fontan手术后蛋白质丢失性肠病:来自越南临床经验的见解和全球合作的需要。

IF 0.9 4区 医学 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS
Cardiology in the Young Pub Date : 2025-05-01 Epub Date: 2025-04-14 DOI:10.1017/S1047951125001581
Dai Dac Tran, Tien Anh Do, Huy Quang Bui, Thanh Ngoc Le, Long Hoang Vo
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引用次数: 0

摘要

蛋白质丢失性肠病仍然是Fontan手术后一个可怕且未被充分认识的并发症,在受影响的患者中显著增加发病率和死亡率。尽管在治疗单心室冠心病方面取得了实质性进展,但蛋白质丢失性肠病仍然是一个主要的临床挑战,特别是在资源有限的环境中,缺乏专门的诊断方法。本文讨论了fontan后患者的患病率、临床表现、诊断障碍和蛋白质丢失性肠病的管理,借鉴了越南的临床经验。在95例fontan术后患者中,蛋白质丢失性肠病的患病率为4.2%,发病率很高,需要进行侵入性干预,在某些情况下导致死亡。诊断局限性,包括限制获得α -1抗胰蛋白酶清除试验,突出了迫切需要提高临床怀疑和实用的诊断方法。我们提倡将低成本、可广泛获得的筛查措施,如常规血清蛋白监测,纳入标准的fontan后护理方案,以加强蛋白质丢失性肠病的早期发现和管理。此外,我们强调全球合作和多中心研究计划的重要性,以解决蛋白质丧失性肠病的全球负担,促进资源共享,并开发基于证据的干预措施。协调一致的国际努力对于缩小诊断和治疗差距,最终改善Fontan循环患者的生存和生活质量至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Protein-losing enteropathy after fontan surgery: insights from Vietnamese clinical experience and the need for global collaboration.

Protein-losing enteropathy remains a formidable and underrecognised complication following Fontan surgery, contributing significantly to morbidity and mortality among affected patients. Despite substantial progress in managing single-ventricle CHDs, protein-losing enteropathy persists as a major clinical challenge, particularly in resource-constrained environments where specialised diagnostic modalities are scarce. This manuscript discusses the prevalence, clinical presentation, diagnostic hurdles, and management of protein-losing enteropathy in post-Fontan patients, drawing from clinical experiences in Vietnam. Among 95 post-Fontan patients reviewed, the prevalence of protein-losing enteropathy was 4.2%, with considerable morbidity necessitating invasive interventions and, in some cases, resulting in mortality. Diagnostic limitations, including restricted access to alpha-1-antitrypsin clearance testing, highlight the urgent need for heightened clinical suspicion and pragmatic diagnostic approaches. We advocate for integrating low-cost, widely accessible screening measures, such as routine serum protein monitoring, into standard post-Fontan care protocols to enhance early detection and management of protein-losing enteropathy. Furthermore, we underscore the critical importance of global collaboration and multicentre research initiatives to address the worldwide burden of protein-losing enteropathy, facilitate resource-sharing, and develop evidence-based interventions. Coordinated international efforts are essential to closing diagnostic and treatment gaps, ultimately improving survival and quality of life for patients living with the Fontan circulation.

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来源期刊
Cardiology in the Young
Cardiology in the Young 医学-小儿科
CiteScore
1.70
自引率
10.00%
发文量
715
审稿时长
4-8 weeks
期刊介绍: Cardiology in the Young is devoted to cardiovascular issues affecting the young, and the older patient suffering the sequels of congenital heart disease, or other cardiac diseases acquired in childhood. The journal serves the interests of all professionals concerned with these topics. By design, the journal is international and multidisciplinary in its approach, and members of the editorial board take an active role in the its mission, helping to make it the essential journal in paediatric cardiology. All aspects of paediatric cardiology are covered within the journal. The content includes original articles, brief reports, editorials, reviews, and papers devoted to continuing professional development.
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