Pediatrics Commons^™

Variability Of M-Mode Versus Two-Dimensional Echocardiography Measurements In Children With Dilated Cardiomyopathy., Caroline K. Lee, Renee Margossian, Lynn A. Sleeper, Charles E. Canter, Shan Chen, Lloyd Y. Tani, Girish S. Shirali, Anita Szwast, Elif Seda Selamet Tierney, M Jay Campbell, Fraser Golding, Yanli Wang, Karen Altmann, Steven D. Colan, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

M-mode and 2-dimensional (2D) echocardiographic imaging are routinely used to quantify left-ventricular (LV) size and function in pediatric patients with dilated cardiomyopathy (DCM). The reproducibility of and correlation between these techniques are unknown. This analysis sought to compare interreader, intrareader, and interacquisition reproducibility of M-mode versus 2D measurements in pediatric DCM patients. The Ventricular Volume Variability study of the Pediatric Heart Network is a multicenter, prospective, observational study assessing the course of chronic DCM in children. Two sonographers performed baseline image acquisitions locally, and two readers performed measurements at the echocardiographic core laboratory. One reader repeated measurements 1 month later. …

Changing The Road To Recovery: A Lesson In Pediatric Surgery Efficiency, Hope L. Johnson Msn, Rn, Cnor

Patient Care Services / Nursing

No abstract provided.

2014 Massachusetts Family Impact Seminar: A Lot On Our Plate; Chronic Health Threats In Massachusetts, Ira S. Ockene M.D., Christina D. Economos Ph.D, Barbara Goldoftas Ph.D, Denise A. Hines Ph.D., Caitlin Alcorn, Jacqueline A. Daigneault, Jordan R. Daley, Maura A. Flanagan, Carsten-Hendrik Rasche, Heather Rios, Julia Tran, Liam Kelly Fleming

Mosakowski Institute for Public Enterprise

A Lot on Our Plate: Chronic Health Threats in Massachusetts is the fifth Massachusetts Family Impact Seminar, and is designed to emphasize a family perspective in policymaking on issues related to childhood obesity, cardiovascular disease, and type 2 diabetes. In general, Family Impact Seminars analyze the consequences an issue, policy, or program may have for families.

The Pediatric Milestones Project: Part Ii, Kris Rooney Md, Diana Drogalis-Kim Do

Department of Pediatrics

No abstract provided.

Microfluidic-Enabled Liposomes Elucidate Size-Dependent Transdermal Transport, Renee R. Hood, Eric L. Kendall, Mariana Junqueira, Wyatt N. Vreeland, Zenaide M.N. Quezado, Julia C. Finkel, Don L. Devoe

Pediatrics Faculty Publications

Microfluidic synthesis of small and nearly-monodisperse liposomes is used to investigate the size-dependent passive transdermal transport of nanoscale lipid vesicles. While large liposomes with diameters above 105 nm are found to be excluded from deeper skin layers past the stratum corneum, the primary barrier to nanoparticle transport, liposomes with mean diameters between 31–41 nm exhibit significantly enhanced penetration. Furthermore, multicolor fluorescence imaging reveals that the smaller liposomes pass rapidly through the stratum corneum without vesicle rupture. These findings reveal that nanoscale liposomes with well-controlled size and minimal size variance are excellent vehicles for transdermal delivery of functional nanoparticle drugs.

Hospitalization Of Children With Down Syndrome, Ariel Tenenbaum, Rana N. Hanna, Diana Averbuch, Isaiah D. Wexler, Maor Chavkin, Joav Merrick

Pediatrics Faculty Publications

INTRODUCTION: Children with Down syndrome present with multiple medical problems in a higher prevalence compared with the general population, which may lead to hospitalizations.

METHODS: Analysis of 560 hospitalizations of 162 children aged 0-16 years with Down syndrome at Hadassah Medical Center during the years 1988-2007 compared with data on children in the general population, hospitalized at the same period. Data was collected from patient files and statistical data from the Ministry of Health.

RESULTS: Respiratory infections were the leading cause for hospitalization of children with Down syndrome. The number of hospitalizations of children with Down syndrome compared to the …

Common And Specific Impairments In Attention Functioning In Girls With Chromosome 22q11.2 Deletion, Fragile X Or Turner Syndromes., Andrea I Quintero, Elliott A Beaton, Danielle J Harvey, Judith L. Ross, Tony J Simon

Department of Pediatrics Faculty Papers

BACKGROUND: Chromosome 22q11.2 deletion syndrome (22q11.2DS), fragile X syndrome (FXS), and Turner syndrome (TS) are complex and variable developmental syndromes caused by different genetic abnormalities; yet, they share similar cognitive impairments in the domains of numbers, space, and time. The atypical development of foundational neural networks that underpin the attentional system is thought to result in further impairments in higher-order cognitive functions. The current study investigates whether children with similar higher-order cognitive impairments but different genetic disorders also show similar impairments in alerting, orienting, and executive control of attention.

METHODS: Girls with 22q11.2DS, FXS, or TS and typically developing (TD) …

The Temporal Version Of The Pediatric Sepsis Biomarker Risk Model, Hector R. Wong, Scott L. Weiss, John S. Giuliano Jr., Mark S. Wainwright, Natalie Z. Cvijanovich, Robert J. Freishtat, +19 Additional Authors

Pediatrics Faculty Publications

Background

PERSEVERE is a risk model for estimating mortality probability in pediatric septic shock, using five biomarkers measured within 24 hours of clinical presentation.

Objective

Here, we derive and test a temporal version of PERSEVERE (tPERSEVERE) that considers biomarker values at the first and third day following presentation to estimate the probability of a “complicated course”, defined as persistence of ≥2 organ failures at seven days after meeting criteria for septic shock, or death within 28 days.

Methods

Biomarkers were measured in the derivation cohort (n = 225) using serum samples obtained during days 1 and 3 of septic shock. …

Selectivity And Potency Of Microcystin Congeners Against Oatp1b1 And Oatp1b3 Expressing Cancer Cells, Timo H.J. Niedermeyer, Abigail Daily, Monika Swiatecka-Hagenbruch, Jeffrey A. Moscow

Pediatrics Faculty Publications

Microcystins are potent phosphatase inhibitors and cellular toxins. They require active transport by OATP1B1 and OATP1B3 transporters for uptake into human cells, and the high expression of these transporters in the liver accounts for their selective hepatic toxicity. Several human tumors have been shown to have high levels of expression of OATP1B3 but not OATP1B1, the main transporter in liver cells. We hypothesized that microcystin variants could be isolated that are transported preferentially by OATP1B3 relative to OATP1B1 to advance as anticancer agents with clinically tolerable hepatic toxicity. Microcystin variants have been isolated and tested for cytotoxicity in cancer cells …

Syphilis: A Forgotten Sexually Transmitted Disease?, Amy Burnett, Hatim A. Omar

Pediatrics Faculty Publications

Syphilis has declined significantly over the last 30 years. Due to that success, many physicians no longer have the skills to recognize its symptoms. We present a case of syphilis as a demonstration of this fact and hope that health professionals in the field of adolescent medicine will think about this diagnosis in their clinical work.

Genomics In Premature Infants: A Non-Invasive Strategy To Obtain High-Quality Dna, Mariam M. Said, Clint D. Cappiello, Joseph M. Devaney, Daniele S. Podini, Alana L. Beres, Stanislav Vukmanovic, Khodayar Rais-Bahrami, Naomi L. Luban, Anthony D. Sandler, Zohreh Tatari-Calderone

Pediatrics Faculty Publications

We used a cost-effective, non-invasive method to obtain high-quality DNA from buccal epithelial-cells (BEC) of premature infants for genomic analysis. DNAs from BEC were obtained from premature infants with gestational age ≤ 36 weeks. Short terminal repeats (STRs) were performed simultaneously on DNA obtained from the buccal swabs and blood from the same patient. The STR profiles demonstrated that the samples originated from the same individual and exclude any contamination by external DNAs. Whole exome sequencing was performed on DNAs obtained from BEC on premature infants with and without necrotizing enterocolitis, and successfully provided a total number of reads and …

Age Related Clinical Manifestation Of Acute Bacterial Meningitis In Children, Jabeen Fayyaz, Arshalooz Rehman, Akhter Hamid, Munawar Khursheed, Nukhbha Zia, Asher Feroze

Department of Emergency Medicine

Objective: To determine the signs and symptoms of acute bacterial meningitis (ABM) in different age grops of a paediatric population.

Methods: The retrospective study comprised patients who had been admitted through the Emergency Department of Aga Khan University Hospital, Karachi with the relevant diagnosis from September 2009 to September 2011. Case record forms were used to collect data from patient files. Data was collected using variables such as age, gender, presenting complaints, clinical signs and symptoms, computed tomography scan findings and final outcome of patients. There was a minimal risk of breach in patient confidentiality. SPSS 19 was used for …

Readmission To Paediatric Intensive Care Unit: Frequency, Causes And Outcome, Muhammad Rehan Khan, Prem Kumar, Sumaira Iram, Anwarul Haque

Department of Paediatrics and Child Health

Readmission to intensive care units is considered to be an important quality indicator in ICU settings. This study was carried out at the paediatric intensive unit (PICU) and step down units of paediatric ward at the Aga Khan University Hospital, Karachi, Pakistan, to assess the frequency, common causes and outcome of patients readmitted in PICU within 48 hours after discharge from unit. During the study period, 1022 patients were admitted in PICU, out of which 24 (2.34%) patients required readmission. Male to female ratio was 1.2:1. The mean length of stay on paediatric floor before readmission was 24 hours. Fifteen …

Headstrong Intervention For Pediatric Migraine Headache: A Randomized Clinical Trial., Michael A. Rapoff, Mark Connelly, Jennifer Bickel, Scott W. Powers, Andrew D. Hershey, Janelle R. Allen, Cynthia W. Karlson, Catrina C. Litzenburg, John M. Belmont

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The purpose of this study was to evaluate the efficacy of a self-guided CD-ROM program ("Headstrong") containing cognitive-behavioral self-management strategies versus an educational CD-ROM program for treating headaches, headache-related disability, and quality of life.

METHODS: Participants were 35 children ages 7-12 years with migraine recruited from one university medical center and two children's hospital headache clinics. Participants were randomly assigned to complete the Headstrong or educational control CD-ROM program over a 4-week period. Data on headache frequency, duration, and severity, migraine-related disability, and quality of life (QOL) were obtained at baseline, post-intervention, and 3-months post-intervention.

RESULTS: At post-intervention, Headstrong …

Prevention Of Arteriovenous Shunt Occlusion Using Microbubble And Ultrasound Mediated Thromboprophylaxis., Shelby Kutty, Juefei Wu, James M. Hammel, Joseph R. Abraham, Jeeva Venkataraman, Ibrahim Abdullah, David A. Danford, Stanley J. Radio, John Lof, Thomas R. Porter

Journal Articles: Pediatrics

BACKGROUND: Palliative shunts in congenital heart disease patients are vulnerable to thrombotic occlusion. High mechanical index (MI) impulses from a modified diagnostic ultrasound (US) transducer during a systemic microbubble (MB) infusion have been used to dissolve intravascular thrombi without anticoagulation, and we sought to determine whether this technique could be used prophylactically to reduce thrombus burden and prevent occlusion of surgically placed extracardiac shunts.

METHODS AND RESULTS: Heparin-bonded ePTFE tubular vascular shunts of 4 mm×2.5 cm (Propaten; W.L Gore) were surgically placed in 18 pigs: a right-sided side-to-side arteriovenous (AV, carotid-jugular) shunt, and a left-sided arterio-arterial (AA, carotid-carotid) interposition shunt …

Neonatal Encephalopathic Cerebral Injury In South India Assessed By Perinatal Magnetic Resonance Biomarkers And Early Childhood Neurodevelopmental Outcome, Peter J. Lally, David L. Price, Shreela S. Pauliah, Alan Bainbridge, Justin Kurien, Neeraja Sivasamy, Frances M. Cowan, Guhan Balraj, Manjula Ayer, Kariyapilly Satheesan, Sreejith Ceebi, Angie Wade, Ravi Swamy, Shaji Padinjattel, Betty Hutchon, Madhava Vijayakumar, Mohandas Nair, Krishnakumar Padinharath, Hui Zhang, Ernest B. Cady, Seetha Shankaran, Sudhin Thayyil

Department of Pediatrics

Although brain injury after neonatal encephalopathy has been characterised well in high-income countries, little is known about such injury in low- and middle-income countries. Such injury accounts for an estimated 1 million neonatal deaths per year. We used magnetic resonance (MR) biomarkers to characterise perinatal brain injury, and examined early childhood outcomes in South India.

Anakinra And Related Drugs Targeting Interleukin-1 In The Treatment Of Cryopyrin-Associated Periodic Syndromes, Inessa Bachove, Christopher Chang

Department of Pediatrics Faculty Papers

Anakinra is an interleukin (IL) receptor antagonist that works by blocking the biological activity of IL-1 by competitively inhibiting binding of IL-1 to the type 1 interleukin receptor. IL-1 production is induced in response to inflammatory stimuli and mediates various physiological mechanisms, including inflammation and immunological reactions. Patients with neonatal onset multisystem inflammatory disease (NOMID) produce excess IL-1β, a major proinflammatory cytokine that regulates innate immune responses. Anakinra binds competitively and this results in a rapid reduction in disease severity. NOMID, also known as chronic infantile neurologic, cutaneous, articular syndrome, is the most severe clinical phenotype in the spectrum of …

Phase 2 Randomized, Flexible Crossover, Double-Blinded, Placebo-Controlled Trial Of The Farnesyltransferase Inhibitor Tipifarnib In Children And Young Adults With Neurofibromatosis Type 1 And Progressive Plexiform Neurofibromas, Brigitte C. Widemann, Eva Dombi, Andrea Gillespie, Pamela L. Wolters, Jean Belasco, Aerang Kim, +13 Additional Authors

Pediatrics Faculty Publications

Background

RAS is dysregulated in neurofibromatosis type 1 (NF1) related plexiform neurofibromas (PNs). The activity of tipifarnib, which blocks RAS signaling by inhibiting its farnesylation, was tested in children and young adults with NF1 and progressive PNs.

Methods

Patients aged 3–25 years with NF1-related PNs and imaging evidence of tumor progression were randomized in a double-blinded fashion to receive tipifarnib (200 mg/m² orally every 12 h) or placebo (phase A) and crossed over to the opposite treatment arm at the time of tumor progression (phase B). PN volumes were measured with MRI, and progression was defined as ≥20% volume …

Rota Virus Diarrhea In Hospitalized Children, Muhammad Irfan Habib, Sayyeda Ghazala Kazi, Khalid Mehmood Ahmed Khan, Nukhba Zia

Department of Emergency Medicine

Objective: To determine the frequency and clinical features of Rota virus diarrhea in children presenting in a tertiary care hospital.

Study Design: A cross-sectional, observational study.

Place and Duration of Study: National Institute of Child Health, Karachi, from January to June 2007.

Methodology: A total of three hundred children of either gender aged 1 month to 5 years, who presented with diarrhea of < 7 days as a primary illness were enrolled. Children with bloody diarrhea or nosocomial gastroenteritis acquired duringhospitalization for other disease were not included. Detection of Rota virus in stool was done by enzyme linked immunoassay.

Results: Out of 300 children, 188 (63%) tested positive and 112 (37%) tested negative for Rota virus. Positive Rota virus cases in 7 – 12 months of age was (n = 34, 18.08%). Overall, 151 (80.3%) children …

Variation In Feeding Practices Following The Norwood Procedure., Linda M. Lambert, Nancy A. Pike, Barbara Medoff-Cooper, Victor Zak, Victoria L. Pemberton, Lisa Young-Borkowski, Martha L. Clabby, Kathryn N. Nelson, Richard G. Ohye, Bethany Trainor, Karen Uzark, Nancy Rudd, Louise Bannister, Rosalind Korsin, David S. Cooper, Christian Pizarro, Sinai C. Zyblewski, Bronwyn H. Bartle, Richard V. Williams, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: To assess variation in feeding practice at hospital discharge after the Norwood procedure, factors associated with tube feeding, and associations among site, feeding mode, and growth before stage II.

STUDY DESIGN: From May 2005 to July 2008, 555 subjects from 15 centers were enrolled in the Pediatric Heart Network Single Ventricle Reconstruction Trial; 432 survivors with feeding data at hospital discharge after the Norwood procedure were analyzed.

RESULTS: Demographic and clinical variables were compared among 4 feeding modes: oral only (n = 140), oral/tube (n = 195), nasogastric tube (N-tube) only (n = 40), and gastrostomy tube (G-tube) only …

Testing The Prognostic Accuracy Of The Updated Pediatric Sepsis Biomarker Risk Model, Hector R. Wong, Scott L. Weiss, John S. Giuliano Jr., Mark S. Wainwright, Robert J. Freishtat, +20 Additional Authors

Pediatrics Faculty Publications

Background

We previously derived and validated a risk model to estimate mortality probability in children with septic shock (PERSEVERE; PEdiatRic SEpsis biomarkEr Risk modEl). PERSEVERE uses five biomarkers and age to estimate mortality probability. After the initial derivation and validation of PERSEVERE, we combined the derivation and validation cohorts (n = 355) and updated PERSEVERE. An important step in the development of updated risk models is to test their accuracy using an independent test cohort.

Objective

To test the prognostic accuracy of the updated version PERSEVERE in an independent test cohort.

Methods

Study subjects were recruited from multiple pediatric intensive …

Consanguineous Marriage And Intellectual And Developmental Disabilities Among Arab Bedouins Children Of The Negev Region In Southern Israel: A Pilot Study, Hassan Abu Saad, Salman Elbedour, Eyad Hallaq, Joav Merrick, Ariel Tenenbaum

Pediatrics Faculty Publications

No abstract provided.

Zinc Deficiency-Associated Dermatitis In Infants During A Nationwide Shortage Of Injectable Zinc--Washington, Dc, And Houston, Texas 2012-2013, Duke Ruktanonchai, Michael Lowe, Scott A. Norton, Tiana Garrett, Lamia Soghier, Edward Weiss, June Hatfield, Jeffrey Lapinski, Steven Abrams, Wanda Barfield

Pediatrics Faculty Publications

No abstract provided.

An Efficient, Free-Breathing Protocolf For Mr Right Heart Catheterization, Jonathan R. Mazal, Toby Rogers, Anthony Z. Faranesh, Peter Kellman, William H. Schenke, Annette M. Stine, Laurie P. Grant, Kanishka Ratnayaka, Robert J. Lederman

Pediatrics Posters and Presentations

No abstract provided.

Technologist Primer For Mri Right Heart Catheterization: The Nih And Cnmc Experience, Jonathan R. Mazal, Kendall O'Brien, William Schenke, Annette Stine, Laurie Grant, Anthony Z. Faranesh, Kanishka Ratnayaka, Robert J. Lederman

Pediatrics Posters and Presentations

No abstract provided.

Quantification Of Right Ventricular Function From Short-Axis Displacement-Encoded Images, Jonathan D. Suever, Gregory J. Wehner, Linyuan Jing, David K. Powell, Christopher M. Haggerty, Xiaodong Zhong, Frederick H. Epstein, Brandon K. Fornwalt

Pediatrics Presentations

Background

Right ventricular (RV) function is important in many disease states, but is difficult to quantify from routine MR imaging. Previous work has shown that long-axis deformation/strain is the most critical contributor to global RV function; however, short-axis datasets allow for better coverage of the RV. Thus it would be ideal to be able to quantify RV long-axis function using short-axis slice orientations. We hypothesized that a stack of three-dimensional (3D) displacement encoded (DENSE) images could reliably quantify longitudinal deformation of the RV to overcome the need for acquiring additional long-axis views of the RV.

Methods

A contiguous stack of …

Assessment Of Intra- And Inter-Ventricular Cardiac Dyssynchrony In Patients With Repaired Tetralogy Of Fallot: A Cardiac Magnetic Resonance Study, Linyuan Jing, Christopher M. Haggerty, Jonathan D. Suever, Ashwin Prakash, Frank Cecchin, Oskar Skrinjar, Tal Geva, Andrew J. Powell, Brandon K. Fornwalt

Pediatrics Presentations

Background

Patients with repaired tetralogy of Fallot (TOF) frequently have right bundle branch block. However, the contribution of cardiac dyssynchrony to dysfunction remains controversial. To better understand this phenomenon and ultimately study therapies, we developed a method to quantify left (LV), right (RV) and inter-ventricular cardiac dyssynchrony using standard cine CMR.

Methods

30 patients with repaired TOF (age 28 ± 16, 46% female) and 17 healthy controls (age 29 ± 7, 12% female) underwent cine CMR. Patients were imaged twice to assess inter-test reproducibility. Circumferential strain vs time curves were generated with a custom feature tracking algorithm for 12 LV …

Two-Dimensional Estimates Of Left Ventricular Strains Are Significantly Affected By Through-Plane Motion, Jonathan D. Suever, Gregory J. Wehner, Linyuan Jing, David K. Powell, Christopher M. Haggerty, Xiaodong Zhong, Frederick H. Epstein, Brandon K. Fornwalt

Pediatrics Presentations

Background

Advanced measures of cardiac mechanics such as left ventricular (LV) strains can be used in conjunction with classical biomarkers to gauge cardiovascular health and improve prediction of patient outcomes. Several imaging techniques, including displacement-encoded magnetic resonance imaging (DENSE), are used to non-invasively assess cardiac mechanics. These data are predominantly acquired in two dimensions (2D) due to simplified post-processing and shorter acquisition times; however, this type of acquisition and subsequent analysis cannot account for through-plane motion caused by longitudinal contraction of the left ventricle. We hypothesized that through-plane motion has a significant effect on 2D strain estimates.

Methods

Cine DENSE …

Characterizing The Temporal Evolution Of Altered Cardiac Mechanics In Diet-Induced Obese Mice Using Cine Dense Cmr, Christopher M. Haggerty, Andrea C. Mattingly, Cassi M. Binkley, Sage P. Kramer, David K. Powell, Frederick H. Epstein, Brandon K. Fornwalt

Pediatrics Presentations

Background

Obesity and metabolic syndrome are associated with increased risk of cardiovascular disease. Research suggests that altered cardiac mechanics (i.e., reduced strains, torsion, and synchrony of contraction) are present in obesity; yet, the causes of this mechanical dysfunction and its relationship to other sequelae of obesity (e.g., hypertension and elevated blood glucose) are not well understood. We hypothesize that diet-induced obesity in mice leads to reductions in measures of left ventricular (LV) mechanics, which develop in acute response to the onset of hyperglycemia, hypertension, and ventricular remodeling.

Methods

Twenty 4-week-old C57BL/6J mice were randomized (n = 10 per group) to …

Sodium-Coupled Neutral Amino Acid Transporter 1 (Snat1) Modulates L-Citrulline Transport And Nitric Oxide (No) Signaling In Piglet Pulmonary Arterial Endothelial Cells, Anna Dikalova, Angela Fagiana, Judy L. Aschner, Michael Aschner, Marshall Summar, Candice D. Fike

Pediatrics Faculty Publications

Rationale

There is evidence that impairments in nitric oxide (NO) signaling contribute to chronic hypoxia-induced pulmonary hypertension. The L-arginine-NO precursor, L-citrulline, has been shown to ameliorate pulmonary hypertension. Sodium-coupled neutral amino acid transporters (SNATs) are involved in the transport of L-citrulline into pulmonary arterial endothelial cells (PAECs). The functional link between the SNATs, L-citrulline, and NO signaling has not yet been explored.

Objective

We tested the hypothesis that changes in SNAT1 expression and transport function regulate NO production by modulating eNOS coupling in newborn piglet PAECs.

Methods and Results

A silencing RNA (siRNA) technique was used to assess the contribution …