View clinical trials related to Common Variable Immunodeficiency.
Filter by:Primary humoral immunodeficiency (PHID), such as common variable immunodefiency, are the most common symptomatic primary immunodeficiency in adults, in France. Patients are more prone to infections (particularly bacterial upper and lower respiratory tract infections), auto-immunity and atopic manifestations. Morbidity and mortality in PHID are mainly linked to the presence of bronchiectasis, which can lead to infections and to chronic respiratory failure. However, bronchiectasis in these patients can be asymptomatic for a long time. There is no known predictive factors to identify patients more susceptible to develop bronchiectasis and notably, there was no link between the number of previous infectious episodes and bronchiectasis. A marked IgM deficiency and switched memory B cell deficiency might be associated with bronchiectasis. Thoracic CT-scan is recommended at PHID diagnosis but there is no guideline for follow-up, thus leading to bronchiectasis being under-diagnosis or leading to delayed diagnosis
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.
The purpose of this study is to determine if the supplement, Fisetin, can be used as a treatment option for common variable immunodeficiency (CVID) by comparing its efficacy to placebo.
This aim of this study is the evaluation of the gut microbiota imbalance occurrence and its characterization in patients with common variable immunodeficiency associated to an enteropathy with or without porto-sinusoidal vascular disease.
The goal of our study is to assess the cellular immune responses of participants with antibody deficiency disease before and after immunization with SARS-CoV-2 mRNA vaccines.
Rationale: Primary antibody deficiencies (PAD) encompass a group of rare heterogeneous diseases. The clinical presentation may vary widely, including infectious and autoimmune symptoms and increased risk of malignancy. Due to the rarity of the diseases and this wide array of symptoms there is often a delay in diagnosis, of up to 12 years on average1-4. Timely diagnosis of PAD reduces morbidity, mortality and health care costs as effective therapies are available. The currently available screening systems for the broader group of primary immunodeficiencies (PID) have been shown to have poor diagnostic performance5-10 and are time consuming. We have thus developed an algorithm to screen patient records in a primary care setting for risk factors specifically for PAD. Patients with a high risk may undergo a laboratory assessment and referral if necessary, thus reducing the diagnostic delay of PAD. The aim of the current study is to validate this algorithm. Objective: Main objective: to validate a screening algorithm for PAD in a primary care setting in the Netherlands. Study design: Mono-centre cohort study based on regular care data Study population: Primary care patients aged 12-70 years with the 100 highest scores based on our algorithm.
Although there is evidence in the literature that gammaglobulin replacement therapy can lead to a reduction in the prevalence of pulmonary infection and improved lung function, there is no published study to guide immunologists regarding the use of spirometry in titrating IG therapy to assist in the management of immunodeficiency patients with regards to gammaglobulin replacement therapy. The investigators propose to study the use of spirometry to identify patients that could potentially benefit from an increase in IGRT. The investigators will identify 22 common variable immune deficiency (CVID) study subjects on stable IGRT replacement therapy equivalent to 0.40 to 0.60 gm/kg per 4 weeks who have evidence of mild to moderate obstruction as assessed by an FEF25-75% between 50% and 80% of predicted. Patients who are on Hizentra will be preferentially recruited. Of these 22, 11 will be identified at random and treated for 6 months at their current dose (control population). The remaining 11 study subjects (treatment group) will have their level of IGRT increased by the equivalent of 0.05 gm/kg in dose per 4 weeks, adjusted for bioavailability as per manufacturer's instructions. On average, rounded up to the nearest gram, this will typically increase their dose of Hizentra by 2 gm per week.
There is no standard of care therapy for patients with granulomatous-lymphocytic interstitial lung disease (GLILD) seen in common variable immunodeficiency (CVID). Abatacept has recently looked promising for the treatment of patients with complex CVID. This study is a multi-site, phase II, randomized, blinded/placebo-controlled clinical trial in pediatric and adult subjects to determine the efficacy of abatacept compared to placebo for treatment of subjects with GLILD in the context of CVID. Funding Source - FDA OOPD
Background: During a transplant, blood stem cells from one person are given to someone else. The cells grow into the different cells that make up the immune system. This can cure people with certain immunodeficiencies. But transplant has many risks and complications. Objective: To see if stem cell transplant can be successfully performed in people with primary immunodeficiency disease and cure them. Eligibility: People ages 4-69 for whom a primary immunodeficiency (PID) or Primary Immune Regulatory Disorder (PIRD), has caused significant health problems and either standard management has not worked or there are no standard management options, along with their donors Design: Donors will be screened under protocol 01-C-0129. They will donate blood or bone marrow. Participants will be screened with: Medical history Physical exam Blood, urine, and heart tests CT or PET scans Before transplant, participants will have dental and eye exams. They will have a bone marrow biopsy. For this, a needle will be inserted through the skin into the pelvis to remove marrow. Participants will be hospitalized before their transplant. They will have a central catheter put into a vein in their chest or neck. They will get medications through the catheter to prevent complications. Participants will get stem cells through the catheter. They will stay in the hospital for at least 4 weeks. They will give blood, urine, bone marrow, and stool samples. They may need blood transfusions. They may need more scans. They will take more medications. Participants will have visits on days 30, 60, 100, 180, and 360, and 24 months after the transplant. Then they will have visits once a year for about 5 years
Background: Blood stem cells in the bone marrow make all the cells to normally defend a body against disease. Allogeneic blood or marrow transplant is when these stem cells are transferred from one person to another. Researchers think this treatment can provide a new, healthy immune system to correct T-cell problems in some people. Objective: To see if allogeneic blood or bone marrow transplant is safe and effective in treating people with T-cell problems. Eligibility: Donors: Healthy people ages 4 and older Recipients: People the same age with abnormal T-cell function causing health problems Design: All participants will be screened with: - Medical history - Physical exam - Blood, heart, and urine tests Donors will also have an electrocardiogram and chest x-ray. They may have veins tested or a pre-anesthesia test. Recipients will also have lung tests. Some participants will have scans and/or bone marrow collected by needle in the hip bones. Donors will learn about medicines and activities to avoid and repeat some screening tests. Some donors will stay in the hospital overnight and have bone marrow collected with anesthesia. Other donors will get shots for several days to stimulate cells. They will have blood removed by plastic tube (IV) in an arm vein. A machine will remove stem cells and return the rest of the blood to the other arm. Recipients will have: - More bone marrow and a small fragment of bone removed - Dental, diet, and social worker consultations - Scans - Chemotherapy and antibody therapy for 2 weeks - Catheter inserted in a chest or neck vein to receive donor stem cells - A hospital stay for several weeks with more medicines and procedures - Multiple follow-up visits