1. To study the possible association between MBL-deficiency and the occurence and severity of infections in MBL-deficient CF patients.2. To study the possible association between MBL-deficiency and the severity of cystic fibrosis.3. To study the…
ID
Source
Brief title
Condition
- Skin and subcutaneous tissue disorders NEC
- Immunodeficiency syndromes
- Hepatobiliary neoplasms malignant and unspecified
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
1. Frequency and severity of infection
2. Lung transplantation
Secondary outcome
Secondary endpoints
1. Severity CF
2. Phagocytosis of micro-organisms, specific for CF
Background summary
About one third of the DUtch population has deficient plasma levels of the
protein mannose-binding lectin (MBL) and is thus MBL-deficient. MBL-deficiency
is associated with an increased susceptibility to infections, especially in
children and the immunocompromized. In patients with cystic fibrosis (CF) an
association has been found between MBL-deficiency and reduced lung function. We
hypothesize that MBL-deficient CF patients also have an increased
susceptibility to infection, possibly due to an increased risk of infection
with specific microorganisms. If there is an association, determination of MBL
phenotype or genotype might lead to the identification of at risk patients in
the future.
Study objective
1. To study the possible association between MBL-deficiency and the occurence
and severity of infections in MBL-deficient CF patients.
2. To study the possible association between MBL-deficiency and the severity of
cystic fibrosis.
3. To study the phagocytosis of different CF-specific microorganisms in
MBL-deficient and MBL-sufficient CF patients.
Study design
Prospective cohort study, multicenter
Study burden and risks
We want to take a blood sample of 5 ml once (10 ml in adults). Since blood is
drawn for routine care at least once a year from most CF patients, the burden
and risks of our research will be minimal.
Meibergdreef 9
1105 AZ Amsterdam
Nederland
Meibergdreef 9
1105 AZ Amsterdam
Nederland
Listed location countries
Age
Inclusion criteria
patients with cystic fibrosis
Exclusion criteria
inability/unwillingness to donate blood
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL12937.018.06 |