Liza Dalma Sümegi, Daniella Nagy, Ádám Vida, Gergely Varga, Emese Mihály, János Mihály Papp, Zoltán Pozsonyi, Ákos Iliás
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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.</p>","PeriodicalId":19911,"journal":{"name":"Orvosi hetilap","volume":"166 9","pages":"351-358"},"PeriodicalIF":0.8000,"publicationDate":"2025-03-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"[Gastrointestinal amyloidosis - retrospective analysis based on data of a center in Budapest].\",\"authors\":\"Liza Dalma Sümegi, Daniella Nagy, Ádám Vida, Gergely Varga, Emese Mihály, János Mihály Papp, Zoltán Pozsonyi, Ákos Iliás\",\"doi\":\"10.1556/650.2025.33238\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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. 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[Gastrointestinal amyloidosis - retrospective analysis based on data of a center in Budapest].
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.
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.