Disorder of antibody production humoral immunodeficiency
Conditions
Interventions
None listed
Sponsors
Eligibility
Inclusion criteria
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
Exclusion criteria: Cystic fibrosis, anatomical abnormalities of the respiratory tract, primairy ciliary dyskinesia. Age below 2 years of age.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Primairy parameters: B-cell differentiation and maturation defects. Immunological outcome: in vivo vaccination response (adults). History of response to vaccination (children). Clinical outcome: The presence of auto-immune disease and the appearance or progression (if already present at the moment of diagnosis) of permanent pulmonary damage. | — |
Secondary
| Measure | Time frame |
|---|---|
| Frequency of pneumonia after diagnosis Number of pneumonias with hospital admission before diagnosis Bronchiectasis before diagnosis Presence or appearance of malignacies Quality of life Nasopharyngeal carriage of bacteria Carriage of abdominal parasites | — |
Countries
Netherlands