Evaluation of Ultrasound Parameters and Liver and Spleen Stiffness for Prediction of Clinically Significant Portal Hypertension in Patients With Common Variable Immunodeficiency Syndrome
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 发起方
- 入组人数
- 250
- 试验地点
- 2
- 主要终点
- Differences in liver stiffness between CVID patients with and without liver involvement and with clinically significant portal hypertension.
研究概览
简要总结
Patients with CVID will be offered to participate in this observational trial during the routine annual visit in the outpatient clinic at the Center of chronic Immunodeficiency (CCI) of the University Medical Center Freiburg, Germany.
Clinical and laboratory data at the time of presentation will be assessed. Additionally, parameters of abdominal ultrasound, duplex sonography of the liver and spleen, and liver and spleen stiffness at the time of presentation will be evaluated. If applicable, clinical and/or interventional parameters indicating clinically significant portal hypertension (i.e. presence of varices or portal-hypertensive gastropathy in esophago-gastroduodenoscopy, presence of ascites) within 12 months prior and after the index visit will be assessed. During the visit, serum/plasma samples and peripheral blood mononuclear cells (PBMC) are collected and stored in an associated biobank.
详细描述
Patients with CVID can be included in the study. The study cohort will consist of three subgroups: 1) Patients with CVID and autoimmunity, but no liver involvement. 2) Patients with CVID and liver involvement, defined by elevated liver enzymes, abnormal ultrasound and/or abnormal liver biopsy, but no signs of clinically significant portal hypertension and 3) Patients with CVID and clinically significant non-cirrhotic portal hypertension. Further, patients with CVID and diagnosed non-cirrhotic portal hypertension between 01/01/2004 and the start of the prospective part of this study (01/01/2023) will be included retrospectively in this study.
Patients will be recruited during the routine annual visit in the outpatient clinic at the Center of chronic Immunodeficiency (CCI) of the University Medical Center Freiburg, Germany. During the visit a standard ultrasound protocol, including size and morphology of liver and spleen, duplex-sonography of the liver, and measurement of liver and spleen stiffness via transient elastography will be performed as a part of the routine clinical diagnostic. Results from endoscopy or other interventions (esophago-gastroduodenoscopy, invasive measurement of hepatic venous-portal pressure gradient, or TIPS implantation, if applicable) within 12 months prior and after the index visit will be assessed. All non-invasive and invasive interventions will be conducted solely based on clinical decisions made independently from the study inclusion as the study is designed as an observational study.
Detailed patient characteristics, epidemiologic, clinical, imaging and laboratory parameters will be assessed and included in an electronic database.
Apart from these data, patients will be asked to participate in biobank sampling including serum/plasma and peripheral blood mononuclear cells (PBMC) samples from the peripheral veins.
All patients recruited in this study will be followed-up for at least 12 months.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Patients with CVID
排除标准
- •no written informed consent
- •concomitant chronic liver disease (viral hepatitis, alcoholic liver disease, steatitic liver disease, hemochromatosis, primary biliary cholangitis, primary sclerosis cholangitis, M. Wilson, alpha-1-antitrypsin deficiency)
研究组 & 干预措施
Patients with CVID and clinically significant non-cirrhotic portal hypertension
Patients with CVID and clinically significant non-cirrhotic portal hypertension (indicated by the presence of ascites or esophageal varices / portal-hypertensive gastropathy).
干预措施: Ultrasound including color doppler ultrasound (Diagnostic Test)
Patients with CVID and autoimmunity and no liver involvement.
Patients with CVID and autoimmune disease, but no liver involvement (normal liver enzymes and normal abdominal ultrasound)
干预措施: Ultrasound including color doppler ultrasound (Diagnostic Test)
Patients with CVID and liver involvement and no clinical significant portal hypertension
Patients with CVID and liver involvement (elevated liver enzymes, abnormal ultrasound and/or abnormal liver biopsy), but no clinically significant portal hypertension
干预措施: Ultrasound including color doppler ultrasound (Diagnostic Test)
结局指标
主要结局
Differences in liver stiffness between CVID patients with and without liver involvement and with clinically significant portal hypertension.
时间窗: Study inclusion, after 12 months
Liver stiffness will be assessed by transient elastography.
Differences in spleen stiffness between CVID patients with and without liver involvement and with clinically significant portal hypertension.
时间窗: Study inclusion, after 12 months
Spleen stiffness will be assessed by transient elastography.
次要结局
- Differences in alanine aminotransfersases (ALT) between CVID patients with and without liver involvement and with clinically significant portal hypertension.(Study inclusion, after 12 months)
- Differences in portal vein diameter between CVID patients with and without liver involvement and with clinically significant portal hypertension.(Study inclusion, after 12 months)
- Differences in aspartat aminotransfersases (AST) between CVID patients with and without liver involvement and with clinically significant portal hypertension.(Study inclusion, after 12 months)
- Differences in portal vein flow velocity between CVID patients with and without liver involvement and with clinically significant portal hypertension.(Study inclusion, after 12 months)
- Differences in liver function tests (bilirubin, albumin, INR) between CVID patients with and without liver involvement and with clinically significant portal hypertension.(Study inclusion, after 12 months)
- Differences in platelets between CVID patients with and without liver involvement and with clinically significant portal hypertension.(Study inclusion, after 12 months)
研究者
Prof. Dr. Dominik Bettinger
Principal Investigator
University Hospital Freiburg