88% (N = 141) had a primary care provider (PCP), and most (70%) saw their PCP 1-2 times. Even so, 86% (N = 139) received medical care from places other than their PCP or GI doctor; 27% in the ED and 45% at urgent care. Children of parents with less than a bachelor's degree, families that lived further from their GI doctor, and children who saw their PCP more often were more likely to utilize ED/urgent care. CONCLUSIONS ED/urgent care utilization in pediatric patients with IBD was greater than expected, potentially contributing to fragmented, costly care and worse outcomes.BACKGROUND Cystic fibrosis-related liver disease (CFLD) is the leading nonpulmonary cause of mortality in cystic fibrosis (CF). We evaluated and compared the burden of disease and nonrespiratory comorbidities of those with severe CFLD and those without (noCFLD). METHODS A retrospective nationwide (Australia) longitudinal review (from 1998 to 2016) of severe CFLD patients compared with noCFLD controls (matched 1  1 for age, genotype, pancreatic insufficiency, and center). RESULTS One hundred sixty-six patients with severe CFLD and 166 with noCFLD were identified. Forced expiratory volume in 1 second percentage of predicted (FEV1%) was significantly lower in CFLD than noCFLD across all ages (estimate [SE] -6.05% [2.12]; P = 0.004). Median (IQR) hospitalizations per patient per year were higher in CFLD than noCFLD for respiratory indications (0.6 [0.2-1.3] vs 0.4 [0.1-0.9]; P = 0.002); gastrointestinal indications (0.09 [0-0.2] vs 0 [0-0.05]; P  less then  0.001); and other indications (0.05 [0-0.2] vs 0 [0-0.1]; P = 0.03). In the CFLD cohort, there was increased use of nasogastric (12.6% vs 5.4%; OR 2.51 [95% CI 1.06-6.46]; P = 0.03) and gastrostomy nutritional supplementation (22.9% vs 13.2%; OR 1.93 [95% CI 1.05-3.63]; P = 0.03). Additionally, the CFLD cohort had a higher frequency of bone diseases, osteopenia (26.5% vs 16.8%; OR 1.77 [95%CI 1.01-3.15]; P = 0.04) and osteoporosis (16.2% vs 8.4%; OR 2.1 [95% CI 1.01-4.52]; P = 0.04), as well as CF-related diabetes (38.5% vs 19.2%; OR 2.61 [95% CI 1.55-4.47[; P = 0.001). CONCLUSIONS Patients with severe CFLD have greater disease burden, with higher number of hospitalizations (both respiratory and nonrespiratory indications), nutritional interventions, and are at higher risk of CF-related bone disease and diabetes.BACKGROUND Anorectal malformations (ARMs) are a group of congenital malformations affecting the lower gastrointestinal, urogenital, and/or gynecological systems. They occur in approximately 1 in 5000 live births, with a slight male predominance. ARMs can appear in isolation or in association with other anomalies. The literature contains little information about the characteristics of ARMs in United States Hispanic populations. METHODS An institutional review board-approved chart review was conducted in all patients with ARMs seen at a single institution in El Paso, Texas, from January 2012 to December 2015. Data regarding demographics, types of ARMs, associated syndromes, and long-term complications were recorded using the Krickenbeck classification system. RESULTS Of the 37 patients included in the study, 20 were boys and 17 were girls. The most common ARMs were rectoperineal fistula (46%), rectourethral fistula, and cloacal malformation. Constipation was the most common long-term complication, especially in patients with recto-perineal fistula. CONCLUSION Children in the Borderland community may have a higher percentage of recto-perineal fistula than previously reported. Postoperative complications in Hispanic children with ARM were comparable to those reported in other populations.OBJECTIVES Acute-on-chronic liver failure (ACLF) is well-studied in adults and characterized by decompensated cirrhosis, multi-organ failure, and early mortality. Studies of ACLF in children are limited. We sought to characterize the prevalence and clinical factors associated with pediatric ACLF (PACLF). METHODS A retrospective review of children 3 months to 18 years listed for liver transplantation and hospitalized for decompensated cirrhosis between January 2007 and December 2017 at a single pediatric hospital. Primary outcome was the development of PACLF, characterized as failure of at least 1 extrahepatic organ (mechanical ventilation, renal replacement therapy, vasoactive medications, grade III/IV hepatic encephalopathy). Characteristics were recorded for each hospitalization. RESULTS Sixty-six patients had 186 hospitalizations with mean age at admission 4.0 ± 5.6 years and diagnosis of biliary atresia (BA) in 65%. PACLF developed in 20 patients during 23 hospitalizations (12%) and respiratory failure was most common (17/23, 74%). Duration of intensive care unit stay, 13.1 ± 1.2 versus 0.6 ± 0.6 days (P  less then  0.001) and length of stay, 24.3 ± 5.0 days versus 7.9 ± 1.9 days (P = 0.003) were longer in PACLF compared with non-PACLF. Plinabulin Mortality during PACLF hospitalizations was 22%. Clinical factors associated with PACLF were reported from a generalized linear mixed model and included increased admission creatinine (P  less then  0.0001), increased aspartate aminotransferase (AST) (P = 0.014), increased international normalized ration (INR) (P = 0.0015), and a positive blood culture (P = 0.007). CONCLUSION In this pediatric series, PACLF developed in 12% of hospitalizations and mortality was high. Admission creatinine, AST, INR, and presence of a positive blood culture were associated with PACLF development.OBJECTIVES Osteopontin (OPN) is a multifunctional protein present abundantly in human milk, but at low levels in bovine milk and infant formula. Bovine milk OPN (bmOPN) is commercially available, and may therefore, be added to formula. OPN exerts its multiple functions by binding to its receptors to activate cell-signaling pathways. The OPN receptor (integrin)-binding site is conserved across species; therefore, bmOPN may exert bioactivities in humans and mice. The objective of the present study was to evaluate bioactivities of bmOPN using an established OPN knock-out (KO) mouse model. METHODS We evaluated bioactivities of bmOPN, including effects on intestinal growth, immune response, and brain development. In the present study, wild-type (WT) pups were nursed by WT dams, KO dams, or KO dams with bmOPN supplementation from postnatal days 1 to 21 (P1--P21). RESULTS Our results show that orally ingested bmOPN is partly resistant to in vivo gastrointestinal digestion, and supplemental bmOPN exhibited similar effects as mouse milk OPN (mmOPN) on promoting growth of the small intestine revealed by histological analysis of duodenum villus height and crypt depth at P10, on modifying TNF-α response against a LPS challenge at P30, as well as promoting brain myelination by increasing expression of myelin-associated glycoprotein (MAG) and myelin basic protein (MBP) and improving cognitive development.