Antibodydeficiencies immune disorders
Conditions
Interventions
None listed
Sponsors
Universitair Medisch Centrum Utrecht
Eligibility
Age
2 Years to 99 Years
Inclusion criteria
Inclusion criteria: In order to be eligible to participate in this study, a subject must meet all of the following criteria: -Age 4-60 years -Diagnosis of Primary Antibody Deficiency / Common Variable Immunodeficiency Disorder (see Appendix A). -Indication for immunoglobulin replacement therapy and/or treated with immunoglobulin replacement therapy -Current IgG dosing 0.4 - 0.6 gr / kg / 3-4 weeks -Receiving treatment and follow up for PAD by one of the physicians in the participating centers -Written informed consent -Normal lung status, or mild to medium severe pulmonary disease (measured by pulmonary function test and on CT scan, scored by an independent radiologist based on the following criteria): * Baseline AD score 5 and/or ILD score > 7 without clinical diagnosis of severe respiratory insufficiency (defined as: saturations in room air 70% expected for age and body weight / length)
Exclusion criteria
Exclusion criteria: A potential subject who meets any of the following criteria will be excluded from participation in this study: -Age above 60 year -Diagnosis of Combined Immunodeficiency (CID) disease at onset of study (see Appendix A). Explanation: Combined Immunodeficiency is featured by the occurrence of more viral infections and reactivations and thus less comparable to PAD. -Severe pulmonary disease, determined by an independent radiologist: * Baseline AD score > 5 and/or ILD score > 7, in combination with: o Saccular bronchiectasis on CT scan, or; o Clinical diagnosis of severe respiratory insufficiency (defined as: (defined as: saturations in room air 70% expected for age and body weight / length)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Main study parameters/endpoints: 1. Progression of lung disease, determined by computed tomography (CT) scanning and pulmonary function test | — |
Secondary
| Measure | Time frame |
|---|---|
| 2. Number of respiratory infections 3. Days missed from school/work. 4. Cost savings 5. Quality of life. | — |
Countries
Netherlands
Outcome results
None listed