The Unique Disease Course of Children with Very Early onset-Inflammatory Bowel Disease. Issue 6 (27th September 2019)

• Record Type: Journal Article
• Authors: Kelsen, Judith R; Conrad, Maire A; Dawany, Noor; Patel, Trusha; Shraim, Rawan; Merz, Audrey; Maurer, Kelly; Sullivan, Kathleen E; Devoto, Marcella
• Is Part Of: Inflammatory bowel diseases. Volume 26:Issue 6(2020)
• Page Start: 909
• Page End: 918
• Publication Date: 2019-09-27
• Subjects: very early onset-inflammatory bowel disease -- growth failure -- disease course; Inflammatory bowel diseases -- Periodicals; Colitis, Ulcerative -- Periodicals; Crohn Disease -- Periodicals; Inflammatory Bowel Diseases -- Periodicals; 616.344
• DOI: 10.1093/ibd/izz214
• Languages: English
• ISSNs: 1078-0998

Abstract

Abstract : In comparing the disease course of newly diagnosed subjects with VEO-IBD with older pediatric IBD, we found that subjects with VEO-IBD had more severe disease than older children. There were more medication failures and colectomies and persistent growth failure. Abstract: Background: Insight into the pathogenesis of very early onset-inflammatory bowel disease (VEO-IBD) has expanded through the identification of causative monogenic defects detected in a subset of patients. However, the clinical course of this population remains uncertain. The study objective is to determine whether VEO-IBD is associated with more severe disease, defined as increased surgical intervention and growth failure, than older pediatric IBD. Secondary outcomes included therapeutic response and hospitalizations. Methods: Subjects with IBD diagnosed younger than 6 years old (VEO-IBD) were compared with children diagnosed 6 to 10 (intermediate-onset) and older than 10 years of age (older-onset IBD). Metadata obtained from the medical record included age of onset, disease phenotype and location, surgeries, medical therapy, and comorbid conditions. Length of follow-up was at least 1 year from diagnosis. Results: There were 229, 221, and 521 subjects with VEO, intermediate-onset, and older-onset IBD, respectively. Very early onset-inflammatory bowel disease subjects underwent more diverting ileostomies ( P < 0.001) and colectomies ( P < 0.001) than the older children. There was less improvement in weight- and height-for-age Z scores during the follow-up period in subjects with VEO-IBD. Additionally, subjects with VEO-IBD had higher rates of medication failure at 1 year and were more frequently readmitted to the hospital. Targeted therapy was successfully used almost exclusively in VEO-IBD. Conclusion: Patients with VEO-IBD can have a more severe disease course with increased surgical interventions and poor growth as compared with older-onset IBD patients. Further, VEO-IBD patients are more likely to be refractory to conventional therapies. Strategies using targeted therapy in these children can improve outcome and, in some cases, be curative.