Gastroenterology Commons^™

Leukocyte Cell-Derived Chemotaxin 2 Correlates With Pediatric Non-Alcoholic Fatty Liver Disease., Diego Paine-Cabrera, Lisa Harvey, Dakota R. Robarts, Michele T. Pritchard, John Thyfault, Steven A. Weinman, Udayan Apte, Voytek Slowik

Manuscripts, Articles, Book Chapters and Other Papers

Non-alcoholic fatty liver disease (NAFLD), newly renamed metabolic dysfunction-associated liver disease (MASLD), is a leading cause of liver disease in children and adults. There is a paucity of data surrounding potential biomarkers and therapeutic targets, especially in pediatric NAFLD. Leukocyte cell-derived chemotaxin 2 (LECT2) is a chemokine associated with both liver disease and skeletal muscle insulin resistance. Our aim was to determine associations between LECT2 and common clinical findings of NAFLD in pediatric patients. Enzyme-linked immunosorbent assay (ELISA) was used to measure serum LECT2 concentrations in children (aged 2-17 years) with and without NAFLD. LECT2 concentrations were then correlated to …

A Case Report And A Review Of Pediatric Hepatoblastoma, Anthony D. Derenzi, Audrey Bowen

HCA Healthcare Journal of Medicine

Introduction

Hepatoblastoma is a rare pediatric cancer. Approximately 100 cases of hepatoblastoma are reported per year. Due to the limited incidence of this disorder an internationally agreed- upon criteria was developed to classify patients as standard or high-risk. Studies involving chemotherapeutic agents, surgery, and liver transplants have been demonstrated to improve the disease-free survival rate. The combination of chemotherapeutic agents and surgery demonstrated the ability of these regimens to downgrade the initial diagnostic staging of tumors and transform previously unresectable tumors into resectable tumors.

Case Presentation

The following case of hepatoblastoma presents a 4-year-old male who presented to the emergency …

Small Intestinal Polyp Burden In Pediatric Peutz-Jeghers Syndrome Assessed Through Capsule Endoscopy: A Longitudinal Study., Jeremy Stewart, Nathan R. Fleishman, Vincent S. Staggs, Mike Thomson, Nicole Stoecklein, Caitlin E. Lawson, Michael P. Washburn, Shahid Umar, Thomas M. Attard

Manuscripts, Articles, Book Chapters and Other Papers

The management of pediatric Peutz-Jeghers Syndrome (PJS) focuses on the prevention of intussusception complicating small intestinal (SI) polyposis. This hinges on the accurate appraisal of the polyp burden to tailor therapeutic interventions. Video Capsule Endoscopy (VCE) is an established tool to study SI polyps in children, but an in-depth characterization of polyp burden in this population is lacking. Methods: We performed a retrospective longitudinal cross-sectional analysis of VCE studies in pediatric PJS patients at our institution (CMKC) from 2010 to 2020. Demographic, clinical, and VCE findings reported by three reviewers in tandem were accrued. Polyp burden variables were modeled as …

Molecular Characterization Of Clostridium Perfringens Isolates From A Tertiary Children's Hospital In Guangzhou, China, Establishing An Association Between Bacterial Colonization And Food Allergies In Infants., Kun-Yi Huang, Bing-Shao Liang, Xiao-Yan Zhang, Huan Chen, Ni Ma, Jiao-Li Lan, Ding-You Li, Zhen-Wen Zhou, Min Yang

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Cow's milk protein allergy (CMPA) is one of the most common types of food allergy in infants. Faecal pathogen cultures showed that the positive rate of Clostridium perfringens was more than 30%, which was significantly higher than that for other bacteria. Therefore, it is speculated that Clostridium perfringens colonization may be one of the pathogenetic factors for CMPA in infants. We conducted a real-world evidence study. Infants aged 0-6 months with diarrhoea and mucoid and/or bloody stools were recruited from a large tertiary hospital in China. Faecal pathogen cultures for the detection of Clostridium perfringens were confirmed by flight …

Contact With Caregivers Is Associated With Composition Of The Infant Gastrointestinal Microbiome In The First 6 Months Of Life, Kyle S. Wiley, Andrew M. Gregg, Molly M. Fox, Venu Lagishetty, Curt A. Sandman, Jonathan P. Jacobs, Laura M. Glynn

Psychology Faculty Articles and Research

Objectives

Little is known about how physical contact at birth and early caregiving environments influence the colonization of the infant gastrointestinal microbiome. We investigated how infant contact with caregivers at birth and within the first 2 weeks of life relates to the composition of the gastrointestinal microbiome in a sample of U.S. infants (n = 60).

Methods

Skin-to-skin and physical contact with caregivers at birth and early caregiving environments were surveyed at 2 weeks postpartum. Stool samples were collected from infants at 2 weeks, 2, 6, and 12 months of age and underwent 16S rRNA sequencing as a proxy …

Rumination Syndrome Diagnosis Supported By Colon Manometry In A Pediatric Patient, Alex Biller, John Rosen, Amornluck Krasaelap

Posters

Introduction: Rumination syndrome is a disorder of gut-brain interaction characterized by effortless repetitive postprandial regurgitation, reswallowing, and/or spitting.(1) This condition is caused by increased intragastric pressure due to abdominal muscle contractions, leading to the opening of the lower esophageal sphincter and the return of gastric content into the esophagus.(1) Rumination is a clinical diagnosis after the exclusion of other etiologies of symptoms. The diagnosis of rumination is clinical, but supportive evidence can be obtained through esophageal or antroduodenal manometry.(2) We present a unique case report of rumination syndrome diagnosis supported by data obtained during colon manometry in a pediatric patient. …

Latency Time From Prior Authorization Submission To Initiation Of Biologic Therapy In Inflammatory Bowel Disease, Shannon Capraro, Janelle R. Noel-Macdonnell Phd, Lauren Disselhoff, Layne Sieve, Rachel Chevalier

Posters

Background For pediatric patients with a diagnosis of inflammatory bowel disease (IBD), initiation of biologic therapy can be essential to attain disease control. Unfortunately, patients often experience delays in receiving the first dose of these medications due to the need for insurance companies to approve medication prior authorizations (PA). Physicians and support staff spend many working hours collating and transferring information regarding urgency to payors. To our knowledge, no studies exist to evaluate the factors that contribute to how quickly a medication is approved in pediatric patients. We set out here to evaluate which demographic and disease factors contribute to …

State Of The Art Bowel Management For Pediatric Colorectal Problems: Spinal Anomalies., Elizaveta Bokova, Ninad Prasade, John Rosen, Irene Isabel P. Lim, Marc A. Levitt, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Patients with spinal abnormalities often struggle with fecal and/or urinary incontinence (up to 87 and 92%, respectively) and require a collaborative approach to bowel management in conjunction.

METHODS: To define existing approaches and propose state-of-the-art bowel management, a literature search was performed using Medline/PubMed, Google Scholar, Cochrane, and EMBASE databases and focusing on the manuscripts published July 2013 and July 2023.

RESULTS: Patients with spinal anomalies have impaired innervation of the rectum and anal canal, decreasing the success rate from laxatives and rectal enemas. Thus, transanal irrigations and antegrade flushes are widely utilized in this group of patients. Based …

Equity And Inclusion In Pediatric Gastroenterology Telehealth: A Study Of Demographic, Socioeconomic, And Digital Disparities., Edward B. Mougey, Wesley C. Judy, Rajitha D. Venkatesh, Elizabeth Berg, Jennifer A. Lee, John Rosen, B U K Li, Alex Koster, Kristin Maiden, Zhongcui Gao, James P. Franciosi

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: The purpose of our study is to compare in-person and telehealth pediatric care ambulatory visits for gastroenterology (GI) at the Nemours Children's Health System in the Delaware Valley (NCH-DV) based on geospatial, demographic, socioeconomic, and digital disparities.

METHODS: Characteristics of 26,565 patient encounters from January 2019 to December 2020 were analyzed. U.S. Census Bureau geographic identifiers were assigned to each participant and aligned with the American Community Survey (2015-2019) socioeconomic and digital outcomes. Reported odds ratios (OR) are telehealth encounter/in-person encounter.

RESULTS: GI telehealth usage increased 145-fold in 2020 compared to 2019 for NCH-DV. Comparing telehealth to in-person usage …

State Of The Art Bowel Management For Pediatric Colorectal Problems: Hirschsprung Disease., Elizaveta Bokova, Ninad Prasade, Sanjana Janumpally, John Rosen, Irene Isabel P. Lim, Marc A. Levitt, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

After an initial pull-though, patients with Hirschsprung disease (HD) can present with obstructive symptoms, Hirschsprung-associated enterocolitis (HAEC), failure to thrive, or fecal soiling. This current review focuses on algorithms for evaluation and treatment in children with HD as a part of a manuscript series on updates in bowel management. In constipated patients, anatomic causes of obstruction should be excluded. Once anatomy is confirmed to be normal, laxatives, fiber, osmotic laxatives, or mechanical management can be utilized. Botulinum toxin injections are performed in all patients with HD before age five because of the nonrelaxing sphincters that they learn to overcome with …

Don't Look Back And Spray! Risk Of Endoscope Adhesion With Hemospray Application In Retroflexion In A Pediatric Patient., Sarah T. Edwards, Diana G. Lerner, Moises Alatorre-Jimenez, Thomas M. Attard

Manuscripts, Articles, Book Chapters and Other Papers

Although the vast majority of recognized pediatric upper gastrointestinal bleeding (GIB) resolves spontaneously, gastrointestinal hemorrhage is the most common indication for urgent or emergent therapeutic endoscopy in pediatric practice. The application of hemostatic powders, including TC-325 (Hemospray, Cook Medical, Winston-Salem, NC, USA), has shown considerable impact on the control of acute bleeding, with the advantage of potentially covering an extensive area and requiring less technical expertise. We report a case of transient adherence of an esophagogastroduodenoscopy following Hemospray application in a 22-month-old with upper GIB. Our experience does not detract from the significant gains in the management of pediatric GIB …

Prevalence, Management And Efficacy Of Treatment In Portal Vein Obstruction After Paediatric Liver Transplantation: Protocol Of The Retrospective International Multicentre Portal Registry., Bader A. Alfares, Hubert P J Van Der Doef, Barbara E. Wildhaber, Thomas Casswall, Greg Nowak, Martin Delle, Denise Aldrian, Valeria Berchtold, Georg F. Vogel, Piotr Kaliciński, Malgorzata Markiewicz-Kijewska, Adam Kolesnik, Jesús Q. Bernabeu, María Mercadal Hally, Mauricio Larrarte K, Paolo Marra, Michela Bravi, Domenico Pinelli, Mureo Kasahara, Seisuke Sakamoto, Hajime Uchida, Vidyadhar Mali, Marion Aw, Stéphanie Franchi-Abella, Emmanuel Gonzales, Florent Guérin, Guillermo Cervio, Julia Minetto, Sergio Sierre, Martin De Santibañes, Victoria Ardiles, Jimmy Walker Uno, Helen Evans, David Duncan, John Mccall, Steffen Hartleif, Ekkehard Sturm, Jai Patel, Marumbo Mtegha, Raj Prasad, Cristina T. Ferreira, Luiza S. Nader, Marco Farina, Catalina Jaramillo, Manuel I. Rodriguez-Davalos, Peter Feola, Amit A. Shah, Phoebe M. Wood, Michael R. Acord, Ryan T. Fischer, Bhargava Mullapudi, Richard J. Hendrickson, Rajeev Khanna, Viniyendra Pamecha, Amar Mukund, Khalid Sharif, Girish Gupte, Simon Mcguirk, Gilda Porta, Marco Spada, Tommaso Alterio, Giuseppe Maggiore, Winita Hardikar, Marisa Beretta, Rudi Dierckx, Ruben H J De Kleine, Reinoud P H Bokkers

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Portal vein obstruction (PVO) consists of anastomotic stenosis and thrombosis, which occurs due to a progression of the former. The aim of this large-scale international study is to assess the prevalence, current management practices and efficacy of treatment in patients with PVO.

METHODS AND ANALYSIS: The Portal vein Obstruction Revascularisation Therapy After Liver transplantation registry will facilitate an international, retrospective, multicentre, observational study, with 25 centres around the world already actively involved. Paediatric patients (agedyears) with a diagnosed PVO between 1 January 2001 and 1 January 2021 after liver transplantation will be eligible for inclusion. The primary endpoints are …

Overactive Bladder Syndrome Symptoms In Youth With Abdominal Pain-Associated Disorders Of Gut-Brain Interaction., Hunter J. Friesen, Pierce Thompson, Jennifer Verrill Schurman, Jennifer Colombo, Craig A. Friesen

Manuscripts, Articles, Book Chapters and Other Papers

The purpose of the current study was to assess the frequency of overactive bladder syndrome (OBS) symptoms and their relationship to gastrointestinal symptoms in youth with abdominal pain-associated disorders of gut-brain interaction (AP-DGBI). This is a retrospective study of 226 youth diagnosed with an AP-DGBI. As part of standard care, all patients completed a symptom questionnaire regarding gastrointestinal and non-gastrointestinal symptoms including increased urinary frequency, nighttime urination, and urinary urgency. Overall, 54% of patients reported at least one OBS symptom. Increased frequency of urination was reported by 19%, urinary urgency by 34%, and nighttime urination by 36%. Increased frequency of …

State Of The Art Bowel Management For Pediatric Colorectal Problems: Functional Constipation., Elizaveta Bokova, Wendy Jo Svetanoff, John Rosen, Marc A. Levitt, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Functional constipation (FC) affects up to 32% of the pediatric population, and some of these patients are referred to pediatric surgery units to manage their constipation and/or fecal incontinence. The aim of the current paper is to report the recent updates on the evaluation and management of children with FC as a part of a manuscript series on bowel management in patients with anorectal malformations, Hirschsprung disease, spinal anomalies, and FC.

METHODS: A literature search was performed using Medline/PubMed, Google Scholar, Cochrane, and EMBASE databases and focusing on the manuscripts published within the last 5-10 years.

RESULTS: The first …

Pharmacogenetic Testing For The Pediatric Gastroenterologist: Actionable Drug-Gene Pairs To Know., Tracy L. Sandritter, Rachel Chevalier, Rebecca Abt, Valentina Shakhnovich

Manuscripts, Articles, Book Chapters and Other Papers

Gastroenterologists represent some of the earlier adopters of precision medicine through pharmacogenetic testing by embracing upfront genotyping for thiopurine S-methyltransferase nucleotide diphosphatase (TPMT) before prescribing 6-mercaptopurine or azathioprine for the treatment of inflammatory bowel disease. Over the last two decades, pharmacogenetic testing has become more readily available for other genes relevant to drug dose individualization. Common medications prescribed by gastroenterologists for conditions other than inflammatory bowel disease now have actionable guidelines, which can improve medication efficacy and safety; however, a clear understanding of how to interpret the results remains a challenge for many clinicians, precluding wide implementation of …

Diagnostic Role Of Fractional Exhaled Nitric Oxide In Pediatric Eosinophilic Esophagitis, Relationship With Gastric And Duodenal Eosinophils., Panamdeep Kaur, Rachel Chevalier, Craig A. Friesen, Jamie Ryan, Ashley K. Sherman, Stephanie Page

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Eosinophilic esophagitis (EoE) is an eosinophilic-predominant inflammation of the esophagus diagnosed by upper endoscopy and biopsies. A non-invasive and cost-effective alternative for management of EoE is being researched. Previous studies assessing utility of fractional exhaled nitric oxide (FeNO) in EoE were low powered. None investigated the contribution of eosinophilic inflammation of the stomach and duodenum to FeNO.

AIM: To assess the utility of FeNO as a non-invasive biomarker of esophageal eosinophilic inflammation for monitoring disease activity.

METHODS: Patients aged 6-21 years undergoing scheduled upper endoscopy with biopsy for suspected EoE were recruited in our observational study. Patients on steroids …

Evaluating The Impact Of Routine Enteral Iron Fortification On Gastrointestinal Dysbiosis In Premature Infants, Matthew Van Ormer

Theses & Dissertations

The gastrointestinal microbiome in preterm infants bears significant influence on optimal outcomes – dysbiosis is shown to substantially increase the risk of the life-threatening necrotizing enterocolitis, whereas optimal microbiome composition improves nutrient digestion and immune function. Iron is a vital nutrient especially during the perinatal window of rapid hemoglobin production, tissue growth, and foundational neurological development. However, excess colonic iron exhibits potent oxidation capacity and alters the competitive environment of microbes – potentially facilitating the proliferation of pathological bacterial strains in the gut. Of note, breastfed preterm infants routinely receive iron fortification starting at 14 days of life and are …

Use, Durability, And Risks For Discontinuation Of Initial And Subsequent Biologics In A Large Pediatric-Onset Ibd Cohort., Jess L. Kaplan, Chunyan Liu, Eileen C. King, Julie A. Bass, Ashish S. Patel, Jeanne Tung, Shiran Chen, Trevor Lissoos, Ninfa Candela, Shehzad Saeed, Richard B. Colletti, Improvecarenow Network

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Biologic medications are recommended for treatment of moderately-to-severely active Crohn disease (CD) or ulcerative colitis (UC) in children. However, many patients require sequential biologic treatment because of nonresponse or loss of response to the initial biologic.

METHODS: We analyzed pediatric inflammatory bowel disease (IBD) data from the ImproveCareNow Network registry between May 2006 and September 2016, including time to biologic initiation, choice of first subsequent biologics, biologic durability, and reasons for discontinuation.

RESULTS: Of 17,649 patients with IBD [CD: 12,410 (70%); UC: 5239 (30%)], 7585 (43%) were treated with a biologic agent before age 18 (CD: 50%; UC: 25%). …

Drug Metabolizing Enzymes And Transporters May Help Determine Effective Budesonide Dosing In Eoe, Laurie Mccann, Lisa Harvey, Norah Almahbub, Wendy Y. Wang, Erin C. Boone, Janelle R. Noel-Macdonnell, Rachel Chevalier

Posters

Background: Eosinophilic esophagitis (EoE) is a chronic inflammatory disorder diagnosed in children with painful or difficult swallowing, vomiting, or poor weight gain.  Current treatment models adopt a trial-and-error approach in regard EoE treatment, including restrictive elimination diets, proton pump inhibitors, and topical budesonide. This approach can delay effective treatment which increases risk of disease progression and increases medical costs to families for frequent clinic visits and endoscopy.   Objectives/Goal: The objective of this study is to determine the CYP3A5 genotype and expression of patients with eosinophilic esophagitis to discover which patients will respond to standard dosing of topical budesonide treatment (1, …

A Metabolic, Mechanical, Multi-Organ Masterpiece: Dural Device Support Bridge To En-Bloc Heart-Liver Transplantation In Propionic Acidemia, Rebecca Juhl, Brian Birnbaum, Aliessa P. Barnes, William Gibson, Bhargava Mullapudi, Beth Lang, Megan Faseler, Daniel E. Heble, Victoria Urban, Ryan T. Fischer, Jennifer L. Gannon, David Sutcliffe

Posters

Introduction: Propionic Acidemia (PA) is a disorder related to abnormal protein and lipid metabolism resulting in progressive neurological injury and dilated cardiomyopathy (DCM). Interventions for PA and secondary disease manifestations can require multi-organ transplantation. Herein we report the case of a child with PA and end-stage DCM requiring left ventricular assist device (LVAD) support with eventual heart-liver transplant. Case Report: A 17 year old male diagnosed in childhood with PA developed chronically progressive DCM culminating in end stage heart failure with acute decompensations. In a recurrent admission, he progressed to require dual inotropic support and systemic anticoagulation for new LV …

Impact Of Duodenal Pathology On Oral Drug Bioavailability And Disease Outcomes In Pediatric Crohn's Disease., Rebecca Casini, Carrie A. Vyhlidal, Julia M. Bracken, Ashley K. Sherman, Atif Ahmed, Vivekanand Singh, Veronica Williams, Valentina Shakhnovich

Manuscripts, Articles, Book Chapters and Other Papers

Background: Crohn's disease with upper gastrointestinal tract involvement occurs more often in children than adults and has the potential to interfere with oral drug absorption. We aimed to compare disease outcomes in children receiving oral azathioprine for the treatment of Crohn's disease with (DP) and without (NDP) duodenal pathology at diagnosis.

Methods: Duodenal villous length, body mass index (BMI), and laboratory studies were compared in DP vs. NDP during the first year post-diagnosis, using parametric/nonparametric tests and regression analysis (SAS v9.4); the data are reported as the median (interquartile range) or the mean ± standard deviation. Thiopurine metabolite concentration (pmol/8 …

Diagnosis And Management Of Cyclic Vomiting Syndrome, Sara Read

Capstone Showcase

Cyclic vomiting syndrome (CVS) is a chronic and debilitating condition that can be encountered in a multitude of clinical settings. It is classified as a functional gastrointestinal disorder and is often misdiagnosed. Proper identification and management of this condition are crucial to patient care and quality of life. This article reviews the hypothesized etiologies, clinical presentation, and diagnosis of CVS as well as recommendations for management in a variety of settings. As an understudied and underdiagnosed condition, it is important that clinicians have a basic understanding of this disorder.

Two Siblings With Microvillous Inclusion Disease, Moises Alatorre-Jimenez, Brandi Weller, Meike Orlick, William San Pablo

Posters

Introduction: Microvillous Inclusion Disease (MVID) (OMIM#251850) is a rare autosomal recessive condition caused by mutations or deletions mostly in the MYH6 gene but also STX3 and STXBP2 genes. It is characterized by protracted diarrhea with severe congenital alteration of the intestinal epithelium resulting in watery diarrhea, metabolic acidosis, failure to thrive, and permanent malabsorption that normally leads to a lifelong dependency on total parenteral nutrition (TPN) and the eventual possibility of bowel transplantation . Histological hallmarks of MVID in small intestinal biopsies are hypoplastic, atrophic, or disorganized villi without crypt hypertrophy, or immune cell infiltrate and, at the intestinal cellular …

Endoscopic Removal Of Safety Pin From Appendiceal Orifice, Alex Biller, Moises Alatorre-Jimenez, Jeremy Stewart, Ruba A. Abdelhadi

Posters

Introduction: Foreign body ingestion in children is considered a common concern in every Emergency Department worldwide. In the United States, it is estimated that foreign body ingestions cause approximately 1,500 deaths yearly (1). Safety pins ingestions are estimated to be 1% of the foreign body ingestions in children (2). In literature, ingested safety pins have been reported to be lodged mostly in the upper gastrointestinal tract, but a few cases have been reported to be lodged in the appendix (2,3,4). Objective: Recommendations for safety pin ingestions when past the second portion of the duodenum. Case: We report the case of …

Intraductal Papillary Mucinous Neoplasm In An 18-Year-Old With Recurrent Pancreatitis, Nadia Ibrahimi, Thomas M. Attard, Moises Alatorre-Jimenez, Karie Robinson, Syed Jafri

Posters

Introduction: Intraductal Papillary Mucinous Neoplasms (IPMNs) of the pancreas are premalignant, intraductal, mucin secreting cystic lesions. IPMNs are a rare entity in the pediatric population and commonly seen in men in their 6 to 7 decade. Pediatric IPMN has been previously reported in a 14-year-old and a neonate, who also had hyperinsulinism and a de novo germline SKIL gene mutation. . Kim et al. did a ten-year single centered pediatric pancreatic malignancy study discovering IPMN in one patient of 35 with pancreatic mass removals . Case: Our patient is an 18-year-old male who initially presented at the age of 15 …

Endoscopic Retrograde Cholangiography Using A Colonoscope In A Pediatric Liver Transplant Patient With Roux-En-Y Biliary Anastomosis, Nadia Ibrahimi, Thomas M. Attard, Ryan T. Fischer, Voytek Slowik, Richard J. Hendrickson, Bhargava Mullapudi, Moises Alatorre-Jimenez, Syed Jafri

Posters

Introduction: Biliary ducal sequelae including occlusions, strictures, leaks, or necrosis frequently complicate pediatric liver transplantation. The underlying etiology includes prolonged intraoperative ischemia or postoperative arterial inflow obstruction. In adult patients the role of endoscopic retrograde cholangiography (ERC) is well established in the management of biliary complications post-liver transplantation. In contrast, in the pediatric population, due to the combination of small patient size and the complexity of Roux-en-Y anastomotic techniques, ERC has been described as difficult or virtually impossible. This limits the options available for managing biliary duct complications to percutaneous techniques Endoscopic management of biliary complications in pediatric patients with …

Inverted Appendix Presenting As Abdominal Pain And Lower Gastrointestinal Bleeding, Moises Alatorre-Jimenez, Thomas M. Attard

Posters

Background: Cecal polyps are challenging insofar as the region represents the highest risk of bleeding and perforation during polypectomy . In addition, polypoid lesions in the cecum may represent a morphologically abnormal ileocecal valve or an inverted appendix. Inadvertent polypectomy in those scenarios would lead to serious sequelae including perforation. Case report: Our patient is a 6-year-old male with a background history of prematurity, lactose intolerance, chronic esophagitis, gastritis, constipation, and painless rectal bleeding. He presented with a acute onset diffuse, severe abdominal pain associated with diarrhea that evolved to include hematochezia. At the time of presentation, he required aggressive …

Characterization Of The Mucosally-Adherent Duodenal Microbiome In Children With And Without Crohn's Disease., Kenneth Schmidt, Janelle R. Noel-Macdonnell Phd, Carrie Vyhlidal, Daniel P. Heruth, Vivekanand Singh, Atif A. Ahmed, Taina Hudson, Veronica Williams, Valentina Shakhnovich

Manuscripts, Articles, Book Chapters and Other Papers

Manipulation of the microbiome is a rational treatment strategy for inflammatory bowel disease (IBD). Compared to the colon and terminal ileum (TI), understanding of the microbial composition in the duodenum is sparse. This gap in knowledge is especially significant for children with Crohn's disease (CD) because the prevalence of duodenal CD is higher in children than in adults. Our aim was to characterize the bacterial composition of the mucosally-adherent duodenal microbiome in children with and without CD as a first step toward development of targeted IBD treatment strategies at this disease location. Fresh-frozen mucosal biopsies were obtained from the duodenum …

Disease Burden And Management Of Crigler-Najjar Syndrome: Report Of A World Registry, Sem J Aronson, Norman Junge, Mediha Trabelsi, Wided Kelmemi, Aurelie Hubert, Karlla W Brigatti, Michael D. Fox, Robert J De Knegt, Johanna C Escher, Virginia M Ginocchio, Raffaele Iorio, Yan Zhu, Figen Özçay, Fakher Rahim, Mortada H F El-Shabrawi, Eyal Shteyer, Angelo Di Giorgio, Lorenzo D'Antiga, Federico Mingozzi, Nicola Brunetti-Pierri, Kevin A Strauss, Philippe Labrune, Ridha Mrad, Ulrich Baumann, Ulrich Beuers, Piter J Bosma

Department of Pediatrics Faculty Papers

Background and Aims Crigler-Najjar syndrome (CNS) is a disorder of bilirubin conjugation leading to brain damage and death without treatment. Although cohort studies of limited size have been published, uncertainty about outcome, co-morbidities, occurence of liver fibrosis and treatment outcome remains. With this worldwide cohort study, we aim to add substantial knowledge to the previously published data.

Methods Anonymized retrospective data of CNS patients were collected in a web-based registry platform.

Results Clinical data of 221 CNS patients (46% female, severe phenotype n = 209) were collected. At the time of analysis, 59 CNS patients were deceased. Mean serum total …

Improving Advanced Therapeutic Endoscopy Performance At An Academic Children's Hospital, Julia Snodgrass, Thomas M. Attard, Donna Ditsch, John Colgan, Amanda Weston, Jessica Koch, Nadia Ibrahimi, Pang Krasaelap, Voytek Slowick, Laura Shroyer

Research Days

Problem Statement/Question: Advanced therapeutic endoscopy is infrequently needed but critical in the management of children with complex gastroenterology disorders. Hands-on experience in specific advanced techniques, set up of endoscopy equipment and familiarity with new ancillary equipment for use during endoscopy have been intra-departmentally identified as potential areas for improvement.

Background/Project Intent: Our aim is to improve provider scoring on an endoscopy practice test based on technical aspects of therapeutic techniques, indications and set up of standard and new endoscopic equipment by 20% over pre-practice session testing.

Methods: The Model for Improvement framework was utilized. A multidisciplinary team was created including …