜重。
DOI：
10.1097/mpg.0000000000003654
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发表时间：
2023
影响因子：
2.9
通讯作者：
Nayagam JS
中科院分区：
文献类型：
--
作者：
Nayagam JS
目的：在成人自身免疫性肝病（AILD）和慢性胆汁淤积中，肝干铜重量升高是公认的。我们的目的是审查肝干铜重量在儿科AILD.Methods：回顾性分析1999年至2018年在我们机构管理的儿科AILD，104例患者肝干铜重量assessments.Results：中位年龄在介绍是13.4岁（四分位数范围，IQR，11.7-14.9），60%的女性，54%自身免疫性肝炎，42%自身免疫性硬化性胆管炎，4%原发性硬化性胆管炎。晚期肝纤维化的组织学特征为68%。中位肝脏干铜重量为51.1 µg/g干重（IQR，28.0-103.8）。51%的患者存在肝干铜重量升高（> 50 µg/g干重），与AILD亚型（P= 0.83）、就诊年龄（P= 0.68）或晚期纤维化（P= 0.53）无关。肝干铜重较高者行肝移植（10%）（148.5 µg/g干重[IQR，39.5-257.3] vs 47.5 [IQR，27.8-91.5]，P= 0.04）;然而，在多变量分析中，这与LT无关（风险比1.002，95%CI 0.999-1.005，P= 0.23）。在8例（7.7%）患者ATP 7 B测序和潜在的致病变异被确定在2例患者，都需要LT。结论：肝干铜重量升高是常见的儿科AILD。与成人不同，它与伴有胆汁淤积的AILD亚型无关。在需要LT的患者中检测到较高的干铜重量。虽然需要进一步的工作来确定铜沉积在儿科AILD中的意义，但我们建议密切监测水平升高的患者进行性肝病。
Objectives:Elevated hepatic dry copper weight is recognized in adults with autoimmune liver disease (AILD) and chronic cholestasis. We aim to review hepatic dry copper weight in pediatric AILD.Methods:Retrospective review of pediatric AILD managed at our institution from 1999 to 2018, and 104 patients with hepatic dry copper weight assessment were included.Results:Median age at presentation was 13.4 years (interquartile range, IQR, 11.7–14.9), 60% female, 54% autoimmune hepatitis, 42% autoimmune sclerosing cholangitis, and 4% primary sclerosing cholangitis. Histological features of advanced liver fibrosis in 68%. Median hepatic dry copper weight was 51.1 µg/g dry weight (IQR, 28.0–103.8). Elevated hepatic dry copper weight (> 50 µg/g dry weight) was present in 51%, and was not associated with AILD subtype (P= 0.83), age at presentation (P= 0.68), or advanced fibrosis (P= 0.53). Liver transplantation (LT) was performed in 10%, who had higher hepatic dry copper weight (148.5 µg/g dry weight [IQR, 39.5–257.3] vs 47.5 [IQR, 27.8–91.5], P= 0.04); however this was not associated with LT on multivariate analysis (hazard ratio 1.002, 95% CI 0.999–1.005, P= 0.23). In 8 (7.7%) patients ATP7B was sequenced and potentially disease causing variants were identified in 2 patients, both who required LT.Conclusions:Elevations in hepatic dry copper weight are common in pediatric AILD. Unlike in adults, it is not associated with AILD subtypes with cholestasis. Higher dry copper weight was detected in patients who required LT. While further work is needed to identify the significance of copper deposition in pediatric AILD, we recommend close monitoring of patients with elevated levels for progressive liver disease.