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Heterotaxia syndrome: when almost nothing is as it should be. 异位综合征:当几乎所有事情都不尽如人意时。
IF 2.2 4区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-01-01 Epub Date: 2024-07-17 DOI: 10.1016/j.gastrohep.2024.502233
Luisa Adán-Merino, Francisco Garrido Gallego, Rodrigo Pastorín-Salis, Ángel Ponferrada-Díaz
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引用次数: 0
Increased arterial stiffness in Crohn's disease: Prevalence, associated factors and impact of anti-TNF therapy. 克罗恩病患者动脉僵化加剧:发病率、相关因素和抗肿瘤坏死因子疗法的影响。
IF 2.2 4区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-01-01 Epub Date: 2024-09-11 DOI: 10.1016/j.gastrohep.2024.502252
Mahrez Fissah, Adel Rechach, Meriem Charifi, Farouk Menzou, Said Taharboucht, Messaouda Djouhri, Leila Talbi, Nadia Touati, Lamine Atif, Mehdi Rabhia, Ahcene Chibane

Introduction: Recent studies have demonstrated the growing interest in cardiovascular risk in Crohn's disease (CD), the aim of our work is to highlight the need for research into the frequency of arterial stiffness (AS) and its link with certain associated factors, particularly those related to inflammation.

Materials and methods: This was a cross-sectional observational study involving 118 patients with CD. Pulse wave velocity (PWV) measured by applanation tonometry was the criterion for calculating AS, the study also investigated the association of AS especially the indicators of inflammation, as well as the impact of anti-TNF alpha therapy on AS.

Results: The prevalence of AS, after adjustment for age and blood pressure level reached more than a quarter of patients compared to the cardiovascular risk which was low. The factors that were strongly associated with AS were age, systolic and diastolic blood pressure. Two parameters related to inflammation emerged as having a highly significant link after multivariate analysis: recurrence in the last year and length of disease with a p=0.008, and an OR of 5 and 9 successively. Patients treated with anti-TNF alpha had a significant reduction in PWV.

Conclusion: The prevalence of AS reached more than a quarter of patients with CD, the duration and recurrence rate of CD appear to be factors linked to inflammation. Treatment with anti-TNF alpha seems to slow down PWV in these patients.

导言:最近的研究表明,人们越来越关注克罗恩病(CD)的心血管风险,我们的工作旨在强调研究动脉僵化(AS)频率及其与某些相关因素(尤其是与炎症有关的因素)之间联系的必要性:这是一项横断面观察研究,涉及 118 名 CD 患者。研究还调查了动脉僵化与炎症指标的关联,以及抗 TNF α 治疗对动脉僵化的影响:结果:在对年龄和血压水平进行调整后,超过四分之一的患者患有强直性脊柱炎,而心血管风险较低。与强直性脊柱炎密切相关的因素是年龄、收缩压和舒张压。经过多变量分析,有两个与炎症相关的参数与强直性脊柱炎有非常显著的联系:去年的复发率和病程,P=0.008,OR 值分别为 5 和 9。接受抗 TNF α 治疗的患者脉搏波速度明显降低。结论 :在CD患者中,强直性脊柱炎的发病率超过四分之一,CD的病程和复发率似乎与炎症有关。抗肿瘤坏死因子α治疗似乎能减缓这些患者的脉搏波速度。
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引用次数: 0
Artificial intelligence-assisted identification and retrieval of chronic hepatitis C patients lost to follow-up in the health area of Pontevedra and O Salnés (Spain). 人工智能辅助识别和检索庞特韦德拉和奥萨尔内斯卫生区(西班牙)失去随访的慢性丙型肝炎患者。
IF 2.2 4区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-01-01 Epub Date: 2024-06-29 DOI: 10.1016/j.gastrohep.2024.502226
Pablo Parada Vázquez, Santiago Pérez-Cachafeiro, Belén Castiñeira Domínguez, Juan Manuel González-Pérez, José Manuel Mera Calviño, Raquel Souto-Rodríguez, Yolanda Falagán Cachafeiro, Indhira Pérez-Medrano, Nuria Vázquez-Temprano, Matilde Trigo, Alba Carrodeguas, José Luis González-Sánchez, Carmen Durán-Parrondo, Juan Turnes

Objective: Direct-acting antivirals (DAAs) to treat hepatitis C virus (HCV) infection offer an opportunity to eliminate the disease. This study aimed to identify and relink to care HCV patients previously lost to medical follow-up in the health area of Pontevedra and O Salnés (Spain) using an artificial intelligence-assisted system.

Patients and methods: Active retrospective search of previously diagnosed HCV cases recorded in the Galician Health Service proprietary health information exchange database using the Herramientas para la EXplotación de la INformación (HEXIN) application.

Results and conclusions: Out of 99 lost patients identified, 64 (64.6%) were retrieved. Of these, 62 (96.88%) initiated DAA treatment and 54 patients (87.1%) achieved a sustained virological response. Mean time from HCV diagnosis was over 10 years. Main reasons for loss to follow-up were fear of possible adverse effects of treatment (30%) and mobility impediments (21%). Among the retrieved patients, almost one in three presented advanced liver fibrosis (F3) or cirrhosis (F4) at evaluation. In sum, HCV patients lost to follow-up can be retrieved by screening past laboratory records. This strategy promotes the achievement of HCV elimination goals.

目的:治疗丙型肝炎病毒(HCV)感染的直接作用抗病毒药物(DAAs)为消除该疾病提供了机会。本研究旨在利用人工智能辅助系统,在庞特韦德拉和奥萨尔内斯(西班牙)卫生保健区发现之前失去医疗随访的丙型肝炎病毒(HCV)患者,并将其重新链接到医疗机构:使用 Herramientas para la EXplotación de la INformación (HEXIN) 应用程序对加利西亚卫生服务专有卫生信息交换数据库中记录的先前诊断的 HCV 病例进行主动回顾性搜索:在已确认的 99 名失访患者中,有 64 人(64.6%)被找回。其中,62 名患者(96.88%)接受了 DAA 治疗,54 名患者(87.1%)获得了持续病毒学应答。从确诊为丙型肝炎病毒到现在的平均时间超过 10 年。失去随访的主要原因是担心治疗可能产生不良反应(30%)和行动不便(21%)。在失访患者中,几乎三分之一的患者在评估时出现了晚期肝纤维化(F3)或肝硬化(F4)。总之,通过筛查过去的实验室记录,可以找回失去随访的 HCV 患者。这一策略有助于实现消除 HCV 的目标。
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引用次数: 0
Adaptation and validation of an abbreviated version of the SIPAT integrated psychosocial risk scale in patients with liver cirrhosis candidates for liver transplantation (SIPAT-11). 肝硬化肝移植候选患者社会心理风险综合量表缩略版(SIPAT-11)的改编与验证。
IF 2.2 4区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-01-01 Epub Date: 2024-06-19 DOI: 10.1016/j.gastrohep.2024.502220
Natalia García Morales, Sagrario Gutiérrez Morato, Carmen Castillo Cejas, Margarita Fernández de la Varga, Martín Menéndez Rodríguez, Victoria Aguilera, Salvador Benlloch, Luis Menéndez Rodríguez, Teresa Seoane Pillado, Joaquín Cubiella

Introduction: Patients with liver cirrhosis who are candidates for liver transplantation must be evaluated both clinically and socially in order to obtain the optimal outcomes and avoid futile therapeutic measures. For the evaluation of the social aspects in these patients, no validated scale in Spanish is available. The SIPAT (Stanford Integrated Psychosocial Assessment for Transplantation) scale is an instrument that measures the social, family and psychological aspects in candidates for solid organ transplantation. The objective of this study is to adapt and validate an abbreviated version of the SIPAT scale in Spanish for patients with liver cirrhosis.

Material and methods: Prospective observational study carried out in the Hepatology Unit of the La Fe Unversity Hospital in Valencia, by questionnaire validation methodology. To analyze the reliability of the questionnaire, the internal consistency of all variables was calculated, for variability an exploratory factor analysis, and for stability the test-retest test was carried out.

Results: 96 patients who were admitted for decompensated cirrhosis to the Hepatology Unit of the La Fe Hospital in Valencia between November 1, 2017 and January 31, 2017 were selected. 84% were men, the mean age was 60.01 (SD 10.12) years. In 73.2% of those admitted, the etiology of cirrhosis was alcoholic. 14.4% had a Child's stage A, 57.7% B and 27.8% C. The internal consistency of all variables reached a Cronbach's Alpha of 0.766. In the exploratory factor analysis, 6 dimensions of the questionnaire were identified that explain 84.27% of the total variability. To see the stability of the instrument, the measurement was repeated at 2 and 6 months of follow-up, obtaining in the test-retest a kappa agreement of 0.612 and 0.565 respectively.

Conclusion: The SIPAT-11 questionnaire has good psychometric characteristics in cirrhotic patients who are candidates for liver transplantation. It is easy to complete and can be administered by professionals who are not specialists in the area of Mental Health.

导言:肝硬化患者是肝脏移植的候选者,必须对其进行临床和社会评估,以获得最佳治疗效果,避免采取徒劳无益的治疗措施。对于这些患者的社会方面评估,目前还没有西班牙文的有效量表。SIPAT(斯坦福移植综合社会心理评估)量表是一种测量实体器官移植候选者的社会、家庭和心理方面的工具。本研究的目的是改编并验证适用于肝硬化患者的西班牙语 SIPAT 量表缩写版:在巴伦西亚拉费大学医院肝病科开展的前瞻性观察研究,采用问卷验证法。为分析问卷的可靠性,计算了所有变量的内部一致性,对变异性进行了探索性因子分析,对稳定性进行了重复测试:选取了2017年11月1日至2017年1月31日期间巴伦西亚拉费医院肝病科收治的96名失代偿期肝硬化患者。84%为男性,平均年龄为60.01岁(标清10.12岁)。73.2%的入院患者的肝硬化病因是酒精性肝硬化。所有变量的内部一致性达到 Cronbach's Alpha 0.766。在探索性因子分析中,确定了问卷的 6 个维度,它们解释了 84.27% 的总变异性。为了解该工具的稳定性,在 2 个月和 6 个月的随访中重复进行了测量,在重复测试中获得的 kappa 一致度分别为 0.612 和 0.565:SIPAT-11问卷对肝移植候选者中的肝硬化患者具有良好的心理测量特性。该问卷易于填写,可由非心理健康领域专家的专业人员进行管理。
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引用次数: 0
Portal hypertension: recommendations for diagnosis and treatment. Consensus document sponsored by the Spanish Association for the Study of the Liver (AEEH) and the Biomedical Research Network Centre for Liver and Digestive Diseases (CIBERehd). 门静脉高压:诊断和治疗的建议。由西班牙肝脏研究协会(AEEH)和肝脏和消化疾病生物医学研究网络中心(CIBERehd)赞助的共识文件。
IF 2.2 4区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-01-01 Epub Date: 2024-09-30 DOI: 10.1016/j.gastrohep.2024.502208
Agustín Albillos, Rafael Bañares, Virginia Hernández-Gea

Portal hypertension is a hemodynamic abnormality that complicates the course of cirrhosis, as well as other diseases that affect the portal venous circulation. The development of portal hypertension compromises prognosis, especially when it rises above a certain threshold known as clinically significant portal hypertension (CSPH). In the consensus conference on Portal Hypertension promoted by the Spanish Association for the Study of the Liver and the Hepatic and Digestive diseases area of the Biomedical Research Networking Center (CIBERehd), different aspects of the diagnosis and treatment of portal hypertension caused by cirrhosis or other diseases were discussed. The outcome of this discussion was a set of recommendations that achieved varying degrees of consensus among panelists and are reflected in this consensus document. The six areas under discussion were: the relevance of CSPH and the non-invasive methods used for its diagnosis and that of cirrhosis, the prevention of the first episode of decompensation and its recurrence, the treatment of acute variceal bleeding and other complications of portal hypertension, the indications for the use of TIPS, and finally, the diagnosis and treatment of liver vascular diseases.

门脉高压是一种血液动力学异常,它使肝硬化和其他影响门静脉循环的疾病的病程复杂化。门脉高压的发展影响预后,特别是当它超过一定阈值时,称为临床显著门脉高压(CSPH)。在西班牙肝脏研究协会和生物医学研究网络中心(CIBERehd)肝脏和消化疾病领域推动的门脉高压共识会议上,讨论了肝硬化或其他疾病引起的门脉高压的诊断和治疗的不同方面。这次讨论的结果是一组建议,这些建议在小组成员之间取得了不同程度的协商一致意见,并反映在本协商一致文件中。讨论的六个方面是:CSPH的相关性及其与肝硬化的无创诊断方法,预防首次失代偿及其复发,治疗急性静脉曲张出血和门静脉高压的其他并发症,TIPS的适应症,最后是肝血管疾病的诊断和治疗。
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引用次数: 0
Cerebral thrombosis as a manifestation of poor control in inflammatory bowel disease. 脑血栓是炎症性肠病控制不佳的一种表现。
IF 2.2 4区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-01-01 Epub Date: 2024-09-28 DOI: 10.1016/j.gastrohep.2024.502256
Marta Fernández Carrasco, Ana Delgado Maroto, Álvaro Hernández Martínez
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引用次数: 0
Combined biologic treatment in patient with chronic spontaneous urticaria and Crohn's disease. 慢性自发性荨麻疹和克罗恩病患者的联合生物治疗。
IF 2.2 4区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-01-01 Epub Date: 2024-05-28 DOI: 10.1016/j.gastrohep.2024.502213
Alejandro García Martínez, María Del Pilar Lobato de la Sierra, Teresa Castro Aguilar-Tablada
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引用次数: 0
Assessment of the quality of life of patients with inflammatory bowel disease. 评估炎症性肠病患者的生活质量。
IF 2.2 4区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-01-01 Epub Date: 2024-05-06 DOI: 10.1016/j.gastrohep.2024.502192
María Del Mar Calvo Bernal, Elena Pérez Campos, Adrián Aparicio Mota, Álvaro Hernández Martínez

Introduction: Ulcerative colitis (UC) and Crohn's disease (CD) are diseases that cause a significant impact on patients' quality of life. The aim of this study is to assess the impact of inflammatory bowel disease (IBD) on health-related quality of life (HRQoL).

Material and methods: Observational, descriptive, cross-sectional study, carried out at Torrecárdenas Hospital (Almería). Patients over 14 years of age diagnosed with CD or UC were included. For the assessment of HRQoL, the reduced 9-item IBDQ-9 questionnaire was used.

Results: 106 patients with a mean age of 44 years were included, with a female predominance. Forty-five percent of the patients in the sample had UC compared to 55% with CD. Of the patients, 69.8% were in clinical remission. The median questionnaire score was 60.8 points out of 100. Statistically significant differences were observed between sexes, with worse HRQoL for females. No differences were observed between patients with UC and CD. Differences were also detected between patients who underwent surgery and those who did not. A negative association was observed between the number of flares and the questionnaire score.

Conclusions: In our study population, there is an acceptable HRQoL, with no differences observed between CD and UC. Female sex, absence of clinical remission, number of previous outbreaks, and surgery have a negative association with HRQoL.

导言:本研究旨在评估炎症性肠病(IBD)对健康相关生活质量(HRQoL)的影响:观察性、描述性、横断面研究,在托雷卡德纳斯医院(阿尔梅里亚)进行。研究纳入了 14 岁以上被诊断为 CD 或 UC 的患者。在评估 HRQoL 时,使用了简化的 9 项 IBDQ-9 问卷:结果:共纳入 106 名患者,平均年龄为 44 岁,其中女性居多。样本中 45% 的患者患有 UC,而 55% 的患者患有 CD。调查问卷的中位数为 60.8 分(满分 100 分)。性别差异具有统计学意义,女性的 HRQoL 更差。UC 和 CD 患者之间没有差异。接受手术治疗和未接受手术治疗的患者之间也存在差异:在我们的研究人群中,患者的 HRQoL 可以接受,CD 和 UC 患者之间没有差异。
{"title":"Assessment of the quality of life of patients with inflammatory bowel disease.","authors":"María Del Mar Calvo Bernal, Elena Pérez Campos, Adrián Aparicio Mota, Álvaro Hernández Martínez","doi":"10.1016/j.gastrohep.2024.502192","DOIUrl":"10.1016/j.gastrohep.2024.502192","url":null,"abstract":"<p><strong>Introduction: </strong>Ulcerative colitis (UC) and Crohn's disease (CD) are diseases that cause a significant impact on patients' quality of life. The aim of this study is to assess the impact of inflammatory bowel disease (IBD) on health-related quality of life (HRQoL).</p><p><strong>Material and methods: </strong>Observational, descriptive, cross-sectional study, carried out at Torrecárdenas Hospital (Almería). Patients over 14 years of age diagnosed with CD or UC were included. For the assessment of HRQoL, the reduced 9-item IBDQ-9 questionnaire was used.</p><p><strong>Results: </strong>106 patients with a mean age of 44 years were included, with a female predominance. Forty-five percent of the patients in the sample had UC compared to 55% with CD. Of the patients, 69.8% were in clinical remission. The median questionnaire score was 60.8 points out of 100. Statistically significant differences were observed between sexes, with worse HRQoL for females. No differences were observed between patients with UC and CD. Differences were also detected between patients who underwent surgery and those who did not. A negative association was observed between the number of flares and the questionnaire score.</p><p><strong>Conclusions: </strong>In our study population, there is an acceptable HRQoL, with no differences observed between CD and UC. Female sex, absence of clinical remission, number of previous outbreaks, and surgery have a negative association with HRQoL.</p>","PeriodicalId":12802,"journal":{"name":"Gastroenterologia y hepatologia","volume":" ","pages":"502192"},"PeriodicalIF":2.2,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140891141","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"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
Vaccine adherence and adverse events of the SARS-COV vaccine in patients with inflammatory bowel disease. 炎症性肠病患者接种 SARS-COV 疫苗的依从性和不良事件。
IF 2.2 4区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2025-01-01 Epub Date: 2024-05-07 DOI: 10.1016/j.gastrohep.2024.502202
Francisca Isabelle da Silva E Sousa, Raiza Lima Silva, Cezar Nilton Rabelo Lemos Filho, Maria Tereza Oliveira Pereira Santos, Luiz Eduardo Soares Martins, Thais Carvalho de Abreu, Leonardo Freire Alves Nogueira, Sâmya Correia Marques, Marcellus Henrique Loiola Ponte de Souza, Lucia Libanez Bessa Campelo Braga

Objective: To assess adherence to and the adverse effects of the SARS-COV vaccine in patients with inflammatory bowel disease (IBD).

Patients and methods: This is an observational, analytical, cross-sectional study. Sociodemographic and clinical data, SARS-COV vaccine data, medications for IBD with use during the vaccination period, and adverse events during the vaccination period were collected. Carried out logistic regressions with robust variance estimation to estimate the odds ratio with the respective 95% confidence intervals (95%CI) to assess the factors associated with non-serious adverse effects following vaccine doses as outcome variables.

Results: 194 patients participated, with vaccine compliance of 78.3% for three doses of any vaccine (n=152). Local symptoms and mild systemic symptoms predominated, regardless of the type of vaccine. The first dose of the SARS-COV vaccine with AstraZeneca had a higher percentage of patients with vaccine symptoms. AstraZeneca vaccine increased the chance of non-serious adverse effects in IBD patients by 2.65 times (95% CI: 1.38-5.08; p=0.003), regardless of age, gender, physical activity, excess weight, use of disease-modifying drugs, immunobiological and corticosteroids. CoronaVac vaccine was associated with asymptomatic patients at the first dose and reduced the chance of adverse effects by 0.28 times (OR: 0.284; 95%CI: 0.13-0.62; p=0.002).

Conclusion: Local symptoms and mild systemic symptoms predominated, regardless of the type of vaccine. Using CoronaVac in the first dose reduced the chances of adverse effects, while AstraZeneca increased the risk of adverse effects.

目的:评估炎症性肠病(IBD)患者接种SARS-COV疫苗的依从性和不良反应:这是一项观察性、分析性、横断面研究。收集了社会人口学和临床数据、SARS-COV 疫苗数据、接种期间使用的治疗 IBD 的药物以及接种期间发生的不良事件。采用稳健方差估计法进行逻辑回归,估算出与相应 95% 置信区间 (95%CI) 的比值比,以评估作为结果变量的接种疫苗后出现非严重不良反应的相关因素:194名患者参与了研究,任何疫苗接种三次的依从性为78.3%(152人)。无论接种哪种疫苗,局部症状和轻微全身症状均占多数。第一剂接种阿斯利康 SARS-COV 疫苗的患者出现疫苗症状的比例较高。阿斯利康疫苗使 IBD 患者出现非严重不良反应的几率增加了 2.65 倍(95% CI:1.38-5.08;P=0.003),与年龄、性别、体力活动、超重、使用疾病调节药物、免疫生物学和皮质类固醇无关。CoronaVac疫苗与首次接种时无症状的患者有关,并将不良反应几率降低了0.28倍(OR:0.284;95%CI:0.13-0.62;p=0.002):结论:无论疫苗类型如何,局部症状和轻微的全身症状均占主导地位。结论:无论疫苗类型如何,局部症状和轻微的全身症状占多数。在第一剂中使用科罗娜疫苗可降低不良反应的发生几率,而使用阿斯利康疫苗则会增加不良反应的风险。
{"title":"Vaccine adherence and adverse events of the SARS-COV vaccine in patients with inflammatory bowel disease.","authors":"Francisca Isabelle da Silva E Sousa, Raiza Lima Silva, Cezar Nilton Rabelo Lemos Filho, Maria Tereza Oliveira Pereira Santos, Luiz Eduardo Soares Martins, Thais Carvalho de Abreu, Leonardo Freire Alves Nogueira, Sâmya Correia Marques, Marcellus Henrique Loiola Ponte de Souza, Lucia Libanez Bessa Campelo Braga","doi":"10.1016/j.gastrohep.2024.502202","DOIUrl":"10.1016/j.gastrohep.2024.502202","url":null,"abstract":"<p><strong>Objective: </strong>To assess adherence to and the adverse effects of the SARS-COV vaccine in patients with inflammatory bowel disease (IBD).</p><p><strong>Patients and methods: </strong>This is an observational, analytical, cross-sectional study. Sociodemographic and clinical data, SARS-COV vaccine data, medications for IBD with use during the vaccination period, and adverse events during the vaccination period were collected. Carried out logistic regressions with robust variance estimation to estimate the odds ratio with the respective 95% confidence intervals (95%CI) to assess the factors associated with non-serious adverse effects following vaccine doses as outcome variables.</p><p><strong>Results: </strong>194 patients participated, with vaccine compliance of 78.3% for three doses of any vaccine (n=152). Local symptoms and mild systemic symptoms predominated, regardless of the type of vaccine. The first dose of the SARS-COV vaccine with AstraZeneca had a higher percentage of patients with vaccine symptoms. AstraZeneca vaccine increased the chance of non-serious adverse effects in IBD patients by 2.65 times (95% CI: 1.38-5.08; p=0.003), regardless of age, gender, physical activity, excess weight, use of disease-modifying drugs, immunobiological and corticosteroids. CoronaVac vaccine was associated with asymptomatic patients at the first dose and reduced the chance of adverse effects by 0.28 times (OR: 0.284; 95%CI: 0.13-0.62; p=0.002).</p><p><strong>Conclusion: </strong>Local symptoms and mild systemic symptoms predominated, regardless of the type of vaccine. Using CoronaVac in the first dose reduced the chances of adverse effects, while AstraZeneca increased the risk of adverse effects.</p>","PeriodicalId":12802,"journal":{"name":"Gastroenterologia y hepatologia","volume":" ","pages":"502202"},"PeriodicalIF":2.2,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140897831","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"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
Assessment of cystic fibrosis related liver disease in a pediatric cohort. 儿童队列中囊性纤维化相关肝病的评估
IF 2.2 4区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-12-13 DOI: 10.1016/j.gastrohep.2024.502321
S Vicente-Santamaría, M E Torres-Guerrero, M García-González, A Tabares-González, C Gascón-Galindo, C M López-Cárdenes, E Blitz-Castro, A Morales-Tirado, M I Mota Goitia, J R Gutiérrez Martínez, C Tutau Gómez, R García Romero, E Salcedo Lobato, L Peña Quintana, A Reyes Domínguez, E Torcuato Rubio, P Ortiz Pérez, A E Fernández-Lorenzo, A Moreno Álvarez, Alfonso Solar Boga, H Romero Rey, M Álvarez Beltrán, E Masip Simo, D González Jiménez

Background: Cystic fibrosis (CF) is an autosomal recessive, chronic, potentially lethal genetic disease. CF manifestations are due to mutations in the CF transmembrane receptor transporter (CFTR) gene which codes for a protein (CFTR) that acts as an anion transporter, mainly chlorine, at epithelial cells where it is expressed. Cystic fibrosis related liver disease (CFRLD) includes a spectrum of hepatobiliary manifestations whose diagnosis and follow-up remains a challenge.

Methods: Cross-sectional, descriptive study from 10 Spanish Cystic fibrosis Units. Clinical and biochemical data obtained. Patients categorized into 3 groups according to liver involvement based on ESPGHAN 2017 criteria. Liver stiffness assessed by transient elastography (TE) and findings from abdominal ultrasound recorded. Statistics performed using SPSS v25.0.

Results: We obtained hepatic TE data from 155 pediatric CF patients. Forty-four classified as CFRLD, 38 (86%) had CFRLD without cirrhosis and 6 (14%) had cirrhosis. Fourteen patients without CFRLD (12%) had ultrasound abnormalities. Mean liver elastography value (kPa) was 4.7 (3.5-5.3) in non-CFRLD and 6.09 (4.4-6.7) in CFRLD (p=0.01;Tstudent [T]).

Conclusions: CFRLD is common in children with CF. Transient elastography is a useful method for diagnosis and follow-up, as higher values of TE are found in patients with CFRLD.

背景:囊性纤维化(CF)是一种常染色体隐性、慢性、潜在致死性遗传病。CF表现是由于CF跨膜受体转运体(CFTR)基因突变所致,该基因编码的蛋白质(CFTR)在其表达的上皮细胞中起阴离子转运作用,主要是氯。囊性纤维化相关肝病(CFRLD)包括一系列肝胆表现,其诊断和随访仍是一项挑战:方法:对西班牙 10 个囊性纤维化单位进行横断面描述性研究。获得临床和生化数据。根据 2017 年 ESPGHAN 标准,根据肝脏受累情况将患者分为 3 组。通过瞬态弹性成像(TE)评估肝脏硬度,并记录腹部超声波检查结果。使用 SPSS v25.0 进行统计:我们获得了155名儿科CF患者的肝脏TE数据。其中 44 例被归类为 CFRLD,38 例(86%)为无肝硬化的 CFRLD,6 例(14%)为肝硬化。14名无 CFRLD 的患者(12%)出现超声波异常。非CFRLD患者的平均肝脏弹性成像值(kPa)为4.7(3.5-5.3),CFRLD患者的平均肝脏弹性成像值(kPa)为6.09(4.4-6.7)(P=0.01;Tstudent [T]):CFRLD在CF儿童中很常见。瞬态弹性成像是诊断和随访的有效方法,因为在 CFRLD 患者中发现了较高的 TE 值。
{"title":"Assessment of cystic fibrosis related liver disease in a pediatric cohort.","authors":"S Vicente-Santamaría, M E Torres-Guerrero, M García-González, A Tabares-González, C Gascón-Galindo, C M López-Cárdenes, E Blitz-Castro, A Morales-Tirado, M I Mota Goitia, J R Gutiérrez Martínez, C Tutau Gómez, R García Romero, E Salcedo Lobato, L Peña Quintana, A Reyes Domínguez, E Torcuato Rubio, P Ortiz Pérez, A E Fernández-Lorenzo, A Moreno Álvarez, Alfonso Solar Boga, H Romero Rey, M Álvarez Beltrán, E Masip Simo, D González Jiménez","doi":"10.1016/j.gastrohep.2024.502321","DOIUrl":"https://doi.org/10.1016/j.gastrohep.2024.502321","url":null,"abstract":"<p><strong>Background: </strong>Cystic fibrosis (CF) is an autosomal recessive, chronic, potentially lethal genetic disease. CF manifestations are due to mutations in the CF transmembrane receptor transporter (CFTR) gene which codes for a protein (CFTR) that acts as an anion transporter, mainly chlorine, at epithelial cells where it is expressed. Cystic fibrosis related liver disease (CFRLD) includes a spectrum of hepatobiliary manifestations whose diagnosis and follow-up remains a challenge.</p><p><strong>Methods: </strong>Cross-sectional, descriptive study from 10 Spanish Cystic fibrosis Units. Clinical and biochemical data obtained. Patients categorized into 3 groups according to liver involvement based on ESPGHAN 2017 criteria. Liver stiffness assessed by transient elastography (TE) and findings from abdominal ultrasound recorded. Statistics performed using SPSS v25.0.</p><p><strong>Results: </strong>We obtained hepatic TE data from 155 pediatric CF patients. Forty-four classified as CFRLD, 38 (86%) had CFRLD without cirrhosis and 6 (14%) had cirrhosis. Fourteen patients without CFRLD (12%) had ultrasound abnormalities. Mean liver elastography value (kPa) was 4.7 (3.5-5.3) in non-CFRLD and 6.09 (4.4-6.7) in CFRLD (p=0.01;Tstudent [T]).</p><p><strong>Conclusions: </strong>CFRLD is common in children with CF. Transient elastography is a useful method for diagnosis and follow-up, as higher values of TE are found in patients with CFRLD.</p>","PeriodicalId":12802,"journal":{"name":"Gastroenterologia y hepatologia","volume":" ","pages":"502321"},"PeriodicalIF":2.2,"publicationDate":"2024-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142828337","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"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
期刊
Gastroenterologia y hepatologia
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