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

Autor:	Guz-Mark A; Institute of Gastroenterology, Nutrition and Liver Diseases, Schneider Children's Medical Center of Israel, Petach Tikva, Tel-Aviv University, Tel-Aviv, Israel., Aloi M; Pediatric and Gastroenterology Unit, Sapienza University of Rome-Umberto I Hospital, Rome, Italy., Scarallo L; Gastroenterology and Nutrition Unit, Meyer children's Hospital, Florence, Italy., Bramuzzo M; Gastroenterology, Digestive Endoscopy and Nutrition Unit, Institute for Maternal and Child Health-IRCCS 'Burlo Garofolo,' Trieste, Italy., Escher JC; Department of Pediatric Gastroenterology, Erasmus MC-Sophia Children's Hospital, Rotterdam, Netherlands., Alvisi P; Pediatric Gastroenterology unit, Maggiore Hospital, Bologna, Italy., Henderson P; Department of Paediatric Gastroenterology and Nutrition, Royal Hospital for Children and Young People, Edinburgh, Scotland., Hojsak I; Children's Hospital Zagreb, University of Zagreb Medical School, Zagreb, Croatia., Lev-Tzion R; Juliet Keidan Institute of Pediatric Gastroenterology Hepatology and Nutrition, Shaare Zedek Medical Center, Jerusalem, Israel., El-Matary W; Pediatric Gastroenterology, University of Manitoba, Winnipeg, Canada., Schwerd T; Department of Pediatrics, Dr von Hauner Children's Hospital, University Hospital, LMU Munich, Munich, Germany., Granot M; Pediatric Gastroenterology Unit, Edmond & Lily Safra Children's Hospital, Sheba Medical Center, Ramat-Gan, Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel., Sladek M; Department of Pediatrics, Gastroenterology and Nutrition, Jagiellonian University Medical College, Krakow, Poland., Strisciuglio C; Department of Woman, Child and General and Specialistic Surgery, University of Campania Vanvitelli, Naples, Italy., Müller KE; Heim Pal National Institute for Pediatrics, Budapest, Institute for Translational Medicine, University of Pécs, and Department of Family Care Methodology, Semmelweis University, Budapest, Hungary., Olbjørn C; Department of Pediatric and Adolescent Medicine, Akershus University Hospital, Lørenskog, Norway., Tzivinikos C; Paediatric Gastroenterology Department, Al Jalila Children's Hospital, Mohammed Bin Rashid University, Dubai, United Arab Emirates., Yerushalmy-Feler A; Pediatric Gastroenterology Institute, 'Dana-Dwek' Children's Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel., Huysentruyt K; Paediatric gastroenterology, KidZ Health Castle, UZ Brussel, Vrije Universiteit Brussel, Brussels, Belgium., Norsa L; Pediatric Hepatology, Gastroenterology and Transplantation, ASST Papa Giovanni XXIII, Bergamo, Italy., Viola I; Pediatric Gastroenterology and Cystic Fibrosis Unit, Department of Human Pathology in Adulthood and Childhood 'G. Barresi,' University of Messina, Italy., de Ridder L; Department of Pediatric Gastroenterology, Erasmus MC-Sophia Children's Hospital, Rotterdam, Netherlands., Shouval DS; Institute of Gastroenterology, Nutrition and Liver Diseases, Schneider Children's Medical Center of Israel, Petach Tikva, Tel-Aviv University, Tel-Aviv, Israel., Lega S; Gastroenterology, Digestive Endoscopy and Nutrition Unit, Institute for Maternal and Child Health-IRCCS 'Burlo Garofolo,' Trieste, Italy., Lionetti P; Department NEUROFARBA, Gastroenterology and Nutrition Unit, Meyer Children's Hospital, University of Florence, Florence, Italy., Catassi G; Pediatric and Gastroenterology Unit, Sapienza University of Rome-Umberto I Hospital, Rome, Italy., Assa A; Juliet Keidan Institute of Pediatric Gastroenterology Hepatology and Nutrition, Shaare Zedek Medical Center, Jerusalem, Israel.; The Hebrew University of Jerusalem, Jerusalem, Israel.
Jazyk:	angličtina
Zdroj:	Pediatrics [Pediatrics] 2024 Aug 01; Vol. 154 (2).
DOI:	10.1542/peds.2023-064546
Abstrakt:	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. (Copyright © 2024 by the American Academy of Pediatrics.)
Databáze:	MEDLINE
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