Clinical features of 55 patients with very early onset inflammatory bowel disease

Objectives To investigate the clinical features of patients with primary diagnosis of very early onset inflammatory bowel disease (VEO-IBD) . Methods Clinical data of 55 patients diagnosed with VEO-IBD in our hospital from January 2010 to December 2018 were retrospectively analyzed. Patients were followed-up by telephone interview. Besides, patients undergoing gene detection were divided into monogenic IBD and non-monogenic IBD and clinical features of these two groups were compared. Results A total of 55 children were diagnosed as VEO-IBD, with a male to female ratio of 2.24∶1, median age of disease onset was 12.00 (2.73, 47.38) months. Among 55 patients, 43 were Crohn′s disease (CD) or CD-like intestinal manifestations (78.18%, 43/55) , 3 were ulcerative colitis (UC) and 9 were IBD unclassified. The clinical manifestation was mainly diarrhea (83.64%) , bloody stool (65.45%) and abdominal pain (43.64%) . The incidence of middle-severe malnutrition and growth retardation accounted for 38.18% and 30.91% respectively. The incidence of perianal diseases was 27.27%. 44.19% of the children were colonic, and 25.58% had intestinal stenosis/perforation. Of the 21 children undergoing gene detection, 9 were monogenic IBD with predominantly IL-10R mutations. Compared with non-monogenic IBD children, the incidence of perianal lesions was higher in monogenic IBD group (77.78% vs. 0, P<0.001) . 16.36% (9/55) of the children died. Conclusions The main clinical manifestations of VEO-IBD were diarrhea and bloody stool. VEO-IBD was easy to develop malnutrition and growth retardation, and its incidence of perianal lesions was high. Gene mutation detection should be performed on children with VEO-IBD to enhance individualized treatment. Key words: Very early onset inflammatory bowel disease; Gene; IL-10R; Clinical feature