The Unique Disease Course of Children with Very Early onset-Inflammatory Bowel Disease

Kelsen JR1,2, Conrad MA1,2, Dawany N3, Patel T1,2, Shraim R1, Merz A1, Maurer K4, Sullivan KE2,4, Devoto M2,5,6. Inflamm Bowel Dis. 2019 Sep 27. pii: izz214. doi: 10.1093/ibd/izz214. [Epub ahead of print]


Author information

Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia.

Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania.

Department of Biomedical and Health Informatics, The Children's Hospital of Philadelphia.

Division of Immunology and Allergy, The Children's Hospital of Philadelphia.

Division of Human Genetics, The Children's Hospital of Philadelphia.

Department of Translational and Precision Medicine, University Sapienza, Rome, Italy.


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.

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