Pediatrics Commons^™

Allied Health Professional Support In Pediatric Inflammatory Bowel Disease: A Survey From The Canadian Children Inflammatory Bowel Disease Network—A Joint Partnership Of Cihr And The Ch.I.L.D. Foundation, Wael El-Matary, Eric I. Benchimol, David Mack, Hien Q. Huynh, Jeff Critch, Anthony Otley, Colette Deslandres, Kevan Jacobson, Jennifer Debruyn, Matthew W. Carroll, Eytan Wine, Johan Van Limbergen, Mary Sherlock, Kevin Bax, Sally Lawrence, Ernest Seidman, Robert Issenman, Thomas D. Walters, Peter Church, Anne M. Griffiths

Paediatrics Publications

Objectives. The current number of healthcare providers (HCP) caring for children with inflammatory bowel disease (IBD) across Canadian tertiary-care centres is underinvestigated. The aim of this survey was to assess the number of healthcare providers (HCP) in ambulatory pediatric IBD care across Canadian tertiary-care centres.

Methods. Using a self-administered questionnaire, we examined available resources in academic pediatric centres within the Canadian Children IBD Network. The survey evaluated the number of HCP providing ambulatory care for children with IBD.

Results. All 12 tertiary pediatric gastroenterology centres participating in the network responded. Median full-time equivalent (FTE) of allied health professionals providing …

Molecular Characterization Of Nrxn1 Deletions From 19,263 Clinical Microarray Cases Identifies Exons Important For Neurodevelopmental Disease Expression, Chelsea Lowther, Marsha Speevak, Christine M Armour, Elaine S Goh, Gail E Graham, Chumei Li, Susan Zeesman, Malgorzata J M Nowaczyk, Lee-Anne Schultz, Antonella Morra, Rob Nicolson, Peter Bikangaga, Dawa Samdup, Mostafa Zaazou, Kerry Boyd, Jack H Jung, Victoria Siu, Manjulata Rajguru, Sharan Goobie, Mark A Tarnopolsky, Chitra Prasad, Paul T Dick, Asmaa S Hussain, Margreet Walinga, Renske G Reijenga, Matthew Gazzellone, Anath C Lionel, Christian R Marshall, Stephen W Scherer, Dimitri J Stavropoulos, Elizabeth Mccready, Anne S Bassett

Paediatrics Publications

PURPOSE: The purpose of the current study was to assess the penetrance of NRXN1 deletions.

METHODS: We compared the prevalence and genomic extent of NRXN1 deletions identified among 19,263 clinically referred cases to that of 15,264 controls. The burden of additional clinically relevant copy-number variations (CNVs) was used as a proxy to estimate the relative penetrance of NRXN1 deletions.

RESULTS: We identified 41 (0.21%) previously unreported exonic NRXN1 deletions ascertained for developmental delay/intellectual disability that were significantly greater than in controls (odds ratio (OR) = 8.14; 95% confidence interval (CI): 2.91-22.72; P < 0.0001). Ten (22.7%) of these had a second clinically relevant CNV. Subjects with a deletion near the 3' end of NRXN1 were significantly more likely to have a second rare CNV than subjects with a 5' NRXN1 deletion (OR = 7.47; 95% CI: 2.36-23.61; P = 0.0006). The prevalence of intronic NRXN1 deletions was not statistically different between cases and controls (P = 0.618). The majority (63.2%) of intronic NRXN1 deletion cases had a second rare CNV at a prevalence twice as high as that for exonic NRXN1 deletion cases (P = 0.0035).

CONCLUSIONS: The results support the importance of exons …

How Does The Social “Get Under The Gums”? The Role Of Socio-Economic Position In The Oral-Systemic Health Link, Noha Gomaa, Belinda Nicolau, Arjumand Siddiqi, Howard Tenenbaum, Michael Glogauer, Carlos Quiñonez

Paediatrics Publications

OBJECTIVES: To evaluate the extent of association between systemic inflammation and periodontal disease in American adults, and to assess whether socio-economic position mediated this relationship. METHODS: We used data from the National Health and Nutrition Examination Survey (NHANES IV) (2001-2010). Systemic inflammation was defined by individual and aggregate (cumulative inflammatory load) biomarkers (C-reactive protein, white blood cell counts, neutrophil counts, and neutrophil: lymphocyte ratio). Loss of attachment and bleeding on probing were used to define periodontal disease. Poverty:income ratio and education were indicators of socio-economic position. Covariates included age, sex, ethnicity, smoking, alcohol, and attendance for dental treatment. Univariate and …

When Crrt On Ecmo Is Not Enough For Potassium Clearance: A Case Report, Janice A. Tijssen, Guido Filler

Paediatrics Publications

Background: Continuous renal replacement therapy (CRRT) is an excellent method used to remove fluid and solutes. It may also reduce the systemic inflammatory response for patients on extracorporeal membrane oxygenation (ECMO) support. Theobjective of this report is to describe a case where CRRT in combination with ECMO was insufficient to control hyperkalemia. Methods: We report the case of an adolescent patient with refractory symptomatic hyperkalemia due to substantial rhabdomyolysis in which CRRT insufficiently cleared the patient's excess potassium. Results: Intermittent hemodialysis (IHD) was added and proved successful. The patient was weaned off ECMO, CRRT, and IHD, and his cardiac and …

Graduate Medical Education 2016-2017 Annual Report, Children's Mercy Hospital

GME Annual Reports

Annual report of the Children's Mercy Hospital Graduate Medical Education department, including pediatric residency, internal medicine/pediatrics residency, fellowship programs, medical students, continuing medical education, and library services.

Measuring The Effects Of Therapeutic Listening - Quickshifts, Hannah Tashjian, Phoebe Taasan, Donielle Hair

Student Research Posters

This study examines a structured protocol to measure the effects of daily individualized use of Therapeutic Listening - Quickshifts (TL-Q) used to improve occupational performance in children with learning and developmental disabilities. The study identifies a standardized practice-based evidence procedure to measure the outcomes of implementing TL-Q within traditional OT practice. OTs worldwide are using sound-based therapies (SBTs) despite limited supporting evidence available regarding the productivity of these interventions. Even fewer studies have been published on the effects of Therapeutic Listening (TL), a novel intervention shown to increase personal and interpersonal skills including sensory processing skills, visual motor integration, and …

Toward A Rapid Production Of Multivirus-Specific T Cells Targeting Bkv, Adenovirus, Cmv, And Ebv From Umbilical Cord Blood, Hema Dave, Min Luo, J.W. Blaney, Shabnum Patel, Cecilia Barese, Conrad Russell Cruz, Elizabeth J. Shpall, Catherine M. Bollard, Patrick J. Hanley

Pediatrics Faculty Publications

Umbilical cord blood (CB) has emerged as an effective alternative donor source for hematopoietic stem cell transplantation. Despite this success, the prolonged duration of immune suppression following CB transplantation and the naiveté of CB T cells leave patients susceptible to viral infections. Adoptive transfer of ex vivo-expanded virus-specific T cells from CB is both feasible and safe. However, the manufacturing process of these cells is complicated, lengthy, and labor-intensive. We have now developed a simplified method to manufacture a single culture of polyclonal multivirus-specific cytotoxic T cells in less than 30 days. It eliminates the need for a live virus …

A Case Of Difficult-To-Treat Asthma Due To Uncontrolled Atypical Gastroesophageal Reflux, Alissa Welsh, Andrey Leonov

Research Day

Introduction: 
In uncontrolled asthma, comorbid conditions should be treated in order to achieve better asthma control. The contribution of gastro-esophageal reflux (GER) may be overlooked when its presentation is atypical. 
Case Report: 
11 year-old male with moderate persistent asthma and allergic rhinitis presented with persistent barky cough, diagnosed as spasmodic croup after bronchoscopy, esophagram, swallow study and magnified airway x-ray failed to show anatomical abnormality. Empiric proton pump inhibitor (PPI) treatment was started with improvement in cough. Patient was referred to Gastroenterology for evaluation of GER, despite lack of heartburn or vomiting. Endoscopy revealed esophageal candida infection which was treated. …

Widening The Differential: A Case Of Hemophagocytic Lymphohistiocytosis Disguised As A Fever Of Unknown Origin, Emily Cordes

Research Day

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is an under recognized, aggressive inflammatory disease characterized by excessive activity of histiocytes and lymphocytes. According to the HLH-2004 guidelines, five of the following eight criteria must be met to diagnose HLH: fever >38.5¡ã C, splenomegaly; cytopenias affecting ¡Ý two cell lines; hypertriglyceridemia/hypofibrinogenemia; evidence of hemophagocytosis in the bone marrow, spleen or lymph nodes; hyperferritinemia; low NK cell activity; or high levels of soluble CD25. Once a diagnosis of HLH is established, investigations for infectious, malignant or autoimmune etiologies must be pursued. We report a case of fever of unknown origin (FUO) that presented multiple times …

A Case Of Native Valve Staphylococcus Epidermidis Endocarditis With Cardiac Abscess Formation, Emily Cordes, Chris Jacob, Mark Loehrke

Research Day

Introduction: Staphylococcus epidermidis is the most common commensal bacteria of the skin; however it can be pathogenic and is the most frequent cause of medical device associated endocarditis. It is also a well-known cause of endocarditis in IV drug abuse (IVDA). However, in individuals without obvious disruption of the skin barrier, it is a relatively rare, but emerging entity of native valve endocarditis. Coagulase negative staphylococci account for only 1-5% of community acquired native valve infective endocarditis and most resolve with treatment. We report a case of a 56 year old male with an aggressive strain of community acquired Staphylococcus …

Atrial Fibrillation Induction In A Pediatric Patient During Adenosine Administration, Jeffrey A. Robinson, Christopher S. Snyder

Journal Articles: Pediatrics

Adenosine by rapid intravenous bolus is frequently utilized in clinical practice as both a pharmacologic treatment for supraventricular (reentrant) tachycardia and in provocative testing for the diagnosis of Wolff-Parkinson-White (WPW) syndrome. This is a case report of an otherwise healthy adolescent female who received adenosine during a provocative test for WPW syndrome. Immediately after receiving adenosine, the patient had a self-limited episode of atrial fibrillation. When administering adenosine, teams should be aware of the potential adverse effects and be prepared to treat appropriately.

Management Of Children With Chronic Wet Cough And Protracted Bacterial Bronchitis Chest Guideline And Expert Panel Report, Anne B. Chang, John J. Oppenheimer, Miles M. Weinberger, Bruce K. Rubin, Cameron C. Grant, Kelly Weir, Richard S. Irwin, Chest Expert Cough Panel

Pediatrics Publications

BACKGROUND: Wet or productive cough is common in children with chronic cough. We formulated recommendations based on systematic reviews related to the management of chronic wet cough in children (aged
METHODS: We used the CHEST expert cough panel's protocol for systematic reviews and the American College of Chest Physicians (CHEST) methodologic guidelines and GRADE framework (the Grading of Recommendations Assessment, Development and Evaluation). Data from the systematic reviews in conjunction with patients' values and preferences and the clinical context were used to form recommendations. Delphi methodology was used to obtain consensus for the recommendations/suggestions made.

RESULTS: Combining data from the …

Children's Mercy Hospital Annual Report 2016, Children's Mercy Hospital

Children's Mercy Annual Reports

Annual report for The Children's Mercy Hospital in Kansas City MO, a pediatric medical center.

Does Lactobacillus Reuteri Dsm 17938 Reduce Daily Crying Times In Infants With Colic?, Taylor Kolle

PCOM Physician Assistant Studies Student Scholarship

Objective: The objective of this selective EBM review is to determine whether or not “Does Lactobacillus reuteri DSM 17938 reduce daily crying times in infants with colic?”

Study Design: Review of all English language primary studies published in 2010, 2013, 2015.

Data Sources: Three randomized, double blind, placebo controlled clinical trials found using PubMed.

Outcomes Measured: Daily average crying times were measured by parents who documented these in a journal.

Results: In all three randomized control studies by Chaeu et al. (2015), Savino et al, (2010), and Szajewska et al. (2013), they found that infants …

Distinct Neural Bases Of Disruptive Behavior And Autism Symptom Severity In Boys With Autism Spectrum Disorder., Y.J. Daniel Yang, Denis G Sukhodolsky, Jiedi Lei, Eran Dayan, Kevin A. Pelphrey, Pamela Ventola

Pediatrics Faculty Publications

BACKGROUND: Disruptive behavior in autism spectrum disorder (ASD) is an important clinical problem, but its neural basis remains poorly understood. The current research aims to better understand the neural underpinnings of disruptive behavior in ASD, while addressing whether the neural basis is shared with or separable from that of core ASD symptoms.

METHODS: Participants consisted of 48 male children and adolescents: 31 ASD (7 had high disruptive behavior) and 17 typically developing (TD) controls, well-matched on sex, age, and IQ. For ASD participants, autism symptom severity, disruptive behavior, anxiety symptoms, and ADHD symptoms were measured. All participants were scanned while …

Pediatric Cardiovascular Care In Uganda: Current Status, Challenges, And Opportunities For The Future., Twalib Olega Aliku, Sulaiman Lubega, Judith Namuyonga, Tom Mwambu, Michael Oketcho, John O Omagino, Craig Sable, Peter Lwabi

Pediatrics Faculty Publications

In many developing countries, concerted action against common childhood infectious diseases has resulted in remarkable reduction in infant and under-five mortality. As a result, pediatric cardiovascular diseases are emerging as a major contributor to childhood morbidity and mortality. Pediatric cardiac surgery and cardiac catheterization interventions are available in only a few of Sub-Saharan African countries. In Uganda, open heart surgeries (OHSs) and interventional procedures for pediatric cardiovascular disease are only possible at the Uganda Heart Institute (UHI), having been started with the help of expatriate teams from the years 2007 and 2012, respectively. Thereafter, independent OHS and cardiac catheterization have …

Nasopharyngeal Microbiome Diversity Changes Over Time In Children With Asthma, Marcos Pérez-Losada, Lamia Alamri, Keith Crandall, Robert J. Freishtat

Pediatrics Faculty Publications

Background

The nasopharynx is a reservoir for pathogens associated with respiratory illnesses such as asthma. Next-generation sequencing (NGS) has been used to characterize the nasopharyngeal microbiome of infants and adults during health and disease; less is known, however, about the composition and temporal dynamics (i.e., longitudinal variation) of microbiotas from children and adolescents. Here we use NGS technology to characterize the nasopharyngeal microbiomes of asthmatic children and adolescents (6 to 18 years) and determine their stability over time.

Methods

Two nasopharyngeal washes collected 5.5 to 6.5 months apart were taken from 40 children and adolescents with asthma living in the …

Boiling Histotripsy Lesion Characterization On A Clinical Magnetic Resonance Imaging-Guided High Intensity Focused Ultrasound System., Avinash Eranki, Navid Farr, Ari Partanen, Karun Sharma, Hong Chen, Christopher T Rossi, Satya V V N Kothapalli, Matthew Oetgen, Aerang Kim, Ayele H Negussie, David Woods, Bradford J Wood, Peter C W Kim, Pavel S. Yarmolenko

Pediatrics Faculty Publications

PURPOSE: High intensity focused ultrasound (HIFU) is a non-invasive therapeutic technique that can thermally ablate tumors. Boiling histotripsy (BH) is a HIFU approach that can emulsify tissue in a few milliseconds. Lesion volume and temperature effects for different BH sonication parameters are currently not well characterized. In this work, lesion volume, temperature distribution, and area of lethal thermal dose were characterized for varying BH sonication parameters in tissue-mimicking phantoms (TMP) and demonstrated in ex vivo tissues.

METHODS: The following BH sonication parameters were varied using a clinical MR-HIFU system (Sonalleve V2, Philips, Vantaa, Finland): acoustic power, number of cycles/pulse, total …

Hypertension In The Pediatric Kidney Transplant Recipient., Olga Charnaya, Asha Moudgil

Pediatrics Faculty Publications

Hypertension after kidney transplant is a frequent occurrence in pediatric patients. It is a risk factor for graft loss and contributes to the significant burden of cardiovascular disease (CVD) in this population. The etiology of posttransplant hypertension is multifactorial including donor factors, recipient factors, medications, and lifestyle factors similar to those prevalent in the general population. Ambulatory blood pressure monitoring has emerged as the most reliable method for measuring hypertension in pediatric transplant recipients, and many consider it to be essential in the care of these patients. Recent technological advances including measurement of carotid intima-media thickness, pulse wave velocity, and …

Endotypes Of Difficult-To-Control Asthma In Inner-City African American Children., K R Brown, R Z Krouse, A Calatroni, C M Visness, U Sivaprasad, C M Kercsmar, E C Matsui, J B West, M M Makhija, M A Gill, H Kim, M Kattan, D Pillai, J E Gern, W W Busse, A Togias, A H Liu, G K Khurana Hershey

Pediatrics Faculty Publications

African Americans have higher rates of asthma prevalence, morbidity, and mortality in comparison with other racial groups. We sought to characterize endotypes of childhood asthma severity in African American patients in an inner-city pediatric asthma population. Baseline blood neutrophils, blood eosinophils, and 38 serum cytokine levels were measured in a sample of 235 asthmatic children (6-17 years) enrolled in the NIAID (National Institute of Allergy and Infectious Diseases)-sponsored Asthma Phenotypes in the Inner City (APIC) study (ICAC (Inner City Asthma Consortium)-19). Cytokines were quantified using a MILLIPLEX panel and analyzed on a Luminex analyzer. Patients were classified as Easy-to-Control or …