胰腺动静脉畸形作为遗传性出血性毛细血管扩张症（Rendu-Osler-Weber 病）的一种表现：系统综述。
完整摘要 · 中文译文
目的：关于遗传性出血性毛细血管扩张症（HHT），即Rendu-Osler-Weber病患者的胰腺动静脉畸形（AVM）数据有限。本系统综述旨在识别所有已报道的HHT患者胰腺AVM病例，并总结患者人口学特征、临床表现、诊断方式、治疗策略及随访情况。 设计：按照系统综述和Meta分析优先报告条目（PRISMA）指南进行的系统综述。 数据来源：系统检索PubMed、Embase、Cochrane Library和Web of Science数据库，截至2025年6月。 纳入标准：所有报道HHT患者胰腺AVM的英文全文研究。仅纳入HHT和胰腺AVM确诊患者。 数据提取与综合：所有识别出的文章由两名评价者独立筛选。收集有关患者人口学特征、临床表现、诊断方式、治疗策略及随访的数据。当报告了个别AVM大小和AVM数量时，计算平均AVM大小。 结果：在429条筛选记录中，46篇文章进行了全文评估。来自13项研究的19例患者符合纳入标准。大多数患者为女性（58%），平均年龄52岁，无症状表现（50%），无实验室检查异常（73%）。CT是最常用的诊断方式（56%），平均AVM大小为7.1 mm。大多数患者接受保守治疗（69%）。Pr 预后通常无法获得（79%）。鉴别诊断主要为神经内分泌肿瘤（75%）。 结论：与一般人群相比，HHT患者的胰腺动静脉畸形似乎更小，且更常无症状。门静脉高压和胃肠道出血等严重并发症较少见。保守治疗最为常见。需要进一步研究以更好地阐明预后和治疗结局，尤其是在有症状的患者中。 PROSPERO注册号：CRD420251077109。
完整摘要 · 英文原文
OBJECTIVES: Limited data are available on pancreatic arteriovenous malformations (AVMs) in patients with hereditary haemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber disease. This systematic review aimed to identify all reported cases of pancreatic AVMs in patients with HHT and summarise patient demographics, clinical presentation, diagnostic modalities, treatment strategies and follow-up. DESIGN: Systematic review in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. DATA SOURCES: Databases PubMed, Embase, Cochrane Library and Web of Science were systematically screened up to June 2025. ELIGIBILITY CRITERIA: All English-language, full-text studies reporting pancreatic AVMs in patients with HHT. Only patients with definite diagnoses of HHT and pancreatic AVM were included. DATA EXTRACTION AND SYNTHESIS: All identified articles were independently screened by two reviewers. Data regarding patient demographics, clinical presentation, diagnostic modalities, treatment strategies and follow-up were collected. The mean AVM size was calculated when individual AVM size and number of AVMs were reported. RESULTS: Of 429 screened records, 46 articles underwent full-text assessment. Nineteen patients from 13 studies met the inclusion criteria. Most patients were female (58%), with a mean age of 52 years, presenting asymptomatically (50%) without laboratory findings (73%). CT was the most frequently used diagnostic modality (56%), and the mean AVM size was 7.1 mm. Most patients were treated conservatively (69%). Prognosis was frequently unavailable (79%). Differential diagnoses consisted mostly of neuroendocrine tumours (75%). CONCLUSIONS: Pancreatic AVMs in patients with HHT appear to be smaller and are more frequently asymptomatic compared with the general population. Severe complications, such as portal hypertension and gastrointestinal bleeding, were less frequent. Conservative management was most common. Additional studies are needed to better characterise prognosis and treatment outcomes, particularly in symptomatic patients. PROSPERO REGISTRATION NUMBER: CRD420251077109.