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Brown bowel syndrome: a systematic review. 褐色肠综合征:系统回顾。
IF 2.1 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-05-01 Epub Date: 2025-04-28 DOI: 10.20524/aog.2025.0965
Rena Hm Cao, Jason Diab, Michael C Grimm, Christophe R Berney

Brown bowel syndrome (BBS) is a rare disorder characterized by brown pigmentation of the intestinal wall, thought to be a consequence of lipofuscin accumulation. Celiac disease and vitamin E deficiency have been postulated to be risk factors. We systematically searched PubMed, Embase, Web of Science and Cochrane to identify all case reports and abstracts reporting clinical information on patients with a confirmed diagnosis of BBS. Forty-two studies met our inclusion criteria, including 63 patients with confirmed BBS. The most common symptoms of BBS were diarrhea (50.8%) and malnutrition (50.8%), followed by abdominal pain (39.7%) and vomiting (22.2%). BBS patients with celiac disease who presented with similar symptoms to non-celiac patients were significantly less likely to be hypoalbuminemic (15.4 vs. 45.5%) and showed a non-significant trend towards a higher mortality rate (36.4% vs. 15.4%). Nineteen (31.7%) BBS patients were also vitamin E deficient. The clinical presentation and outcomes in BBS patients with vitamin E deficiency and celiac disease were similar to those without vitamin E deficiency and celiac disease. Further studies are warranted to better define the diagnostic-therapeutic approach to patients with BBS.

棕色肠综合征(BBS)是一种罕见的疾病,以肠壁棕色色素沉着为特征,被认为是脂褐素积累的结果。乳糜泻和维生素E缺乏被认为是危险因素。我们系统地检索PubMed、Embase、Web of Science和Cochrane,以确定所有报告BBS确诊患者临床信息的病例报告和摘要。42项研究符合我们的纳入标准,包括63例确诊的BBS患者。BBS最常见的症状是腹泻(50.8%)和营养不良(50.8%),其次是腹痛(39.7%)和呕吐(22.2%)。伴有乳糜泻的BBS患者如果表现出与非乳糜泻患者相似的症状,低白蛋白血症的可能性显著降低(15.4%对45.5%),且死亡率呈非显著性升高趋势(36.4%对15.4%)。19例(31.7%)BBS患者同时缺乏维生素E。伴有维生素E缺乏和乳糜泻的BBS患者的临床表现和结果与没有维生素E缺乏和乳糜泻的患者相似。需要进一步的研究来更好地确定BBS患者的诊断和治疗方法。
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引用次数: 0
Induction with upadacitinib in Crohn's disease: real-world experience from an early-access program in Greece. upadacitinib诱导治疗克罗恩病:来自希腊早期准入项目的真实世界经验
IF 2.1 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-05-01 Epub Date: 2025-04-28 DOI: 10.20524/aog.2025.0969
Evgenia Papathanasiou, Alexandros Ioannou, Pavlos Pardalis, Giorgos Leonidakis, George Michalopoulos, Spilios Manolakopoulos, Spyridon Siakavellas, Angeliki Theodoropoulou, Athanasia Tasovasili, Olga Giouleme, Maria Tzouvala, Eftychia Tsironi, Nikos Viazis, Spyridon Michopoulos, Evanthia Zampeli

Background: Upadacitinib is a selective Janus kinase-1 inhibitor, approved for the management of Crohn's disease (CD) by the United States Food & Drug Administration. In Greece, upadacitinib was initially available through an early-access program. Our goal was to describe the real practice experience.

Methods: This was a multicenter retrospective cohort study of patients with moderate-to-severe CD. The primary endpoint was clinical response, defined as a reduction ≥3 in the Harvey-Bradshaw index. Secondary endpoints included biochemical improvement. Outcomes were assessed at 4, 8 and 12 weeks.

Results: A total of 24 CD patients received upadacitinib and were included in the analysis. Their mean age was 42.2 years (range 24-63). Eleven patients (45.8%) had ileocolonic CD and 5 (20.8%) CD colitis. Fourteen patients had active extraintestinal manifestations. The majority of patients (19/24) had ≥3 failed biologics. All of them had failed treatment with anti-tumor necrosis factor and 19 (79%) with ustekinumab. At 12 weeks, nearly all patients achieved a clinical response (85%). Of 13 patients with C-reactive protein >5 mg/L at baseline, 11 (84.6%) achieved normalization by week 8. Adverse events occurred in 3 patients (14.2%).

Conclusion: In a small cohort of resistant CD patients, the short-term clinical efficacy of upadacitinib was high.

背景:Upadacitinib是一种选择性Janus激酶-1抑制剂,已被美国食品和药物管理局批准用于克罗恩病(CD)的治疗。在希腊,upadacitinib最初是通过早期获取计划获得的。我们的目标是描述真实的实践经验。方法:这是一项针对中重度CD患者的多中心回顾性队列研究。主要终点是临床反应,定义为Harvey-Bradshaw指数降低≥3。次要终点包括生化改善。在4周、8周和12周时评估结果。结果:共有24例CD患者接受了upadacitinib治疗,并被纳入分析。平均年龄42.2岁(24-63岁)。11例(45.8%)有回结肠CD, 5例(20.8%)有CD结肠炎。14例患者有活跃的肠外表现。大多数患者(19/24)有≥3种失败的生物制剂。抗肿瘤坏死因子治疗全部失败,ustekinumab治疗19例(79%)。在12周时,几乎所有患者(85%)都获得了临床缓解。在13例基线时c -反应蛋白bbb5 mg/L的患者中,11例(84.6%)在第8周达到正常化。不良事件3例(14.2%)。结论:在一小部分耐药乳糜泻患者中,upadacitinib的短期临床疗效较高。
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引用次数: 0
Incidence of ileus and associated factors in patients with acute pancreatitis: a nationwide analysis. 急性胰腺炎患者肠梗阻发生率及相关因素:一项全国性分析。
IF 2.1 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-05-01 Epub Date: 2025-04-17 DOI: 10.20524/aog.2025.0957
Anmol Singh, Ritika Dhruve, Carol Singh, Vikash Kumar, Aalam Sohal, Divyesh Sejpal

Background: Ileus is a well-known complication of acute pancreatitis (AP). There are limited data on the factors associated with ileus, as well as its impact on AP patients. We aimed to investigate the incidence and clinical predictors of ileus in hospitalized AP patients.

Methods: We queried the 2016-2019 National Inpatient Sample (NIS) database using the International Classification of Diseases (ICD)-10 codes. Adult patients diagnosed with AP (ICD-10 K85) were included, excluding those with chronic pancreatitis. Demographics, comorbidities, complications and interventions were stratified by the presence of ileus. Multivariate analysis identified factors associated with ileus, adjusting for patient and hospital characteristics, comorbidities, and pancreatitis complications.

Results: Among 1,386,390 AP patients, 50,170 (3.6%) developed ileus. Female sex was associated with a lower risk (adjusted odds ratio [aOR] 0.56, 95% confidence interval [CI] 0.53-0.58; P<0.001). Hispanic patients had the lowest risk (aOR 0.82, 95%CI 0.76-0.88), while older age groups had a higher risk. Pseudocysts (P<0.001), sepsis (P<0.001) and portal vein thrombosis (P<0.001) were significant predictors. Pancreatic drainage was associated with ileus (P=0.007), but endoscopic retrograde cholangiopancreatography was not. Patients with ileus had greater mortality (P<0.001), longer hospital stays (+4.9 days, P<0.001), and higher costs ($67,855.91, P<0.001).

Conclusions: This study highlights age, sex and racial disparities in the development of ileus in patients with AP. It also reveals a significant association of ileus with pseudocysts, portal vein thrombosis, and pancreatic drainage. Early recognition and timely enteral feeding are crucial to prevent disease progression and improve outcomes.

背景:肠梗阻是众所周知的急性胰腺炎(AP)并发症。与肠梗阻相关的因素及其对AP患者的影响的数据有限。我们的目的是调查住院AP患者肠梗阻的发生率和临床预测因素。方法:使用国际疾病分类(ICD)-10代码查询2016-2019年国家住院患者样本(NIS)数据库。纳入诊断为AP (ICD-10 K85)的成年患者,不包括慢性胰腺炎患者。人口统计学、合并症、并发症和干预措施根据肠梗阻的存在进行分层。多变量分析确定了与肠梗阻相关的因素,调整了患者和医院的特征、合并症和胰腺炎并发症。结果:在1,386,390例AP患者中,50,170例(3.6%)发生肠梗阻。女性与较低的风险相关(调整优势比[aOR] 0.56, 95%可信区间[CI] 0.53-0.58;结论:本研究强调了AP患者肠梗阻发展的年龄、性别和种族差异。它还揭示了肠梗阻与假性囊肿、门静脉血栓形成和胰腺引流的显著关联。早期识别和及时肠内喂养对于预防疾病进展和改善预后至关重要。
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引用次数: 0
Efficacy and safety of esophageal stenting for esophageal perforation: a systematic review and meta-analysis. 食管支架置入术治疗食管穿孔的疗效和安全性:系统回顾和荟萃分析。
IF 2.1 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-03-01 Epub Date: 2025-02-28 DOI: 10.20524/aog.2025.0943
Adnan Malik, Muhammad Imran Malik, Sadia Javaid, Shahbaz Qureshi, Aboud Kaliounji, Abdul Nadir, Douglas G Adler

Background: Esophageal perforations are managed with endoscopic stenting. However, surgical repair is still employed in many centers, if they lack endoscopic services, or for complex perforations.

Methods: We searched PubMed, Scopus, and Web of Science for relevant clinical trials and observational studies. Quality assessment was evaluated according to GRADE. The studies included were assessed based on the National Heart, Lung, and Blood Institute criteria. We included the following outcomes: leak after primary repair, operative repair after endoscopic therapy, stent migration, length of hospital stay (days), and mortality. We analyzed continuous data using mean differences and 95% confidence intervals (CI), while dichotomous data were analyzed using odds ratios and 95%CI. Statistical heterogeneity was assessed using the I 2 statistic.

Results: Eight studies were analyzed and found to include 95 patients with esophageal perforation. Mortality rates decreased over time from 16.3% (Abbas, 2009) to 6.7% (Heel, 2020). Re-operative procedures were highest at 51.4%(D'Cunha, 2011) and lower in later studies. Stent migration rates varied from 16.2-22.3%. Leakage rates ranged from 8.8-16.2%. Hospital stays ranged from 5.0 days (D'Cunha, 2011) to 15.3 days (Law, 2017), with significant variability across studies.

Conclusion: Esophageal stenting is considered an efficient and well-tolerated method for managing esophageal perforation.

背景:食管穿孔是通过内镜支架置入治疗的。然而,手术修复仍然在许多中心,如果他们缺乏内窥镜服务,或复杂的穿孔。方法:检索PubMed、Scopus和Web of Science相关临床试验和观察性研究。质量评价按GRADE进行评价。纳入的研究是根据国家心脏、肺和血液研究所的标准进行评估的。我们纳入了以下结果:初次修复后的渗漏、内镜治疗后的手术修复、支架迁移、住院时间(天)和死亡率。我们使用平均差异和95%置信区间(CI)分析连续数据,而使用优势比和95%CI分析二分类数据。使用i2统计量评估统计异质性。结果:对8项研究进行分析,发现95例食管穿孔患者。随着时间的推移,死亡率从16.3% (Abbas, 2009年)降至6.7% (Heel, 2020年)。再手术率最高,为51.4%(D’cunha, 2011),后期研究中比例更低。支架迁移率从16.2-22.3%不等。泄漏率为8.8-16.2%。住院时间从5.0天(D’cunha, 2011年)到15.3天(Law, 2017年)不等,各研究之间存在显著差异。结论:食管支架置入术是治疗食管穿孔的一种有效且耐受性良好的方法。
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引用次数: 0
Efficacy and safety of full-thickness versus circular peroral endoscopic myotomy for treatment of achalasia: a systematic review and meta-analysis. 全层与环形经口内窥镜下肌切开术治疗贲门失弛缓症的疗效和安全性:一项系统综述和荟萃分析。
IF 2.1 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-03-01 Epub Date: 2025-02-25 DOI: 10.20524/aog.2025.0946
Sudheer Dhoop, Mohammed Abu-Rumaileh, Wasef Sayeh, Sami Ghazaleh, Conner Lombardi, Manthanbhai Patel, Bisher Sawaf, Wade Lee-Smith, Adrian Zhou, Ali Nawras, Yaseen Alastal

Background: Peroral endoscopic myotomy (POEM) is a treatment for esophageal achalasia with 2 variations in myotomy depth: full-thickness myotomy (FTM) and circular myotomy (CM). This systematic review and meta-analysis compares the efficacy and safety of these variations.

Methods: Major health databases and registers, including Embase, MEDLINE and Cochrane were searched systematically. The primary outcome was clinical success, while secondary outcomes included change in achalasia severity scores, post-POEM gastroesophageal reflux disease (GERD) measures, procedural time, and adverse events. Meta-analysis was conducted using random-effects models, with risk ratios (RR) and mean differences (MD) calculated for dichotomous and continuous variables, respectively.

Results: Nine observational studies compared FTM and CM in 1,203 patients. FTM was performed in more severe achalasia and demonstrated similar clinical success to CM (RR 1.01, 95% confidence interval [CI] 0.98-1.04; P=0.55; n=6) and procedural time (MD 3.49 min, 95%CI -2.79-9.78; P=0.28, I 2=66%; n=3). FTM was associated with increased post-POEM GERD outcomes, post-POEM pain (RR 1.94, 95%CI 1.27-2.95; P=0.002; n=2), and length of stay (LOS) (MD 0.85 days, 95%CI 0.11-1.59; P=0.02; I 2=0%; n=2); however, association with esophagitis disappeared when proton pump inhibitors use was accounted for (RR 1.68, 95%CI 0.89-3.16; P=0.11; I 2=23%; n=4). CM was associated with higher rates of subcutaneous emphysema (RR 0.59, 95%CI 0.43-0.81; P=0.001; n=5).

Conclusions: FTM and CM have comparable observed clinical efficacy and procedural time, with minimal differences in complications. FTM may be preferred in more severe achalasia and its association with post-POEM GERD may have been overestimated, but it may increase post-POEM pain and LOS.

背景:经口内窥镜下肌切开术(POEM)是一种治疗食管贲门失弛缓症的方法,有两种不同的肌切开术深度:全层肌切开术(FTM)和环形肌切开术(CM)。本系统综述和荟萃分析比较了这些变异的疗效和安全性。方法:系统检索Embase、MEDLINE、Cochrane等主要卫生数据库和注册库。主要结局是临床成功,次要结局包括贲门失弛缓症严重程度评分、poem后胃食管反流病(GERD)测量、手术时间和不良事件的变化。采用随机效应模型进行meta分析,分别计算二分类变量和连续变量的风险比(RR)和平均差异(MD)。结果:9项观察性研究比较了1203例患者的FTM和CM。在更严重的贲门失弛缓症中进行FTM,其临床成功率与CM相似(RR 1.01, 95%可信区间[CI] 0.98-1.04;P = 0.55;n=6)和手术时间(MD 3.49 min, 95%CI -2.79 ~ 9.78;P=0.28, i2 =66%;n = 3)。FTM与poem后胃食管反流结局、poem后疼痛增加相关(RR 1.94, 95%CI 1.27-2.95;P = 0.002;n=2),住院时间(LOS) (MD 0.85天,95%CI 0.11-1.59;P = 0.02;我2 = 0%;n = 2);然而,当使用质子泵抑制剂时,与食管炎的相关性消失(RR 1.68, 95%CI 0.89-3.16;P = 0.11;我2 = 23%;n = 4)。CM与较高的皮下肺气肿发生率相关(RR 0.59, 95%CI 0.43-0.81;P = 0.001;n = 5)。结论:FTM和CM具有相当的临床疗效和手术时间,并发症差异极小。在更严重的失弛缓症中,FTM可能是首选,它与诗后反流的关联可能被高估了,但它可能增加诗后疼痛和LOS。
{"title":"Efficacy and safety of full-thickness versus circular peroral endoscopic myotomy for treatment of achalasia: a systematic review and meta-analysis.","authors":"Sudheer Dhoop, Mohammed Abu-Rumaileh, Wasef Sayeh, Sami Ghazaleh, Conner Lombardi, Manthanbhai Patel, Bisher Sawaf, Wade Lee-Smith, Adrian Zhou, Ali Nawras, Yaseen Alastal","doi":"10.20524/aog.2025.0946","DOIUrl":"10.20524/aog.2025.0946","url":null,"abstract":"<p><strong>Background: </strong>Peroral endoscopic myotomy (POEM) is a treatment for esophageal achalasia with 2 variations in myotomy depth: full-thickness myotomy (FTM) and circular myotomy (CM). This systematic review and meta-analysis compares the efficacy and safety of these variations.</p><p><strong>Methods: </strong>Major health databases and registers, including Embase, MEDLINE and Cochrane were searched systematically. The primary outcome was clinical success, while secondary outcomes included change in achalasia severity scores, post-POEM gastroesophageal reflux disease (GERD) measures, procedural time, and adverse events. Meta-analysis was conducted using random-effects models, with risk ratios (RR) and mean differences (MD) calculated for dichotomous and continuous variables, respectively.</p><p><strong>Results: </strong>Nine observational studies compared FTM and CM in 1,203 patients. FTM was performed in more severe achalasia and demonstrated similar clinical success to CM (RR 1.01, 95% confidence interval [CI] 0.98-1.04; P=0.55; n=6) and procedural time (MD 3.49 min, 95%CI -2.79-9.78; P=0.28, <i>I</i> <sup>2</sup>=66%; n=3). FTM was associated with increased post-POEM GERD outcomes, post-POEM pain (RR 1.94, 95%CI 1.27-2.95; P=0.002; n=2), and length of stay (LOS) (MD 0.85 days, 95%CI 0.11-1.59; P=0.02; <i>I</i> <sup>2</sup>=0%; n=2); however, association with esophagitis disappeared when proton pump inhibitors use was accounted for (RR 1.68, 95%CI 0.89-3.16; P=0.11; <i>I</i> <sup>2</sup>=23%; n=4). CM was associated with higher rates of subcutaneous emphysema (RR 0.59, 95%CI 0.43-0.81; P=0.001; n=5).</p><p><strong>Conclusions: </strong>FTM and CM have comparable observed clinical efficacy and procedural time, with minimal differences in complications. FTM may be preferred in more severe achalasia and its association with post-POEM GERD may have been overestimated, but it may increase post-POEM pain and LOS.</p>","PeriodicalId":7978,"journal":{"name":"Annals of Gastroenterology","volume":"38 2","pages":"143-155"},"PeriodicalIF":2.1,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11928898/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143690993","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Effective endoscopic management of gastric neoplastic complications in patients with autoimmune gastritis: results of a monocentric study of 88 patients. 自身免疫性胃炎患者胃肿瘤并发症的有效内镜治疗:88例患者的单中心研究结果
IF 2.1 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-03-01 Epub Date: 2025-02-25 DOI: 10.20524/aog.2025.0947
Solène Hoibian, Jean-Philippe Ratone, Alexey Solovyev, Yanis Dahel, Emmanuel Mitry, Flora Poizat, Jerome Guiramand, Fabrice Caillol, Marc Giovannini

Background: We evaluated the efficacy of endoscopic treatment (ET) for gastric neoplastic complications of autoimmune gastritis (AIG). We also assessed the safety of ET and the risk factors for the occurrence of neuroendocrine tumors (NETs) and gastric adenocarcinoma (GA).

Methods: This was a retrospective, single-center, observational study. All patients diagnosed with AIG between 1987 and 2019 and had at least 1 upper endoscopy available were included.

Results: The study population comprised 88 patients (68.2% female). The median follow up was 5 years (range 1-28). A total of 132 NETs were diagnosed in 39/88 patients (44.3%) (median age 50.0 years, range 27.0-85.0 years). The mean lesion size was 7.1 mm (range 1-30); there were 80 G1 NETs and 52 G2 NETs. Among the 132 lesions, 86.3% (114/132) were endoscopically resected, mostly by endoscopic mucosal resection (105/114, 92.1%), without complications. Only 1 patient underwent surgery. Twelve patients (13.6%) (7 females; median age, 76.0 years; range, 53.0-90.0 years) presented with GA. Of these, 66.7% (8/12) needed surgery, while 4 patients underwent exclusive endoscopic resection. Only 2 patients presented with NETs and GA (2.8%). Patients who presented with NETs were significantly younger at AIG diagnosis than patients with GA: 52.0 (18.0-85.0) vs. 67.0 (44.0-81.0) years (P=0.008). Patients who presented with GA were significantly older than those who presented with NETs: 76.0 (53.0-90.0) vs. 50.0 (27.0-85.0) years (P<0.001).

Conclusion: ET of NETs for AIG is effective and safe. GA is rarer, occurs in significantly older patients, and usually requires surgery.

背景:我们评估内镜治疗(ET)对自身免疫性胃炎(AIG)胃肿瘤并发症的疗效。我们还评估了ET的安全性以及发生神经内分泌肿瘤(NETs)和胃腺癌(GA)的危险因素。方法:回顾性、单中心、观察性研究。所有1987年至2019年间诊断为AIG且至少有1次上颌内窥镜检查的患者均被纳入研究。结果:研究人群包括88例患者(68.2%为女性)。中位随访时间为5年(1-28年)。88例患者中有39例(44.3%)诊断出132例NETs(中位年龄50.0岁,范围27.0-85.0岁)。平均病变大小为7.1 mm(范围1-30);G1 NETs 80支,G2 NETs 52支。132例病变中,86.3%(114/132)经内镜切除,以内镜粘膜切除居多(105/114,92.1%),无并发症发生。只有1例患者接受了手术。12例(13.6%),其中女性7例;中位年龄76.0岁;年龄范围为53.0 ~ 90.0岁)。其中66.7%(8/12)需要手术治疗,4例患者行内镜切除。只有2例患者出现NETs和GA(2.8%)。出现NETs的患者在AIG诊断时明显比GA患者年轻:52.0(18.0-85.0)比67.0(44.0-81.0)岁(P=0.008)。表现为GA的患者明显比表现为NETs的患者年龄大:76.0(53.0-90.0)比50.0(27.0-85.0)岁。GA是罕见的,发生在明显老年患者,通常需要手术。
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引用次数: 0
Hepatocellular carcinoma in Fontan-associated liver disease. 丰坦相关肝病中的肝细胞癌
IF 2.1 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-03-01 Epub Date: 2025-02-26 DOI: 10.20524/aog.2025.0948
Joanne D'Amato, Eliezer Zahra Bianco, Jade Camilleri, Emma Debattista, Pierre Ellul

Fontan-associated liver disease (FALD) is a significant complication in patients with Fontan palliation. The improved longevity following Fontan palliation has led to wider recognition of FALD and its association with hepatocellular carcinoma (HCC). This review examines the intricate link between FALD and HCC development, emphasizing the unique hemodynamic changes in Fontan circulation that promote hepatic congestion, fibrosis and cirrhosis, thereby facilitating carcinogenesis. The review comprehensively analyzes the existing literature, highlighting key risk factors, pathophysiological mechanisms, and diagnostic challenges in FALD-related HCC. While HCC incidence in FALD remains relatively low (1.5-5.0%), its higher mortality rate of 29.4% necessitates a thorough understanding of contributing factors and screening requirements. The management of FALD involves multidisciplinary approaches, addressing cardiac and hepatic aspects, with regular surveillance for liver disease progression and HCC using advanced imaging and biomarkers. Therapeutic considerations include interventions to manage hepatic congestion and fibrosis, although balancing these with the unique cardiac needs of Fontan circulation remains challenging. Interestingly, FALD management often mirrors that of other liver diseases, underscoring the need for tailored approaches. In severe cases, combined heart-liver transplantation offers a comprehensive solution for FALD-HCC. This review consolidates current knowledge on the epidemiology, pathogenesis and comprehensive management of HCC in the specific context of FALD, ultimately improving outcomes for this unique patient population.

丰坦相关肝病(FALD)是丰坦姑息治疗患者的一个重要并发症。Fontan姑息治疗后寿命的延长使人们更广泛地认识到FALD及其与肝细胞癌(HCC)的关系。本综述探讨了FALD与HCC发展之间的复杂联系,强调Fontan循环中独特的血流动力学变化可促进肝脏充血、纤维化和肝硬化,从而促进癌变。本文综合分析了现有文献,强调了fld相关HCC的关键危险因素、病理生理机制和诊断挑战。虽然FALD的HCC发病率仍然相对较低(1.5-5.0%),但其29.4%的高死亡率需要彻底了解影响因素和筛查要求。FALD的治疗涉及多学科方法,涉及心脏和肝脏方面,并使用先进的成像和生物标志物定期监测肝脏疾病进展和HCC。治疗方面的考虑包括干预以控制肝充血和纤维化,尽管平衡这些与Fontan循环独特的心脏需求仍然具有挑战性。有趣的是,FALD的管理往往反映了其他肝脏疾病,强调需要量身定制的方法。在重症病例中,心脏-肝联合移植为FALD-HCC提供了全面的解决方案。这篇综述巩固了目前关于肝细胞癌流行病学、发病机制和在FALD特定背景下的综合治疗的知识,最终改善了这一独特患者群体的预后。
{"title":"Hepatocellular carcinoma in Fontan-associated liver disease.","authors":"Joanne D'Amato, Eliezer Zahra Bianco, Jade Camilleri, Emma Debattista, Pierre Ellul","doi":"10.20524/aog.2025.0948","DOIUrl":"10.20524/aog.2025.0948","url":null,"abstract":"<p><p>Fontan-associated liver disease (FALD) is a significant complication in patients with Fontan palliation. The improved longevity following Fontan palliation has led to wider recognition of FALD and its association with hepatocellular carcinoma (HCC). This review examines the intricate link between FALD and HCC development, emphasizing the unique hemodynamic changes in Fontan circulation that promote hepatic congestion, fibrosis and cirrhosis, thereby facilitating carcinogenesis. The review comprehensively analyzes the existing literature, highlighting key risk factors, pathophysiological mechanisms, and diagnostic challenges in FALD-related HCC. While HCC incidence in FALD remains relatively low (1.5-5.0%), its higher mortality rate of 29.4% necessitates a thorough understanding of contributing factors and screening requirements. The management of FALD involves multidisciplinary approaches, addressing cardiac and hepatic aspects, with regular surveillance for liver disease progression and HCC using advanced imaging and biomarkers. Therapeutic considerations include interventions to manage hepatic congestion and fibrosis, although balancing these with the unique cardiac needs of Fontan circulation remains challenging. Interestingly, FALD management often mirrors that of other liver diseases, underscoring the need for tailored approaches. In severe cases, combined heart-liver transplantation offers a comprehensive solution for FALD-HCC. This review consolidates current knowledge on the epidemiology, pathogenesis and comprehensive management of HCC in the specific context of FALD, ultimately improving outcomes for this unique patient population.</p>","PeriodicalId":7978,"journal":{"name":"Annals of Gastroenterology","volume":"38 2","pages":"133-142"},"PeriodicalIF":2.1,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11928897/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143691031","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Patients' and gastroenterologists' preferences regarding outcomes and medication attributes in ulcerative colitis. 溃疡性结肠炎患者和胃肠病学家对预后和药物属性的偏好。
IF 2.1 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-03-01 Epub Date: 2025-02-25 DOI: 10.20524/aog.2025.0944
Anastasia Katsoula, Olga Giouleme, Paschalis Paschos, Maria Toumpalidou, Maria Mainou, Konstantinos Malandris, Anna-Bettina Haidich, Apostolos Tsapas

Background: Patients' and gastroenterologists' views on the relative importance of treatment outcomes and medication attributes for ulcerative colitis (UC) may differ. We aimed to explore which treatment outcomes and medication attributes are considered important by both for therapeutic decisions.

Methods: Eight gastroenterologists and 23 patients with UC in Greece participated in semi-structured interviews and focus groups, respectively. The focus groups and interviews were audio-recorded, transcribed and coded, utilizing thematic analysis until data saturation was achieved.

Results: Themes that were discussed included the impact of UC on daily life, UC-related outcomes, drug-related attributes and the patient-doctor relationship. Within these themes, disparities between the perspectives of gastroenterologists and patients were evident on 2 main issues. Gastroenterologists prioritized clinical remission and emphasized long-term objectives, such as mucosal healing, while patients focused on shorter-term outcomes, such as the early and sustained relief of symptoms. Regarding medication attributes, important factors for patients were primarily those that impacted their daily life, such as route of administration, dosage and the need for hospital visits. In contrast, gastroenterologists were more concerned about potential adverse events and non-responsiveness to treatment. There was a consensus regarding the importance of shared decision-making for UC management, emphasized by both patients and clinicians.

Conclusions: Gastroenterologists mostly prioritize objective measures of remission, while patients mainly focus on factors related to their quality of life and overall well-being. Enhancing communication regarding different goals and expectations may strengthen the physician-patient relationship, ultimately resulting in better shared therapeutic decision-making.

背景:患者和胃肠病学家对溃疡性结肠炎(UC)治疗结果和药物属性的相对重要性的看法可能不同。我们的目的是探讨哪些治疗结果和药物属性对治疗决策都是重要的。方法:希腊8名胃肠病学家和23名UC患者分别参加了半结构化访谈和焦点小组。焦点小组和访谈录音、转录和编码,利用专题分析,直到达到数据饱和。结果:讨论的主题包括UC对日常生活的影响、UC相关结果、药物相关属性和医患关系。在这些主题中,胃肠病学家和患者的观点在两个主要问题上存在明显差异。胃肠病学家优先考虑临床缓解并强调长期目标,如粘膜愈合,而患者则关注短期结果,如早期和持续缓解症状。在药物属性方面,患者的重要因素主要是那些影响其日常生活的因素,如给药途径、剂量和就诊需要。相比之下,胃肠病学家更关注潜在的不良事件和对治疗无反应。患者和临床医生都强调了共同决策对UC管理的重要性,这是一个共识。结论:胃肠病学家大多优先考虑缓解的客观措施,而患者主要关注与他们的生活质量和整体健康相关的因素。就不同的目标和期望加强沟通可以加强医患关系,最终产生更好的共同治疗决策。
{"title":"Patients' and gastroenterologists' preferences regarding outcomes and medication attributes in ulcerative colitis.","authors":"Anastasia Katsoula, Olga Giouleme, Paschalis Paschos, Maria Toumpalidou, Maria Mainou, Konstantinos Malandris, Anna-Bettina Haidich, Apostolos Tsapas","doi":"10.20524/aog.2025.0944","DOIUrl":"10.20524/aog.2025.0944","url":null,"abstract":"<p><strong>Background: </strong>Patients' and gastroenterologists' views on the relative importance of treatment outcomes and medication attributes for ulcerative colitis (UC) may differ. We aimed to explore which treatment outcomes and medication attributes are considered important by both for therapeutic decisions.</p><p><strong>Methods: </strong>Eight gastroenterologists and 23 patients with UC in Greece participated in semi-structured interviews and focus groups, respectively. The focus groups and interviews were audio-recorded, transcribed and coded, utilizing thematic analysis until data saturation was achieved.</p><p><strong>Results: </strong>Themes that were discussed included the impact of UC on daily life, UC-related outcomes, drug-related attributes and the patient-doctor relationship. Within these themes, disparities between the perspectives of gastroenterologists and patients were evident on 2 main issues. Gastroenterologists prioritized clinical remission and emphasized long-term objectives, such as mucosal healing, while patients focused on shorter-term outcomes, such as the early and sustained relief of symptoms. Regarding medication attributes, important factors for patients were primarily those that impacted their daily life, such as route of administration, dosage and the need for hospital visits. In contrast, gastroenterologists were more concerned about potential adverse events and non-responsiveness to treatment. There was a consensus regarding the importance of shared decision-making for UC management, emphasized by both patients and clinicians.</p><p><strong>Conclusions: </strong>Gastroenterologists mostly prioritize objective measures of remission, while patients mainly focus on factors related to their quality of life and overall well-being. Enhancing communication regarding different goals and expectations may strengthen the physician-patient relationship, ultimately resulting in better shared therapeutic decision-making.</p>","PeriodicalId":7978,"journal":{"name":"Annals of Gastroenterology","volume":"38 2","pages":"174-181"},"PeriodicalIF":2.1,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11928895/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143691033","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Primary biliary cholangitis: a summary of pathogenesis and therapies. 原发性胆道胆管炎:发病机理及治疗综述。
IF 2.1 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-03-01 Epub Date: 2025-02-28 DOI: 10.20524/aog.2025.0953
Imen Jallouli, Michael Doulberis, Jannis Kountouras

Primary biliary cholangitis (PBC) is a progressive autoimmune liver disease characterized by chronic inflammation and destruction of interlobular bile ducts. Its pathogenesis involves a complex interplay of genetic predisposition, environmental triggers, and immune-mediated mechanisms, particularly T-helper cell activity, leading to bile duct damage. First-line therapy includes ursodeoxycholic acid (UDCA), which improves liver biochemistry and slows disease progression, with obeticholic acid (OCA) as an option for non-responders. Double and/or triple therapy, including UDCA, OCA, and fibrates, appears to be superior in achieving therapeutic benefits in UDCA-nonresponsive PBC patients. Emerging therapies, such as peroxisome proliferator-activated receptor-α agonists, biologics such as dacetuzumab and rituximab, and experimental approaches such as stem-cell therapy, offer promising advances in managing PBC. Liver transplantation remains a final treatment option for advanced cases.

原发性胆管炎(PBC)是一种进行性自身免疫性肝病,以慢性炎症和小叶间胆管破坏为特征。其发病机制涉及遗传易感性、环境触发和免疫介导机制的复杂相互作用,特别是t辅助细胞活性,导致胆管损伤。一线治疗包括熊去氧胆酸(UDCA),它可以改善肝脏生物化学并减缓疾病进展,奥比胆酸(OCA)是无反应的一种选择。双联和/或三联治疗,包括UDCA、OCA和贝特类,在UDCA无反应的PBC患者中似乎更能获得治疗效果。新兴疗法,如过氧化物酶体增殖激活受体-α激动剂,生物制剂如达妥珠单抗和利妥昔单抗,以及实验性方法如干细胞治疗,在治疗PBC方面提供了有希望的进展。肝移植仍然是晚期病例的最后治疗选择。
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引用次数: 0
Age of onset of inflammatory bowel disease is the strongest risk factor for the development of malignancy. 炎症性肠病的发病年龄是恶性肿瘤发生的最大风险因素。
IF 2.1 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-03-01 Epub Date: 2025-02-28 DOI: 10.20524/aog.2025.0952
Nicole Sciberras, Lara Miruzzi, Luke Bugeja, Adrienne Gatt, Suzanne Cauchi, Zane Attard, Pierre Ellul, Stefania Chetcuti Zammit

Background: Inflammatory bowel disease (IBD), which includes Crohn's disease and ulcerative colitis, is a multifactorial inflammatory disorder of the gastrointestinal system that impairs the patient's quality of life. Its presentation includes a spectrum of symptoms that may also be secondary to IBD complications, such as malignancy. On the other hand, immunosuppressive treatment to maintain remission also carries a risk of malignancy, which can cause patients distress due to the risk/benefit balance of IBD control and malignancy.

Methods: In this nationwide retrospective study, we aimed to elucidate which patient and treatment factors have the greatest impact on the development of malignancy in IBD patients. Statistical analysis was performed on patient factors, including treatment types, and nominal regression analysis was carried out to assess the effects of multiple risk factors on the incidence of malignancy in patients with IBD.

Results: Age at diagnosis of IBD correlated significantly with malignancy development, as did the diagnosis of ulcerative colitis. IBD patients diagnosed with malignancy had an older age of onset of IBD than those who did not develop malignancy. Sex, treatment type, treatment duration, and extent or location of disease did not correlate significantly with malignancy development.

Conclusion: We conclude that age of onset of IBD plays the greatest role in malignancy development, whilst immunosuppressive treatment is not a significant risk factor.

背景:炎症性肠病(IBD)包括克罗恩病和溃疡性结肠炎,是一种多因素引起的胃肠道系统炎症性疾病,会影响患者的生活质量。其表现包括一系列症状,也可能继发于 IBD 并发症,如恶性肿瘤。另一方面,为维持缓解而进行的免疫抑制治疗也存在恶性肿瘤的风险,这可能会因 IBD 控制和恶性肿瘤的风险/收益平衡而给患者带来困扰:在这项全国性的回顾性研究中,我们旨在阐明哪些患者和治疗因素对 IBD 患者恶性肿瘤的发生影响最大。我们对患者因素(包括治疗类型)进行了统计分析,并进行了名义回归分析,以评估多种风险因素对 IBD 患者恶性肿瘤发病率的影响:结果:诊断 IBD 时的年龄与恶性肿瘤的发生有显著相关性,诊断为溃疡性结肠炎时的年龄也与恶性肿瘤的发生有显著相关性。确诊为恶性肿瘤的 IBD 患者的发病年龄比未患恶性肿瘤的患者大。性别、治疗类型、治疗时间、疾病程度或部位与恶性肿瘤的发生无明显相关性:我们得出的结论是,IBD的发病年龄在恶性肿瘤的发生中起着最大的作用,而免疫抑制治疗并不是一个重要的风险因素。
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引用次数: 0
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Annals of Gastroenterology
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