EIMs as defined in this study occur in approximately one quarter of pediatric patients with IBD. Disease type and disease severity were commonly associated with the occurrence of EIMs.

In this study, disease outcomes were generally favorable. Discontinuation of antifungal treatment after clinical response and an appropriate duration of therapy, probably at least 12 months, appears safe if pharmacologic immunosuppression has been held. Resumption of TNF-α blocker therapy also appears safe, assuming that the initial antifungal therapy was administered for 12 months.

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Histoplasmosis is an important complication of treatment with TNF-alpha neutralizing agents. Children with IBD treated with aTNF therapy who develop the infection may present with minimal pulmonary symptoms. While discontinuation of aTNF therapy is important initially, few data exist to determine when and how aTNF therapy can be reinstituted. Recognition of Histoplasma capsulatum is often delayed due to the overlap of symptoms with some of the extraintestinal manifestations of IBD and other more prevalent infectious complications.

Background Racial disparities in care and outcomes contribute to mortality and morbidity in children however the role in pediatric Crohn’s disease (CD) is unclear. In this study, we compared cohorts of Black and White children with CD to determine the extent race is associated with differences in readmissions, complications, and procedures among hospitalizations in the United States. Methods Data were extracted from the Pediatric Health Information System (January 1, 2004–June 30, 2012) for patients ≤21 years of age hospitalized with a diagnosis of CD. White and Black cohorts were randomly selected in a 2:1 ratio by hospital. The primary outcome was time from index hospital discharge to readmission. The most frequent complications and procedures were evaluated by race. Results There were 4377 patients. Black children had a shorter time to first readmission and higher probability of readmission (p=0.009), and a 16% increase in risk of readmission compared to White children (p=0.01). Black children had longer length of stay and higher frequency of overall and late (30 days–12 months post discharge) readmissions (p<0.001). During index hospitalization, more Black children had perianal disease and anemia (p<0.001). During any hospitalization, Black children had higher incidence of perianal disease, anemia, and vitamin D deficiency, and greater number of perianal procedures, endoscopies, and blood product transfusion (p<0.001). Conclusions There are differences in hospital readmissions, complications, and procedures among hospitalized children related to race. It is unclear whether these differences are due to genetic differences, worse intrinsic disease, adherence, access to treatment, or treatment disparities.

Background and Aims Ileal strictures are the major indication for resective surgery in Crohn’s disease (CD). We aimed to define ileal gene programs present at diagnosis linked with future stricturing behavior during five year follow-up, and to identify potential small molecules to reverse these gene signatures. Methods Antimicrobial serologies and pre-treatment ileal gene expression were assessed in a representative subset of 249 CD patients within the RISK multicenter pediatric CD inception cohort study, including 113 that are unique to this report. These data were used to define genes associated with stricturing behavior and for model testing to predict stricturing behavior. A bioinformatics approach to define small molecules which may reverse the stricturing gene signature was applied. Results 19 of the 249 patients developed isolated B2 stricturing behavior during follow-up, while 218 remained B1 inflammatory. Using deeper RNA sequencing than in our prior report, we have now defined an inflammatory gene signature including an oncostatin M co-expression signature, tightly associated with extra-cellular matrix (ECM) gene expression in those who developed stricturing complications. We further computationally prioritize small molecules targeting macrophage and fibroblast activation and angiogenesis which may reverse the stricturing gene signature. A model containing ASCA and CBir1 serologies and a refined eight ECM gene set was significantly associated with stricturing development by year five after diagnosis (AUC (95th CI) = 0.82 (0.7-0.94)). Conclusion An ileal gene program for macrophage and fibroblast activation is linked to stricturing complications in treatment naïve pediatric CD, and may inform novel small molecule therapeutic approaches.

Objectives The Pediatric Ulcerative Colitis Activity Index (PUCAI) is a non-invasive disease activity index developed as a clinical trial endpoint. More recently, practice guidelines have recommended the use of PUCAI in routine clinical care. We therefore sought to evaluate the feasibility, validity and responsiveness of PUCAI in a large, diverse collection of pediatric gastroenterology practices. Methods We extracted data from the two most recent encounters for patients with ulcerative colitis in the ImproveCareNow registry. Feasibility was determined by the percent of patients for whom all PUCAI components were recorded, validity by correlation of PUCAI scores across Physician Global Assessment (PGA) categories, and responsiveness to change by the correlation between the change in PUCAI and PGA scores between visits. Results 2503 patients were included (49.5% male, age 15.2±4.1 years, disease duration 3.7±3.2 years). All items in the PUCAI were completed for 96% of visits. PUCAI demonstrated excellent discriminatory ability between remission, mild and moderate disease; discrimination between moderate and severe disease was less robust. There was good correlation with PGA [r=0.76 (p<0.001), weighted kappa k=0.73 (p<0.001)]. The PUCAI change scores correlated well with PGA change scores (p<0.001). Test-retest reliability of the PUCAI was good (intra-class correlation coefficient=0.72 [95% CI 0.70–0.75], p<0.001). Guyatt’s responsiveness statistic was 1.18 and the correlation of ΔPUCAI with ΔPGA was 0.69 (p<0.001). Conclusions The PUCAI is feasible to use in routine clinical settings. Evidence of its validity and responsiveness support its use as a clinical tool for monitoring disease activity for patients with ulcerative colitis.