Idiopathic fibrosing pancreatitis: A rare cause of obstructive jaundice.

Jaundice is an important physiologic manifestation of both benign and insidious diseases. We report on the case of an 11-year-old male who presented with diffuse pruritis, jaundice, and later abdominal pain. Initial work-up revealed an obstructive cholestatic pattern, warranting investigation for structural anomalies. Extensive imaging revealed a lesion on the pancreatic head, and biopsy of the lesion confirmed the diagnosis of idiopathic fibrosing pancreatitis (IFP). Temporary stenting of the common bile duct successfully treated our patient's symptomatic IFP.

High Prevalence of Elevated Alanine Aminotransferase in American Indian Children Seen in a Pediatric Weight Management Clinic.

INTRODUCTION: Elevated alanine aminotransferase (ALT) can indicate risk of metabolic dysfunction-associated steatotic liver disease. However, there is little data about the prevalence of elevated ALT in American Indian (AI) children. METHODS: Baseline data from children attending the pediatric weight management clinic were used to describe the prevalence of elevated ALT, stratified by race and ethnicity. RESULTS: The prevalence of elevated ALT was higher among children who were male, were older, had higher body mass index Z scores, and were non-Hispanic and AI. DISCUSSION: In this clinic, AI children with overweight and obesity had a disproportionately high prevalence of elevated ALT; root causes of this disparity should be identified.

Esophageal Microbiome in Healthy Children and Esophageal Eosinophilia.

OBJECTIVES: There is limited knowledge about the role of esophageal microbiome in pediatric esophageal eosinophilia (EE). We aimed to characterize the esophageal microbiome in pediatric patients with and without EE. METHODS: In the present prospective study, esophageal mucosal biopsies were obtained from 41 children. Of these, 22 had normal esophageal mucosal biopsies ("healthy"), 6 children had reflux esophagitis (RE), 4 had proton pump inhibitor (PPi)-responsive esophageal eosinophilia (PPi-REE), and 9 had eosinophilic esophagitis (EoE). The microbiome composition was analyzed using 16S rRNA gene sequencing. The age median (range) in years for the healthy, RE, PPi-REE, and EoE group were 10 (1.5-18), 6 (2-15), 6.5 (5-15), and 9 (1.5-17), respectively. RESULTS: The bacterial phylum Actinobacteria, Bacteroidetes, Firmicutes, Fusobacteria, and Proteobacteria were the most predominant. The Epsilonproteobacteria, Betaproteobacteria, Flavobacteria, Fusobacteria, and Sphingobacteria class were underrepresented across groups. The Vibrionales was predominant in healthy and EoE group but lower in RE and PPi-REE groups. The genus Streptococcus, Rahnella, and Leptotrichia explained 29.65% of the variation in the data with an additional 10.86% variation in the data was explained by Microbacterium, Prevotella, and Vibrio genus. The healthy group had a higher diversity and richness index compared to other groups, but this was not statistically different. CONCLUSIONS: The pediatric esophagus has an abundant and diverse microbiome, both in the healthy and diseased states. The healthy group had a higher, but not significantly different, diversity and richness index compared to other groups.

North American Society for Pediatric Gastroenterology, Hepatology and Nutrition and the Society for Pediatric Radiology Joint Position Paper on Noninvasive Imaging of Pediatric Pancreatitis: Literature Summary and Recommendations.

ABSTRACT: The reported incidence of pediatric pancreatitis is increasing. Noninvasive imaging, including ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI), play important roles in the diagnosis, staging, follow-up, and management of pancreatitis in children. In this position paper, generated by members of the Pancreas Committee of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) and the Abdominal Imaging Committee of The Society for Pediatric Radiology (SPR), we review the roles of noninvasive imaging in pediatric acute, acute recurrent, and chronic pancreatitis. We discuss available evidence related to noninvasive imaging, highlighting evidence specific to pediatric populations, and we make joint recommendations for use of noninvasive imaging. Further, we highlight the need for research to define the performance and role of noninvasive imaging in pediatric pancreatitis.

Effect of Octreotide on Colonic Motility in Pediatric Patients With Chronic Recalcitrant Constipation.

OBJECTIVE: The aim of the present study was to study the effect of octreotide on colonic motility in pediatric patients with recalcitrant chronic constipation/encopresis and other suspected colonic motility disorders. METHODS: This was a nonrandomized, single-center, open-label, prospective study evaluating the effect of a single subcutaneous dose of octreotide on colonic motility. RESULTS: Thirteen patients (5 boys) were enrolled in the study. The age range was 4.6 to 16.2 years. Eleven patients (84%) had normal colonic manometry and 2 patients (16%) had colonic neuropathy. Motility Index (MI) (mmHg) for the 15 minutes before and after octreotide infusion was 6.03 ±â€Š1.26 (95% confidence interval [CI] 5.35-6.72) and 5.32 ±â€Š1.66 (95% CI 4.42-6.23), respectively, with P value of 0.08. MI for the 30 minutes before and after octreotide infusion was 6.89 ±â€Š1.37 (95% CI 6.14-7.64) and 6.71 ±â€Š1.47 (95% CI 5.91-7.52), respectively, with P value of 0.55. MI for the 45 minutes before and after octreotide infusion was 7.73 ±â€Š1.32 (95% CI 7.01-8.45) and 7.53 ±â€Š1.38 (95% CI 6.78-8.28), respectively, with P value of 0.8. CONCLUSION: Our study showed that the administration of octreotide resulted in no significant changes in colonic MI in pediatric patients with chronic recalcitrant constipation.

Clinical, endoscopic, and histologic features of eosinophilic inflammation of the gastrointestinal tract in pediatric liver transplant patients.

Immunosuppression during the post-transplantation period has led to dramatic outcome improvements in PLTR patients. There have been reports describing the development of food allergies and an increased predilection for development of EGI in PLTR. We aimed to identify the clinical, endoscopic and histologic features of EGI in PLTR patients. In this retrospective case series we analyzed medical record of all PLTR who underwent EGD and/or colonoscopy at our institution from 2000 to 2006. From 2000 to 2006, 32 PLTR patients underwent endoscopic evaluation. Seventeen (53%) of 32 patients were diagnosed with EGI. Endoscopic abnormalities were seen in the esophagus, stomach, and small intestine in 11 (65%), 11 (65%), and four (24%) patients, respectively. Eosinophilic inflammation was seen in the esophagus, stomach, and small intestine in 13 (76%), 10 (59%), and five (29%) patients, respectively. Nine of 17 patients underwent colonoscopy and endoscopic abnormalities were seen in four (44%) patients. Five patients (56%) had eosinophilic inflammation. In conclusion, we have characterized the clinical, endoscopic, and histologic features of EGI. Histologic and endoscopic examination reveals that, when present, EGI is often found at multiple segments along the gastrointestinal tract.

Vomiting.

Vomiting can be the presenting symptom of a variety of disorders, ranging from self-limited diseases to life-threatening diseases. The causes of vomiting vary with age of presentation, and pediatricians should develop the skill to identify serious conditions at the earliest stage based on the age of presentation. Bilious emesis at any age is a sign of intestinal obstruction until proven otherwise and needs immediate attention. Vomiting is not always due to a GI disorder, and pediatricians should look for causes outside the GI tract if no GI disease is identified.

Pancreatic stent migration into the portal vein causing portal vein thrombosis: a rare complication of a prophylactic pancreatic stent.

This report describes the first case of a novel complication of prophylactic pancreatic stents used to reduce the risk of post-endoscopic retrograde cholangiopancreatography pancreatitis, whereby the stent migrated into the portal vein resulting in portal vein thrombosis. We review the literature and discuss the potential mechanisms that caused this complication. The approach and prompt treatment that resulted in a favorable clinical outcome are described.

Eosinophilic esophagitis: epithelial mesenchymal transition contributes to esophageal remodeling and reverses with treatment.

BACKGROUND: Mechanisms underlying esophageal remodeling with subepithelial fibrosis in subjects with eosinophilic esophagitis (EoE) have not been delineated. OBJECTIVES: We sought to explore a role for epithelial mesenchymal transition (EMT) in subjects with EoE and determine whether EMT resolves with treatment. METHODS: Esophageal biopsy specimens from 60 children were immunostained for epithelial (cytokeratin) and mesenchymal (vimentin) EMT biomarkers, and EMT was quantified. Subjects studied had EoE (n = 17), indeterminate EoE (n = 15), gastroesophageal reflux disease (n = 7), or normal esophagus (n = 21). EMT was analyzed for relationships to diagnosis, eosinophil counts, and indices of subepithelial fibrosis, eosinophil peroxidase, and TGF-ß immunostaining. EMT was assessed in pretreatment and posttreatment biopsy specimens from 18 subjects with EoE treated with an elemental diet, 6-food elimination diet, or topical corticosteroids (n = 6 per group). RESULTS: TGF-ß1 treatment of esophageal epithelial cells in vitro for 24 hours induced upregulation of mesenchymal genes characteristic of EMT, including N-cadherin (3.3-fold), vimentin (2.1-fold), and fibronectin (7.5-fold). EMT in esophageal biopsy specimens was associated with EoE (or indeterminate EoE) but not gastroesophageal reflux disease or normal esophagus and was correlated to eosinophil counts (r = 0.691), eosinophil peroxidase (r = 0.738), and TGF-ß (r = 0.520) immunostaining and fibrosis (r = 0.644) indices. EMT resolved with EoE treatments that induced clinicopathologic remission with reduced eosinophil counts. EMT decreased significantly after treatment by 74.1% overall in the 18 treated subjects with EoE; pretreatment versus posttreatment EMT scores were 3.17 ± 0.82 versus 0.82 ± 0.39 (P < .001), with similar decreases within treatment groups. Pretreatment/posttreatment EMT was strongly correlated with eosinophil counts for combined (r = 0.804, P < .001) and individual treatment groups. CONCLUSIONS: EMT likely contributes to subepithelial fibrosis in subjects with EoE and resolves with treatments that decrease esophageal inflammation, and its resolution correlates with decreased numbers of esophageal eosinophils.

Letter: Perioral dermatitis in a child associated with an inhalation steroid.

Perioral dermatitis, also known as periorificial dermatitis, is characterized by a papular rash involving the perioral, perinasal and periorbital areas of the skin. There are multiple agents that may cause these lesions, with topical steroids being the most common. Inhaled steroids are rarely implicated as a cause of perioral dermatitis. Our case is illustrative because there was a clear association of perioral dermatitis with the use of inhaled steroids and a quick response to the treatment regimen, which included discontinuation of the offending agent.

Infliximab therapy in pediatric Crohn's disease: a review.

Anti-tumor necrosis factor alpha (TNF-α) therapy has re-defined our treatment paradigms in managing patients with Crohn's disease (CD) and ulcerative colitis. Although the ACCENT studies showed proven efficacy in the induction and maintenance of disease remission in adult patients with moderate to severe CD, the pediatric experience was instrumental in bringing forth the notion of "top-down" therapy to improve overall clinical response while reducing the risk of complications resulting from long-standing active disease. Infliximab has proven efficacy in the induction and maintenance of disease remission in children and adolescents with CD. In an open-labeled study of 112 pediatric patients with moderate to severe CD, 58% achieved clinical remission on induction of infliximab (5 mg/kg) therapy. Among those patients who achieved disease remission, 56% maintained disease remission on maintenance (5 mg/kg every 8 weeks) therapy. Longitudinal follow-up studies have also shown that responsiveness to infliximab therapy also correlates well with reduced rates of hospitalization, and surgery for complication of long-standing active disease, including stricture and fistulae formation. Moreover, these children have also been shown to improve overall growth while maintaining an effective disease remission. The pediatric experience has been instructive in suggesting that the early introduction of anti-TNF-α therapy may perhaps alter the natural history of CD in children, an observation that has stimulated a great deal of interest among gastroenterologists who care for adult patients with CD.