十七年小儿肝移植治疗肝硬化和肝细胞癌的经验。

IF 1.9 4区 生物学 Q2 MARINE & FRESHWATER BIOLOGY
Figen Özçay, Oya Balci Sezer, Faik Sarialioğlu, Fatih Boyvat, Mehmet Coşkun, Nihan Haberal Reyhan, Mehmet Haberal
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引用次数: 0

摘要

目的这是一项对肝硬化和肝细胞癌儿科患者进行肝移植的回顾性分析:2004年至2021年期间,14名患有慢性肝病和肝细胞癌的儿童患者接受了肝移植手术。先天性疾病包括酪氨酸血症(6 例)、进行性家族性肝内胆汁淤积 2 型(2 例)和 3 型(3 例)、隐源性肝硬化(2 例)、乙型和丁型肝炎(1 例)以及胆道闭锁(1 例):平均年龄为(9.43 ± 4.9)岁(13个月至16岁)。3名患者有1个肿瘤,4名患者有2个肿瘤,7名患者有多个(≥3个)病灶。部分患者在移植前(4例)或移植后(3例)接受了全身化疗,或在移植前接受了经动脉化疗栓塞术和微波消融术(1例)。3名患者(21%)分别在移植后23、47和108个月复发肝细胞癌。复发部位为网膜(1 例)和肝移植(2 例)。其中一名患者接受了肝切除、射频消融和放疗,另一名患者则因移植物肿瘤复发接受了射频消融和化疗。患者2年、4年和10年的无复发生存率分别为92%、82.5%和72.2%。四名受者(28.5%)死亡;移植后死亡原因分别是19个月(1例)和188个月(1例)时感染或79个月(1例)和165个月(1例)时肝细胞癌复发。中位随访时间为 178 个月(13-204 个月)。平均估计生存期为 171.25 ± 16.6 个月。患者移植后1年、2年、5年、10年和15年的总生存率分别为100%、92.3%、92.3%、83%和72%:结论:在我们的儿科病例中,肝细胞癌主要与遗传性肝病有关。肝移植为患有肝硬化和肝细胞癌的儿童患者提供了长期生存优势。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Seventeen Years of Pediatric Liver Transplantation Experience for Cirrhosis and Hepatocellular Carcinoma.

Objectives: This was a retrospective analysis of liver transplant for pediatric patients with liver cirrhosis and hepatocellular carcinoma.

Materials and methods: Fourteen pediatric patients with chronic liver disease and hepatocellular carcinoma underwent liver transplant from 2004 to 2021. Preexisting diseases were tyrosinemia (n = 6), progressive familial intrahepatic cholestasis type 2 (n = 2) and type 3 (n = 3), cryptogenic cirrhosis (n = 2), hepatitis B and D (n = 1), and biliary atresia (n = 1).

Results: Mean age was 9.43 ± 4.9 years (range, 13 months to 16 years). Three patients had 1 tumor, 4 had 2 tumors, and 7 had multiple (≥3) lesions. Six patients were classified as Pretreatment Extent of Disease Staging System for Hepatoblastoma (PRETEXT) stage IV, 3 as stage II, and 5 as stage I. Some patients received systemic chemotherapy before (n = 4) or after transplant (n = 3) or transarterial chemoembolization and microwave ablation pretransplant (n = 1). Hepatocellular carcinoma posttransplant recurrence was observed at 23, 47, and 108 months in 3 patients (21%). Recurrence sites were omentum (n = 1) and liver graft (n = 2). One patient was treated with hepatic resection, radiofrequency ablation, and radiotherapy, while the other received radiofrequency ablation and chemotherapy for graft tumor recurrence. Relapse-free patient survival rates were 92%, 82.5%, and 72.2% at 2, 4, and 10 years, respectively. Four recipients (28.5%) died; posttransplant cause of death was infection at 19 (n = 1) and 188 months (n = 1) or hepatocellular carcinoma recurrence at 79 (n = 1) and 165 months (n = 1). Median follow-up was 178 months (range, 13-204 months). Mean estimated survival was 171.25 ± 16.6 months. Overall patient posttransplant survival was 100%, 92.3%, 92.3%, 83%, and 72% at 1, 2, 5, 10, and 15 years, respectively.

Conclusions: Hepatocellular carcinoma was mainly associated with inherited liver diseases in our pediatric series. Liver transplant provided a long-term survival advantage to pediatric patients with preexisting cirrhosis and hepatocellular carcinoma.

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来源期刊
Journal of Molluscan Studies
Journal of Molluscan Studies 生物-动物学
CiteScore
3.00
自引率
8.30%
发文量
36
审稿时长
3 months
期刊介绍: The Journal of Molluscan Studies accepts papers on all aspects of the study of molluscs. These include systematics, molecular genetics, palaeontology, ecology, evolution, and physiology. Where the topic is in a specialized field (e.g. parasitology, neurobiology, biochemistry, molecular biology), submissions will still be accepted as long as the mollusc is the principal focus of the study, and not incidental or simply a convenient experimental animal. Papers with a focus on fisheries biology, aquaculture, and control of molluscan pests will be accepted only if they include significant advances in molluscan biology. While systematic papers are encouraged, descriptions of single new taxa will only be considered if they include some ‘added value’, for example in the form of new information on anatomy or distribution, or if they are presented in the context of a systematic revision or phylogenetic analysis of the group.
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