June 28, 2010 Hankje Escher USA registries European registries - PowerPoint PPT Presentation

June 28, 2010 Hankje Escher

USA registries  European registries  Longterm registry (Centocor) 

Pediatric IBD consortium (Heyman)  Start 2000: prevalent and incident cases, ± 1600  patients 6 sites: San Francisco, Chicago, Atlanta, Houston,  Philadelphia, Boston Publications on:  ▪ Phenotype ▪ IFX premedication and infusion reactions ▪ Initial surgery risk in CD ▪ Gender related phenotype and disease course ▪ Age at onset-related phenotype and disease course ▪ Racial/ethnic differences ▪ Extraintestinal manifestations ▪ Complications of CD

Pediatric IBD Collaborative Research Group  (Hyams) Start 2002, consecutive incident cases < 16 years, ± 250  cases in 18 Eastern US and Canadian centers  prospective registration by research nurses  Publications on:  ▪ Response to CS in UC ▪ Response to CS and influence of IFX in CD ▪ QoL (IMPACT) at diagnosis and 1 year follow-up ▪ Lab tests at diagnosis ▪ Perianal disease in CD ▪ IFX maintenance in CD ▪ Appraisal of PUCAI ▪ IFX maintenance in UC

EPIMAD (Colombel)  and adult IBD, ± Start 1988, pediatric 500 pediatric  patients Population based inception cohort in Northern France  Interviewer practitioners collect data from pediatric  gastroenterologists Publications on:  ▪ Environmental risk factors in pediatric IBD ▪ Incidence and disease location ▪ Natural history of CD ▪ Malnutrition and Growth in CD

CEDATA (Buderus)  Germany and Austria, start 2004, ± 600  patients Prevalent and incident cases, reported by  pediatric gastroenterologists Data on:  ▪ disease phenotype at presentation ▪ first treatment

SIGENP IBD Study Group  Italian Pediatric National pediatric IBD register  start 1996, patients at diagnosis, ± 1500  patients all 40 ped gastroenterology centers in Italy  retrospective and prospective  Publication on:  ▪ Incidence and phenotype

EUROKIDS (Escher)  Web-based registry of ESPGHAN IBD working group  in 18 European countries and Israel  incident cases < 18 years, registration at diagnosis  Start 2004, ongoing, now ± 3000 patients  Continued registration of new patients  Open for new participants, new countries  Data on:  ▪ Diagnostic work-up ▪ Disease location in CD ▪ Disease location in UC and IBD-U ▪ Height and weight at diagnosis ▪ Colitis in the very young

Initiative of IBD working group of ESPGHAN  Start May 2004 with 21 centres in 12 countries  now 18 countries  Prospective registration of newly diagnosed IBD  patients Data entry:  2004 – 2006: electronic forms sent by email • 2006 – now: web-based registry • Coordination in Rotterdam, The Netherlands 

Norway Norway Sweden Sweden Latvia Latvia Denmark Denmark Netherlands Netherlands United Kingdom United Kingdom Germany Germany Poland Poland Belgium Belgium Czech Republic Czech Republic France France Hungary Hungary Slovenia Slovenia Croatia Croatia Portugal Portugal Italy Italy Greece Greece Israel Israel

Cohort May 2004 – May 2009 n=2280 Exclusion: Exclusion: 143 patients retrospectively 12 patients without diagnosis included Exclusion: Exclusion: 34 patients with incorrect date of 4 patients > 18 years at diagnosis diagnosis* Study cohort n=2087 CD (n=1227) IC (n=195) UC (n=665) * Date of diagnosis > 1 month after date of registration

Childrens University Hospital Riga Childrens Hospital Brescia Koningin Paola Kinderziekenhuis Antwerpen ZNA Children's Hospital, Technical University Dresden Stiftung Deutsche Klinik fur Diagnostik GM BH Wiesbaden Akademiska Barnsjukhuset Uppsala Hospital Robert Debre, AP-HP Paris UZ Gent Childrens hospital Ljublana Universita degli Studi di Napoli Federico II Napoli Hospital S. Joao Porto Semmelweis Egyetem I. sz. Gyermekklinika Budapest Edmond &amp; Lili Safra Children's Hospital, Sheba M edical Centre Tel Hashomer University Hospital Prague-M otol Ostfold Central Hospital Hopital Necker-Enfants M alades Paris E. Wolfson M edical Centre Tel Aviv M eyer Hospital Florence Queen M ary's Hospital for Children Surrey University Children's M edical Centre Bonn University of Athens Charles University Teaching Hospital Hradec Kralove Children's Hospital Zagreb University of Rome La Sapienza Chelsea and Westminster Hospital London The Children's M emorial Health Institute Warsaw M edical University of Warsaw Erasmus M C-Sophia Children's Hospital Rotterdam Dr. v. Haunersches Kinderspital, Ludwig-M aximillians-University M unich Hvidovre Hospital Copenhagen Karolinska Institute Stockholm Polish-American Children's Hospital, Jagiellonian University Cracow Birmingham Children's Hospital Royal Hospital for Children Bristol 0 50 100 150 200 250 Total patients patients with missing/wrong data

Mean age at diagnosis 12.5 years  Mean diagnostic delay 5 months  Ethnicity 86% Caucasian  1 st degree family history in 10.6%  Extraintestinal symptoms in 18.3%  Perianal disease in 9.1%  Stenosis in 13.6% 

57% had complete Porto work-up  = upper GI + ileocolonoscopy + small bowel imaging  88% upper GI endoscopy  88% colonoscopy  70% terminal ileum  70% imaging of small bowel (SBFT, MRI)  95% terminal ileum visualised  by endoscopy or radiology 

6% ileum 4% ileum + upper GI 18% colon 12% colon + upper GI 29% ileocolon 29% ileocolon + upper GI 1% only upper GI 0% 5% 10% 15% 20% 25% 30% 35% N=990

Centocor; FDA and EMEA mandate  USA (start 2007?)  6 European (start 2009) countries  Europe 10-15 sites  France, Italy, Germany, UK, Belgium,  Netherlands 5000 patients, 20 year follow-up  2000 Crohn’s disease, IFX treated  2000 Crohn’s disease, not IFX treated  1000 ulcerative colitis/unclassified 