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Bronchopulmonary Dysplasia: Comparison Between The Two Most Used Diagnostic Criteria, Enrique Gomez Pomar, Vanessa A. Concina, Aaron Samide, Philip M. Westgate, Henrietta S. Bada Dec 2018

Bronchopulmonary Dysplasia: Comparison Between The Two Most Used Diagnostic Criteria, Enrique Gomez Pomar, Vanessa A. Concina, Aaron Samide, Philip M. Westgate, Henrietta S. Bada

Pediatrics Faculty Publications

Objectives: To compare the Shennan's and the consensus definition of Bronchopulmonary Dysplasia (BPD) from the National Institutes of Health (NIH) workshop and analyze specific risk factors associated with each definition.

Study design: Retrospective analysis of records of 274 infants admitted to a level IV intensive care unit. Infants were classified as having BPD or no BPD by both definitions. Differences in incidence and risk factors were analyzed. Statistical methods included descriptive statistics, comparative tests, and marginal logistic regression modeling.

Results: The estimated difference in prevalence was 32% [95% CI: (26%, 37%), (p < 0.0001)] between both criteria. The prevalence of BPD was 80% higher based on the NIH criteria [RR = 1.80; 95% CI: (1.58, 2.06)]. Infants with no BPD by the Shennan definition were breathing room air with or without positive or continuous pressure support and were most likely to be discharged home on oxygen [OR = 4.47, 95% CI: (1.20, 16.61), p = 0.03]. Gestational age, birth weight, …


Dual-Display Laparoscopic Laser Speckle Contrast Imaging For Real-Time Surgical Assistance, Jaepyeong Cha, Corey Zheng, Lung Wai Lau Nov 2018

Dual-Display Laparoscopic Laser Speckle Contrast Imaging For Real-Time Surgical Assistance, Jaepyeong Cha, Corey Zheng, Lung Wai Lau

Pediatrics Faculty Publications

Laser speckle contrast imaging (LSCI) utilizes the speckle pattern of a laser to determine the blood flow in tissues. The current approaches for its use in a clinical setting require a camera system with a laser source on a separate optical axis making it unsuitable for minimally invasive surgery (MIS). With blood flow visualization, bowel viability, for example, can be determined. Thus, LSCI can be a valuable tool in gastrointestinal surgery. In this work, we develop the first-of-its-kind dual-display laparoscopic vision system integrating LSCI with a commercially available 10mm rigid laparoscope where the laser has the same optical axis as …


Blood Transfusions In Preterm Infants: Changes On Perfusion Index And Intermittent Hypoxemia, Katrina T. Ibonia, Henrietta S. Bada, Philip M. Westgate, Enrique Gomez Pomar, Prasad Bhandary, Abhijit R. Patwardhan, Elie G. Abu Jawdeh Nov 2018

Blood Transfusions In Preterm Infants: Changes On Perfusion Index And Intermittent Hypoxemia, Katrina T. Ibonia, Henrietta S. Bada, Philip M. Westgate, Enrique Gomez Pomar, Prasad Bhandary, Abhijit R. Patwardhan, Elie G. Abu Jawdeh

Pediatrics Faculty Publications

BACKGROUND

Red blood cell (RBC) transfusion decreases intermittent hypoxemia (IH) events beyond the first week of life. This benefit may be related to improved perfusion to the respiratory control network. Perfusion index (PI) is a perfusion measure provided by the pulse oximeter. We hypothesized that the benefit in IH after RBC transfusion is associated with an increase in PI. In addition, we assessed the value of PI and clinical measures in predicting the effect of RBC transfusion on IH.

STUDY DESIGN AND METHODS

We prospectively enrolled infants less than 30 weeks' gestation age. PI and oxygen saturation (SpO2) …


Testing Longitudinal Relationships Between Internet Addiction And Well-Being In Hong Kong Adolescents: Cross-Lagged Analyses Based On Three Waves Of Data, Lu Yu, Daniel T. L. Shek Oct 2018

Testing Longitudinal Relationships Between Internet Addiction And Well-Being In Hong Kong Adolescents: Cross-Lagged Analyses Based On Three Waves Of Data, Lu Yu, Daniel T. L. Shek

Pediatrics Faculty Publications

Using a panel design, this study examined the prospective relationships between Internet addiction and life satisfaction as well as hopelessness in a representative sample of Hong Kong adolescents. Starting from 2009/10 academic year, 3328 Secondary 1 students in 28 secondary schools in Hong Kong participated in this longitudinal study (Mean age = 12.59 years; SD = 0.74 years). All participants responded to a questionnaire that includes the Internet Addiction Test, Life Satisfaction Scale, and Hopelessness Scale on a yearly basis. Cross-lagged analyses based on three waves of data collected during three junior adolescent years showed that Internet addiction measured at …


Progression Of Gastric Acid Production In Preterm Neonates: Utilization Of In-Vitro Method, Murali R. Palla, Shashidhar Harohalli, Tim N. Crawford, Nirmala Desai Aug 2018

Progression Of Gastric Acid Production In Preterm Neonates: Utilization Of In-Vitro Method, Murali R. Palla, Shashidhar Harohalli, Tim N. Crawford, Nirmala Desai

Pediatrics Faculty Publications

Background: Limited studies are done regarding ability to produce gastric acid in preterm infants and most studies used in vivo method of assessing gastric pH.

Objectives: To assess the feasibility of using an in vitro method of measuring gastric pH in babies ≤ 28 weeks gestational age (GA) and determine whether changes in gastric pH differ with gestational age, mode of delivery, and use of antenatal steroids.

Design/Methods: Prospective study that enrolled extremely low birth weight (ELBW) babies. Gastric aspirate collected before feeding. In vitro testing of gastric aspirates for pH were done on days of life 1, 3, 5, …


Results From A 78-Week, Single-Arm, Open-Label Phase 2 Study To Evaluate Ux007 In Pediatric And Adult Patients With Severe Long-Chain Fatty Acid Oxidation Disorders (Lc-Faod)., Jerry Vockley, Barbara Burton, Gerard T Berry, Nicola Longo, John Phillips, Pranoot Tanpaiboon, +Several Additional Authors Jul 2018

Results From A 78-Week, Single-Arm, Open-Label Phase 2 Study To Evaluate Ux007 In Pediatric And Adult Patients With Severe Long-Chain Fatty Acid Oxidation Disorders (Lc-Faod)., Jerry Vockley, Barbara Burton, Gerard T Berry, Nicola Longo, John Phillips, Pranoot Tanpaiboon, +Several Additional Authors

Pediatrics Faculty Publications

Long-chain fatty acid oxidation disorders (LC-FAOD) are rare disorders characterized by acute crises of energy metabolism and severe energy deficiency that may present with cardiomyopathy, hypoglycemia, and/or rhabdomyolysis, which can lead to frequent hospitalizations and early death. An open-label Phase 2 study evaluated the efficacy of UX007, an investigational odd-carbon medium-chain triglyceride, in 29 subjects with severe LC-FAOD. UX007 was administered over 78 weeks at a target dose of 25-35% total daily caloric intake (mean 27.5%). The frequency and duration of major clinical events (hospitalizations, emergency room visits, and emergency home interventions due to rhabdomyolysis, hypoglycemia, and cardiomyopathy) occurring during …


Economic Evaluation Of Patient Navigation Programs In Colorectal Cancer Care, A Systematic Review., Chloé Gervès-Pinquié, Anne Girault, Serena Phillips, Sarah Raskin, Mandi Pratt-Chapman Jun 2018

Economic Evaluation Of Patient Navigation Programs In Colorectal Cancer Care, A Systematic Review., Chloé Gervès-Pinquié, Anne Girault, Serena Phillips, Sarah Raskin, Mandi Pratt-Chapman

Pediatrics Faculty Publications

Patient navigation has expanded as a promising approach to improve cancer care coordination and patient adherence. This paper addresses the need to identify the evidence on the economic impact of patient navigation in colorectal cancer, following the Health Economic Evaluation Publication Guidelines. Articles indexed in Medline, Cochrane, CINAHL, and Web of Science between January 2000 and March 2017 were analyzed. We conducted a systematic review of the literature using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The quality assessment of the included studies was based on the Consolidated Health Economic Evaluation Reporting Standards (CHEERS) checklist. Inclusion criteria …


Lung Cancer Risk And Low (≤50 Μg/L) Drinking Water Arsenic Levels For Us Counties (2009⁻2013)-A Negative Association., Steven H Lamm, Isabella J Boroje, Hamid Ferdosi, Jaeil Ahn Jun 2018

Lung Cancer Risk And Low (≤50 Μg/L) Drinking Water Arsenic Levels For Us Counties (2009⁻2013)-A Negative Association., Steven H Lamm, Isabella J Boroje, Hamid Ferdosi, Jaeil Ahn

Pediatrics Faculty Publications

While epidemiologic studies clearly demonstrate drinking water with high levels of arsenic as a significant risk factor for lung cancer, the evidence at low levels (≤50 μg/L) is uncertain. Therefore, we have conducted an ecological analysis of recent lung cancer incidence for US counties with a groundwater supply of <50 μg/L, the historical limit for both the EPA and WHO. Data sources used included USGS for arsenic exposure, NCI for lung cancer outcome, and CDC and US Census Bureau forcovariates. Poisson log-linear models were conducted for male, female, and total populations using for exposure median county arsenic level, maximum arsenic level ≤50 μg/L, and ≥80% population groundwater dependency. Statistically significant negative associations were found in each of the six models in which the exposure was limited to those who had major exposure (≥80% dependency) to low-levels of arsenic (≤50 μg/L). This is the first large ecological study of lung cancer risk from drinking water arsenic levels that specifically examined the dose-response slope for populations whose exposure was below the historical limit of ≤50 μg/L. The models for each of the three populations (total; male; female) demonstrated an association that is both negative and statistically significant.


Phenotypical Characterization Of Human Rhinovirus Infections In Severely Premature Children, Geovanny F. Perez, Bassem Kurdi, Rosemary Megalaa, Krishna Pancham, Shehlanoor Huseni, Natalia Isaza, Carlos E. Rodriguez-Martinez, Dinesh Pillai, Gustavo Nino Jun 2018

Phenotypical Characterization Of Human Rhinovirus Infections In Severely Premature Children, Geovanny F. Perez, Bassem Kurdi, Rosemary Megalaa, Krishna Pancham, Shehlanoor Huseni, Natalia Isaza, Carlos E. Rodriguez-Martinez, Dinesh Pillai, Gustavo Nino

Pediatrics Faculty Publications

Background: Human Rhinovirus (HRV) has been identified as the most common cause of acute respiratory infections and hospitalizations in premature children. It is unclear if premature children are more susceptible to HRV due to their decreased pulmonary reserve or because they have enhanced lower airway reactivity to HRV.

Methods: We conducted a retrospective analysis of the clinical respiratory presentation of all PCR-confirmed HRV infections in full-term and premature children aged ≤ 3 years in our institution. Standardized respiratory distress scores were developed to examine lower airway obstruction (i.e., wheezing, hyperinflation, and sub-costal retractions) along with markers of decreased pulmonary reserve …


First-Pass Cyp3a-Mediated Metabolism Of Midazolam In The Gut Wall And Liver In Preterm Neonates., Janneke M Brussee, Huixin Yu, Elke H J Krekels, Berend De Roos, Margreke J E Brill, Johannes N Van Den Anker, Amin Rostami-Hodjegan, Saskia N De Wildt, Catherijne A J Knibbe Jun 2018

First-Pass Cyp3a-Mediated Metabolism Of Midazolam In The Gut Wall And Liver In Preterm Neonates., Janneke M Brussee, Huixin Yu, Elke H J Krekels, Berend De Roos, Margreke J E Brill, Johannes N Van Den Anker, Amin Rostami-Hodjegan, Saskia N De Wildt, Catherijne A J Knibbe

Pediatrics Faculty Publications

To predict first-pass and systemic cytochrome P450 (CYP) 3A-mediated metabolism of midazolam in preterm neonates, a physiological population pharmacokinetic model was developed describing intestinal and hepatic midazolam clearance in preterm infants. On the basis of midazolam and 1-OH-midazolam concentrations from 37 preterm neonates (gestational age 26-34 weeks) receiving midazolam orally and/or via a 30-minute intravenous infusion, intrinsic clearance in the gut wall and liver were found to be very low, with lower values in the gut wall (0.0196 and 6.7 L/h, respectively). This results in a highly variable and high total oral bioavailability of 92.1% (range, 67-95%) in preterm neonates, …


Admission Eeg Findings In Diverse Paediatric Cerebral Malaria Populations Predict Outcomes., Douglas G Postels, Xiaoting Wu, Chenxi Li, Peter W Kaplan, Karl B Seydel, Terrie E Taylor, Youssef A Kousa, Richard Idro, Robert Opoka, Chandy C John, Gretchen L Birbeck May 2018

Admission Eeg Findings In Diverse Paediatric Cerebral Malaria Populations Predict Outcomes., Douglas G Postels, Xiaoting Wu, Chenxi Li, Peter W Kaplan, Karl B Seydel, Terrie E Taylor, Youssef A Kousa, Richard Idro, Robert Opoka, Chandy C John, Gretchen L Birbeck

Pediatrics Faculty Publications

BACKGROUND: Electroencephalography at hospital presentation may offer important insights regarding prognosis that can inform understanding of cerebral malaria (CM) pathophysiology and potentially guide patient selection and risk stratification for future clinical trials. Electroencephalogram (EEG) findings in children with CM in Uganda and Malawi were compared and associations between admission EEG findings and outcome across this diverse population were assessed. Demographic, clinical and admission EEG data from Ugandan and Malawian children admitted from 2009 to 2012 with CM were gathered, and survivors assessed for neurological abnormalities at discharge.

RESULTS: 281 children were enrolled (Uganda n = 122, Malawi n = 159). …


Disruption Of Neonatal Cardiomyocyte Physiology Following Exposure To Bisphenol-A., Manelle Ramadan, Meredith Sherman, Rafael Jaimes, Ashika Chaluvadi, Luther Swift, Nikki Gillum Posnack May 2018

Disruption Of Neonatal Cardiomyocyte Physiology Following Exposure To Bisphenol-A., Manelle Ramadan, Meredith Sherman, Rafael Jaimes, Ashika Chaluvadi, Luther Swift, Nikki Gillum Posnack

Pediatrics Faculty Publications

Bisphenol chemicals are commonly used in the manufacturing of polycarbonate plastics, polyvinyl chloride plastics, resins, and thermal printing applications. Humans are inadvertently exposed to bisphenols through contact with consumer products and/or medical devices. Recent reports have shown a link between bisphenol-a (BPA) exposure and adverse cardiovascular outcomes; although these studies have been limited to adult subjects and models. Since cardiac physiology differs significantly between the developing and adult heart, we aimed to assess the impact of BPA exposure on cardiac function, using a neonatal cardiomyocyte model. Neonatal rat ventricular myocytes were monitored to assess cell viability, spontaneous beating rate, beat …


The Influence Of Parental Control And Parent-Child Relational Qualities On Adolescent Internet Addiction: A 3-Year Longitudinal Study In Hong Kong, Daniel T. L. Shek, Xiaoqin Zhu, Cecilia M. S. Ma May 2018

The Influence Of Parental Control And Parent-Child Relational Qualities On Adolescent Internet Addiction: A 3-Year Longitudinal Study In Hong Kong, Daniel T. L. Shek, Xiaoqin Zhu, Cecilia M. S. Ma

Pediatrics Faculty Publications

This study investigated how parental behavioral control, parental psychological control, and parent-child relational qualities predicted the initial level and rate of change in adolescent internet addiction (IA) across the junior high school years. The study also investigated the concurrent and longitudinal effects of different parenting factors on adolescent IA. Starting from the 2009/2010 academic year, 3,328 Grade 7 students (Mage = 12.59 ± 0.74 years) from 28 randomly selected secondary schools in Hong Kong responded on a yearly basis to a questionnaire measuring multiple constructs including socio-demographic characteristics, perceived parenting characteristics, and IA. Individual growth curve (IGC) analyses …


Amygdala Corticofugal Input Shapes Mitral Cell Responses In The Accessory Olfactory Bulb, Livio Oboti, Eleonora Russo, Tuyen Tran, Daniel Durstewitz, Joshua G. Corbin May 2018

Amygdala Corticofugal Input Shapes Mitral Cell Responses In The Accessory Olfactory Bulb, Livio Oboti, Eleonora Russo, Tuyen Tran, Daniel Durstewitz, Joshua G. Corbin

Pediatrics Faculty Publications

Interconnections between the olfactory bulb and the amygdala are a major pathway for triggering strong behavioral responses to a variety of odorants. However, while this broad mapping has been established, the patterns of amygdala feedback connectivity and the influence on olfactory circuitry remain unknown. Here, using a combination of neuronal tracing approaches, we dissect the connectivity of a cortical amygdala [posteromedial cortical nucleus (PmCo)] feedback circuit innervating the mouse accessory olfactory bulb. Optogenetic activation of PmCo feedback mainly results in feedforward mitral cell (MC) inhibition through direct excitation of GABAergic granule cells. In addition, LED-driven activity of corticofugal afferents increases …


A Global Health Research Checklist For Clinicians., Rasha D Sawaya, Kristen A Breslin, Eiman Abdulrahman, Jennifer I Chapman, Dafina M Good, Lili Moran, Paul C Mullan, Oluwakemi Badaki-Makun Apr 2018

A Global Health Research Checklist For Clinicians., Rasha D Sawaya, Kristen A Breslin, Eiman Abdulrahman, Jennifer I Chapman, Dafina M Good, Lili Moran, Paul C Mullan, Oluwakemi Badaki-Makun

Pediatrics Faculty Publications

Global health research has become a priority in most international medical projects. However, it is a difficult endeavor, especially for a busy clinician. Navigating the ethics, methods, and local partnerships is essential yet daunting.To date, there are no guidelines published to help clinicians initiate and complete successful global health research projects. This Global Health Research Checklist was developed to be used by clinicians or other health professionals for developing, implementing, and completing a successful research project in an international and often low-resource setting. It consists of five sections: Objective, Methodology, Institutional Review Board and Ethics, Culture and partnerships, and Logistics. …


Characterization Of Sex-Based Dna Methylation Signatures In The Airways During Early Life., Cesar L Nino, Geovanny F Perez, Natalia Isaza, Maria J Gutierrez, Jose L Gomez, Gustavo Nino Apr 2018

Characterization Of Sex-Based Dna Methylation Signatures In The Airways During Early Life., Cesar L Nino, Geovanny F Perez, Natalia Isaza, Maria J Gutierrez, Jose L Gomez, Gustavo Nino

Pediatrics Faculty Publications

Human respiratory conditions are largely influenced by the individual's sex resulting in overall higher risk for males. Sex-based respiratory differences are present at birth suggesting a strong genetic component. Our objective was to characterize early life sex-based genomic signatures determined by variable X-chromosome methylation in the airways. We compared male versus female genome-wide DNA methylation in nasal airway samples from newborns and infants aged 1-6 months (N = 12). We analyzed methylation signals across CpG sites mapped to each X-linked gene using an unsupervised classifier (principal components) followed by an internal evaluation and an exhaustive cross-validation. Results were validated in …


Self-Reported Risk And Delinquent Behavior And Problem Behavioral Intention In Hong Kong Adolescents: The Role Of Moral Competence And Spirituality, Daniel T. L. Shek, Xiaoqin Zhu Mar 2018

Self-Reported Risk And Delinquent Behavior And Problem Behavioral Intention In Hong Kong Adolescents: The Role Of Moral Competence And Spirituality, Daniel T. L. Shek, Xiaoqin Zhu

Pediatrics Faculty Publications

Based on the six-wave data collected from Grade 7 to Grade 12 students (N = 3,328 at Wave 1), this pioneer study examined the development of problem behaviors (risk and delinquent behavior and problem behavioral intention) and the predictors (moral competence and spirituality) among adolescents in Hong Kong. Individual growth curve models revealed that while risk and delinquent behavior accelerated and then slowed down in the high school years, adolescent problem behavioral intention slightly accelerated over time. After controlling the background socio-demographic factors, moral competence and spirituality were negatively associated with risk and delinquent behavior as well as problem …


Communicating Effectively In Pediatric Cancer Care: Translating Evidence Into Practice., Lindsay J Blazin, Cherilyn Cecchini, Catherine Habashy, Erica C Kaye, Justin N Baker Mar 2018

Communicating Effectively In Pediatric Cancer Care: Translating Evidence Into Practice., Lindsay J Blazin, Cherilyn Cecchini, Catherine Habashy, Erica C Kaye, Justin N Baker

Pediatrics Faculty Publications

Effective communication is essential to the practice of pediatric oncology. Clear and empathic delivery of diagnostic and prognostic information positively impacts the ways in which patients and families cope. Honest, compassionate discussions regarding goals of care and hopes for patients approaching end of life can provide healing when other therapies have failed. Effective communication and the positive relationships it fosters also can provide comfort to families grieving the loss of a child. A robust body of evidence demonstrates the benefits of optimal communication for patients, families, and healthcare providers. This review aims to identify key communication skills that healthcare providers …


Continuous Training And Certification In Neonatal Resuscitation In Remote Areas Using A Multi-Platform Information And Communication Technology Intervention, Compared To Standard Training: A Randomized Cluster Trial Study Protocol, Carlos Alberto Delgado, Enrique M. Gómez Pomar, Pablo Velásquez, Víctor Sánchez, Roberto Shimabuku, Luis Huicho, Rcpneoperu Study Group Mar 2018

Continuous Training And Certification In Neonatal Resuscitation In Remote Areas Using A Multi-Platform Information And Communication Technology Intervention, Compared To Standard Training: A Randomized Cluster Trial Study Protocol, Carlos Alberto Delgado, Enrique M. Gómez Pomar, Pablo Velásquez, Víctor Sánchez, Roberto Shimabuku, Luis Huicho, Rcpneoperu Study Group

Pediatrics Faculty Publications

Background: About 10% of all newborns may have difficulty breathing and require support by trained personnel. In Peru, 90% of deliveries occur in health facilities. However, there is not a national neonatal resuscitation and certification program for the public health sector. In addition, the Andes and the Amazon regions concentrate large rural remote areas, which further limit the implementation of training programs and the accomplishment of continuous certification. Neonatal resuscitation training through the use of Information, Communication and Technology (ICT) tools, running on computers, tablets or mobile phones, may overcome such limitations. This strategy allows online and offline access …


Teaching The Principles Of Pediatric Critical Care To Non-Intensivists In Resource Limited Settings: Challenges And Opportunities, Michael F. Canarie, Asha N. Shenoi Mar 2018

Teaching The Principles Of Pediatric Critical Care To Non-Intensivists In Resource Limited Settings: Challenges And Opportunities, Michael F. Canarie, Asha N. Shenoi

Pediatrics Faculty Publications

No abstract provided.


Maladjustment Of Programmable Ventricular Shunt Valves By Inadvertent Exposure To A Common Hospital Device, R. Fujimura, Robert M. Lober, K. Kamian, L. Kleiner Mar 2018

Maladjustment Of Programmable Ventricular Shunt Valves By Inadvertent Exposure To A Common Hospital Device, R. Fujimura, Robert M. Lober, K. Kamian, L. Kleiner

Pediatrics Faculty Publications

Background: Programmable ventricular shunt valves are commonly used to treat hydrocephalus. They can be adjusted to allow for varying amounts of cerebrospinal fluid (CSF) flow using an external magnetic programming device, and are susceptible to maladjustment from inadvertent exposure to magnetic fields.Case Description: We describe the case of a 3‑month‑old girl treated for hydrocephalus with a programmable StrataTM II valve found at the incorrect setting on multiple occasions during her hospitalization despite frequent reprogramming and surveillance. We found that the Vocera badge, a common hands‑free wireless communication system worn by our nursing staff, had a strong enough magnetic field to …


Risk Factors For Major Early Adverse Events Related To Cardiac Catheterization In Children And Young Adults With Pulmonary Hypertension: An Analysis Of Data From The Impact (Improving Adult And Congenital Treatment) Registry., Michael L O'Byrne, Kevin F Kennedy, Joshua P Kanter, John T Berger, Andrew C Glatz Feb 2018

Risk Factors For Major Early Adverse Events Related To Cardiac Catheterization In Children And Young Adults With Pulmonary Hypertension: An Analysis Of Data From The Impact (Improving Adult And Congenital Treatment) Registry., Michael L O'Byrne, Kevin F Kennedy, Joshua P Kanter, John T Berger, Andrew C Glatz

Pediatrics Faculty Publications

BACKGROUND: Cardiac catheterization is the gold standard for assessment and follow-up of patients with pulmonary hypertension (PH). To date, there are limited data about the factors that influence the risk of catastrophic adverse events after catheterization in this population.

METHODS AND RESULTS: A retrospective multicenter cohort study was performed to measure risk of catastrophic adverse outcomes after catheterization in children and young adults with PH and identify risk factors for these outcomes. All catheterizations in children and young adults, aged 0 to 21 years, with PH at hospitals submitting data to the IMPACT (Improving Adult and Congenital Treatment) registry between …


Impaired Type I Interferon Regulation In The Blood Transcriptome Of Recurrent Asthma Exacerbations., Jose L Gomez, Maria P Diaz, Gustavo Nino, Clemente J Britto Feb 2018

Impaired Type I Interferon Regulation In The Blood Transcriptome Of Recurrent Asthma Exacerbations., Jose L Gomez, Maria P Diaz, Gustavo Nino, Clemente J Britto

Pediatrics Faculty Publications

BACKGROUND: Asthma exacerbations are an important cause of morbidity in asthma. Respiratory infections are often involved in asthma exacerbations in both children and adults. Some individuals with asthma have increased susceptibility to viral infections and as a result increased rates of asthma exacerbations. We sought to identify a transcriptomic signature in the blood associated with asthma exacerbations triggered by respiratory infections (AETRI) and determine its association with increased risk for asthma exacerbations.

METHODS: We conducted a two-step study using publicly available, previously generated transcriptomic signatures in peripheral blood mononuclear cells (PBMCs) from asthmatics to identify novel markers of increased risk …


The Epidemic Of Neonatal Abstinence Syndrome, Historical References Of Its Origins, Assessment, And Management, Enrique Gomez-Pomar, Loretta P. Finnegan Feb 2018

The Epidemic Of Neonatal Abstinence Syndrome, Historical References Of Its Origins, Assessment, And Management, Enrique Gomez-Pomar, Loretta P. Finnegan

Pediatrics Faculty Publications

Neonatal abstinence syndrome (NAS) refers to a constellation of signs that are present in some newborn infants resulting from the abrupt cessation of passive transfer of maternal opioids used during pregnancy. The classic NAS refers to infants born to mothers who used opioids during pregnancy, but the term has broadened to include infants whose mothers have used or abused other psychoactive substances during pregnancy that contribute to the expression of the syndrome. Pregnant women who use opioids do so illicitly, and/or as medically prescribed for pain relief, and/or as medication assisted treatment for opioid dependence. The first case of NAS …


Predicting Cyp3a-Mediated Midazolam Metabolism In Critically Ill Neonates, Infants, Children And Adults With Inflammation And Organ Failure., Janneke M Brussee, Nienke J Vet, Elke H J Krekels, Abraham J Valkenburg, Evelyne Jacqz-Aigrain, Joop M A Van Gerven, Eleonora L Swart, Johannes N Van Den Anker, Dick Tibboel, Matthijs De Hoog, Saskia N De Wildt, Catherijne A J Knibbe Feb 2018

Predicting Cyp3a-Mediated Midazolam Metabolism In Critically Ill Neonates, Infants, Children And Adults With Inflammation And Organ Failure., Janneke M Brussee, Nienke J Vet, Elke H J Krekels, Abraham J Valkenburg, Evelyne Jacqz-Aigrain, Joop M A Van Gerven, Eleonora L Swart, Johannes N Van Den Anker, Dick Tibboel, Matthijs De Hoog, Saskia N De Wildt, Catherijne A J Knibbe

Pediatrics Faculty Publications

No abstract provided.


Cardiovascular Effects Of Metabolic Syndrome After Transplantation: Convergence Of Obesity And Transplant-Related Factors., Kristen Sgambat, Sarah Clauss, Asha Moudgil Feb 2018

Cardiovascular Effects Of Metabolic Syndrome After Transplantation: Convergence Of Obesity And Transplant-Related Factors., Kristen Sgambat, Sarah Clauss, Asha Moudgil

Pediatrics Faculty Publications

Children are at increased risk of developing metabolic syndrome (MS) after kidney transplantation, which contributes to long-term cardiovascular (CV) morbidities and decline in allograft function. While MS in the general population occurs due to excess caloric intake and physical inactivity, additional chronic kidney disease and transplant-related factors contribute to the development of MS in transplant recipients. Despite its significant health consequences, the interplay of the individual components in CV morbidity in pediatric transplant recipients is not well understood. Additionally, the optimal methods to detect early CV dysfunction are not well defined in this unique population. The quest to establish clear …


Teenage Exercise Is Associated With Earlier Symptom Onset In Dysferlinopathy: A Retrospective Cohort Study., Ursula R Moore, Marni Jacobs, Roberto Fernandez-Torron, Jiji Jang, Meredith K James, Diana X Bharucha-Goebel, +Several Additional Authors Jan 2018

Teenage Exercise Is Associated With Earlier Symptom Onset In Dysferlinopathy: A Retrospective Cohort Study., Ursula R Moore, Marni Jacobs, Roberto Fernandez-Torron, Jiji Jang, Meredith K James, Diana X Bharucha-Goebel, +Several Additional Authors

Pediatrics Faculty Publications

No abstract provided.


Mindfulness And Multiple Sclerosis, Mandakini Sadhir Jan 2018

Mindfulness And Multiple Sclerosis, Mandakini Sadhir

Pediatrics Faculty Publications

Multiple sclerosis is a chronic, debilitating autoimmune condition with unknown etiology. It can have significant impact on quality of life and overall sense of wellbeing. It can be associated with poor sleep, fatigue, mental health disorders such as anxiety, depression and affect interpersonal relationships. Impact of mindfulness based interventions in mental health conditions, chronic illness as well as multiple sclerosis in adults has been widely studied. Several studies have concluded that mindfulness based intervention can be beneficial in improving quality of life, mental health and physical symptoms such as fatigue in adult patients with multiple sclerosis. Current literature suggest that …


Aberrant Brain Functional Connectivity In Newborns With Congenital Heart Disease Before Cardiac Surgery, Josepheen De Asis-Cruz, Mary T. Donofrio, Gilbert Vezina, Catherine Limperopoulos Jan 2018

Aberrant Brain Functional Connectivity In Newborns With Congenital Heart Disease Before Cardiac Surgery, Josepheen De Asis-Cruz, Mary T. Donofrio, Gilbert Vezina, Catherine Limperopoulos

Pediatrics Faculty Publications

Newborns with congenital heart disease (CHD) requiring open heart surgery are at increased risk for neurodevelopmental disabilities. Recent quantitative MRI studies have reported disrupted growth, microstructure, and metabolism in fetuses and newborns with complex CHD. To date, no study has examined whether functional brain connectivity is altered in this high-risk population after birth, before surgery. Our objective was to compare whole-brain functional connectivity of resting state networks in healthy, term newborns (n = 82) and in term neonates with CHD before surgery (n = 30) using graph theory and network-based statistics. We report for the first time intact global network …


Genotype-Phenotype Correlation In Nf1: Evidence For A More Severe Phenotype Associated With Missense Mutations Affecting Nf1 Codons 844-848, M Koczkowska, Y Chen, T Callens, A Gomes, A Sharp, Rhonda Schonberg, +Several Additional Authors Jan 2018

Genotype-Phenotype Correlation In Nf1: Evidence For A More Severe Phenotype Associated With Missense Mutations Affecting Nf1 Codons 844-848, M Koczkowska, Y Chen, T Callens, A Gomes, A Sharp, Rhonda Schonberg, +Several Additional Authors

Pediatrics Faculty Publications

Neurofibromatosis type 1 (NF1), a common genetic disorder with a birth incidence of 1:2,000–3,000, is characterized by a highly variable clinical presentation. To date, only two clinically relevant intragenic genotype-phenotype correlations have been reported for NF1 missense mutations affecting p.Arg1809 and a single amino acid deletion p.Met922del. Both variants predispose to a distinct mild NF1 phenotype with neither externally visible cutaneous/plexiform neurofibromas nor other tumors. Here, we report 162 individuals (129 unrelated probands and 33 affected relatives) heterozygous for a constitutional missense mutation affecting one of five neighboring NF1 codons—Leu844, Cys845, Ala846, Leu847, and Gly848—located in the cysteine-serine-rich domain (CSRD). …