Role of phlebotomy in the treatment of liver damage related to erythropoietic porphyria.
Role of phlebotomy in the treatment of liver damage related to erythropoietic porphyria.
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DOI：
10.1038/s41598-022-10089-z
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发表时间：
2022-04-12
影响因子：
4.6
通讯作者：
Kudo M
中科院分区：
文献类型：
--
作者：
Hagiwara S;Nishida N;Ida H;Ueshima K;Minami Y;Takita M;Aoki T;Morita M;Chishina H;Komeda Y;Yoshida A;Park AM;Sato M;Kawada A;Nakano H;Nakagawa H;Kudo M
肝损害影响红细胞生成性原卟啉症（EPP）患者的预后。然而，对于伴有严重肝损害的EPP患者，尚无根治性治疗方法。本研究旨在探讨静脉切开术在严重肝损伤患者中的有效性。我们检查了2010年至2020年期间诊断为EPP和肝损伤的7名患者。7例中3例肝脏病变严重者行静脉切开术，均观察到肝脏病变改善效果。此外，作为额外的研究，我们还研究了肝损伤变得更严重的机制。肝活检标本用苏木精-伊红染色，免疫组化法检测三磷酸腺苷结合转运蛋白G2（ABCG 2）的表达。7例EPP患者中3例进行了肝活检。在这3例患者中，2例患者的ABCG 2表达较低，尤其是在原卟啉（PP）沉积区。两名ABCG 2表达降低的患者随后发生了严重的肝损伤。但ABCG 2表达降低与肝损害加重之间的因果关系尚未得到直接证明，有待于今后进一步研究。本研究证实了静脉切开术在伴有严重肝损伤的EPP患者中的有效性。
Liver damage affects the prognosis of patients with erythropoietic protoporphyria (EPP). However, there is no radical cure for EPP patients with severe liver damage. This study aims to investigate the effectiveness of phlebotomy in patients with severe liver damage. We examined seven patients diagnosed with EPP and liver damage between 2010 and 2020. Of the 7 cases, phlebotomy was performed in 3 cases with severe hepatic disorder, and the improvement effect of hepatic disorder was observed in all cases. In addition, as an additional study, we also investigated the mechanism by which liver damage becomes more severe. Liver biopsy samples were stained with hematoxylin and eosin and immunohistochemistry was used to examine the expression of adenosine triphosphate-binding transporter G2 (ABCG2). Liver biopsies were performed in 3 of 7 patients with EPP. Of these three patients, ABCG2 expression was low in two patients, especially in the protoporphyrin (PP) deposition area. Two patients with reduced ABCG2 expression subsequently developed severe liver damage. However, the causal relationship between the decreased expression of ABCG2 and the exacerbation of liver damage has not been directly proved, and further investigation is required in the future. This study demonstrated the effectiveness of phlebotomy in EPP patients with severe liver damage.
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影响因子：
1.2
作者：
Yoshida, Akihiro;Hagiwara, Satoru;Kudo, Masatoshi
通讯作者：
Kudo, Masatoshi
影响因子：
2.9
作者：
Bloomer, J;Wang, YM;Risheg, H
通讯作者：
Risheg, H
影响因子：
13.6
作者：
Wang, Pengcheng;Sachar, Madhav;Ma, Xiaochao
通讯作者：
Ma, Xiaochao
影响因子：
20.3
作者：
Egan, Daniel N.;Yang, Zhantao;Abkowitz, Janis L.
通讯作者：
Abkowitz, Janis L.
影响因子：
13.5
作者：
BLOOMER, JR
通讯作者：
BLOOMER, JR