Risk models for predicting the health-related quality of life of caregivers of youth with gastrointestinal concerns.

Purpose: To determine the usefulness of cumulative and additive risk models in predicting the healthy-related quality of life (HRQOL) of caregivers of youth with chronic gastrointestinal conditions.

Methods: 203 caregivers (82.8% mothers; 77.

Key Determinants for Achieving Enteral Autonomy and Reduced Parenteral Nutrition Exposure in Pediatric Intestinal Failure.

Background: We sought to evaluate the relationship between determinants of intestinal failure (IF) and achieving enteral autonomy from parenteral nutrition (PN) in a large single-center cohort of children.

Methods: This is a retrospective chart review of pediatric subjects enrolled in a database for the Center for Advanced Intestinal Rehabilitation at Children's of Alabama from 1989 to 2016. IF was defined as dependence on PN for >60 days.

Disease-Related Predictors of Health-Related Quality of Life in Youth With Eosinophilic Esophagitis.

Objective: To evaluate relations between health-related quality of life (HRQoL) and clinical symptom presentation in youth with eosinophilic esophagitis (EoE). We hypothesized that presence of dysphagia, reflux, nausea/vomiting, and epigastric pain would be related to poorer HRQoL. In predictive models, it was hypothesized that dysphagia, reflux, nausea/vomiting, and epigastric pain would each significantly and uniquely predict poorer HRQoL.

Evidence of Disturbed Sleep in Children With Eosinophilic Esophagitis and Persistent Epigastric Pain.

Objective: To determine whether the experience of persistent epigastric pain is associated with sleep disturbances in children with eosinophilic esophagitis (EoE). We hypothesized that children with EoE and persistent epigastric pain would (1) self-report greater current and worst pain severity, and (2) experience more disturbed sleep on objective and subjective measures in comparison with children with EoE but no persistent pain and healthy children.

Methods: Fifty children with EoE were recruited for this cross-sectional study, of which 24 (48%) reported experiencing persistent epigastric pain.

Outcomes from a 12-Week, Open-Label, Multicenter Clinical Trial of Teduglutide in Pediatric Short Bowel Syndrome.

Objective: To determine safety and pharmacodynamics/efficacy of teduglutide in children with intestinal failure associated with short bowel syndrome (SBS-IF).

Study Design: This 12-week, open-label study enrolled patients aged 1-17 years with SBS-IF who required parenteral nutrition (PN) and showed minimal or no advance in enteral nutrition (EN) feeds. Patients enrolled sequentially into 3 teduglutide cohorts (0.

A Heterozygous RAB27A Mutation Associated with Delayed Cytolytic Granule Polarization and Hemophagocytic Lymphohistiocytosis.

Frequently fatal, primary hemophagocytic lymphohistiocytosis (HLH) occurs in infancy resulting from homozygous mutations in NK and CD8 T cell cytolytic pathway genes. Secondary HLH presents after infancy and may be associated with heterozygous mutations in HLH genes. We report two unrelated teenagers with HLH and an identical heterozygous RAB27A mutation (c.

Topical Review: Eosinophilic Esophagitis in Children: Implications for Health-Related Quality of Life and Potential Avenues for Future Research.

Objective: To characterize the health-related quality of life (HRQoL) of children with eosinophilic esophagitis (EoE) as well as generate novel hypotheses for future research in this pediatric population.

Method: A literature review was completed using PubMed and the keywords below.

Results: Research has shown that for children with EoE and their parents, symptom experiences and recommended treatments can have a negative impact on HRQoL.

Cytokines associated with necrotizing enterocolitis in extremely-low-birth-weight infants.

Background: The goal was to identify cytokines associated with necrotizing enterocolitis (NEC). Based on our earlier reports of decreased tissue expression of transforming growth factor (TGF)-β, we hypothesized that infants with NEC also have low blood TGF-β levels. We further hypothesized that because fetal inflammation increases the risk of NEC, infants who develop NEC have elevated blood cytokine levels in early neonatal period.

Baclofen for the treatment of pediatric GERD.

Background And Aim: Baclofen, a γ-aminobutyric acid receptor agonist, has been shown to reduce the episodes of gastroesophageal reflux (GER) by reducing the incidence of transient lower esophageal sphincter relaxations. Although baclofen has been shown to reduce reflux symptoms in adults, data in pediatric patients are limited. The aim of the study was to evaluate the efficacy of baclofen in children with refractory GER.

Mortality and intestinal failure in surgical necrotizing enterocolitis.

Background/purpose: To examine whether as initial surgical intervention for necrotizing enterocolitis, primary peritoneal drainage as compared to primary laparotomy is associated with increased mortality or intestinal failure.

Methods: Retrospective observational study of 240 infants with surgical necrotizing enterocolitis.

Results: There was no difference concerning the composite outcome of mortality before discharge or survival with intestinal failure after adjusting for known covariates (Odds Ratio 1.

Cryptosporidial infection in children with inflammatory bowel disease.

Background And Aims: Cryptosporidiosis is usually a self-limiting illness in healthy patients. However, it can cause severe life threatening complications in immunocompromised patients. The effect of cryptosporidial infection on inflammatory bowel disease (IBD) has not been well studied and available literature is largely restricted to adult case reports.

Crohn's disease with pulmonary manifestations in children: 2 case reports and review of the literature.

Crohn's disease (CD) is a chronic granulomatous disease of unknown etiology that affects primarily the gastrointestinal system but can be associated with extraintestinal manifestations. Latent pulmonary involvement in children with CD has been described, but symptomatic pulmonary disease has rarely been reported in children. In this review, we report two pediatric cases, one with pleural effusion at the time of CD diagnosis and the other with bilateral cavitary lesions in a previously diagnosed CD patient.

Critical role for P-glycoprotein expression in hematopoietic cells in the FVB.Mdr1a(-/-) model of colitis.

Objective: P-glycoprotein (P-gp), the functional product of the multidrug resistance gene (MDR), is a transmembrane protein that extrudes substrates from the intracellular environment. P-gp is expressed on the apical surface of epithelial cells and on cells from the hematopoietic lineage. Human MDR polymorphisms have been associated with the increased risk of inflammatory bowel disease, and FVB/N animals deficient in mdr1a expression develop spontaneous colitis.

Absence of N addition facilitates B cell development, but impairs immune responses.

The programmed, stepwise acquisition of immunocompetence that marks the development of the fetal immune response proceeds during a period when both T cell receptor and immunoglobulin (Ig) repertoires exhibit reduced junctional diversity due to physiologic terminal deoxynucleotidyl transferase (TdT) insufficiency. To test the effect of N addition on humoral responses, we transplanted bone marrow from TdT-deficient (TdT(-/-)) and wild-type (TdT(+/+)) BALB/c mice into recombination activation gene 1-deficient BALB/c hosts. Mice transplanted with TdT(-/-) cells exhibited diminished humoral responses to the T-independent antigens α-1-dextran and (2,4,6-trinitrophenyl) hapten conjugated to AminoEthylCarboxymethyl-FICOLL, to the T-dependent antigens NP(19)CGG and hen egg lysozyme, and to Enterobacter cloacae, a commensal bacteria that can become an opportunistic pathogen in immature and immunocompromised hosts.

TGF-β2 suppresses macrophage cytokine production and mucosal inflammatory responses in the developing intestine.

Background & Aims: Premature neonates are predisposed to necrotizing enterocolitis (NEC), an idiopathic, inflammatory bowel necrosis. We investigated whether NEC occurs in the preterm intestine due to incomplete noninflammatory differentiation of intestinal macrophages, which increases the risk of a severe mucosal inflammatory response to bacterial products.

Methods: We compared inflammatory properties of human/murine fetal, neonatal, and adult intestinal macrophages.

Role of postnatal acquisition of the intestinal microbiome in the early development of immune function.

Objectives: Therapy with broad-spectrum antibiotics is a common practice for premature infants. This treatment can reduce the biodiversity of the fecal microbiota and may be a factor in the cause of necrotizing enterocolitis. In contrast, probiotic treatment of premature infants reduces the incidence of necrotizing enterocolitis.

Toll-like receptor 4 is protective against neonatal murine ischemia-reperfusion intestinal injury.

Purpose: Premature infants receiving probiotics have a decreased incidence of necrotizing enterocolitis. This may be mediated by intestinal bacterial signaling via toll-like receptors (TLRs) 2 and 4 maintaining intestinal homeostasis. We hypothesized that TLRs 2 and 4 are protective against ischemia-reperfusion (I/R) intestinal injury.

Acute kidney injury and renal replacement therapy independently predict mortality in neonatal and pediatric noncardiac patients on extracorporeal membrane oxygenation.

Objective: To determine the independent impact of acute kidney injury (AKI) and renal replacement therapy (RRT) in infants and children who receive extracorporeal membrane oxygenation. Despite continued expertise/technological advancement, patients who receive extracorporeal membrane oxygenation have high mortality. AKI and RRT portend poor outcomes independent of comorbidities and illness severity in several critically ill populations.

Contribution of surgical evacuation of a large subgaleal hematoma to the resolution of severe conjugated hyperbilirubinemia in a neonate. Case report.

Subgaleal hematomas (SGHs) in neonates may result from trauma to the scalp sustained during delivery. In the majority of cases, these lesions will resolve on their own without serious or long-term consequences. The authors report on a case of SGH resulting in hypotension, anemia, coagulopathy, and eventually direct hyperbilirubinemia in a neonate.

Expression of CXCL15 (Lungkine) in murine gastrointestinal, urogenital, and endocrine organs.

The ELR(+) chemokine CXCL15, which recruits neutrophils during pulmonary inflammation, is also known as lungkine due to its reported exclusive expression in the lung. We now report that CXCL15 mRNA and protein are also expressed in other mucosal and endocrine organs including the gastrointestinal and urogenital tracts and the adrenal gland. Our results indicate that CXCL15 is expressed throughout the gastrointestinal tract, with the exception of the cecum.

A potential biomarker in the cord blood of preterm infants who develop retinopathy of prematurity.

Preterm infants are at risk of developing sepsis, necrotizing enterocolitis (NEC), chronic lung disease (CLD), and retinopathy of prematurity (ROP). We used high-throughput mass spectrometry to investigate whether cord blood proteins can be used to predict development of these morbidities. Cord blood plasma from 44 infants with a birth weight of <1500 g was analyzed by surface-enhanced laser desorption/ionization time-of-flight mass spectrometry (SELDI-TOF).

Estimating blood needs for very-low-birth-weight infants.

Background: Red blood cell (RBC) transfusions are crucial for the care of very-low-birth-weight (VLBW) infants. These infants frequently require multiple, small-volume RBC transfusions, with potential exposure to multiple donors. Optimal protocols provide dedicated RBC units to reduce exposures and avoid RBC wastage.

Laparotomy versus peritoneal drainage for necrotizing enterocolitis and perforation.

Background: Perforated necrotizing enterocolitis is a major cause of morbidity and mortality in premature infants, and the optimal treatment is uncertain. We designed this multicenter randomized trial to compare outcomes of primary peritoneal drainage with laparotomy and bowel resection in preterm infants with perforated necrotizing enterocolitis.

Methods: We randomly assigned 117 preterm infants (delivered before 34 weeks of gestation) with birth weights less than 1500 g and perforated necrotizing enterocolitis at 15 pediatric centers to undergo primary peritoneal drainage or laparotomy with bowel resection.

Characterization of ureaplasmas isolated from preterm infants with and without bronchopulmonary dysplasia.

A PCR assay was used to analyze endotracheal aspirates from preterm infants for Ureaplasma parvum versus U. urealyticum. U.