Pediatrics Commons^™

Ileal Atresia, Obiyo O. Osuchukwu, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

Ileal and jejunal atresias are usually described together as jejunoileal atresia (JIA). JIA is a common cause of intestinal obstruction in neonates. It is seen in 1 in 5000 to 1 in 14000 live births. Intestinal atresia can occur in any location on the small bowel as a solitary or even multiple lesions. Distally located atresia usually presents with delayed symptoms compared to proximal ones. Occasionally, JIA is associated with other malformations such as cardiac anomalies, gastroschisis, and cystic fibrosis. Evaluation can be initiated before birth with prenatal diagnosis using ultrasound findings of evidence of intestinal obstruction reported in 29% …

Patent Urachus, Kayla B. Briggs, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

atent urachus refers to one condition in a rare spectrum of disorders referred to as urachal anomalies. These conditions result from the failure of the involution of normal embryologic tissues that serve to empty the fetal bladder. The location and amount of persistent tissue dictate the presenting symptoms. Some of these urachal anomalies are obvious at birth, while others are more subtle and not diagnosed until adulthood or only incidentally discovered after imaging is obtained for other reasons. Historically, surgical resection of urachal anomalies was routinely undertaken, given the potential for malignancy in retained ectopic tissue. Early surgical resection has …

Pentalogy Of Cantrell, Muhammad Khawar Sana, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

Pentalogy of Cantrell (POC) is a collection of five congenital midline birth anomalies that present a distinctive challenge for clinicians and surgeons. Those five defects are of the heart, pericardium, diaphragm, sternum, and abdominal wall. This condition has been divided into two categories, complete or partial. Complete, as the name indicates, refers to the presence of all five defects, while others may present with only partial defects. It is also referred to as thoracoabdominal ectopia cordis, a condition where the heart is covered by an omphalocele-like membrane. Ectopia cordis (EC) is often found in fetuses with POC. Infants usually have …

Two Months Of Global Health In Maseru, Lesotho, Rachel Hildebrand

Posters

Describes her experience working with infants and children at the Center of Excellence and Adolescents at Queen Mamohato Memorial Hospital in Maseru, Lesotho.

Global Health Immersive Elective In The Philippines, Lisa Godfrey

Posters

Describes her experiences working at Fabella Memorial NICU and the Philippines General Hospital.

Gastroschisis, Rebecca M. Rentea, Vikas Gupta

Manuscripts, Articles, Book Chapters and Other Papers

Gastroschisis is a paraumbilical, full-thickness abdominal wall defect associated with protrusion of the bowel through the defect. It is rarely associated with genetic conditions. A membrane does not cover the bowel exposed in utero and, as a result, may be matted, dilated, and covered with a fibrinous inflammatory rind. Infants have a high proportion of intrauterine growth restriction. Diagnosis is often made on the 20-week ultrasound with free-floating bowel loops in the uterine cavity. Maternal serum alpha-fetoprotein (AFP) is elevated in pregnancies with gastroschisis. Compared with other abdominal wall defects diagnosed prenatally such as omphalocele, only 10 percent of cases …

Torsion Of An Accessory Spleen In A Child With Biliary Atresia Splenic Malformation Syndrome., David A. Simon, Nathan R. Fleishman Md, Pamala Choi, Jason D. Fraser, Ryan T. Fischer

Manuscripts, Articles, Book Chapters and Other Papers

Torsion of an accessory spleen is an exceedingly rare cause of abdominal pain in pediatric patients. The diagnosis is frequently challenging as presentation is variable and diagnostic imaging can be aspecific. The current case describes an unusual presentation of a torted accessory spleen in a 5-year-old girl with biliary atresia splenic malformation syndrome who initially presented with non-specific abdominal symptoms and fever. The diagnosis was made following fine-needle aspiration of a suspected intraabdominal abscess. The case highlights the diagnostic challenge of accessory splenic torsion and stresses the importance of its inclusion on the differential diagnosis of pediatric patients, especially those …

Hypomorphic Si Genetic Variants Are Associated With Childhood Chronic Loose Stools., Bruno P Chumpitazi, Jeffery Lewis, Derick Cooper, Mauro D'Amato, Joel Lim, Sandeep Gupta, Adrian Miranda, Natalie Terry, Devendra Mehta, Ann Scheimann, Molly O'Gorman, Neelesh Tipnis, Yinka Davies, Joel Friedlander, Heather Smith, Jaya Punati, Julie Khlevner, Mala Setty, Carlo Di Lorenzo

Manuscripts, Articles, Book Chapters and Other Papers

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.

METHODS: A prospective study conducted at 18 centers enrolled 308 non-Hispanic white children ≤18 years old who were experiencing chronic, idiopathic, loose stools at least once per week for >4 weeks. Data on demographics, GI …

Philippines: An Int-Erupted Experience, Susamita Kesh

Posters

Describes her experience working in the NICU of the Fabella Memorial Hospital in Philippines.

Fit Clinical Decision-Making: Uhl's Anomaly: An Uncommon Cause Of Fetal Cardiomegaly, Bethany Runkel, Atif Ahmed, Tara Swanson, Maria Kiaffas

Posters

No abstract provided.

An Unexpected Global Health Experience In Manila, Susan Mcanany

Posters

Describes her experience in the NICU of the Fabella Memorial Hospital in Manila, Philippines.

Global Health Elective In Santiago Atitlan, Guatemala, Sarah Greenberg

Posters

Describes her experience working in Hospitalito Atitlan in Santiago Atitlan, Guatemala.

Fit Clinical Decision-Making Unusual Echocardiographic Findings Of Myocarditis Mimicking An Aortic Runoff Lesion, Sarah Studyvin, Christine Symes, Barbara A. Pahud, Nitin Madan

Posters

No abstract provided.

Allergic Reaction In Hem/Onc Patient, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Incidence Of Initial Renal Replacement Therapy Over The Course Of Kidney Disease In Children., Derek K. Ng, Matthew B. Matheson, Bradley A. Warady, Susan R. Mendley, Susan L. Furth, Alvaro Muñoz

Manuscripts, Articles, Book Chapters and Other Papers

The Chronic Kidney Disease in Children Study, a prospective cohort study with data collected from 2003 to 2018, provided the first opportunity to characterize the incidence of renal replacement therapy (RRT) initiation over the life course of pediatric kidney diseases. In the current analysis, parametric generalized gamma models were fitted and extrapolated for RRT overall and by specific treatment modality (dialysis or preemptive kidney transplant). Children were stratified by type of diagnosis: nonglomerular (mostly congenital; n = 650), glomerular-hemolytic uremic syndrome (HUS; n = 49), or glomerular-non-HUS (heterogeneous childhood onset; n = 216). Estimated durations of time to RRT after …

Anticipation, Accompaniment, And A Good Death In Perinatal Care., Bryanna S. Moore, Brian S. Carter, Bryan Beaven, Katie House, Joel House

Manuscripts, Articles, Book Chapters and Other Papers

The ethics of perinatal care, and the experiences of families who receive such care, remains a nascent area of inquiry. It can be hard to see how existing "good death" constructs apply to the experiences of fetal patients and their families. In this paper, we explore two themes raised by a case at our fetal health center: anticipation and accompaniment. In this case, a mother presented to our fetal health center; her unborn son, our fetal patient, was diagnosed with life-threatening hypoplastic left heart syndrome and endocardial fibroelastosis. The parents were told that their son's life expectancy, upon birth, was …

Transanal-Only Swenson-Like Pull-Through For Late Diagnosed Hirschsprung Disease., Rebecca M. Rentea, Devin R. Halleran, Hira Ahmad, Elias Maloof, Richard J. Wood, Marc A. Levitt

Manuscripts, Articles, Book Chapters and Other Papers

Hirschsprung disease (HD) is an obstructive colonic process usually diagnosed in the neonatal period. A small subset of cases are diagnosed late, present with severe constipation without enterocolitis and have low rectosigmoid disease. A transanal-only pull-through is a well-described approach but in the newborn period risks a situation whereby the transition zone is higher than the sigmoid. We present our experience with the unique patient population of older HD patients in whom the transition zone was reliably reachable via a single-stage transanal approach, performed in prone position. Patients between 2 and 6 years of age with a rectal or sigmoid …

Dr. Ahmed Abdelmoity To Receive Endowed Chair, Children's Mercy Hospital

Our Story Continues

Former Children’s Mercy CEO Rand O’Donnell, PhD, and his wife, Melva, are establishing an endowed chair position that will be awarded to Ahmed Abdelmoity, MD, FAAP, Division Director of Child Neurology, Section Chief of Epilepsy and Neurophysiology. Dr. Abdelmoity will become our 31st endowed chair at Children’s Mercy.

Unraveling Complexity About Childhood Obesity And Nutritional Interventions: Modeling Interactions Among Psychological Factors., Keith Feldman, Gisela M B Solymos, Maria Paula De Albuquerque, Nitesh V. Chawla

Manuscripts, Articles, Book Chapters and Other Papers

As the global prevalence of childhood obesity continues to rise, researchers and clinicians have sought to develop more effective and personalized intervention techniques. In doing so, obesity interventions have expanded beyond the traditional context of nutrition to address several facets of a child's life, including their psychological state. While the consideration of psychological features has significantly advanced the view of obesity as a holistic condition, attempts to associate such features with outcomes of treatment have been inconclusive. We posit that such uncertainty may arise from the univariate manner in which features are evaluated, focusing on a particular aspect such as …

An Electronic Medical Records-Based Approach To Identify Idiosyncratic Drug-Induced Liver Injury In Children., Tracy L. Sandritter, Jennifer Goldman, Clayton J Habiger, James F. Daniel, Jennifer Lowry, Ryan T. Fischer

Manuscripts, Articles, Book Chapters and Other Papers

Drug-induced liver injury (DILI) is the leading cause of liver failure in the United States and the most common cause of drug recall. As opposed to the recognized direct toxicity of super-therapeutic acetaminophen or chemotherapeutic agents in children, limited data exists for pediatric populations on the incidence of idiosyncratic DILI (iDILI) that may develop independently of drug dose or duration of administration. To improve the detection of adverse drug reactions at our hospital, we utilized electronic medical records-based automated trigger tools to alert providers of potential iDILI. Clinical criteria concerning for iDILI were defined as serum ALT > 5x or serum …

Pneumothorax: What’S A Pediatric Pulmonologist To Do?, Terrence W. Carver

Manuscripts, Articles, Book Chapters and Other Papers

A pneumothorax is a collection of air in the pleural space commonly associated with respiratory symptoms. Patients may be relatively asymptomatic but may present with life-threatening distress. Confirmation of a clinically suspected pneumothorax is most often by chest x-ray when patients have been stabilized. Pneumothoraces are first categorized as primary, secondary, or iatrogenic and informs the evaluation and treatment plan. However, despite management guidelines in the literature, their applicability for pediatric patients is limited. Understanding of the historical risk factors including the acute clinical course and how radiographs, both chest X-ray and CT chest illuminate which patients are at highest …

The Early "Unnatural" History Following Surgical Repair Of Ventricular Septal Defects., Sathish M. Chikkabyrappa, Justin T. Tretter, Arpan R. Doshi, Sujatha Buddhe, Puneet Bhatla, Achi Ludomirsky

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: Surgical outcomes for simple ventricular septal defects (VSD) have been excellent in the past three decades. For this project, the timing of resolution of left-sided dilation and mitral regurgitation (MR) following VSD repair was assessed.

Methods: Echocardiographic data surrounding surgery of 42 consecutive children who underwent surgical patch repair of a VSD were reviewed. The echocardiograms were reviewed up to a mean of 12 months post-operatively (range 9 - 14 months). Quantitative data indexed to body surface area including left atrial (LA) volume, mitral valve annulus diameter, and left ventricular end-diastolic dimension (LVEDD) was analyzed.

Results: The majority of …

Gamna-Gandy Bodies Present As A Spleen Mass In A Patient With Hereditary Spherocytosis, Chandni Dargan, Sahibu Sultan M Habeebu, Grace S. Mitchell, Weijie Li

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Pain, Functional Disability, And Their Association In Juvenile Fibromyalgia Compared To Other Pediatric Rheumatic Diseases., Mark Connelly, Jennifer E Weiss, Carra Registry Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Severe pain and impairments in functioning are commonly reported for youth with juvenile fibromyalgia. The prevalence and impact of pain in other diseases commonly managed in pediatric rheumatology comparatively have been rarely systematically studied. The objective of the current study was to determine the extent to which high levels of pain and functional limitations, and the strength of their association, are unique to youth with juvenile primary fibromyalgia syndrome/JPFS) relative to other pediatric rheumatic diseases.

METHODS: Using data from 7753 patients enrolled in the multinational Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry, we compared the levels and …

Failure To Rescue As An Outcome Metric For Pediatric And Congenital Cardiac Catheterization Laboratory Programs: Analysis Of Data From The Impact Registry., Michael L. O'Byrne, Kevin F. Kennedy, Natalie Jayaram, Lisa J. Bergersen, Matthew J. Gillespie, Yoav Dori, Jeffrey H. Silber, Steven M. Kawut, Jonathan J. Rome, Andrew C. Glatz

Manuscripts, Articles, Book Chapters and Other Papers

Background Risk-adjusted adverse event (AE) rates have been used to measure the quality of pediatric and congenital cardiac catheterization laboratories. In other settings, failure to rescue (FTR) has demonstrated utility as a quality metric. Methods and Results A multicenter retrospective cohort study was performed using data from the IMPACT (Improving Adult and Congenital Treatment) Registry between January 2010 and December 2016. A modified FTR metric was developed for pediatric and congenital cardiac catheterization laboratories and then compared with pooled AEs. The associations between patient- and hospital-level factors and outcomes were evaluated using hierarchical logistic regression models. Hospital risk standardized ratios …

Pharmacometabolomics Of Respiratory Phenotypic Response To Dexamethasone In Preterm Infants At Risk For Bronchopulmonary Dysplasia., Tamorah R. Lewis Md Phd, Prabhakar Chalise, Cheri Gauldin, William E. Truog

Manuscripts, Articles, Book Chapters and Other Papers

A prospective cohort study was performed in preterm infants less than 32 weeks gestation at birth who were treated with dexamethasone for developing or established bronchopulmonary dysplasia (BPD). Respiratory phenotype (Respiratory Severity Score (RSS)), serum, and urine metabolomics were assessed before and after treatment. Ten infants provided nine matched serum and nine matched urine samples. There was a significant decrease in RSS with steroid treatment. Serum gluconic acid had the largest median fold change (140 times decreased, P = 0.008). In metabolite set enrichment analysis, in both serum and urine, the urea cycle, ammonia recycling, and malate-aspartate shuttle pathways were …

Standardizing Aminoglycoside Induced Ototoxicity Monitoring, Claire Elson, Christopher M. Oermann, Stephanie Duehlmeyer, Ellen Meier

Posters

Aminoglycoside (AG) antibiotics are essential for the treatment of cystic fibrosis (CF) lung infections. Pseudomonas aeruginosa. Nontuberculous mycobacteria. Monitoring is critical secondary to potential nephrotoxicity and ototoxicity. Children’s Mercy Kansas City (CMKC), standardized nephrotoxicity monitoring in 2016. Observed variable ototoxicity monitoring practices. Prevalence of ototoxicity, 2016 CFF Patient Registry, 1.1% in pediatric patients (less than or equal to 18 years). 2.2% in pediatric and adult population. National Institute of Deafness and Other Communication Disorders, 13% in US population equal to or less than 12 years old. A standardized AG induced ototoxicity monitoring algorithm (AIOA) was developed and implemented at CMKC …

How To Use Peanut Ige Levels To Predict Challenge Outcomes In The Pediatric Population, Nalin U.G., Jay Portnoy, Jodi Shroba, Salman Aljubran

Posters

Introduction: The accepted clinical practice is to consider oral challenge when peanut IgE levels are 2.0 kUA/L or less in patients with a history of a major reaction or less than 5.0 kUA/L in patients with only sensitization. Certain populations are predisposed to having increased IgE levels, and these patients are often not offered challenges due to elevated numbers.

Methods: We reviewed unidentified patients information from January 2017 to July 2019 on peanut IgE levels and oral challenge outcomes through REDCap database. A ROC using SPSS from IBM was used and the points from the ROC curve were used to …

The Pediatrician's Role In Eliminating Racial And Ethnic Disparities In Sleep-Related Infant Deaths., Jeffrey D. Colvin, Rachel Y. Moon

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Current Practice Patterns In The Diagnosis And Management Of Sleep-Disordered Breathing In Infants., Rachana Kombathula, David G. Ingram Md, Zarmina Ehsan Md

Manuscripts, Articles, Book Chapters and Other Papers

STUDY OBJECTIVES: Currently, there are no universally accepted guidelines for diagnosis and management of sleep-disordered breathing (SDB) in infants. The purpose of this study was to survey pediatric sleep medicine providers regarding their current practice patterns for diagnosis and management of SDB in infants.

METHODS: An anonymous, web-based survey with 71 questions was distributed via the PEDSLEEP and Ped-Lung listserv, which serve as a hub of communication for pediatric sleep and pulmonary medicine providers worldwide.

RESULTS: Fifty-four providers from eight countries completed the survey. Ninety-six percent of providers reported performing sleep studies in infants with 53% performing more than 30 …