Low immunoglobulin levels in patients with cystic fibrosis: reduced inflammation or sign of common variable immunodeficiency syndrome?
Details
Serval ID
serval:BIB_E036DCE9131C
Type
Inproceedings: an article in a conference proceedings.
Publication sub-type
Abstract (Abstract): shot summary in a article that contain essentials elements presented during a scientific conference, lecture or from a poster.
Collection
Publications
Institution
Title
Low immunoglobulin levels in patients with cystic fibrosis: reduced inflammation or sign of common variable immunodeficiency syndrome?
Title of the conference
Joint annual meeting of the Swiss Society for Pediatrics, Swiss Society of Pediatric Pneumology
Address
Crans Montana, Switzerland, June 17-18, 2010
ISBN
1424-7860
Publication state
Published
Issued date
2010
Peer-reviewed
Oui
Volume
140
Series
Swiss Medical Weekly
Pages
27S
Language
english
Notes
Meeting Abstract
Abstract
Introduction: In children with cystic fibrosis (CF), low immunoglobulin
(IgG) levels have been reported to be associated with significantly less
severe lung disease. However, decreased IgG can be a sign for common
variable immunodeficiency (CVID) and affect clinical outcome. The aim of
this study was to analyze clinical and serological data of patients having
low IgG levels in routine blood tests at annual assessment, particularly
their antibody response to polysaccharide antigens.
Method: Retrospective chart review of demographic data of CF
patients followed at the pediatric CF clinic throughout 2009. Clinical
parameters (genotype, pancreas sufficiency, FEV1), presence of
Pseudomonas aeruginosa (PA) and number of exacerbations per year
were correlated with immunoglobulin and vaccination antibodies levels
(antibodies to pneumococcal serotypes 14, 19, 23, 1, 5 and 7F
measured by enzyme-linked immune-sorbent assay).
Results: 4 out of 60 patients (6.7%) had lower IgG-levels for age.
Ages ranged from 1 year 8 months to 11 years, 2 boys, 2 girls. Three
patients were delF508 homozygotes, one heterozygote composite
delF508/G542X. All were pancreatic insufficient. FEV1 ranged from 74
to 108%. One patient never had colonization by PA, 2 had intermittent
PA colonization and one was chronically infected. After conjugated
vaccination all patients had protective antibodies against serotypes 14,
19, 23F. For serotypes not included in the vaccine, only one patient
had protective titers for 1 out of 3 serotypes. None of the patients had
received unconjugated pneumococcal vaccine. There was no
significant clinical difference in FEV1, PA colonization or number of
exacerbations according to IgG and vaccination antibody levels.
Conclusion: Cystic Fibrosis patients with low immunoglobulin levels have
normal antibody response to protein antigens. However, despite recurrent
infections, there seems to be delayed or deficient antibody response to
polysaccharide antigens. Prospective studies are needed to evaluate
the development of polysaccharide antibody responses in CF-patients
to monitor for CVID. With early detection of CF by newborn screening
program, long term follow up could be started early in childhood.
(IgG) levels have been reported to be associated with significantly less
severe lung disease. However, decreased IgG can be a sign for common
variable immunodeficiency (CVID) and affect clinical outcome. The aim of
this study was to analyze clinical and serological data of patients having
low IgG levels in routine blood tests at annual assessment, particularly
their antibody response to polysaccharide antigens.
Method: Retrospective chart review of demographic data of CF
patients followed at the pediatric CF clinic throughout 2009. Clinical
parameters (genotype, pancreas sufficiency, FEV1), presence of
Pseudomonas aeruginosa (PA) and number of exacerbations per year
were correlated with immunoglobulin and vaccination antibodies levels
(antibodies to pneumococcal serotypes 14, 19, 23, 1, 5 and 7F
measured by enzyme-linked immune-sorbent assay).
Results: 4 out of 60 patients (6.7%) had lower IgG-levels for age.
Ages ranged from 1 year 8 months to 11 years, 2 boys, 2 girls. Three
patients were delF508 homozygotes, one heterozygote composite
delF508/G542X. All were pancreatic insufficient. FEV1 ranged from 74
to 108%. One patient never had colonization by PA, 2 had intermittent
PA colonization and one was chronically infected. After conjugated
vaccination all patients had protective antibodies against serotypes 14,
19, 23F. For serotypes not included in the vaccine, only one patient
had protective titers for 1 out of 3 serotypes. None of the patients had
received unconjugated pneumococcal vaccine. There was no
significant clinical difference in FEV1, PA colonization or number of
exacerbations according to IgG and vaccination antibody levels.
Conclusion: Cystic Fibrosis patients with low immunoglobulin levels have
normal antibody response to protein antigens. However, despite recurrent
infections, there seems to be delayed or deficient antibody response to
polysaccharide antigens. Prospective studies are needed to evaluate
the development of polysaccharide antibody responses in CF-patients
to monitor for CVID. With early detection of CF by newborn screening
program, long term follow up could be started early in childhood.
Web of science
Create date
08/09/2010 14:53
Last modification date
20/08/2019 17:04
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