Clinical Analysis Of 10 Cases Of Very Early Onset Inflammatory Bowel Disease

Objective: To summarize the clinical features,gene diagnosis,treatment and prognosis of very early onset inflammatory bowel disease(VEO-IBD).That will have some guiding significance for pediatricians when they encounter the patients with long term diarrhea,bloody stools,Fever,poor weight loss and other clinical features in clinical work.Methods: A retrospective analysis was performed on the clinical data which including gender,age of onset,diarrhea,blood in the stool,fever,nutritional status and related treatment,prognosis of 10 children with very early onset inflammatory bowel disease admitted to the Department of Pediatric Gastroenterology of Shengjing Hospital Affiliated to China Medical University from January 2014 to January 2018.To Summary the frequency of each characteristic and calculate average age of onset,meanwhile Responsive laboratory data range by mean±standard deviation.Results: The average age of onset in 10 children included in the study was 8.13 months.Eight of the eight cases(80%)had different degrees of diarrhea in the first symptom,of which 5 cases had bloody stools(50%)(3 cases were muco-purulent and bloody stools and 1 case had bloody sputum).6 cases(60%)had different degrees of fever,7 cases(70%)had malnutrition(4 cases of moderate malnutrition,3 cases of mild malnutrition),2 cases(20%)of vaginal rectal fistula,and there was 1 perianal abscess.Two cases had retching as the first symptom,and one case had abdominal pain as the first symptom.Eight patients(80%)had elevated leukocytes,10 patients(100%)had elevated C-reactive protein,and 8 cases(80%)had decreased hemoglobin levels,There were 8 cases(80%)having increased platelet levels and 8 cases(80%)having decreased albumin.Eight patients with colonoscopy showed extensive colon involvement,and the other 2 patients were diagnosed with surgical treatment of intestinal obstruction.According to the modified Porto criteria,7 cases were diagnosed with Crohn's disease and 3 cases were diagnosed as ulcerative colitis.Among them,8 cases were perfected for geneexamination.There were 4 gene defects in the test results,all of which were IL-10 RA mutations,of which 3 were mixed heterozygous mutations and 1 was heterozygous mutations.During following up,2 children with genetic defects finally died.The other 1patient was treated with long-term oral administration of mesalazine and thalidomide,but there were still diarrhea,intermittent fever,and malnutrition.The remaining 6 patients were active growth under control of the drug.Conclusions: 1.The age of onset of VEO-IBD is early,and there are serious clinical manifestations such as long-term diarrhea,bloody stools,fever,poor weight gain and often accompanied by perianal lesion.2.Imaging examination has some meaning in the diagnosis of VEO-IBD.3.Endoscopic examination often shows extensive colon involvement and according pathological changes.4.The VEO-IBD in young children are resistant to many of the standard medications for IBD,which is particularly true in infantile patients with genetic defects.