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Co-morbidity of cystic fibrosis and celiac disease in Scandinavian cystic fibrosis patients

Fluge, G.; Olesen, H. V.; Gilljam, M.; Meyer, Peter LU ; Pressler, T.; Storrosten, O. T.; Karpati, F. and Hjelte, L. (2009) In Journal of Cystic Fibrosis 8(3). p.198-202
Abstract
Background: The co-morbidity of cystic fibrosis (CF) and celiac disease (CD) has been reported sporadically since the 1960s. To our knowledge, this is the first time a systematic screening is performed in a large cohort of CF patients. Methods: Transglutaminase-IgA (TGA), endomysium-IgA (EMA) and total IgA in serum were measured in 790 CF patients (48% females, 86% with pancreatic insufficiency). Six patients were diagnosed with CD prior to the Study, all receiving a gluten-free diet. Patients with elevated TGA (>50 Units/mL) and a positive EMA test were offered a gastroscopy obtaining mucosal biopsies from the duodenum. Results: Four new cases of CD were diagnosed. Two additional patients had positive serological tests, but normal... (More)
Background: The co-morbidity of cystic fibrosis (CF) and celiac disease (CD) has been reported sporadically since the 1960s. To our knowledge, this is the first time a systematic screening is performed in a large cohort of CF patients. Methods: Transglutaminase-IgA (TGA), endomysium-IgA (EMA) and total IgA in serum were measured in 790 CF patients (48% females, 86% with pancreatic insufficiency). Six patients were diagnosed with CD prior to the Study, all receiving a gluten-free diet. Patients with elevated TGA (>50 Units/mL) and a positive EMA test were offered a gastroscopy obtaining mucosal biopsies from the duodenum. Results: Four new cases of CD were diagnosed. Two additional patients had positive serological tests, but normal biopsies. In total, 10 cases of CD (1.2%, 1:83) indicate a prevalence rate about three times higher than the general prevalence of CD in Norway and Sweden. No CD patients were detected in the Danish CF cohort. Patients diagnosed with untreated CD reported symptoms typical of both CF and CD (poor weight gain, loose and/or fatty stools, fatigue, irritability, abdominal pain). They improved after introduction of a gluten-free diet. Conclusions: Systematic screening for CD in a Scandinavian cohort of CF patients revealed a higher prevalence of CD than in the general population. Clinical signs of CD are difficult to differentiate from CF with malabsorption, and patients may go undiagnosed for a long time. In a population where CD is common we recommend screening for CD in patients with CF. (C) 2009 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Prevalence, Co-morbidity, Cystic fibrosis, Celiac disease
in
Journal of Cystic Fibrosis
volume
8
issue
3
pages
198 - 202
publisher
Elsevier
external identifiers
  • wos:000266461700006
  • scopus:67349246238
ISSN
1873-5010
DOI
10.1016/j.jcf.2009.02.002
language
English
LU publication?
yes
id
421a8039-8874-4040-b6e8-3e5a5823d79a (old id 1425058)
date added to LUP
2009-07-02 16:44:39
date last changed
2017-11-12 03:23:20
@article{421a8039-8874-4040-b6e8-3e5a5823d79a,
  abstract     = {Background: The co-morbidity of cystic fibrosis (CF) and celiac disease (CD) has been reported sporadically since the 1960s. To our knowledge, this is the first time a systematic screening is performed in a large cohort of CF patients. Methods: Transglutaminase-IgA (TGA), endomysium-IgA (EMA) and total IgA in serum were measured in 790 CF patients (48% females, 86% with pancreatic insufficiency). Six patients were diagnosed with CD prior to the Study, all receiving a gluten-free diet. Patients with elevated TGA (>50 Units/mL) and a positive EMA test were offered a gastroscopy obtaining mucosal biopsies from the duodenum. Results: Four new cases of CD were diagnosed. Two additional patients had positive serological tests, but normal biopsies. In total, 10 cases of CD (1.2%, 1:83) indicate a prevalence rate about three times higher than the general prevalence of CD in Norway and Sweden. No CD patients were detected in the Danish CF cohort. Patients diagnosed with untreated CD reported symptoms typical of both CF and CD (poor weight gain, loose and/or fatty stools, fatigue, irritability, abdominal pain). They improved after introduction of a gluten-free diet. Conclusions: Systematic screening for CD in a Scandinavian cohort of CF patients revealed a higher prevalence of CD than in the general population. Clinical signs of CD are difficult to differentiate from CF with malabsorption, and patients may go undiagnosed for a long time. In a population where CD is common we recommend screening for CD in patients with CF. (C) 2009 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.},
  author       = {Fluge, G. and Olesen, H. V. and Gilljam, M. and Meyer, Peter and Pressler, T. and Storrosten, O. T. and Karpati, F. and Hjelte, L.},
  issn         = {1873-5010},
  keyword      = {Prevalence,Co-morbidity,Cystic fibrosis,Celiac disease},
  language     = {eng},
  number       = {3},
  pages        = {198--202},
  publisher    = {Elsevier},
  series       = {Journal of Cystic Fibrosis},
  title        = {Co-morbidity of cystic fibrosis and celiac disease in Scandinavian cystic fibrosis patients},
  url          = {http://dx.doi.org/10.1016/j.jcf.2009.02.002},
  volume       = {8},
  year         = {2009},
}