P263 Very early onset Inflammatory Bowel Diseases: Review of the Genoa-Messina joint clinical experience. Clinical history: Part I

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Abstract Background Pediatric inflammatory bowel diseases (pIBD) have specific phenotypes compared to Inflammatory Bowel Diseases (IBD) in adults. The Very Early Onset-IBD (VEO-IBD) is considered a diagnosis of IBD in children before age 6 years old with 15% of prevalence. Methods The objective of this study was to compare the clinical and endoscopic features of VEO-IBD compared to pediatric IBD (diagnosis > 6 years old) also in terms of natural history and response to treatment. The VEO patients followed at the IBD-Centers of Messina and Gaslini were enrolled retrospectively. We compared the results of this population with what is reported in the literature in terms of clinical characteristics at onset (symptoms, age at onset, age at diagnosis), comorbidities, complications, associated immunodeficiencies, endoscopic models. Percentage comparison and chi-square test of data was performed. Results 74 VEO-IBD patients were enrolled. The onset is around 3 years, a predominantly colonic localization, inflammatory pattern. Positive family history for IBD in 8.1%. Histology is very nonspecific and is characterized above all by basal plasmacytosis (CD 68,4%; CU 72,7%) and hypereosinophilia (CD 36,8%; CU 40%). At diagnosis, IBD-U (= Unclassified) is prevalent (47,2%), the frequency of which will decrease over the years (3 years after onset, 32.4%), differentiating into CU and CD (table 1). The main clinical manifestations at onset are chronic diarrhea (CD 89,5%; CU 91%), blood in the stool and / or hematochezia (CD 78,9%; CU 96,4%; table 2). Sensitive onset tests for IBD are ESR, fecal calprotectin and iron deficiency anemia (positive respectively in CD 75-60-71,4%%; CU 58,8-84,6-66,7%). The most frequent extraintestinal manifestations are arthritis (CD 22,2%) and sclerosing cholangitis (CU 7,3%). To highlight the significant statistical association (p <0.05) between VEO-IBD and neuro / nephrological diseases (major renal malformations and nephropathies, autism and neuropsychiatric disorders; table 3). Among the most common complications are severe anemia (CD 31,6%; CU 42,6%), acute attack of severe colitis, malnutrition and fistulization. A monogenic form of VEO-IBD was found in 5.4% frequently linked to immunedeficiency, 2 cases required allogenic HSC transplantation (XIAP, WAS). Conclusion VEO-IBDs represent a challenge for the pediatric gastroenterologist. The clinical course is no more severe than pediatric inflammatory bowel diseases, with the exception of monogenic forms. A genetic association between nephro / neurological comorbidities and VEO-IBDs is likely, the genetics is still to be discovered. Further studies will be needed to define the best therapeutic and diagnostic approach for these diseases.

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