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Pediatric IBD-unclassified Is Less Common than Previously Reported; Results of an 8-Year Audit of the EUROKIDS Registry

Winter, Dwight A. MD1; Karolewska-Bochenek, Katarzyna MD, PhD2; Lazowska-Przeorek, Izabella MD, PhD2; Lionetti, Paolo MD, PhD3; Mearin, M. Luisa MD, PhD4; Chong, Sonny K. MD5; Roma-Giannikou, Eleftheria MD, PhD6; Maly, Jan MD, PhD7; Kolho, Kaija-Leena MD, PhD8; Shaoul, Ron MD, PhD9; Staiano, Annamaria MD, PhD10; Damen, Gerard M. MD, PhD11; de Meij, Tim MD12; Hendriks, Daniëlle MD13; George, Elvira K. MD, PhD14; Turner, Dan MD, PhD15; Escher, Johanna C. MD, PhD1the Paediatric IBD Porto Group of ESPGHAN

doi: 10.1097/MIB.0000000000000483
Original Clinical Articles

Background: Inflammatory bowel disease–unclassified (IBD-U) is diagnosed in ∼10% of pediatric and adolescent onset IBD patients. The EUROKIDS registry (2004) initiated by the Porto IBD working group of ESPGHAN prospectively monitors diagnostic workup of newly diagnosed pediatric and adolescent onset IBD patients. We aimed to describe diagnostic workup, phenotype, and change of diagnosis over time in pediatric IBD-U patients.

Methods: Data were collected on children from 52 centers across 20 European countries and Israel, diagnosed with IBD from May 2005 through November 2013. Full endoscopy plus small bowel radiology was considered complete diagnostic workup. Participating centers reporting IBD-U patients were queried in 2014 for follow-up data.

Results: IBD-U was the provisional first diagnosis in 265 of 3461 children (7.7%) (91/158 [58%] with pancolitis; 140 [53%] male), diagnosed more frequently under the age of 10 (median age 12.3 years, 89 [34%] under 10 years). Half (48%) had undergone complete diagnostic workup. Lack of small bowel radiology was the prevailing reason for incomplete workup. As a result of reinvestigations (endoscopy in 54%, radiology in 38%) during a median follow-up of 5.7 years (interquartile range, 2.5–7.8), a change in diagnosis from IBD-U to Crohn's disease (12%) or ulcerative colitis (20%) was reported.

Conclusions: Only half of patients reported as IBD-U in EUROKIDS had undergone complete diagnostic workup. Follow-up with reinvestigations resulted in a reduction of IBD-U rate to 5.6%. A diagnosis of IBD-U becomes less likely in case of complete diagnostic workup. Implementation of clear diagnostic criteria will further reduce the rate of IBD-U in the future.

Article first published online 9 July 2015.Supplemental Digital Content is Available in the Text.

1Pediatric Gastroenterology, Department of Pediatrics, Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, the Netherlands;

2Department of Pediatric Gastroenterology and Nutrition, The Medical University of Warsaw, Warsaw, Poland;

3Gastroenterology and Nutrition Unit, Meyer Pediatric Hospital, Florence, Italy;

4Department of Pediatrics, Leiden University Medical Center, Leiden, the Netherlands;

5Department of Pediatric Gastroenterology, Queen Mary's Hospital for Children, Surrey, United Kingdom;

6First Department of Pediatrics, Athens University, Athens, Greece;

7Department of Pediatrics, Charles University in Prague, Hradec Kralove, Czech Republic;

8Department of Pediatric Gastroenterology, Helsinki Children's Hospital, Helsinki, Finland;

9Pediatric Gastroenterology Unit, Meyer Children's Hospital, Haifa, Israel;

10Department of Pediatrics, University of Naples, Naples, Italy;

11Department of Pediatric Gastroenterology, Radboud University Medical Centre, Nijmegen, the Netherlands;

12Department of Pediatric Gastroenterology, VU University Medical Center, Amsterdam, the Netherlands;

13Department of Pediatrics, Juliana Children's Hospital, The Hague, the Netherlands;

14Department of Pediatrics, Medical Center Alkmaar, Alkmaar, the Netherlands; and

15Pediatric Gastroenterology Unit, Shaare Zedek Medical Center, The Hebrew University of Jerusalem, Jerusalem, Israel.

Reprints: Johanna C. Escher, MD, PhD, Pediatric Gastroenterology, Department of Pediatrics, Erasmus Medical Center-Sophia Children's Hospital, P.O. Box 2060, 3000 CB, Rotterdam, the Netherlands 7036811 (e-mail:

These findings are the result of data collected in the EUROKIDS registry, supported by the Paediatric IBD Porto Group of ESPGHAN. The views expressed in this article are those of the authors, no official endorsement by the Paediatric IBD Porto Group of ESPGHAN is intended or should be inferred.

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Web site (

The overview of contributing investigators from the Paediatric IBD Porto Group of ESPGHAN are listed in the Table, Supplemental Digital Content 1,

The authors have no conflicts of interest to disclose.

Received February 18, 2015

Accepted April 17, 2015

© Crohn's & Colitis Foundation of America, Inc.
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