Medicine and Health Sciences Commons^™

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 …

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 …

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 …

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 …

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 …

Natural Killer Cells In Liver Transplantation: Can We Harness The Power Of The Immune Checkpoint To Promote Tolerance?, Jennifer Halma, Stephen Pierce, Rebecca Mclennan, Todd Bradley, Ryan T. Fischer

Manuscripts, Articles, Book Chapters and Other Papers

The roles that natural killer (NK) cells play in liver disease and transplantation remain ill-defined. Reports on the matter are often contradictory, and the mechanisms elucidated are complex and dependent on the context of the model tested. Moreover, NK cell attributes, such as receptor protein expression and function differ among species, make study of primate or rodent transplant models challenging. Recent insights into NK function and NK-mediated therapy in the context of cancer therapy may prove applicable to transplantation. Of specific interest are immune checkpoint molecules and the mechanisms by which they modulate NK cells in the tumor micro-environment. In …

Pediatric Growth Patterns In Youth-Onset Type 2 Diabetes Mellitus: Implications For Physiologically-Based Pharmacokinetic Models., Chelsea M. Hosey, Kelsee Halpin, Valentina Shakhnovich, Chengpeng Bi, Brooke Sweeney, Yun Yan, J Steven Leeder

Manuscripts, Articles, Book Chapters and Other Papers

An accurate understanding of the changes in height and weight of children with age is critical to the development of models predicting drug concentrations in children (i.e., physiologically-based pharmacokinetic models). However, curves describing the growth of a typical population of children may not accurately characterize growth of children with various conditions, such as obesity. Therefore, to develop height and weight versus age growth curves for youth who were diagnosed with type 2 diabetes, we extracted data from electronic medical records. Robust nonlinear models were parameterized to the equations describing height and weight versus age as defined by the Centers for …

Caregiver Mental Health Screening In A Pediatric Feeding Clinic, Dana Bakula, Laura Slosky, Kelsey Dean, Ann M. Davis, Sarah T. Edwards

Posters

Introduction: One in four adults have mental health (MH) problems. Caregivers of children with feeding problems are at even higher risk for mental health (MH) problems, including depression, anxiety, and posttraumatic stress. Caregiver MH concerns have a negative impact on caregiver and child quality of life and are specifically linked to suboptimal child feeding practices. Thus, it is critical to identify caregiver MH problems in pediatric feeding settings. However, it can be challenging to comprehensively screen for caregiver MH in a busy pediatric feeding clinic. This emphasizes the importance of a feasible and brief method for screening caregiver MH. Methods: …

Presence Of Increased Mast Cells In Infants And Children With Volume And Variety Limited Intake., Amy Issa, Jensen Edwards, Meenal Singh, Craig A. Friesen, Sarah T. Edwards

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Reports indicate patients with feeding difficulties demonstrate signs of inflammation on biopsies, notably eosinophilia, but it is unknown whether mast cell density contributes to variety or volume limitation symptoms. The aim of our study was to evaluate eosinophil and mast cell density of EGD biopsies in pediatric patients with symptoms of decreased volume or variety of ingested foods.

METHODS: We conducted a single-center, retrospective chart review of EMRs for all new feeding clinic patients between 0 and 17 years of age. Patients were categorized by symptoms at the initial visit as well as eosinophil and mast cell densities in …

Use And Safety Of Prophylactic Endoscopy From A Single Center Serving Urban And Rural Children With Portal Hypertension., Voytek Slowik, Anissa Bernardez, Heather Wasserkrug, Ryan T. Fischer, James F. Daniel, Tassos Grammatikopoulos

Manuscripts, Articles, Book Chapters and Other Papers

Prophylactic endoscopy is routine in adults with portal hypertension (PHTN), but there is limited data in pediatrics. We sought to describe our experience with prophylactic endoscopy in pediatric PHTN. This is a retrospective study of 87 children who began surveillance endoscopy prior to gastrointestinal bleeding (primary prophylaxis) and 52 who began after an episode of bleeding (secondary prophylaxis) from 01/01/1994 to 07/01/2019. Patients who underwent primary prophylaxis had a lower mean number of endoscopies (3.897 vs 6.269, p = 0.001). The primary prophylaxis group was less likely to require a portosystemic shunt (6% vs 15%, p < 0.001) with no difference in immediate complications (1% vs 2%, p = 0.173) or 2-week complications (1% vs 2%, p = 0.097). No deaths were related to variceal bleeding or endoscopy. Kaplan-Meier Survival Curve suggests improved transplant and shunt free survival in the primary prophylaxis group (log-rank p < 0.001). Primary and secondary endoscopic prophylaxis should be considered safe for the prevention of variceal hemorrhage in pediatric portal hypertension. There are differences in outcomes in primary and secondary prophylaxis, but unclear if this is due to patient characteristics versus treatment strategy. Further study is needed to compare safety and efficacy to watchful waiting.