[Gastrointestinal amyloidosis - retrospective analysis based on data of a center in Budapest].

IF 0.8 4区 医学 Q3 MEDICINE, GENERAL & INTERNAL
Liza Dalma Sümegi, Daniella Nagy, Ádám Vida, Gergely Varga, Emese Mihály, János Mihály Papp, Zoltán Pozsonyi, Ákos Iliás
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引用次数: 0

Abstract

Introduction: In case of amyloidosis, an abnormal protein is formed which deposits and leads to dysfunction. Any organ can be affected. The most common digestive system symptoms are gastrointestinal dysmotility, dysphagia, satiety, gastroparesis, bleeding and constipation. Objective: Review of amyloidosis patients with gastrointestinal involvement treated at Semmelweis University, taking symptoms, endoscopic and histological results into account. Method: 15 amyloidosis patients with gastrointestinal involvement treated between 2009 and 2024 were collected using the hospital’s computerized registry system. Results: In 12 patients, amyloid light chain amyloidosis was confirmed, while transthyretin amyloidosis variant was described in 1 case, lysozyme amyloidosis in 1, and amyloid A amyloidosis in 1 patient. The median age at diagnosis and death was 65 years, reflecting the late-stage diagnosis, and the high Mayo-score (mean value: 2.67) predicted a poor prognosis for light chain amyloidosis. Other affected organs were heart (73.3%), kidneys (33.3%) and peripheral nerves (40%). The leading gastrointestinal symptoms were unintentional weight loss (20%), dysphagia (6.6%), hematochezia (6.6%), abdominal pain (6.6%), and diarrhea (6.6%). Common laboratory findings were anemia and chronic renal failure, while the most frequently described gastroscopic abnormalities were gastroduodenitis, gastroparesis, ulcers and polypoid formations. Discussion: A clear understanding of the mechanisms underlying gastrointestinal dysfunction is still lacking. Gastroparesis, diarrhea, polyneuropathy, and malabsorption can occur as a result of dysfunction resulting from amyloid deposition that damages blood vessels and nerves. Abnormalities seen during endoscopy may correspond to those seen in amyloidosis, and ulcers may be the source of hematochezia, occult bleeding, and anemia. Conclusion: Systemic amyloidosis is a disease with a poor prognosis that can affect many organs. The symptoms, laboratory parameters and endoscopic findings are varied, but not specific, so close cooperation of the allied professions is essential for the fastest diagnosis. Orv Hetil. 2025; 166(9): 351–358.

[胃肠道淀粉样变性-基于布达佩斯某中心数据的回顾性分析]。
在淀粉样变的情况下,会形成一种异常的蛋白质沉积并导致功能障碍。任何器官都可能受到影响。最常见的消化系统症状是胃肠动力障碍、吞咽困难、饱腹感、胃轻瘫、出血和便秘。目的:回顾Semmelweis大学治疗的淀粉样变性伴胃肠道病变患者,包括症状、内镜和组织学结果。方法:收集2009 ~ 2024年间收治的15例累及胃肠道的淀粉样变性患者。结果:12例确诊为淀粉样蛋白轻链淀粉样变,其中转甲状腺素淀粉样变变1例,溶菌酶淀粉样变变1例,淀粉样A淀粉样变1例。诊断和死亡的中位年龄为65岁,反映诊断晚期,高mayo评分(平均值2.67)预示轻链淀粉样变预后较差。其他受累器官为心脏(73.3%)、肾脏(33.3%)和周围神经(40%)。主要胃肠道症状为体重减轻(20%)、吞咽困难(6.6%)、便血(6.6%)、腹痛(6.6%)和腹泻(6.6%)。常见的实验室结果是贫血和慢性肾衰竭,而最常见的胃镜异常是胃十二指肠炎、胃轻瘫、溃疡和息肉形成。讨论:对胃肠功能障碍的机制仍缺乏清晰的认识。由于淀粉样蛋白沉积损害血管和神经而导致功能障碍,可发生胃轻瘫、腹泻、多神经病变和吸收不良。内窥镜检查所见的异常可能与淀粉样变所见的异常相对应,溃疡可能是便血、隐血和贫血的来源。结论:全身性淀粉样变性是一种预后较差的疾病,可累及多脏器。症状、实验室参数和内窥镜检查结果各不相同,但并不具体,因此相关专业的密切合作对于最快的诊断至关重要。Orv Hetil. 2025;166(9): 351 - 358。
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来源期刊
Orvosi hetilap
Orvosi hetilap MEDICINE, GENERAL & INTERNAL-
CiteScore
1.20
自引率
50.00%
发文量
274
期刊介绍: The journal publishes original and review papers in the fields of experimental and clinical medicine. It covers epidemiology, diagnostics, therapy and the prevention of human diseases as well as papers of medical history. Orvosi Hetilap is the oldest, still in-print, Hungarian publication and also the one-and-only weekly published scientific journal in Hungary. The strategy of the journal is based on the Curatorium of the Lajos Markusovszky Foundation and on the National and International Editorial Board. The 150 year-old journal is part of the Hungarian Cultural Heritage.
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