Influencing Progression of Airway Disease in Primary Antibody Deficiency

Withdrawn

Conditions: Antibodydeficiencies
immune disorders
10021460

Registration Number: NL-OMON49108

Lead Sponsor: niversitair Medisch Centrum Utrecht

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Withdrawn

Sex: Not specified

Target Recruitment: 100

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 ILD score < 7, or;
* Baseline AD score > 5 and/or ILD score > 7 without clinical diagnosis of
severe respiratory insufficiency (defined as: saturations in room air <92% and
/ or oxygen dependency).
* Baseline pulmonary function (FEV1 and FVC >70% expected for age and body
weight / length)

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 <92%, and/ or oxygen dependency).
* Baseline pulmonary function (FEV1 and FVC >70% expected for age and body
weight / length)

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p><br /><br>Main study parameters/endpoints: 1. Progression of lung disease, determined by<br /><br>computed tomography (CT) scanning and pulmonary function test </p><br>

Secondary Outcome Measures