Infantile and Very Early Onset Inflammatory Bowel Disease: A Multicenter Study

Author:

Guz-Mark Anat1,Aloi Marina2,Scarallo Luca3,Bramuzzo Matteo4,Escher Johanna C.5,Alvisi Patrizia6,Henderson Paul7,Hojsak Iva8,Lev-Tzion Raffi9,El-Matary Wael10,Schwerd Tobias11,Granot Maya12,Sladek Malgorzata13,Strisciuglio Caterina14,Müller Katalin E.15,Olbjørn Christine16,Tzivinikos Christos17,Yerushalmy-Feler Anat18,Huysentruyt Koen19,Norsa Lorenzo20,Viola Irene21,de Ridder Lissy5,Shouval Dror S.1,Lega Sara4,Lionetti Paolo22,Catassi Giulia2,Assa Amit923,

Affiliation:

1. aInstitute of Gastroenterology, Nutrition and Liver Diseases, Schneider Children’s Medical Center of Israel, Petach Tikva, Tel-Aviv University, Tel-Aviv, Israel

2. bPediatric and Gastroenterology Unit, Sapienza University of Rome-Umberto I Hospital, Rome, Italy

3. cGastroenterology and Nutrition Unit, Meyer children’s Hospital, Florence, Italy

4. dGastroenterology, Digestive Endoscopy and Nutrition Unit, Institute for Maternal and Child Health-IRCCS “Burlo Garofolo,” Trieste, Italy

5. eDepartment of Pediatric Gastroenterology, Erasmus MC-Sophia Children’s Hospital, Rotterdam, Netherlands

6. fPediatric Gastroenterology unit, Maggiore Hospital, Bologna, Italy

7. gDepartment of Paediatric Gastroenterology and Nutrition, Royal Hospital for Children and Young People, Edinburgh, Scotland

8. hChildren’s Hospital Zagreb, University of Zagreb Medical School, Zagreb, Croatia

9. iJuliet Keidan Institute of Pediatric Gastroenterology Hepatology and Nutrition, Shaare Zedek Medical Center, Jerusalem, Israel

10. jPediatric Gastroenterology, University of Manitoba, Winnipeg, Canada

11. kDepartment of Pediatrics, Dr von Hauner Children’s Hospital, University Hospital, LMU Munich, Munich, Germany

12. lPediatric Gastroenterology Unit, Edmond & Lily Safra Children’s Hospital, Sheba Medical Center, Ramat-Gan, Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel

13. mDepartment of Pediatrics, Gastroenterology and Nutrition, Jagiellonian University Medical College, Krakow, Poland

14. nDepartment of Woman, Child and General and Specialistic Surgery, University of Campania Vanvitelli, Naples, Italy

15. oHeim Pal National Institute for Pediatrics, Budapest, Institute for Translational Medicine, University of Pécs, and Department of Family Care Methodology, Semmelweis University, Budapest, Hungary

16. pDepartment of Pediatric and Adolescent Medicine, Akershus University Hospital, Lørenskog, Norway

17. qPaediatric Gastroenterology Department, Al Jalila Children’s Hospital, Mohammed Bin Rashid University, Dubai, United Arab Emirates

18. rPediatric Gastroenterology Institute, “Dana-Dwek” Children’s Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel

19. sPaediatric gastroenterology, KidZ Health Castle, UZ Brussel, Vrije Universiteit Brussel, Brussels, Belgium

20. tPediatric Hepatology, Gastroenterology and Transplantation, ASST Papa Giovanni XXIII, Bergamo, Italy

21. uPediatric Gastroenterology and Cystic Fibrosis Unit, Department of Human Pathology in Adulthood and Childhood “G. Barresi,” University of Messina, Italy

22. vDepartment NEUROFARBA, Gastroenterology and Nutrition Unit, Meyer Children’s Hospital, University of Florence, Florence, Italy

23. wThe Hebrew University of Jerusalem, Jerusalem, Israel

Abstract

OBJECTIVES This study described disease characteristics and long-term outcomes in patients diagnosed with very early onset inflammatory bowel disease (VEOIBD) (diagnosed before 6 years of age) and infantile-IBD (before 2 years). METHODS Cases from 21 centers worldwide diagnosed with VEOIBD (2008–2018), with minimum 2 years of follow-up, were retrospectively reviewed. RESULTS The cohort included 243 patients (52% males, median follow-up of 5.8 [range 2−18] years, including 69 [28%]) with infantile-IBD. IBD subtypes included Crohn’s disease (CD), ulcerative colitis (UC), or IBD-unclassified (IBDU) in 30%, 59%, and 11%, respectively. Among patients with CD, 94% had colonic involvement, and among patients with UC/IBDU, 75% had pancolitis. Patients with infantile-IBD presented with higher rates of IBDU, lower hemoglobin and albumin levels, and higher C-reactive protein, and had lower response rates to first-induction therapy and corticosteroids therapy (P < .05 for all). Colectomy and diversion surgeries were performed in 11% and 4%, respectively, with no significant differences between age groups. Corticosteroid-free remission rates were 74% and 78% after 3 and 5 years, respectively, and 86% at end of follow-up. Genetic testing was performed in 96 (40%) patients. Among tested population, 15 (16%) were identified with monogenic disease. This group demonstrated lower response rates to induction therapies, higher rates of surgical intervention, and higher rates of major infections (P < .05 for all). CONCLUSIONS Patients with VEOIBD, including infantile-IBD, exhibit low rate of complications and surgical interventions at the long term. Patients with monogenic IBD are at risk for more severe disease course.

Publisher

American Academy of Pediatrics (AAP)

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