In view of the increasing rate of pediatric inflammatory bowel disease (IBD) and increasing knowledge and experience with this condition, the NASPGHAN (North American Society for Pediatric Gastroenterology, Hepatology and… Click to show full abstract
In view of the increasing rate of pediatric inflammatory bowel disease (IBD) and increasing knowledge and experience with this condition, the NASPGHAN (North American Society for Pediatric Gastroenterology, Hepatology and Nutrition) has come out with a position paper on very early onset (VEO) IBD. It describes the epidemiology classification, genetic etiologies, phenotypes and treatment modalities for VEO-IBD. IBD diag-nosed in the first 2 years of life is called infantile-onset IBD while that diagnosed between 2 to 6 years is known as VEO-IBD. Monogenic defects can be detected in 15-20% of children with VEO-IBD. The genes involved are those of the intestinal epithelial barrier function and immunological disorders. Detailed phenotypic characterization including clinical evaluation, endoscopy and biopsies can help narrow down the plausible genetic defects, which can further be identified by targeted or whole exome sequencing. The current data on VEO-IBD is limited to case reports and small case series. The available treatment modalities include immunomodulators such as methotrexate and azathioprine, biologi-cals such as infliximab and abatacept. The child may also require surgical interventions or hematopoietic stem cell transplant. It is important to have a collaborative multidisciplinary team to manage these patients.
               
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