[Analysis on clinical features and risk factors for mortality in children with very early-onset inflammatory bowel dsease].

Objective: To investigate the clinical characteristics and risk factors for mortality in children with very early-onset inflammatory bowel disease (VEO-IBD). Methods: The children with VEO-IBD was retrospectively selected from the Children's Hospital Affiliated to Zhengzhou University between January 2014 and December 2022. The children were stratified into the deceased group and the survival group based on clinical outcomes. The differences in clinical characteristics, laboratory findings, nutritional indicators, and treatment regimens were compared between the two groups. Multivariate logistic regression analysis was employed to identify risk factors associated with mortality in children with VEO-IBD. Results: A total of 67 patients were included, with 21 in the deceased group [17 males, 4 females; onset age M (Q1, Q3) 7.5 (0.5, 35.2) months] and 46 in the survival group [31 males, 15 females; onset age 19.5 (1.0, 51.5) months]. The deceased group exhibited higher incidences of single gene mutation (IL-10RA, IL-10RB, CYBB, DOCK8, BTK, TNFAIP3, ELANE, WASP) [100.0% (21/21) vs 32.5% (15/46), P<0.001], perianal disease behavior of the disease behavior [100.0% (21/21) vs 34.8% (16/46), P=0.003], severe malnutrition [85.7% (18/21) vs 32.5% (22/46), P=0.008], and growth impairment [90.5% (19/21) vs 23.9% (11/46), P=0.011] compared to the survival group. The deceased group showed elevated levels of erythrocyte sedimentation rate [(66±18) vs (31±9) mm/1 h], fecal calprotectin [(1 860±257) vs (916±274) μg/g], and interleukin (IL)-6 [(159±35) vs (103±27) ng/L] (all P<0.05). In contrast, weight-for-height Z-score (WHZ) [(-2.95±0.98) vs (-1.12±0.85) scores], weight-for-age Z-score (WAZ) [(-2.96±1.17) vs (-1.23±1.06) scores], and height-for-age Z-score (HAZ) [(-2.87±1.14) vs (-1.16±0.77) scores] in the deceased group were lower than those in the survival group (all P<0.05). Single gene mutation (IL-10RA、IL-10RB、CYBB、DOCK8、BTK、TNFAIP3、ELANE、WASP) (OR=25.753, 95%CI: 9.373-83.465), age<7.5 months (OR=33.528, 95%CI: 10.132-114.152), severe malnutrition (OR=6.534, 95%CI: 3.493-12.819), and a history of critical surgical intervention (OR=10.396, 95%CI: 7.501-20.313) were risk factors for mortality in children with VEO-IBD. Conclusions: The children with VEO-IBD who died of VEO-IBD exhibited a state of hyperinflammation. Single gene mutation (IL-10RA、IL-10RB、CYBB、DOCK8、BTK、TNFAIP3、ELANE、WASP), age<7.5 months, severe malnutrition, and a history of critical surgical intervention are risk factors for mortality in children with VEO-IBD.