Digestive and Liver Disease最新文献

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Predictive value of renal shear wave elastography in liver cirrhosis. 肾剪切波弹性成像对肝硬化的预测价值
IF 4 3区 医学
Digestive and Liver Disease Pub Date : 2024-10-26 DOI: 10.1016/j.dld.2024.10.018
Christian Hagel, Michael Hirth, Jan Bißbort, Andreas Teufel, Svetlana Hetjens, Matthias P Ebert, Christoph Antoni
{"title":"Predictive value of renal shear wave elastography in liver cirrhosis.","authors":"Christian Hagel, Michael Hirth, Jan Bißbort, Andreas Teufel, Svetlana Hetjens, Matthias P Ebert, Christoph Antoni","doi":"10.1016/j.dld.2024.10.018","DOIUrl":"https://doi.org/10.1016/j.dld.2024.10.018","url":null,"abstract":"<p><strong>Background: </strong>In liver cirrhosis, prognosis is profoundly affected by renal function. This study evaluates the usefulness of renal stiffness measurement by point-shear wave elastography and renal perfusion by duplex.</p><p><strong>Methods: </strong>In this case-control study, organ stiffness was quantified using point-shear wave elastography and duplex sonography to calculate the arterial resistive (RI) and pulsatility indices (PI) in 123 patients, including patients with liver cirrhosis with (LC+A) and without ascites (LC-A) and patients without liver cirrhosis (NLC).</p><p><strong>Results: </strong>PI and RI were significantly increased in LC-A and LC+A compared to NLC (p<0.001) and correlated with MELD and Child Pugh scores. Point-shear wave elastography showed significantly lower renal stiffness in LC+A compared to LC-A (p<0.01) and NLC (p<0.001). Renal stiffness correlated inversely with MELD and Child Pugh scores. Reduced renal stiffness, but not PI or RI, was associated with the presence of hepatorenal syndrome (p<0.001). Reduced renal stiffness was associated with an increased risk of death due to complications of liver cirrhosis within 3 years (p<0.01).</p><p><strong>Conclusion: </strong>Point-shear wave elastography and duplex sonography correlate with progression of liver cirrhosis, but only shear wave elastography represents a valuable prognostic tool for hepatorenal syndrome and mortality in patients with liver cirrhosis.</p>","PeriodicalId":11268,"journal":{"name":"Digestive and Liver Disease","volume":null,"pages":null},"PeriodicalIF":4.0,"publicationDate":"2024-10-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142496946","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Full Title Page /Editorial Board 完整扉页/编辑栏
IF 4 3区 医学
Digestive and Liver Disease Pub Date : 2024-10-25 DOI: 10.1016/S1590-8658(24)01026-0
{"title":"Full Title Page /Editorial Board","authors":"","doi":"10.1016/S1590-8658(24)01026-0","DOIUrl":"10.1016/S1590-8658(24)01026-0","url":null,"abstract":"","PeriodicalId":11268,"journal":{"name":"Digestive and Liver Disease","volume":null,"pages":null},"PeriodicalIF":4.0,"publicationDate":"2024-10-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142532624","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Types, behaviour and therapeutic requirements of inflammatory pouch disorders: Results from the RESERVO study of GETECCU. 炎性肠袋疾病的类型、行为和治疗要求:GETECCU的RESERVO研究结果。
IF 4 3区 医学
Digestive and Liver Disease Pub Date : 2024-10-24 DOI: 10.1016/j.dld.2024.09.023
F Mesonero, Y Zabana, A Fernández-Clotet, A Solá, B Caballol, E Leo-Carnerero, M J García, F Bertoletti, G Bastida, G Suris, B Casis, R Ferreiro-Iglesias, M Calafat, I Jiménez, J Miranda-Bautista, L J Lamuela, I Fajardo, L Torrealba, R Nájera, R M Sáiz-Chumillas, I González-Partida, M Vicuña, N García-Morales, A Gutiérrez, A López-García, J M Benítez, C Rubín de Célix, C Tejido, E Brunet, A Hernandez-Camba, C Suárez, I Rodríguez-Lago, M Piqueras, A Castaño, L Ramos, A Sobrino, M C Rodríguez-Grau, A Elosua, M Montoro, R Baltar, J M Huguet, B Hermida, A Caballero-Mateos, L Sánchez-Guillén, A Bouhmidi, R Pajares, I Baston-Rey, A López-Sanromán, A Albillos, M Barreiro-de Acosta
{"title":"Types, behaviour and therapeutic requirements of inflammatory pouch disorders: Results from the RESERVO study of GETECCU.","authors":"F Mesonero, Y Zabana, A Fernández-Clotet, A Solá, B Caballol, E Leo-Carnerero, M J García, F Bertoletti, G Bastida, G Suris, B Casis, R Ferreiro-Iglesias, M Calafat, I Jiménez, J Miranda-Bautista, L J Lamuela, I Fajardo, L Torrealba, R Nájera, R M Sáiz-Chumillas, I González-Partida, M Vicuña, N García-Morales, A Gutiérrez, A López-García, J M Benítez, C Rubín de Célix, C Tejido, E Brunet, A Hernandez-Camba, C Suárez, I Rodríguez-Lago, M Piqueras, A Castaño, L Ramos, A Sobrino, M C Rodríguez-Grau, A Elosua, M Montoro, R Baltar, J M Huguet, B Hermida, A Caballero-Mateos, L Sánchez-Guillén, A Bouhmidi, R Pajares, I Baston-Rey, A López-Sanromán, A Albillos, M Barreiro-de Acosta","doi":"10.1016/j.dld.2024.09.023","DOIUrl":"https://doi.org/10.1016/j.dld.2024.09.023","url":null,"abstract":"<p><strong>Background and aims: </strong>Inflammatory pouch disorders exhibit a heterogeneous clinical spectrum and therapeutic requirements have not been properly studied.</p><p><strong>Methods: </strong>This retrospective, multicentre study included ulcerative colitis patients with ileal pouch construction and were later diagnosed with an inflammatory pouch disorder between 1995 and 2020. Classifications, behaviour and therapies applied were recorded and compared in the long-term.</p><p><strong>Results: </strong>Overall, 338 patients were recruited. The most common disorders were pouchitis (n = 258, 76%), Crohn's disease of the pouch (n = 55, 16%) and cuffitis (n = 25, 7%). Pouchitis presented mainly as chronic (65.2%) and recurrent (87%) forms. Crohn's disease manifested as stricturing/penetrating in 53% of cases and perianal disease in 42%. Patients received multiple therapies: 86% antibiotics, 42% steroids, 27% immunosuppressants, 43% biologics and 27% surgery. Compared with pouchitis, Crohn's disease of the pouch was characterised by a later diagnosis (99 vs. 55 months, p < 0.001) and greater needs for immunosuppressants (OR 3.53, 1.79-6.94, p < 0.0001), biologics (OR 5.45, 2.78-10.6, p < 0.0001) and surgeries (OR 2.65, 1.43-4.89, p < 0.001).</p><p><strong>Conclusions: </strong>Chronic pouchitis is the most common pouch disorder presentation. These entities have diverse therapeutics requirements, particularly for Crohn's disease of the pouch.</p>","PeriodicalId":11268,"journal":{"name":"Digestive and Liver Disease","volume":null,"pages":null},"PeriodicalIF":4.0,"publicationDate":"2024-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142496949","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Response to letter to the editor: Enhancing the applicability and rigor of precision surgical techniques in laparoscopic hepatectomy. 回复致编辑的信:提高腹腔镜肝切除术中精确手术技术的适用性和严谨性。
IF 4 3区 医学
Digestive and Liver Disease Pub Date : 2024-10-22 DOI: 10.1016/j.dld.2024.10.004
Zejiang Xu, Yaqing Lv, Hao Zou, Yuanfang Jia, Wenhao Du, Jinbin Lu, Yang Liu, Zemin Shao, Hao Zhang, Chuandong Sun, Chengzhan Zhu
{"title":"Response to letter to the editor: Enhancing the applicability and rigor of precision surgical techniques in laparoscopic hepatectomy.","authors":"Zejiang Xu, Yaqing Lv, Hao Zou, Yuanfang Jia, Wenhao Du, Jinbin Lu, Yang Liu, Zemin Shao, Hao Zhang, Chuandong Sun, Chengzhan Zhu","doi":"10.1016/j.dld.2024.10.004","DOIUrl":"https://doi.org/10.1016/j.dld.2024.10.004","url":null,"abstract":"","PeriodicalId":11268,"journal":{"name":"Digestive and Liver Disease","volume":null,"pages":null},"PeriodicalIF":4.0,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142496947","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Pancreatic steatosis is a strong risk factor for post-ERCP pancreatitis: An emerging concept. 胰腺脂肪变性是ERCP术后胰腺炎的一个重要风险因素:一个新概念。
IF 4 3区 医学
Digestive and Liver Disease Pub Date : 2024-10-22 DOI: 10.1016/j.dld.2024.10.005
Caroline Prouvot, Myriam Boumaiza, Khawla Maoui, Anne Sophie Peaucelle, Soiwafi Mohamed, Hanae Boutallaka, Claire Boutet, Xavier Roblin, Jean-Marc Phelip, Rémi Grange, Nicolas Williet
{"title":"Pancreatic steatosis is a strong risk factor for post-ERCP pancreatitis: An emerging concept.","authors":"Caroline Prouvot, Myriam Boumaiza, Khawla Maoui, Anne Sophie Peaucelle, Soiwafi Mohamed, Hanae Boutallaka, Claire Boutet, Xavier Roblin, Jean-Marc Phelip, Rémi Grange, Nicolas Williet","doi":"10.1016/j.dld.2024.10.005","DOIUrl":"https://doi.org/10.1016/j.dld.2024.10.005","url":null,"abstract":"<p><strong>Objective: </strong>The Endoscopic Retrograde Cholangiopancreatography (ERCP) is the treatment of choice for biliary obstruction but is associated with post-ERCP pancreatitis (PEP) in around 5 % of cases. No radiological criteria have been evaluated for predicting PEP risk.</p><p><strong>Design: </strong>This retrospective study examined records of 1365 patients who underwent ERCP at our center between 2014-2023. Only sphincterotomy-naïve patients were included. CT scans within 30 days of ERCP were reviewed for radiological criteria. The optimal pancreatic density cut-off was determined using AUROC and Youden index. Logistic regression was used for analyses.</p><p><strong>Results: </strong>PEP occurred in 75 patients (6.1 %). The CT scan was performed before ERCP for 565 of the total population. A fatty pancreas, defined as a spontaneous density less than -50HU, was statistically associated with PEP (OR: 7.35; 95 % CI: 1.56-26.5 p = 0.004), as well as with biliary obstruction due to stones (OR: 0.61; 95 % CI: 0.38-0.98; P = 0.04), the need for precut (OR: 2.19; 95 % CI: 1.35-3.51; P = 0.001), cannulation of the main pancreatic duct (OR: 2.23; 95 % CI: 1.36-3.59; P = 0.001), and the use of a pancreatic stent (OR: 2.48; 95 % CI: 1.29-4.47; P = 0.004). In multivariate analyses, only obstruction unrelated to gallstones (OR = 2.63; 95 % CI: 1.16-6.25; P = 0.024) and a low pancreatic density (<-50HU) (OR=7.94, 95 %CI: 1.59-31.09; P = 0.005) remains significantly associated with the risk of PEP, including after adjustment for age and sex (P = 0.006).</p><p><strong>Conclusion: </strong>A very low pancreatic fat density could be a significant risk factor for post-ERCP pancreatitis with potential clinical and research implications. Further validation is needed.</p>","PeriodicalId":11268,"journal":{"name":"Digestive and Liver Disease","volume":null,"pages":null},"PeriodicalIF":4.0,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142496945","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Author's reply: "A critical appraisal of the safety and efficacy of P-ESWL in treating pancreatic stones". 作者回复:"对 P-ESWL 治疗胰腺结石的安全性和有效性的严格评估"。
IF 4 3区 医学
Digestive and Liver Disease Pub Date : 2024-10-19 DOI: 10.1016/j.dld.2024.10.006
Yu Liu, Liang-Hao Hu
{"title":"Author's reply: \"A critical appraisal of the safety and efficacy of P-ESWL in treating pancreatic stones\".","authors":"Yu Liu, Liang-Hao Hu","doi":"10.1016/j.dld.2024.10.006","DOIUrl":"https://doi.org/10.1016/j.dld.2024.10.006","url":null,"abstract":"","PeriodicalId":11268,"journal":{"name":"Digestive and Liver Disease","volume":null,"pages":null},"PeriodicalIF":4.0,"publicationDate":"2024-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142460420","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
An unusual polypoid lesion of the rectal ampulla. 直肠安瓿的不寻常息肉样病变。
IF 4 3区 医学
Digestive and Liver Disease Pub Date : 2024-10-18 DOI: 10.1016/j.dld.2024.10.003
Maria Cristina Spinella, Giovanni Fellegara
{"title":"An unusual polypoid lesion of the rectal ampulla.","authors":"Maria Cristina Spinella, Giovanni Fellegara","doi":"10.1016/j.dld.2024.10.003","DOIUrl":"https://doi.org/10.1016/j.dld.2024.10.003","url":null,"abstract":"","PeriodicalId":11268,"journal":{"name":"Digestive and Liver Disease","volume":null,"pages":null},"PeriodicalIF":4.0,"publicationDate":"2024-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142460419","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The association analysis between exposure to volatile organic compounds and fatty liver disease in US Adults. 美国成年人接触挥发性有机化合物与脂肪肝之间的关联分析。
IF 4 3区 医学
Digestive and Liver Disease Pub Date : 2024-10-18 DOI: 10.1016/j.dld.2024.09.027
Xiaoxia Duan, Zhenhua Chen, Juan Liao, Mingsheng Wen, Yong Yue, Li Liu, Xiaojing Li, Lu Long
{"title":"The association analysis between exposure to volatile organic compounds and fatty liver disease in US Adults.","authors":"Xiaoxia Duan, Zhenhua Chen, Juan Liao, Mingsheng Wen, Yong Yue, Li Liu, Xiaojing Li, Lu Long","doi":"10.1016/j.dld.2024.09.027","DOIUrl":"https://doi.org/10.1016/j.dld.2024.09.027","url":null,"abstract":"<p><strong>Background: </strong>Limited epidemiological research has explored the associations between ambient volatile organic compounds (VOCs) and fatty liver disease (FLD). This study aimed to explore the associations between VOCs and FLD and liver function biomarkers. We obtained urinary concentrations of VOCs metabolites from NHANES.</p><p><strong>Methods: </strong>Weighted logistic regression models were employed to investigate the relationships between VOCs and FLD risk, including alcoholic FLD (AFLD) and non-alcoholic FLD (NAFLD). The associations of VOCs and liver function biomarkers were also investigated using weighted linear regression.</p><p><strong>Results: </strong>Among the 2050 participants, 774 were classified as having FLD. After adjustment, each log-transformed SD increase in N-Acetyl-S-(2-carboxyethyl)-<sub>l</sub>-cysteine (CEMA), 2-Aminothiazoline-4-carboxylicacid (ATCA), and trans-trans-muconic-acid (MUCA) had a OR (95%CI) of 1.30 (1.06-1.61; P-trend=0.014), 1.34 (1.12-1.61; P-trend=0.002), and 1.22 (1.01-1.47; P-trend=0.035), respectively. ATCA and MUCA were associated with higher risks of NAFLD (OR=1.47, 95%CI: 1.20-1.79, and OR=1.26, 95%CI: 1.02-1.56, respectively). VOCs were positively associated with gamma glutamyl transaminase (GGT) and C-reactive protein (CRP), while inversely associated with albumin, total protein and alanine aminotransferase (ALT) (P < 0.05).</p><p><strong>Conclusions: </strong>Urinary metabolites of VOCs have been found to be strongly correlated with a higher risk of FLD and NFALD, and impaired liver function. These novel findings merit further prospective studies to comprehend the effect of VOCs on liver diseases.</p>","PeriodicalId":11268,"journal":{"name":"Digestive and Liver Disease","volume":null,"pages":null},"PeriodicalIF":4.0,"publicationDate":"2024-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142460425","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Measured glomerular filtration rate predicts liver related deaths better than estimated glomerular filtration rate in advanced chronic liver disease. 在晚期慢性肝病患者中,测量肾小球滤过率比估计肾小球滤过率更能预测与肝脏相关的死亡。
IF 4 3区 医学
Digestive and Liver Disease Pub Date : 2024-10-18 DOI: 10.1016/j.dld.2024.09.016
Carlos González-Alayón, M Hernández-Guerra, Sergio Luis-Lima, Coriolano Cruz Perera Lima, Andrea Santana-Delgado, Carlos Díaz-Mesa, Andrea Morant-Domínguez, Laura Díaz Martín, Federico González-Rinne, Alberto Hernández-Bustabad, Miguel Moreno, Flavio Gaspari, Esteban Porrini
{"title":"Measured glomerular filtration rate predicts liver related deaths better than estimated glomerular filtration rate in advanced chronic liver disease.","authors":"Carlos González-Alayón, M Hernández-Guerra, Sergio Luis-Lima, Coriolano Cruz Perera Lima, Andrea Santana-Delgado, Carlos Díaz-Mesa, Andrea Morant-Domínguez, Laura Díaz Martín, Federico González-Rinne, Alberto Hernández-Bustabad, Miguel Moreno, Flavio Gaspari, Esteban Porrini","doi":"10.1016/j.dld.2024.09.016","DOIUrl":"https://doi.org/10.1016/j.dld.2024.09.016","url":null,"abstract":"<p><strong>Background & aims: </strong>Renal dysfunction is prevalent in advanced chronic liver disease (aCLD) and is associated to liver-related death (LRD). This makes a reliable evaluation of renal function (RF) a crucial aspect. RF can be estimated by formulas or measured by gold standard method. Estimated RF is not reliable in aCLD. However, there is a lack of information on the reliability of formulas in the prediction of LRD.</p><p><strong>Methods: </strong>We analysed a cohort of patients with aCLD in whom RF was measured by the plasma clearance of iohexol (mGFR) and estimated (eGFR) by formulas: MDRD, CKD-EPI, Royal Free Hospital (RFHC), GRAIL and Mindikoglu-eGFR. LRD was defined as death from hepatic causes. Multivariable analysis was used to evaluate association of mGFR or eGFR with LRD.</p><p><strong>Results: </strong>161 patients were evaluated, with median follow-up of 28 months, 58 died from LRD. In overall group mGFR (OR 0.99; p = 0.022) and formulas: CKD-EPI (OR 0.98; p = 0.044), GRAIL (OR 0.98; p = 0.038) was associated with LRD. In patients with normal creatinine levels (≤ 1.1 mg/dL), mGFR (OR 0.99; p = 0.031) was whereas any formula was not associated with LRD.</p><p><strong>Conclusions: </strong>eGFR appears as an unreliable method for predicting LRDs in aCLD, especially in those with lower creatinine levels. By contrast, mGFR seems to be a superior predictor.</p>","PeriodicalId":11268,"journal":{"name":"Digestive and Liver Disease","volume":null,"pages":null},"PeriodicalIF":4.0,"publicationDate":"2024-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142460422","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Blue rubber bleb nevus syndrome: A European multicenter cohort study. 蓝色橡胶斑痣综合征:欧洲多中心队列研究。
IF 4 3区 医学
Digestive and Liver Disease Pub Date : 2024-10-18 DOI: 10.1016/j.dld.2024.10.001
Aymeric Becq, Annouk Bisdorff, Maria Elena Riccioni, Sophie Blaise, Stéphanie Mallet, Ervin Toth, Annabel Maruani, Edward Despott, Christine Labreze, Pablo Cortegoso Valdivia, Emmanuele Rondonotti, Cristina Carrretero Ribón, Laetitia Goffinet, Elise Coffin, Bruno Rosa, Cynthia Medlij, Jean-Christophe Saurin, Xavier Dray
{"title":"Blue rubber bleb nevus syndrome: A European multicenter cohort study.","authors":"Aymeric Becq, Annouk Bisdorff, Maria Elena Riccioni, Sophie Blaise, Stéphanie Mallet, Ervin Toth, Annabel Maruani, Edward Despott, Christine Labreze, Pablo Cortegoso Valdivia, Emmanuele Rondonotti, Cristina Carrretero Ribón, Laetitia Goffinet, Elise Coffin, Bruno Rosa, Cynthia Medlij, Jean-Christophe Saurin, Xavier Dray","doi":"10.1016/j.dld.2024.10.001","DOIUrl":"https://doi.org/10.1016/j.dld.2024.10.001","url":null,"abstract":"<p><strong>Background and aim: </strong>Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare congenital vascular entity, mainly characterized by cutaneous and gastrointestinal venous malformations. BRBNS remains relatively unexplored and only limited descriptive data is available. The aim of this study was to evaluate the clinical features, diagnostic work-up and therapeutic management in current practice.</p><p><strong>Methods: </strong>A multicenter, European, cohort study was performed to investigate the BRBNS population. Patient demographics, clinical presentation and management data were collected.</p><p><strong>Results: </strong>In this multicenter cohort study including 44 patients, BRBNS is diagnosed at a median age of 12 years, mainly based on clinical presentation (65.9 %). The majority of patients present cutaneous (68.2 %) and digestive (79.5 %) lesions, mainly in the colon and small bowel. d-dimer and fibrinogen levels are checked in <50 % of patients at diagnosis. Tie2/TEK mutation testing is rarely performed. Gastrointestinal bleeding is the most common complication (54.3 %), requiring endoscopic treatment (36.4 %) by various techniques.</p><p><strong>Conclusions: </strong>This is the largest cohort study on BRBNS. Diagnosis is mainly based on clinical presentation. d-dimer, fibrinogen and Tie2/TEK mutation testing should be performed in case of suspected BRBNS as it could help confirm the diagnosis.</p>","PeriodicalId":11268,"journal":{"name":"Digestive and Liver Disease","volume":null,"pages":null},"PeriodicalIF":4.0,"publicationDate":"2024-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142460421","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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