Lubiprostone for Pediatric Functional Constipation: Randomized, Controlled, Double-Blind Study With Long-term Extension.

Background & Aims: Pediatric functional constipation (PFC) is a common problem in children that causes distress and presents treatment challenges to health care professionals. We conducted a randomized, placebo-controlled trial (study 1) in patients with PFC (6-17 years of age) to evaluate the efficacy and safety of lubiprostone, followed by an open-label extension for those who completed the placebo-controlled phase (study 2).

Methods: Study 1 (NCT02042183) was a phase 3, multicenter, randomized, double-blind, placebo-controlled, 12-week study evaluating the efficacy and safety of lubiprostone 12 μg twice daily (BID) and 24 μg BID.

Hypomorphic SI genetic variants are associated with childhood chronic loose stools.

Objective: The SI gene encodes the sucrase-isomaltase enzyme, a disaccharidase expressed in the intestinal brush border. Hypomorphic SI variants cause recessive congenital sucrase-isomaltase deficiency (CSID) and related gastrointestinal (GI) symptoms. Among children presenting with chronic, idiopathic loose stools, we assessed the prevalence of CSID-associated SI variants relative to the general population and the relative GI symptom burden associated with SI genotype within the study population.

Adherence to Biopsy and Follow-Up Guidelines in a Population-Based Cohort of Children With Eosinophilic Esophagitis.

Eosinophilic esophagitis (EoE) is a chronic, immune-mediated condition that is becoming more widely recognized in children. Recent EoE practice guidelines provide clear recommendations on adequate biopsy sampling at diagnostic endoscopy and necessity of close follow-up endoscopy with biopsy to ensure mucosal healing with therapy. Despite these recommendations, adherence to biopsy guidelines, time to first follow-up endoscopy, and overall surveillance endoscopy rates have not been robustly studied.

Sensitivity and Specificity of Administrative Medical Coding for Pediatric Eosinophilic Esophagitis.

Objectives: Eosinophilic esophagitis (EoE) is a delayed-type hypersensitivity with increasing rates among pediatric populations. Although studies have used International Classification of Diseases (ICD) coding to define local cohorts and report disease epidemiology, the accuracy of the EoE ICD code for pediatric EoE is unknown.

Methods: We searched the Intermountain Healthcare Database for pediatric cases with the EoE ICD code over a 5-year period.

Avoidant/Restrictive Food Intake Disorder in Diet-treated Children With Eosinophilic Esophagitis.

Eosinophilic esophagitis (EoE) is an inflammatory condition of the esophagus with rising incidence in children. Owed to potential adverse effects and high costs of EoE medications, strict elimination diets are often employed as a mainstay of long-term EoE therapy in children. Currently, there are no effective tests to pinpoint food protein triggers in children with EoE.

Aspiration in the otherwise healthy Infant-Is there a natural course for improvement?

Objectives/hypothesis: Timing and indication for surgical intervention is a major challenge in managing pediatric oropharyngeal dysphagia. No study has evaluated a natural course of swallowing dysfunction in otherwise healthy infants. Our objective was to review the outcomes and time to resolution of abnormal swallow in infants with aspiration.

Incidence and Prevalence of Pediatric Eosinophilic Esophagitis in Utah Based on a 5-Year Population-Based Study.

Background & Aims: Eosinophilic esophagitis (EoE) is often detected in children and is considered to be a rare disease, with prevalence values reported to be below 60 cases per 100,000 persons. To determine whether the incidence of EoE in children in Utah exceeds estimates from regional reports, we calculated incidence and prevalence values over a 5-year period.

Methods: Using consensus guidelines for the diagnosis of EoE, we reviewed pathology records from the Intermountain Healthcare pathology database, from July 1, 2011 through June 31, 2016.

Safety and Pharmacokinetic Study of Fidaxomicin in Children With Clostridium difficile-Associated Diarrhea: A Phase 2a Multicenter Clinical Trial.

Background: Fidaxomicin is an approved therapy for Clostridium difficile-associated diarrhea (CDAD) in adults. The safety of fidaxomicin in children has not been reported.

Methods: In this study (ClinicalTrials.

Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI.

We report the first observation of a patient with contgenital chylous ascites (CCA) and Ehlers-Danlos syndrome type VI due to primary lymphatic defect with additional vascular anomaly. CCA is a rare condition, and there is limited understanding of its pathophysiology and treatment options. We also review the patient's treatment course mitigated with octreotide and total parenteral nutritional support, as there are no current established guidelines for CCA.

Eosinophilic esophagitis in adults is associated with IgG4 and not mediated by IgE.

Background & Aims: Eosinophilic esophagitis is usually triggered by foods, by unclear mechanisms. We evaluated the roles of IgE and IgG4 in the development of eosinophilic esophagitis.

Methods: We performed a prospective, randomized, double-blind, placebo-controlled trial of adults with eosinophilic esophagitis given an antibody against IgE (omalizumab, n = 16) or placebo (n = 14) every 2-4 weeks for 16 weeks, based on weight and serum level of IgE.

Efficacy, dose reduction, and resistance to high-dose fluticasone in patients with eosinophilic esophagitis.

Background & Aims: We evaluated the efficacy and safety of high-dose swallowed fluticasone propionate (FP) and dose reduction in patients with eosinophilic esophagitis (EoE) and analyzed esophageal transcriptomes to identify mechanisms.

Methods: We conducted a randomized, multisite, double-blind, placebo-controlled trial of daily 1760 mcg FP in participants age 3-30 years with active EoE. Twenty-eight participants received FP, and 14 participants received placebo.

Effect of gastrojejunal feedings on visits and costs in children with neurologic impairment.

Objective: The objective of the present study was to determine the effect of gastrojejunal tube (GJT) feedings in children with neurologic impairment (NI) on gastroesophageal reflux disease (GERD)- and/or dysfunctional swallowing-related visits and their associated costs.

Methods: The present study is a retrospective cohort study of children with NI and GERD who underwent GJT placement at the study hospital from December 1999 to October 2006. Visits (emergency department, radiology, and hospitalizations) were reviewed from the time of birth until 1 year following GJT placement and classified as either not GERD and/or dysfunctional swallowing related or GERD and/or dysfunctional swallowing related (eg, pneumonias).

Reslizumab in children and adolescents with eosinophilic esophagitis: results of a double-blind, randomized, placebo-controlled trial.

Background: Eosinophilic esophagitis is a chronic allergic disease with insufficient treatment options. Results from animal studies suggest that IL-5 induces eosinophil trafficking in the esophagus.

Objective: We sought to evaluate the effect of reslizumab, a neutralizing antibody against IL-5, in children and adolescents with eosinophilic esophagitis.

A multicenter, randomized, open-label, pharmacokinetics and safety study of pantoprazole tablets in children and adolescents aged 6 through 16 years with gastroesophageal reflux disease.

Children with gastroesophageal reflux disease (GERD) may benefit from gastric acid suppression with proton pump inhibitors such as pantoprazole. Effective treatment with pantoprazole requires correct dosing and understanding of the drug's kinetic profile in children. The aim of these studies was to characterize the pharmacokinetic (PK) profile of single and multiple doses of pantoprazole delayed-release tablets in pediatric patients with GERD aged 6 to 11 years (study 1) and 12 to 16 years (study 2).

Natural killer cell lymphoma in a pediatric patient with inflammatory bowel disease.

Tumor necrosis factor α (TNF-α) antibody agents are an effective therapy for the treatment of inflammatory bowel disease (IBD); however, because of the potential for immune suppression with these drugs, TNF-α antibody agents can increase the risk of malignancy. We report here the case of an 11-year-old boy who presented with bowel obstruction. He also had a history of periodic fever, aphthous stomatitis, and cervical adenitis (PFAPA).

Clinical variables as prognostic tools in pediatric-onset ulcerative colitis: a retrospective cohort study.

Background: Clinical variables may identify a subset of patients with pediatric-onset ulcerative colitis (UC) (≤18 years at diagnosis) at risk for adverse outcomes. We postulated that routinely measured clinical variables measured at diagnosis would predict colectomy in patients with pediatric-onset UC.

Methods: We conducted a chart review of patients with pediatric-onset UC at a single center over a 10-year period.

Reflux related hospital admissions after fundoplication in children with neurological impairment: retrospective cohort study.

Objective: To examine the impact of fundoplication on reflux related hospital admissions for children with neurological impairment.

Design: Retrospective, observational cohort study. Setting 42 children's hospitals in the United States.

Impact of fundoplication versus gastrojejunal feeding tubes on mortality and in preventing aspiration pneumonia in young children with neurologic impairment who have gastroesophageal reflux disease.

Objective: Aspiration pneumonia is the most common cause of death in children with neurologic impairment who have gastroesophageal reflux disease. Fundoplications and gastrojejunal feeding tubes are frequently employed to prevent aspiration pneumonia in this population. Which of these approaches is more effective in preventing aspiration pneumonia and/or improving survival is unknown.

Quality of life of children with neurological impairment who receive a fundoplication for gastroesophageal reflux disease.

Background: Children with neurological impairment (NI) commonly have gastroesophageal reflux disease (GERD) treated with a fundoplication. The impact of this procedure on quality of life is poorly understood.

Objectives: To examine the quality of life of children with NI who have received a fundoplication for GERD and of their caregivers.

Correlation of duodenal histology with tissue transglutaminase and endomysial antibody levels in pediatric celiac disease.

Background & Aims: IgA antibodies against tissue transglutaminase (TTGA) and endomysium (EMA) are sensitive and specific markers for celiac disease (CD). Data correlating TTGA and EMA levels with degree of villous atrophy are limited. We compared duodenal histopathology in pediatric CD patients with TTGA and EMA serologies, symptoms, height, and weight.

Percutaneous cholecysto-cholangiography in the diagnosis of obstructive jaundice in infants.

Background: Once it is established that a jaundiced infant has an elevated direct bilirubin level, the principal diagnostic concern is the differentiation of hepatocellular from obstructive cholestasis, of disorders of physiology from disorders of anatomy, and of disease that is managed medically from disease that is managed surgically. Traditional tests such as ultrasonography, liver biopsy, and technotium 99m HIDA scan are often not sufficiently discriminating. General anesthesia is required for invasive imaging with endoscopic retrograde cholangio pancreatography (ERCP) or operative cholangiogram.

Decreased oxidative DNA damage and accelerated cell turnover in woodchuck hepatitis virus infected liver.

Infection of newborn woodchucks with woodchuck hepatitis virus (WHV) results in hepatocellular carcinoma (HCC). Since oxidative damage may be carcinogenic, we investigated the relationship between WHV infection and oxidative damage to hepatic lipids and DNA. Eastern woodchucks were infected with WHV.

High-dose steroids, ursodeoxycholic acid, and chronic intravenous antibiotics improve bile flow after Kasai procedure in infants with biliary atresia.

Background/purpose: Early reports suggest that the use of steroids after Kasai portoenterostomy may improve bile flow and outcome in infants with biliary atresia.

Methods: Of 28 infants with biliary atresia, half received adjuvant high-dose steroids, and half received standard therapy. Infants in the steroid group (n = 14) received intravenous solumedrol (taper of 10, 8, 6, 5, 4, 3, 2 mg/kg/d), followed by 8 to 12 weeks of prednisone (2 mg/kg/d).

Gastrointestinal complications of Russell-Silver syndrome: a pilot study.

Russell-Silver syndrome (RSS) is a genetic syndrome with clinical manifestations of intrauterine and postnatal growth retardation, normal head circumference, body asymmetry, and distinctive facial appearance. We followed an infant diagnosed with RSS who had occurrence of multiple gastrointestinal complications. Although there are a number of published reports describing gastrointestinal problems associated with RSS, specific gastrointestinal diseases have not been recognized as major features.