Phenotypic and genotypic characterization of inflammatory bowel disease in children under six years of age in China

AIM To analyze clinical differences between monogenic and nonmonogenic very-early-onset inflammatory bowel disease(VEO-IBD) and to characterize monogenic IBD phenotypically and genotypically via genetic testing. METHODS A retrospective analysis of children aged 0 to 6 years diagnosed with VEO-IBD in a tertiary hospital in southern China from 2005 to 2017 was performed. Clinical data for VEO-IBD patients were collected, and genetic characteristics were analyzed using whole exome sequencing or target gene panel sequencing. RESULTS A total of 54 VEO-IBD patients were included in this study. A diagnosis of Crohn's disease(CD) or CDlike intestinal manifestations accounted for 72.2% of the VEO-IBD cases. Nine patients(16.7%) were identified by genetic testing as having monogenic IBD. The median age of diagnosis in the monogenic group was younger than that of the nonmonogenic IBD group, at 18 mo(interquartile range(IQR): 4 to 78) and 43.5 mo(IQR: 3 to 173), respectively; the P-value was 0.021. The incidence of perianal disease in the monogenic group was higher than that in the nonmonogenic group(P = 0.001). However, there were no significant differences between weight-forage and height-for-age Z-scores between the two groups, and similar laboratory results were obtained for the two groups. Five patients were found to have IL10 receptor mutation, two patients had chronic granulomatous disease, one patient had common variable immunodeficiency disease, and one patient had X-linked inhibitor of apoptosis protein deficiency. CONCLUSION A high proportion of monogenic IBD was observed in the VEO-IBD group, especially with disease onset before the age of 6 mo. Monogenic IBD and nonmonogenic IBD exhibited similar clinical features. Furthermore, next-generation sequencing played an important role in the diagnosis of monogenic IBD, and IL10 receptor mutation was predominant in this cohort.

Nasogastric or nasojejunal feeding in pediatric acute pancreatitis: a randomized controlled trial

Background The aim of this study was to compare nasogastric(NG)feeding with nasojejunal(NJ)feeding when treating pediatric patients with acute pancreatitis(AP)..Methods We performed a single-center,prospective,randomized,active-controlled trial involving 77 pediatric patients with AP from April 2014 to December 2017.The patients were randomized into two groups:the NG tube feeding group(34 patients)and the NJ tube feeding group(33 patients).The primary outcome measures included the enteral nutrition intoler-ance,the length of tube feeding time,the recurrent pain of pancreatitis and complications.Results A total of 62 patients with AP(31 patients for each group)came into the final analysis.No differences were found in baseline characteristics,pediatric AP score and computed tomography severity score between the lwo groups.Three(9.7%)patients in the NG group and one(3.2%)patient in the NJ group developed intolerance(relative risk=3.00,95%confidence interval 0.33-27.29,P=0.612).The tube feeding time and length of hospital stay of the NG group were significantly shorter than those of the NJ group(P=0.016 and 0.027,respectively).No patient died in the trial.No significant differences were found in recurrent pain,complications,nutrition delivery efficacy,and side effects between the two groups.Conclusions NG tube feeding appears to be effective and safe for acute pediatric pancreatitis compared with NJ tube feeding.In addition,high qualifed,large sample sized,randomized controlled trials in pediatric population are needed.