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

June 28, 2010 Hankje Escher

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USA registries

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European registries

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Longterm registry (Centocor)

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Pediatric IBD consortium (Heyman)

• Start 2000: prevalent and incident cases, ±

1600 patients

• 6 sites: San Francisco, Chicago, Atlanta, Houston,

Philadelphia, Boston

• Publications
• n:

▪ 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

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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

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EPIMAD (Colombel)

• Start 1988, pediatric

and adult IBD, ± 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

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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

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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

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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

• f 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

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Initiative of IBD working group of ESPGHAN

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Start May 2004 with 21 centres in 12 countries

• now 18 countries

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Prospective registration of newly diagnosed IBD patients

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Data entry:

• 2004 –

2006: electronic forms sent by email

• 2006 –

now: web-based registry

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Coordination in Rotterdam, The Netherlands

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

Cohort May 2004 – May 2009 n=2280 Study cohort n=2087

Exclusion: 4 patients > 18 years at diagnosis Exclusion: 143 patients retrospectively included Exclusion: 12 patients without diagnosis Exclusion: 34 patients with incorrect date of diagnosis*

CD (n=1227) IC (n=195) UC (n=665)

* Date of diagnosis > 1 month after date of registration

50 100 150 200 250

Royal Hospital for Children Bristol Birmingham Children's Hospital Polish-American Children's Hospital, Jagiellonian University Cracow Karolinska Institute Stockholm Hvidovre Hospital Copenhagen

• Dr. v. Haunersches Kinderspital, Ludwig-M aximillians-University M unich

Erasmus M C-Sophia Children's Hospital Rotterdam M edical University of Warsaw The Children's M emorial Health Institute Warsaw Chelsea and Westminster Hospital London University of Rome La Sapienza Children's Hospital Zagreb Charles University Teaching Hospital Hradec Kralove University of Athens University Children's M edical Centre Bonn Queen M ary's Hospital for Children Surrey M eyer Hospital Florence

• E. Wolfson M edical Centre Tel Aviv

Hopital Necker-Enfants M alades Paris Ostfold Central Hospital University Hospital Prague-M otol Edmond &amp; Lili Safra Children's Hospital, Sheba M edical Centre Tel Hashomer Semmelweis Egyetem I. sz. Gyermekklinika Budapest Hospital S. Joao Porto Universita degli Studi di Napoli Federico II Napoli Childrens hospital Ljublana UZ Gent Hospital Robert Debre, AP-HP Paris Akademiska Barnsjukhuset Uppsala Stiftung Deutsche Klinik fur Diagnostik GM BH Wiesbaden Children's Hospital, Technical University Dresden Koningin Paola Kinderziekenhuis Antwerpen ZNA Childrens Hospital Brescia Childrens University Hospital Riga

Total patients patients with missing/wrong data

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Mean age at diagnosis 12.5 years

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Mean diagnostic delay 5 months

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Ethnicity 86% Caucasian

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1st degree family history in 10.6%

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Extraintestinal symptoms in 18.3%

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Perianal disease in 9.1%

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Stenosis in 13.6%

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57% had complete Porto work-up

• = upper GI + ileocolonoscopy

+ small bowel imaging

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88% upper GI endoscopy

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88% colonoscopy

• 70% terminal ileum

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70% imaging

• f small

bowel (SBFT, MRI)

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95% terminal ileum visualised

• by

endoscopy

• r

radiology

1% 29% 29% 12% 18% 4% 6% 0% 5% 10% 15% 20% 25% 30% 35%

• nly upper GI

ileocolon + upper GI ileocolon colon + upper GI colon ileum + upper GI ileum

N=990

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Centocor; FDA and EMEA mandate

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USA (start 2007?)

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6 European (start 2009) countries

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Europe 10-15 sites

• France, Italy, Germany, UK, Belgium,

Netherlands

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5000 patients, 20 year follow-up

• 2000 Crohn’s

disease, IFX treated

• 2000 Crohn’s

disease, not IFX treated

• 1000 ulcerative

colitis/unclassified