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Disease phenotype at diagnosis in pediatric Crohn's disease: 5-year analyses of the EUROKIDS Registry

(2013) INFLAMMATORY BOWEL DISEASES. 19(2). p.378-385
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Abstract
Background: It has been speculated that pediatric Crohn's disease (CD) is a distinct disease entity, with probably different disease subtypes. We therefore aimed to accurately phenotype newly diagnosed pediatric CD by using the pediatric modification of the Montreal classification, the Paris classification. Methods: Information was collected from the EUROKIDS registry, a prospective, web-based registry of new-onset pediatric IBD patients in 17 European countries and Israel. When a complete diagnostic workup was performed (ileocolonoscopy, upper gastrointestinal [GI] endoscopy, small bowel imaging), CD patients were evaluated for ileocolonic disease extent, esophagogastroduodenal involvement, and jejunal/proximal ileal involvement. Disease behavior and the occurrence of granulomas were also analyzed. Results: In all, 582 pediatric CD patients could be classified according to the Paris classification. Isolated terminal ileal disease (+/- limited cecal disease) was seen at presentation in 16%, isolated colonic disease in 27%, ileocolonic disease in 53%, and isolated upper GI disease in 4% of patients. In total, 30% had esophagogastroduodenal involvement and 24% jejunal/proximal ileal disease. Patients with L2 disease were less likely to have esophagogastroduodenal involvement or stricturing disease than patients with L1 or L3 disease. Terminal ileal disease and stricturing disease behavior were more common in children diagnosed after 10 years of age than in younger patients. Granulomas were identified in 43% of patients. Conclusions: Accurate phenotyping is essential in pediatric CD, as this affects the management of individual patients. Disease phenotypes differ according to age at disease onset. The Paris classification is a useful tool to capture the variety of phenotypic characteristics of pediatric CD.
Keywords
POPULATION-BASED COHORT, ONSET, PREVALENCE, granulomas, INFLAMMATORY-BOWEL-DISEASE, Paris classification, Crohn's disease, pediatrics, disease phenotype, CHILDREN, PEYERS-PATCHES, INCREASING INCIDENCE, CHANGING PATTERN, UPDATE, AGE

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MLA
de Bie, Charlotte I, Anders Paerregaard, Sanja Kolacek, et al. “Disease Phenotype at Diagnosis in Pediatric Crohn’s Disease: 5-year Analyses of the EUROKIDS Registry.” INFLAMMATORY BOWEL DISEASES 19.2 (2013): 378–385. Print.
APA
de Bie, C. I., Paerregaard, A., Kolacek, S., Ruemmele, F. M., Koletzko, S., Fell, J. M., Escher, J. C., et al. (2013). Disease phenotype at diagnosis in pediatric Crohn’s disease: 5-year analyses of the EUROKIDS Registry. INFLAMMATORY BOWEL DISEASES, 19(2), 378–385.
Chicago author-date
de Bie, Charlotte I, Anders Paerregaard, Sanja Kolacek, Frank M Ruemmele, Sibylle Koletzko, John ME Fell, Johanna C Escher, the EUROKIDS Porto IBD Working Group of ESPGHAN, and Stephanie Van Biervliet. 2013. “Disease Phenotype at Diagnosis in Pediatric Crohn’s Disease: 5-year Analyses of the EUROKIDS Registry.” Inflammatory Bowel Diseases 19 (2): 378–385.
Chicago author-date (all authors)
de Bie, Charlotte I, Anders Paerregaard, Sanja Kolacek, Frank M Ruemmele, Sibylle Koletzko, John ME Fell, Johanna C Escher, the EUROKIDS Porto IBD Working Group of ESPGHAN, and Stephanie Van Biervliet. 2013. “Disease Phenotype at Diagnosis in Pediatric Crohn’s Disease: 5-year Analyses of the EUROKIDS Registry.” Inflammatory Bowel Diseases 19 (2): 378–385.
Vancouver
1.
de Bie CI, Paerregaard A, Kolacek S, Ruemmele FM, Koletzko S, Fell JM, et al. Disease phenotype at diagnosis in pediatric Crohn’s disease: 5-year analyses of the EUROKIDS Registry. INFLAMMATORY BOWEL DISEASES. 2013;19(2):378–85.
IEEE
[1]
C. I. de Bie et al., “Disease phenotype at diagnosis in pediatric Crohn’s disease: 5-year analyses of the EUROKIDS Registry,” INFLAMMATORY BOWEL DISEASES, vol. 19, no. 2, pp. 378–385, 2013.
@article{4407525,
  abstract     = {Background: It has been speculated that pediatric Crohn's disease (CD) is a distinct disease entity, with probably different disease subtypes. We therefore aimed to accurately phenotype newly diagnosed pediatric CD by using the pediatric modification of the Montreal classification, the Paris classification. 
Methods: Information was collected from the EUROKIDS registry, a prospective, web-based registry of new-onset pediatric IBD patients in 17 European countries and Israel. When a complete diagnostic workup was performed (ileocolonoscopy, upper gastrointestinal [GI] endoscopy, small bowel imaging), CD patients were evaluated for ileocolonic disease extent, esophagogastroduodenal involvement, and jejunal/proximal ileal involvement. Disease behavior and the occurrence of granulomas were also analyzed. 
Results: In all, 582 pediatric CD patients could be classified according to the Paris classification. Isolated terminal ileal disease (+/- limited cecal disease) was seen at presentation in 16%, isolated colonic disease in 27%, ileocolonic disease in 53%, and isolated upper GI disease in 4% of patients. In total, 30% had esophagogastroduodenal involvement and 24% jejunal/proximal ileal disease. Patients with L2 disease were less likely to have esophagogastroduodenal involvement or stricturing disease than patients with L1 or L3 disease. Terminal ileal disease and stricturing disease behavior were more common in children diagnosed after 10 years of age than in younger patients. Granulomas were identified in 43% of patients. 
Conclusions: Accurate phenotyping is essential in pediatric CD, as this affects the management of individual patients. Disease phenotypes differ according to age at disease onset. The Paris classification is a useful tool to capture the variety of phenotypic characteristics of pediatric CD.},
  author       = {de Bie, Charlotte I and Paerregaard, Anders and Kolacek, Sanja and Ruemmele, Frank M and Koletzko, Sibylle and Fell, John ME and Escher, Johanna C and EUROKIDS Porto IBD Working Group of ESPGHAN, the and Van Biervliet, Stephanie},
  issn         = {1078-0998},
  journal      = {INFLAMMATORY BOWEL DISEASES},
  keywords     = {POPULATION-BASED COHORT,ONSET,PREVALENCE,granulomas,INFLAMMATORY-BOWEL-DISEASE,Paris classification,Crohn's disease,pediatrics,disease phenotype,CHILDREN,PEYERS-PATCHES,INCREASING INCIDENCE,CHANGING PATTERN,UPDATE,AGE},
  language     = {eng},
  number       = {2},
  pages        = {378--385},
  title        = {Disease phenotype at diagnosis in pediatric Crohn's disease: 5-year analyses of the EUROKIDS Registry},
  url          = {http://dx.doi.org/10.1002/ibd.23008},
  volume       = {19},
  year         = {2013},
}

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