Hepatocellular carcinoma in Gaucher disease: an international case series.
Hepatocellular carcinoma in Gaucher disease: an international case series.
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DOI：
10.1007/s10545-018-0142-y
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发表时间：
2018-09
影响因子：
4.2
通讯作者：
Hollak CEM
中科院分区：
文献类型：
--
作者：
Regenboog M;van Dussen L;Verheij J;Weinreb NJ;Santosa D;Vom Dahl S;Häussinger D;Müller MN;Canbay A;Rigoldi M;Piperno A;Dinur T;Zimran A;Mistry PK;Salah KY;Belmatoug N;Kuter DJ;Hollak CEM
戈谢病（GD）与恶性肿瘤风险增加相关。除了血液恶性肿瘤，还描述了几种器官中实体瘤的发展。肝脏是参与GD发病机制的主要储存部位之一，也受到肝脏特异性并发症的影响。在这个病例系列中，我们描述了来自世界各地八个不同转诊中心的16例发生肝细胞癌（HCC）的GD 1型（GD 1）患者。研究了GD患者中导致HCC风险增加的潜在因素。11名患者过去接受过脾切除术。肝硬化是HCC发展的主要危险因素之一，在14例有数据的患者中有9例存在肝硬化。7例受检患者中有3例转铁蛋白饱和度> 45%。在这三名患者中，肝脏组织病理学检查后发现存在铁过载。14例检查病例中有3例存在慢性丙型肝炎感染。我们总结了所有的研究结果，并与文献进行了比较。我们建议GD患者，特别是那些有脾切除术或铁超载的患者，应评估肝纤维化的体征，如果发现HCC，应监测其发展。
Gaucher disease (GD) is associated with an increased risk for malignancies. Next to hematological malignancies, the development of solid tumors in several organs has been described. The liver is one of the major storage sites involved in GD pathogenesis, and is also affected by liver-specific complications. In this case series, we describe 16 GD type 1 (GD1) patients from eight different referral centers around the world who developed hepatocellular carcinoma (HCC). Potential factors contributing to the increased HCC risk in GD patients are studied. Eleven patients had undergone a splenectomy in the past. Liver cirrhosis, one of the main risk factors for the development of HCC, was present in nine out of 14 patients for whom data was available. Three out of seven examined patients showed a transferrin saturation > 45%. In these three patients the presence of iron overload after histopathological examination of the liver was shown. Chronic hepatitis C infection was present in three of 14 examined cases. We summarized all findings and made a comparison to the literature. We recommend that GD patients, especially those with prior splenectomy or iron overload, be evaluated for signs of liver fibrosis and if found to be monitored for HCC development.
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影响因子：
2.4
作者：
Schuette, Kerstin;Schulz, Christian;Malfertheiner, Peter
通讯作者：
Malfertheiner, Peter
影响因子：
6.5
作者：
Regenboog, Martine;Bohte, Anneloes E.;Hollak, Carla E. M.
通讯作者：
Hollak, Carla E. M.
影响因子：
158.5
作者：
BARTON, NW;BRADY, RO;YU, KT
通讯作者：
YU, KT
影响因子：
3
作者：
FLESHNER, PR;AUFSES, AH;ELIAS, R
通讯作者：
ELIAS, R
影响因子：
29.4
作者：
El-Serag, HB;Tran, T;Everhart, JE
通讯作者：
Everhart, JE