Abstract:
OBJECTIVE:X-linked inhibitor of apoptosis (xIAP) deficiency is a primary immune deficiency disorder associated with hemophagocytic lymphohistiocytosis. About 17% of xIAP-deficient patients present with very early onset severe colitis with high mortality. We hypothesized that xIAP deficiency leads to defective generation and/or survival of T regulatory cells (Treg) through its involvement in transforming growth factor-β signaling. METHODS AND RESULTS:We used a T-cell transfer model of chronic colitis and observed a mild increase in colitis severity induced by naïve CD4 T cells from xIAP mice compared with colitis induced by naïve CD4 T cells from WT mice. We did not observe any significant difference in the induction of Treg cells in these studies. We next tested whether xIAP is required for Treg cell function by co-transferring xIAP or WT Treg cells with naïve WT CD4 cells in this model. We demonstrate that XIAP-deficient Treg cells were able to prevent disease similarly to WT Treg cells. In these experiments we, however, found a significantly decreased percentage of IL-17A-producing CD4 T cells in mice receiving Tregs from xIAP mice. CONCLUSIONS:xIAP appears dispensable for the generation of induced Treg cells as well as function of natural Treg cells. There appeared to be a role of xIAP in generation of IL-17-producing cells from either naïve CD4 T cells or Treg cells. Further research is needed to explore the role of xIAP in generation of IL-17-producing cells.
journal_name
J Pediatr Gastroenterol Nutrjournal_title
Journal of pediatric gastroenterology and nutritionauthors
Gurram B,Hammelev E,Syverson G,Haribhai D,Yan K,Simpson P,Salzman N,Verbsky JWdoi
10.1097/MPG.0000000000001122subject
Has Abstractpub_date
2016-08-01 00:00:00pages
218-25issue
2eissn
0277-2116issn
1536-4801journal_volume
63pub_type
杂志文章abstract:OBJECTIVE:The coexistence of celiac disease (CeD) and eosinophilic esophagitis (EoE) in pediatric patients has been increasingly recognized. In the current study, we have aimed to assess the outcomes of therapeutic dietary interventions in a cohort of pediatric patients with CeD and EoE. METHODS:Pediatric patient reco...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章
doi:10.1097/MPG.0000000000002343
更新日期:2019-08-01 00:00:00
abstract::The clinical efficacy of twice-daily cimetidine (20 mg/kg/day) was retrospectively studied in 19 children with gastric ulcer (GU) and 23 with duodenal ulcer (DU) in the active stage. Endoscopically confirmed cumulative healing rates at 2, 4, and 8 weeks of treatment were 53.5, 73.7, and 94.7% in the GU group and 15.4,...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199404000-00007
更新日期:1994-04-01 00:00:00
abstract::Human breast milk contains many enzymes, some of which may assist in digestion; other enzymes may have other diverse functions. The loci and regulation of milk enzyme synthesis remain to be elucidated, but multiple loci and complex hormonal regulation seem to be likely. In a similar fashion, multiple mechanisms of pro...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198300201-00015
更新日期:1983-01-01 00:00:00
abstract:OBJECTIVES:We aimed to identify early noninvasive predictors of clinical and endoscopic remission in children with Crohn disease (CD) under infliximab (IFX). METHODS:Prospective observational study conducted in children with moderate-to-severe CD starting IFX. All patients underwent weighted pediatric CD activity inde...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002262
更新日期:2019-06-01 00:00:00
abstract:OBJECTIVE:To determine the natural history of treated and untreated eosinophilic esophagitis (EE) and examine the presenting symptoms of EE. PATIENTS AND METHODS:Retrospective and prospective chart review of all patients diagnosed with EE at The Children's Hospital of Philadelphia. EE was defined as greater than 20 eo...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181788282
更新日期:2009-01-01 00:00:00
abstract::The underlying causes of chronic diarrhea beginning early in life are increasingly well defined. Infectious and post-infectious enteropathies and food sensitive/allergic enteropathy account for the majority of cases. Recent attention has focused on characterizing defined entities, which cause protracted diarrhea in in...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/00005176-200401000-00007
更新日期:2004-01-01 00:00:00
abstract:OBJECTIVES:The present study aims to identify the genotype-phenotype correlation in children with Peutz-Jeghers Syndrome (PJS) through the analysis of STK11 gene mutations in the context of clinical and pathological characteristics. METHOD:In this observational cohort study, the clinical characteristics of 18 families...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002166
更新日期:2019-02-01 00:00:00
abstract:BACKGROUND:Starch is an important source of energy for children with cystic fibrosis, but little is known about their capacity to digest it. METHODS:A 13C breath test was used to measure starch digestion and oxidation in 16 children with cystic fibrosis (median [range] age, 7.9 [4-15] years; 7 girls, 9 boys) and 5 nor...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199909000-00016
更新日期:1999-09-01 00:00:00
abstract:OBJECTIVES:We prospectively evaluated incidence of prolonged (>28 days) parenteral nutrition (PN), associated complications, and significance of parenteral plant sterols (PS) in neonatal intestinal failure-associated liver disease (IFALD) compared with children. METHODS:We recruited 28 neonates (mean age 50 days, rang...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3182474118
更新日期:2012-06-01 00:00:00
abstract::Proton pump inhibitors such as lansoprazole are used in the treatment of gastroesophageal reflux disease (GERD), but dosing guidelines for infants have not been determined. The objective of this study was to assess the clinical efficacy of 2 dosing regimens of lansoprazole in infants with GERD using the revised infant...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,随机对照试验
doi:10.1097/MPG.0b013e31815667d7
更新日期:2008-03-01 00:00:00
abstract:OBJECTIVES:Lutein and zeaxanthin are dietary carotenoids that may influence visual and cognitive development. The objective of this study was to provide the first data on distribution of carotenoids in the infant brain and compare concentrations in preterm and term infants. METHODS:Voluntarily donated brain tissues fr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000389
更新日期:2014-11-01 00:00:00
abstract:OBJECTIVES:Gastroesophageal reflux is common but remains a controversial disease to diagnose and treat and little is known about the role of reflux testing in predicting clinical outcomes, particularly in children at risk for extraesophageal reflux complications. The aim of this study was to determine if rates of hospi...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章
doi:10.1097/MPG.0000000000001092
更新日期:2016-08-01 00:00:00
abstract::The incidence of inflammatory bowel disease (IBD) is steadily on the rise in Western and in developing countries paralleling the increase of westernized diets, characterized by high protein and fat as well as excessive sugar intake, with less vegetables and fiber. Furthermore, nutrition is involved in several aspects ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0000000000001785
更新日期:2018-03-01 00:00:00
abstract::Serum carboxyterminal propeptide of type I procollagen (PICP) and cross-linked carboxyterminal telopeptide of type I collagen (ICTP), new markers of bone collagen type I biosynthesis and degradation, have not been studied in small for gestational age (SGA) infants. In an earlier study, we found a lower bone mineral co...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199610000-00004
更新日期:1996-10-01 00:00:00
abstract::R-1 anti-reticulin antibodies (ARA) were present in sera of 10 of the 12 children who subsequently fulfilled the ESPGAN diagnostic criteria for celiac disease (CD), but was not found in any children in the age-matched control groups, viz., severely malnourished (n = 20), acute gastroenteritis (n = 23), normally nouris...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:
更新日期:1988-11-01 00:00:00
abstract:BACKGROUND:Premature infants receiving alimentation with cow milk-based formulas run a considerably high risk of incipient late metabolic acidosis, an early stage developing of manifest late metabolic acidosis. Is bone metabolism involved in pathophysiologic mechanisms characterizing this early stage of retention acido...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1097/00005176-200111000-00010
更新日期:2001-11-01 00:00:00
abstract::A report on a case of rickets in a very low birth weight infant (VLBWI) is presented. The infant had no high-risk factors for rickets and was fed a specialized preterm formula with vitamin D supplementation (200 IU daily) by 10 days of age. Feeds were advanced so that an enteral intake of 120 cal/kg/day was achieved b...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198910000-00023
更新日期:1989-10-01 00:00:00
abstract:OBJECTIVES:Eosinophilic esophagitis (EoE) can present as food selectivity or feeding disorders in children. Children with autism spectrum disorders (ASDs) commonly demonstrate behavioral food selectivity in type and texture, which often leads to the diagnosis of feeding disorder. We sought to evaluate the association o...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001282
更新日期:2016-10-01 00:00:00
abstract:OBJECTIVES:Inflammatory bowel disease (IBD) is a chronic lifelong condition and is related to poor quality of life (QoL). The aim of this study was to evaluate the QoL of Asian pediatric patients with IBD and to determine the clinical factors that can influence QoL. METHODS:Children and adolescents aged 9 to 18 years ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,多中心研究
doi:10.1097/MPG.0000000000002119
更新日期:2019-01-01 00:00:00
abstract:OBJECTIVE:The aim of the study was to investigate the in vitro effects of sera from children with acute liver failure (ALF) who had received N-acetylcysteine (NAC) on the metabolic and synthetic activity of cryopreserved human hepatocytes. MATERIALS AND METHODS:Cryopreserved human hepatocytes were plated on collagen-c...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31819114da
更新日期:2009-05-01 00:00:00
abstract::Ventriculoperitoneal shunts are currently a standard therapy for obstructive hydrocephalus. These shunts are associated with a variety of abdominal complications, one of which is the development of ascites. We report an 11-year-old girl with a ventriculoperitoneal shunt in whom a low-grade peritoneal infection present...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198809000-00028
更新日期:1988-09-01 00:00:00
abstract:OBJECTIVES:The aim of the present study was to determine the incidence of peptic ulcer bleeding (PUB) in pediatric patients. METHODS:A hospital inpatient database, Premier Perspective, and an insurance claims database, MarketScan, were analyzed to estimate upper and lower limits for the annual incidence of PUB in the ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31824fb7f9
更新日期:2012-06-01 00:00:00
abstract:OBJECTIVES:Small for gestational age (SGA) infants are more susceptible to infectious morbidity and growth faltering compared to their appropriate for gestational age (AGA) counterparts. Zinc supplementation of SGA infants may be beneficial but the underlying susceptibility to zinc deficiency of SGA infants has not bee...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001778
更新日期:2018-03-01 00:00:00
abstract:OBJECTIVE:A previously unrecognized high prevalence of celiac disease (CD) has been found by screening among European and North American patients with type 1 diabetes mellitus (DM 1). The prevalence of CD among Brazilian children with DM1 is not known. This study was conducted to determine the prevalence of CD in Brazi...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000181400.57884.c3
更新日期:2005-11-01 00:00:00
abstract:OBJECTIVES:The aim of the present study was to evaluate whether polymorphisms of the mannose-binding lectin (MBL-2) gene and MBL serum levels on admission to neonatal intensive care unit are associated with necrotizing enterocolitis (NEC) in preterm infants and to verify MBL expression in NEC bowels. METHODS:In this r...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31824e5f7a
更新日期:2012-08-01 00:00:00
abstract::The effect of a lipid emulsion containing long-chain triglycerides (LCT) and supplemented with L-carnitine on plasma lipids and bilirubin in premature neonates on total parenteral nutrition was compared to that of lipid emulsions containing either LCT or a mixture of LCT and medium-chain triglycerides (MCT). In a doub...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1097/00005176-199507000-00004
更新日期:1995-07-01 00:00:00
abstract::Twenty children with active ulcerative colitis were assessed before and after 8 weeks of medical therapy with 5-aminosalicylic acid (5-ASA) derivatives and corticosteroids. Local therapy was given for distal disease (seven cases); other disease was treated with oral prednisolone (1-2 mg/kg/day, maximum 40 mg). Eightee...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199605000-00006
更新日期:1996-05-01 00:00:00
abstract:OBJECTIVES:To evaluate the safety of lansoprazole in children between 1 and 11 years of age. METHODS:In a phase I/II, open-label, multicenter (11 sites) study, children with symptomatic gastroesophageal reflux disease (GERD), erosive esophagitis (> or = grade 2), and/or esophageal pH < 4 for > 4.2% of the 24-hour peri...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1097/00005176-200211004-00002
更新日期:2002-01-01 00:00:00
abstract::The nutritional management of intractable diarrhea and short bowel syndrome remains a challenging problem. The advantage of continuous nasogastric infusion is undisputed, but what to feed remains in question, and no studies, to these authors' knowledge, have yet compared two widely used specially designed protein hydr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1097/00005176-198801000-00015
更新日期:1988-01-01 00:00:00
abstract:OBJECTIVES:Liver nodularity occurs across the spectrum of cystic fibrosis liver disease (CFLD), from regenerative nodules to cirrhosis, and can occur without liver enzyme abnormalities. Our aims were to determine if incorporating abdominal ultrasound (US) with annual laboratory testing improves the detection of CFLD an...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002412
更新日期:2019-10-01 00:00:00