B-cell differentiation defects and clinical complications in antibody deficiency syndromes with a focus on Common Variable Immunodeficiency
Completed
- Conditions
- Disorder of antibody productionhumoral immunodeficiency100214291000381610019815
Recruitment & Eligibility
- Status
- Completed
- Sex
- Not specified
- Target Recruitment
- 130
Inclusion Criteria
Patients with antibody deficiency syndromes and patients with clinical complications that can be the result of antibody deficency syndromes like bronchiectasis of unknown origin or repeated severe ENT infections without known cause despite extensive examinations.
Exclusion Criteria
Cystic fibrosis, anatomical abnormalities of the respiratory tract, primairy ciliary dyskinesia. Age below 2 years of age.
Study & Design
- Study Type
- Observational non invasive
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method <p>Primairy parameters:<br /><br>B-cell differentiation and maturation defects.<br /><br><br /><br>Immunological outcome: in vivo vaccination response (adults). History of<br /><br>response to vaccination (children).<br /><br><br /><br>Clinical outcome:<br /><br>The presence of auto-immune disease and the appearance or progression (if<br /><br>already present at the moment of diagnosis) of permanent pulmonary damage.</p><br>
- Secondary Outcome Measures
Name Time Method <p>Frequency of pneumonia after diagnosis<br /><br>Number of pneumonias with hospital admission before diagnosis<br /><br>Bronchiectasis before diagnosis<br /><br>Presence or appearance of malignacies<br /><br>Quality of life<br /><br>Nasopharyngeal carriage of bacteria<br /><br>Carriage of abdominal parasites</p><br>