Medical Specialties Commons^™

The Reliability Of Histamine Pharmacodynamic Response Phenotype Classification In Children With Allergic Disease., Shaun S Kumar, Xiaoxi Liu, Catherine M Sherwin, Bridgette Jones

Manuscripts, Articles, Book Chapters and Other Papers

We have identified distinct histamine pharmacodynamic response phenotypes in children with allergic disease utilizing histamine iontophoresis with laser Doppler (HILD). These response phenotypes may be relevant in guiding therapeutic decision making for agents targeting the allergic response pathways. However, the reliability of these response phenotypes has not been assessed. Therefore, we performed HILD in children with allergic rhinitis and/or asthma on two to three separate occasions. HILD response-time data were analyzed in NONMEM using a linked effect PKPD model. Examination of observed vs. classified response phenotypes predicted response plots and the sum of residuals. The intraclass correlation coefficient (ICC) was …

Development Of An International Data Repository And Research Resource: The Prospective Studies Of Acute Child Trauma And Recovery (Pact/R) Data Archive, Nancy Kassam-Adams, Justin A. Kenardy, Douglas L. Delahanty, Meghan L. Marsac, Richard Meiser-Stedman, Reginald D. V. Nixon, Markus A. Landolt, Patrick A. Palmieri

Pediatrics Faculty Publications

Background: Studies that identify children after acute trauma and prospectively track risk/protective factors and trauma responses over time are resource-intensive; small sample sizes often limit power and generalizability. The Prospective studies of Acute Child Trauma and Recovery (PACT/R) Data Archive was created to facilitate more robust integrative cross-study data analyses.

Objectives: To (a) describe creation of this research resource, including harmonization of key variables; (b) describe key study- and participant-level variables; and (c) examine retention to follow-up across studies.

Methods: For the first 30 studies in the Archive, we described study-level (design factors, retention rates) and participant-level …

Musculoskeletal Pain Outcomes Pre- And Post Intrathecal Baclofen Pump Implant In Children With Cerebral Palsy: A Prospective Cohort Study., Chantel C. Barney, Alyssa M. Merbler, Jean Stansbury, Linda E. Krach, Michael D. Partington, Patrick Graupman, Peter D. Kim, Debbie Song, Frank J. Symons

Manuscripts, Articles, Book Chapters and Other Papers

Objective: To characterize musculoskeletal pain intensity, duration, frequency, and interference with activities of daily living in children with cerebral palsy (CP) before and after intrathecal baclofen pump placement.

Design: Prospective cohort study.

Setting: Children's tertiary hospital.

Participants: Participants were children with CP (N=32; 53% male; mean age, 9.9y; age range, 4-17y). The majority of participants had a CP diagnosis of quadriplegia (76%) and relied on wheeled mobility (91%).

Interventions: Assessments were completed pre- and post intrathecal baclofen pump implant.

Main outcome measures: Because of considerable patient heterogeneity, both pain measures (Brief Pain Inventory, Dalhousie Pain Interview) were completed by proxy …

Relationship Between Movement Quality, Functional Ambulation Status, And Spatiotemporal Gait Parameters In Children With Myelomeningocele., Megan Flores, Kathleen Manella, Elizabeth Ardolino

Physical Therapy Collection

Aims: We investigated relationships among the Pediatric Neuromuscular Recovery Scale (Peds NRS), modified Hoffer Scale, and spatiotemporal gait parameters in children with myelomeningocele (MMC).

Methods: 21 children with MMC, age 5.3 years (SD = 2.6), were assessed by three clinicians using the Peds NRS and modified Hoffer Scale. In eight children, gait parameters were also measured.

Results: The Peds NRS summary score demonstrated good correlation with modified Hoffer Scale score (r = -0.64, p = 0.002) that accounted for 41% of variation in summary score. Six Peds NRS seated/standing items exhibited good relationships with modified Hoffer Scale …

Therapeutic Potential Of Citrulline As An Arginine Supplement: A Clinical Pharmacology Review, Jahidur Rashid, Shaun S. Kumar, Kathleen M. Job, Xiaoxi Liu, Candice D. Fike, Catherine M. T. Sherwin

Pediatrics Faculty Publications

Supplemental arginine has shown promise as a safe therapeutic option to improve endogenous nitric oxide (NO) regulation in cardiovascular diseases associated with endothelial dysfunction. L-arginine, an endogenous amino acid, was reported in clinical studies in adults to improve cardiovascular function in hypertension, pulmonary hypertension, pre-eclampsia, angina, and mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome. L-citrulline, a natural precursor of L-arginine, is more bioavailable than L-arginine because of hepatic first-pass metabolism avoidance and longer circulation time. Although not yet well studied, arginine/citrulline has immense therapeutic potential in some life-threatening diseases of children. However, optimal clinical development of arginine or …

Concussion, Anthony Shadiack

Sports Medicine

No abstract provided.

Assessing Physical Activity In People With Mental Illness: 23-Country Reliability And Validity Of The Simple Physical Activity Questionnaire (Simpaq), S Rosenbaum, R Morell, A Abdel-Baki, M Ahmadpanah, T V. Anilkumar, L Baie, A Bauman, S Bender, Zahra Hoodbhoy, Romaina Iqbal, Ayesha Mian

Department of Paediatrics and Child Health

Background: Physical inactivity is a key contributor to the global burden of disease and disproportionately impacts the wellbeing of people experiencing mental illness. Increases in physical activity are associated with improvements in symptoms of mental illness and reduction in cardiometabolic risk. Reliable and valid clinical tools that assess physical activity would improve evaluation of intervention studies that aim to increase physical activity and reduce sedentary behaviour in people living with mental illness.
Methods: The five-item Simple Physical Activity Questionnaire (SIMPAQ) was developed by a multidisciplinary, international working group as a clinical tool to assess physical activity and sedentary behaviour in …

New Horizons In The Genetic Etiology Of Systemic Lupus Erythematosus And Lupus-Like Disease: Monogenic Lupus And Beyond, Erkan Demirkaya, Sezgin Sahin, Micol Romano, Qing Zhou, Ivona Aksentijevich

Paediatrics Publications

Systemic lupus erythematosus (SLE) is a clinically and genetically heterogeneous autoimmune disease. The etiology of lupus and the contribution of genetic, environmental, infectious and hormonal factors to this phenotype have yet to be elucidated. The most straightforward approach to unravel the molecular pathogenesis of lupus may rely on studies of patients who present with early-onset severe phenotypes. Typically, they have at least one of the following clinical features: childhood onset of severe disease (<5 >years), parental consanguinity, and presence of family history for autoimmune diseases in a first-degree relative. These patients account for a small proportion of patients with lupus …

Evaluation Of Dna Methylation Episignatures For Diagnosis And Phenotype Correlations In 42 Mendelian Neurodevelopmental Disorders., Erfan Aref-Eshghi, Jennifer Kerkhof, Victor P Pedro, Mouna Barat-Houari, Nathalie Ruiz-Pallares, Jean-Christophe Andrau, Didier Lacombe, Julien Van-Gils, Patricia Fergelot, Christèle Dubourg, Valerie Cormier-Daire, Sophie Rondeau, François Lecoquierre, Pascale Saugier-Veber, Gaël Nicolas, Gaetan Lesca, Nicolas Chatron, Damien Sanlaville, Antonio Vitobello, Laurence Faivre, Christel Thauvin-Robinet, Frederic Laumonnier, Martine Raynaud, Mariëlle Alders, Marcel Mannens, Peter Henneman, Raoul C Hennekam, Guillaume Velasco, Claire Francastel, Damien Ulveling, Andrea Ciolfi, Simone Pizzi, Marco Tartaglia, Solveig Heide, Delphine Héron, Cyril Mignot, Boris Keren, Sandra Whalen, Alexandra Afenjar, Thierry Bienvenu, Philippe M Campeau, Justine Rousseau, Michael A Levy, Lauren Brick, Mariya Kozenko, Tugce B Balci, Victoria Mok Siu, Alan Stuart, Mike Kadour, Jennifer Masters, Kyoko Takano, Tjitske Kleefstra, Nicole De Leeuw, Michael Field, Marie Shaw, Jozef Gecz, Peter J Ainsworth, Hanxin Lin, David I Rodenhiser, Michael J Friez, Matt Tedder, Jennifer A Lee, Barbara R Dupont, Roger E Stevenson, Steven A Skinner, Charles E Schwartz, David Genevieve, Bekim Sadikovic

Paediatrics Publications

Genetic syndromes frequently present with overlapping clinical features and inconclusive or ambiguous genetic findings which can confound accurate diagnosis and clinical management. An expanding number of genetic syndromes have been shown to have unique genomic DNA methylation patterns (called "episignatures"). Peripheral blood episignatures can be used for diagnostic testing as well as for the interpretation of ambiguous genetic test results. We present here an approach to episignature mapping in 42 genetic syndromes, which has allowed the identification of 34 robust disease-specific episignatures. We examine emerging patterns of overlap, as well as similarities and hierarchical relationships across these episignatures, to highlight …

Apthous Ulcers, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Predictors Of Bicuspid Aortic Valve-Associated Aortopathy In Childhood: A Report From The Mibava Consortium, Michael Grattan, Andrea Prince, Rawan K Rumman, Conall Morgan, Michele Petrovic, Amanda Hauck, Luciana Young, Anders Franco-Cereceda, Bart Loeys, Salah A Mohamed, Harry Dietz, Seema Mital, Chun-Po Steve Fan, Cedric Manlhiot, Gregor Andelfinger, Luc Mertens

Paediatrics Publications

BACKGROUND: Bicuspid aortic valve (BAV) is the most prevalent congenital heart defect affecting 1% to 2% of the population. It is associated with ascending aorta dilatation. Valve morphology, aortic stenosis (AS), and aortic insufficiency (AI) have been proposed as potential risk factors; however, evaluating their role is difficult, as these factors are inherently related. The aim of this study was to determine whether BAV morphology and dysfunction are independent determinants for ascending aorta dilatation in pediatric patients.

METHODS: A multicenter, retrospective, cross-sectional study of pediatric BAV patients followed since 2004 was performed. Imaging data were assessed for BAV morphology, severity …

Bulging Anterior Fontanelle And Dense Bones In An Infant., Camilla Raya Halgren, Jenna Lakhani, Samantha Colaiacovo, Chitra Prasad

Paediatrics Publications

No abstract provided.

Blau Syndrome And Early-Onset Sarcoidosis: A Six Case Series And Review Of The Literature., Ayşenur Paç Kisaarslan, Betül Sözeri̇, Nihal Şahi̇n, Sümeyra Özdemi̇r Çi̇çek, Zübeyde Gündüz, Erkan Demi̇rkaya, Afig Berdeli̇, Serdal Sadet Özcan, Hakan Porazoğlu, Ruhan Düşünsel

Paediatrics Publications

Objectives: This study aims to discuss the clinical, laboratory and genetic findings, and treatment options for six patients who were diagnosed with Blau syndrome (BS)/early-onset sarcoidosis (EOS).

Patients and methods: The study included four patients (2 males,2 females; mean age 7 years; range 4 to 10 years) with EOS and two siblings (1 male, 1 female; mean age 10 years; range, 9 to 11 years) with BS. Age, age of initial symptoms, age of diagnosis; articular involvement, presence of uveitis, dermatitis, or fever, other organ involvement, laboratory findings, results of metabolic tests for mucopolysaccharidosis and mucolipidosis, results of genetic, pathologic, …

Osteochondritis Dissecans In Pediatric Patients With Juvenile Idiopathic Arthritis., Bradley C Jackson, Debra L Bartley, Roberta A Berard

Paediatrics Publications

No abstract provided.

Impressions On Reliability And Students’ Perceptions Of Learning In A Peer-Based Osce, Rishad Khan, Saad Chahine, Steven Macaluso, Ricardo Viana, Caitlin Cassidy, Thomas Miller, Debra Bartley, Michael Payne

Paediatrics Publications

Background: Peer assessment of performance in the objective structured clinical examination (OSCE) is emerging as a learning instrument. While peers can provide reliable scores, there may be a trade-off with students’ learning. The purpose of this study is to evaluate a peer-based OSCE as a viable assessment instrument and its potential to promote learning and explore the interplay between these two roles. Methods: A total of 334 medical students completed an 11-station OSCE from 2015 to 2016. Each station had 1–2 peer examiners (PE) and one faculty examiner (FE). Examinees were rated on a 7-point scale across 5 dimensions: Look, …

Application Of Protection Motivation Theory To Clinical Trial Enrolment For Pediatric Chronic Conditions, Stephanie P. Brooks, Tania M. Bubela

Office of the Provost

Background: Parents of children living with chronic but manageable conditions hope for improved therapies or cures, including Advanced Therapy Medicinal Products (ATMPs). Multiple pediatric clinical trials for ATMPs are underway, but the risk profile of ATMPs for chronic conditions is largely unknown and likely different than for terminal pediatric illnesses. Applying Protection Motivation Theory modified to the context of pediatric ATMP clinical trial enrollment, our study analyses information needs of parents of children living with chronic manageable conditions: Type 1 Diabetes (T1D) or Inherited Retinal Diseases (IRD).
Methods: We conducted semi-structured interviews with 15 parents of children living with T1D …

Evaluation Of Non-Invasive Continuous Physiological Monitoring Devices For Neonates In Nairobi, Kenya: A Research Protocol, Amy Sarah Ginsburg, Evangelyn Nkwopara, William Macharia, Roseline Ochieng, Mary Waiyego, Guohai Zhou, Roman Karasik, Shuai Xu, J Mark Ansermino

Paediatrics and Child Health, East Africa

Introduction: Continuous physiological monitoring devices are often not available for monitoring high-risk neonates in low-resource settings. Easy-to-use, non-invasive, multiparameter, continuous physiological monitoring devices could be instrumental in providing appropriate care and improving outcomes for high-risk neonates in these low-resource settings.

Methods and analysis: The purpose of this prospective, observational, facility-based evaluation is to provide evidence to establish whether two existing non-invasive, multiparameter, continuous physiological monitoring devices developed by device developers, EarlySense and Sibel, can accurately and reliably measure vital signs in neonates (when compared with verified reference devices). We will also assess the feasibility, usability and acceptability of these devices …

Energy And Protein Requirements For Children With Ckd Stages 2-5 And On Dialysis-Clinical Practice Recommendations From The Pediatric Renal Nutrition Taskforce., Vanessa Shaw, Nonnie Polderman, José Renken-Terhaerdt, Fabio Paglialonga, Michiel Oosterveld, Jetta Tuokkola, Caroline Anderson, An Desloovere, Laurence Greenbaum, Dieter Haffner, Christina Nelms, Leila Qizalbash, Johan Vande Walle, Bradley A. Warady, Rukshana Shroff, Lesley Rees

Manuscripts, Articles, Book Chapters and Other Papers

Dietary management in pediatric chronic kidney disease (CKD) is an area fraught with uncertainties and wide variations in practice. Even in tertiary pediatric nephrology centers, expert dietetic input is often lacking. The Pediatric Renal Nutrition Taskforce (PRNT), an international team of pediatric renal dietitians and pediatric nephrologists, was established to develop clinical practice recommendations (CPRs) to address these challenges and to serve as a resource for nutritional care. We present CPRs for energy and protein requirements for children with CKD stages 2-5 and those on dialysis (CKD2-5D). We address energy requirements in the context of poor growth, obesity, and different …

Immunogenicity In Clinical Practice And Drug Development: When Is It Significant?, Valentina Shakhnovich, Bernd Meibohm, Amy Rosenberg, Andrzej M. Kierzek, Rachel Hasenkamp, Ryan S. Funk, Craig J. Thalhauser, Piet H. Van Der Graaf, Yow Ming C. Wang, Lora Hamuro

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

The Dietary Management Of Calcium And Phosphate In Children With Ckd Stages 2-5 And On Dialysis-Clinical Practice Recommendation From The Pediatric Renal Nutrition Taskforce., Louise Mcalister, Pearl Pugh, Laurence Greenbaum, Dieter Haffner, Lesley Rees, Caroline Anderson, An Desloovere, Christina Nelms, Michiel Oosterveld, Fabio Paglialonga, Nonnie Polderman, Leila Qizalbash, José Renken-Terhaerdt, Jetta Tuokkola, Bradley A. Warady, Johan Vande Walle, Vanessa Shaw, Rukshana Shroff

Manuscripts, Articles, Book Chapters and Other Papers

In children with chronic kidney disease (CKD), optimal control of bone and mineral homeostasis is essential, not only for the prevention of debilitating skeletal complications and achieving adequate growth but also for preventing vascular calcification and cardiovascular disease. Complications of mineral bone disease (MBD) are common and contribute to the high morbidity and mortality seen in children with CKD. Although several studies describe the prevalence of abnormal calcium, phosphate, parathyroid hormone, and vitamin D levels as well as associated clinical and radiological complications and their medical management, little is known about the dietary requirements and management of calcium (Ca) and …

Pediatric Congenital Heart Diseases: Patterns Of Presentation To The Emergency Department Of A Tertiary Care Hospital, Surraiya Bano, Saleem Akhtar, Uzma R. Khan

Department of Emergency Medicine

Objective: To observe presentation of Pediatric congenital cardiac defects to the Emergency Department (ED) of a tertiary care hospital in Pakistan.
Methods: This is a retrospective chart review of patients under the age of 16 years with congenital cardiac defects presenting to the Emergency Department of Aga Khan University Hospital over a period of eighteen months, from January 2012 to June 2013. Study population was divided into two groups; first group constituted children with undiagnosed congenital cardiac defects, whereas second group constituted children with diagnosed congenial cardiac defects presented to ED. In previously diagnose cases each visit was counted as …

Does The Oral-Anal Transit Test Correlate With Colonic Manometry Findings In Children With Refractory Constipation?, Jason Dranove, Nathan Fleishman, Saigopala Reddy, Steven Teich

Manuscripts, Articles, Book Chapters and Other Papers

Purpose: The Oral-anal Transit Test (OTT) is a simple method of obtaining information about colonic transit. We aim to assess the correlation of OTT with the neuromuscular integrity of the colon determined by colonic manometry (CM).

Methods: All patients who had OTT followed by CM were evaluated. Less than 6 of 24 markers remaining on OTT was considered normal. CM was performed per previously published guidelines. A normal CM was defined as at least one High Amplitude Propagating Contraction progressing from the most proximal sensor through the sigmoid colon.

Results: A total of 34 patients underwent both OTT and CM …

Mainstreaming Nutrition Services: Stabilization Centers And Outpatient Therapeutic Program Centers, Ashar Muhammad Malik

Community Health Sciences

No abstract provided.

Remarks About The Study On Infection-Associated Relapses In Children With Nephrotic Syndrome: A Short-Term Outcome Study, Akbar Shoukat Ali, Raheel Allana, Rahim Jandani

Department of Medicine

No abstract provided.

A Descriptive Study Of Double Burden Of Malnutrition In Mothers Of Children With Severe Acute Malnutrition Admitted In Children Hospital And Institute Of Child Health, Multan, Saadia Khan, Umme Ammara, Reema Arshad, Farah Naz, Kamran Ishfaq

Department of Paediatrics and Child Health

Objective: To assess the nutritional status of mothers of severely malnourished children, and to evaluate the factors associated with inadequate caloric intake of children with severe acute malnutrition versus sufficient caloric intake by mothers.
Methods: The cross-sectional descriptive study was conducted from January to June 2016 at Children Hospital and Institute of Child Health, Multan, Pakistan, and comprised mothers of severely malnourished children admitted for treatment. Data was collected using a questionnaire administered through interviews. Elements noted included body mass index, 24-hour recall, dietary restriction during pregnancy, lactation and complimentary feeding patterns of the children. The mothers were also examined …

Mail-Back Envelopes For Retrieval Of Opioids After Pediatric Surgery, Adam C Adler, Ammar N Yamani, Caitlin D Sutton, Danielle M Guffey, Arvind Chandrakantan

Faculty, Staff and Students Publications

BACKGROUND: Opioid overprescription has the potential to lead to harmful medications remaining in homes and to a rise in accidental or deliberate ingestion by children and adolescents. Although methods for opioid disposal are available, many are costly or require greater than minimal effort for the patient. In this study, we used a mail-back return envelope to retrieve unused opioids after ambulatory pediatric surgery.

METHODS: This feasibility study was performed to assess the rate of opioid return by using a mail-back envelope for children ages 0 to 18 prescribed opioids after outpatient surgery. Participants were provided a return envelope as well …

Safety And Efficacy Profile Of Ozenoxacin 1% Cream In Pediatric Patients With Impetigo, Adelaide A Hebert, Theodore Rosen, Núria Albareda López, Ilonka Zsolt, Xavier Masramon

Faculty, Staff and Students Publications

BACKGROUND: Ozenoxacin is a topical antibiotic approved in the United States for treatment of impetigo in adults and children age ≥2 months. This analysis evaluated the efficacy and safety of ozenoxacin in specific pediatric age groups.

METHODS: Data for children aged 2 months to <18 years recruited from eight countries who had participated in phase 1 and 3 trials of ozenoxacin were extracted and analyzed by age range.

RESULTS: Across studies, 644 pediatric patients with impetigo received ozenoxacin 1% cream (n = 287) or vehicle (n = 247). One study included retapamulin 1% ointment as the internal validity control (n = 110). The clinical success rate at the end of treatment and bacterial eradication rates after 3 to 4 days of treatment and at …

The Role Of Alternative Mrna Splicing In Heart Development, Douglas Bittel, Nataliya Kibiryeva, Naoya Kenmochi, Prakash Patil, Tamayo Uechi, Brenda Rongish, Mike Filla, Jennifer A. Marshall, Michael Artman, Rajasingh Johnson, James E. O'Brien

Manuscripts, Articles, Book Chapters and Other Papers

Research in the last 10 years has led to improved understanding of the genetic regulation of vertebrate heart development, but despite this effort, approximately 70% of all congenital heart defects (CHDs) still have an unknown etiology. Alternative splicing of mRNA has been documented to play roles in normal and abnormal development. Dysregulated splicing of mRNA has been shown to cause heart defects in mice, however a link between mRNA splicing and CHDs has not yet been shown in humans. We reported that more than 50% of genes associated with heart development were alternatively spliced in the right ventricle (RV) of …

The Great Imitator Strikes Again: A Case Of A Lupus Flare-Up Presenting Like An Acute Abdomen, Emmanouil Kiriakopoulos, Vanessa Perez, Robyn Hoelle

HCA Healthcare Journal of Medicine

Systemic lupus erythematosus (SLE) is an autoimmune disease affecting all age groups and can manifest in various forms, often making the initial or successive presentations difficult to diagnose. Peritonitis secondary to lupus is a rare manifestation of this disease and the prevalence is said to be much lower in children. We present a case report of an adolescent male with a known history of lupus who presented to the emergency department with a clinical picture consistent with an acute surgical abdomen and underwent an appendectomy. Subsequent workup identified the culprit as a lupus-related peritonitis requiring corticosteroids for resolution.

Using The Medical Expenditure Panel Survey To Explore The Association Between High Healthcare Utilization And Behavioral Health Diagnoses In A Pediatric Population With Chronic Conditions, Marlon D. Satchell, Phd, Mph

Population Health Science Program Doctoral Dissertations

The 2010 Affordable Care Act (ACA) introduced an era of changing healthcare delivery and utilization, which is still evolving and changing to this day. Healthcare providers and healthcare systems need to identify strategies to provide better care to patients, that results in better patient outcomes, in a cost effective way. One strategy to accomplish this goal is to focus on managing the care of the most vulnerable patients, particularly including pediatric patients with one or more chronic conditions. The objective of this study was to assess the extent to which a behavioral health diagnosis in addition to another chronic condition …