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Full-Text Articles in Medical Specialties

Directional Secretory Response Of Double Stranded Rna-Induced Thymic Stromal Lymphopoetin (Tslp) And Ccl11/Eotaxin-1 In Human Asthmatic Airways, Gustavo Nino, Shehlanoor Huseni, Geovanny F. Perez, Krishna Pancham, Humaira Mubeen, Aleeza Abeesi, Justin Wang, Stephen Eng, Anamaris M. Colberg-Poley, Mary C. Rose Dec 2014

Directional Secretory Response Of Double Stranded Rna-Induced Thymic Stromal Lymphopoetin (Tslp) And Ccl11/Eotaxin-1 In Human Asthmatic Airways, Gustavo Nino, Shehlanoor Huseni, Geovanny F. Perez, Krishna Pancham, Humaira Mubeen, Aleeza Abeesi, Justin Wang, Stephen Eng, Anamaris M. Colberg-Poley, Mary C. Rose

Pediatrics Faculty Publications

Background

Thymic stromal lymphoproetin (TSLP) is a cytokine secreted by the airway epithelium in response to respiratory viruses and it is known to promote allergic Th2 responses in asthma. This study investigated whether virally-induced secretion of TSLP is directional in nature (apical vs. basolateral) and/or if there are TSLP-mediated effects occurring at both sides of the bronchial epithelial barrier in the asthmatic state.

Methods

Primary human bronchial epithelial cells (HBEC) from control (n = 3) and asthmatic (n = 3) donors were differentiated into polarized respiratory tract epithelium under air-liquid interface (ALI) conditions and treated apically with dsRNA (viral surrogate) …


Integrative Therapy Use For Management Of Side Effects And Toxicities Experienced By Pediatric Oncology Patients, Shana Jacobs Dec 2014

Integrative Therapy Use For Management Of Side Effects And Toxicities Experienced By Pediatric Oncology Patients, Shana Jacobs

Pediatrics Faculty Publications

Integrative Therapies (IT), otherwise known as Complementary and Alternative Medicine, are widely used among pediatric oncology patients, despite a paucity of available evidence. This review summarizes surveys that describe the prevalence of IT use by pediatric oncology patients, both during therapy and in survivorship, as well as the modalities being used. Additionally, the evidence that exists for specific treatments that appear to be efficacious in controlling specific symptoms is described. Finally, there are recommendations for practitioners on how to best counsel patients about IT use.


Corticosteroids And Pediatric Septic Shock Outcomes: A Risk Stratified Analysis, Sarah J. Atkinson, Natalie Z. Cvijanovich, Neal J. Thomas, Geoffrey L. Allen, Nick Anas, Robert J. Freishtat, +17 Additional Authors Nov 2014

Corticosteroids And Pediatric Septic Shock Outcomes: A Risk Stratified Analysis, Sarah J. Atkinson, Natalie Z. Cvijanovich, Neal J. Thomas, Geoffrey L. Allen, Nick Anas, Robert J. Freishtat, +17 Additional Authors

Pediatrics Faculty Publications

Background

The potential benefits of corticosteroids for septic shock may depend on initial mortality risk.

Objective

We determined associations between corticosteroids and outcomes in children with septic shock who were stratified by initial mortality risk.

Methods

We conducted a retrospective analysis of an ongoing, multi-center pediatric septic shock clinical and biological database. Using a validated biomarker-based stratification tool (PERSEVERE), 496 subjects were stratified into three initial mortality risk strata (low, intermediate, and high). Subjects receiving corticosteroids during the initial 7 days of admission (n = 252) were compared to subjects who did not receive corticosteroids (n = 244). Logistic regression …


The Translational Biology Of Remyelination: Past, Present, And Future, Robin J.M. Franklin, Vittorio Gallo Nov 2014

The Translational Biology Of Remyelination: Past, Present, And Future, Robin J.M. Franklin, Vittorio Gallo

Pediatrics Faculty Publications

Amongst neurological diseases, multiple sclerosis (MS) presents an attractive target for regenerative medicine. This is because the primary pathology, the loss of myelin-forming oligodendrocytes, can be followed by a spontaneous and efficient regenerative process called remyelination. While cell transplantation approaches have been explored as a means of replacing lost oligodendrocytes, more recently therapeutic approaches that target the endogenous regenerative process have been favored. This is in large part due to our increasing understanding of (1) the cell types within the adult brain that are able to generate new oligodendrocytes, (2) the mechanisms and pathways by which this achieved, and (3) …


Vitamin A, Vitamin E, Iron And Zinc Status In A Cohort Of Hiv-Infected Mothers And Their Uninfected Infants, Jacqueline Pontes Monteiro, Maria Leticia Santos Cruz, Marisa Marcia Mussi-Pinhata, Roberta Garcia Salomao, Alceu Jordao Junior, Jennifer S. Read, Jose Henrique Da Silva Pilotto, Rachel Ann Cohen, Sonia Karolina Stoszek, George Kelly Siberry Nov 2014

Vitamin A, Vitamin E, Iron And Zinc Status In A Cohort Of Hiv-Infected Mothers And Their Uninfected Infants, Jacqueline Pontes Monteiro, Maria Leticia Santos Cruz, Marisa Marcia Mussi-Pinhata, Roberta Garcia Salomao, Alceu Jordao Junior, Jennifer S. Read, Jose Henrique Da Silva Pilotto, Rachel Ann Cohen, Sonia Karolina Stoszek, George Kelly Siberry

Pediatrics Faculty Publications

Introduction

We hypothesized that nutritional deficiency would be common in a cohort of postpartum, human immunodeficiency virus (HIV)-infected women and their infants.

Methods

Weight and height, as well as blood concentrations of retinol, α-tocopherol, ferritin, hemoglobin, and zinc, were measured in mothers after delivery and in their infants at birth and at 6-12 weeks and six months of age. Retinol and α-tocopherol levels were quantified by high performance liquid chromatography, and zinc levels were measured by atomic absorption spectrophotometry. The maternal body mass index during pregnancy was adjusted for gestational age (adjBMI).

Results

Among the 97 women 19.6% were underweight. …


Coagulopathy In Newborns With Hypoxic Ischemic Encephalopathy (Hie) Treated With Therapeutic Hypothermia: A Retrospective Case-Control Study, Katie R. Forman, Yaser A. Diab, Edward C.C. Wong, Stephen Baumgart, Naomi L. Luban, An Nguyen Massaro Nov 2014

Coagulopathy In Newborns With Hypoxic Ischemic Encephalopathy (Hie) Treated With Therapeutic Hypothermia: A Retrospective Case-Control Study, Katie R. Forman, Yaser A. Diab, Edward C.C. Wong, Stephen Baumgart, Naomi L. Luban, An Nguyen Massaro

Pediatrics Faculty Publications

Background

Newborns with hypoxic ischemic encephalopathy (HIE) are at risk for coagulopathy due to systemic oxygen deprivation. Additionally, therapeutic hypothermia (TH) slows enzymatic activity of the coagulation cascade, leading to constitutive prolongation of routinely assessed coagulation studies. The level of laboratory abnormality that predicts bleeding is unclear, leading to varying transfusion therapy practices.

Methods

HIE infants treated with TH between 2008–2012 were included in this retrospective study. Initial, minimum (min) and maximum (max) values of International Normalized Ratio (INR), activated partial thromboplastin time (aPTT), fibrinogen (Fib) and platelet (PLT) count (measured twice daily during TH) were collected. Bleeding was defined …


Neonatal Nmda Receptor Blockade Disrupts Spike Timing And Glutamatergic Synapses In Fast Spiking Interneurons In A Nmda Receptor Hypofunction Model Of Schizophrenia, Kevin S. Jones, Joshua G. Corbin, Molly M. Huntsman Oct 2014

Neonatal Nmda Receptor Blockade Disrupts Spike Timing And Glutamatergic Synapses In Fast Spiking Interneurons In A Nmda Receptor Hypofunction Model Of Schizophrenia, Kevin S. Jones, Joshua G. Corbin, Molly M. Huntsman

Pediatrics Faculty Publications

The dysfunction of parvalbumin-positive, fast-spiking interneurons (FSI) is considered a primary contributor to the pathophysiology of schizophrenia (SZ), but deficits in FSI physiology have not been explicitly characterized. We show for the first time, that a widely-employed model of schizophrenia minimizes first spike latency and increases GluN2B-mediated current in neocortical FSIs. The reduction in FSI first-spike latency coincides with reduced expression of the Kv1.1 potassium channel subunit which provides a biophysical explanation for the abnormal spiking behavior. Similarly, the increase in NMDA current coincides with enhanced expression of the GluN2B NMDA receptor subunit, specifically in FSIs. In this study mice …


Tps Mutational Status Is A Potential Marker For Risk Stratification In Wilms Tumour With Diffuse Anaplasia, Mariana Maschietto, Richard D. Williams, Tasnim Chagtai, Sergey D. Popov, Neil J. Sebire, Gordan Vujanic, Elizabeth Perlman, James R. Anderson, Paul Grundy, Jeffrey S. Dome, Kathy Pritchard-Jones Oct 2014

Tps Mutational Status Is A Potential Marker For Risk Stratification In Wilms Tumour With Diffuse Anaplasia, Mariana Maschietto, Richard D. Williams, Tasnim Chagtai, Sergey D. Popov, Neil J. Sebire, Gordan Vujanic, Elizabeth Perlman, James R. Anderson, Paul Grundy, Jeffrey S. Dome, Kathy Pritchard-Jones

Pediatrics Faculty Publications

Purpose

The presence of diffuse anaplasia in Wilms tumours (DAWT) is associated with TP53 mutations and poor outcome. As patients receive intensified treatment, we sought to identify whetherTP53 mutational status confers additional prognostic information.

Patients and Methods

We studied 40 patients with DAWT with anaplasia in the tissue from which DNA was extracted and analysed for TP53 mutations and 17p loss. The majority of cases were profiled by copy number (n = 32) and gene expression (n = 36) arrays. TP53 mutational status was correlated with patient event-free and overall survival, genomic copy number instability and gene expression profiling. …


A Delphi Process To Optimize Quality And Performance Of Drug Evaluation In Neonates, Frederic Legrand, Rym Boulkedid, Valery Elie, Stephanie Leroux, Elizabeth Valls, Adolfo Valls-I-Soler, John N. Van Den Anker Sep 2014

A Delphi Process To Optimize Quality And Performance Of Drug Evaluation In Neonates, Frederic Legrand, Rym Boulkedid, Valery Elie, Stephanie Leroux, Elizabeth Valls, Adolfo Valls-I-Soler, John N. Van Den Anker

Pediatrics Faculty Publications

Background

Neonatal trials remain difficult to conduct for several reasons: in particular the need for study sites to have an existing infrastructure in place, with trained investigators and validated quality procedures to ensure good clinical, laboratory practices and a respect for high ethical standards. The objective of this work was to identify the major criteria considered necessary for selecting neonatal intensive care units that are able to perform drug evaluations competently.

Methodology and Main Findings

This Delphi process was conducted with an international multidisciplinary panel of 25 experts from 13 countries, selected to be part of two committees (a scientific …


Lin28a Expression Reduces Sickling Of Cultured Human Erythrocytes, Jaira F. De Vasconcellos, Ross M. Fasano, Y. Terry Lee, Megha Kaushal, Colleen Burns, Emily Riehm Meier, Molly Anderson, Antoinette Rabel, Raul Braylan, David F. Stroncek, Jeffery L. Miller Sep 2014

Lin28a Expression Reduces Sickling Of Cultured Human Erythrocytes, Jaira F. De Vasconcellos, Ross M. Fasano, Y. Terry Lee, Megha Kaushal, Colleen Burns, Emily Riehm Meier, Molly Anderson, Antoinette Rabel, Raul Braylan, David F. Stroncek, Jeffery L. Miller

Pediatrics Faculty Publications

Induction of fetal hemoglobin (HbF) has therapeutic importance for patients with sickle cell disease (SCD) and the beta-thalassemias. It was recently reported that increased expression of LIN28 proteins or decreased expression of its target let-7 miRNAs enhances HbF levels in cultured primary human erythroblasts from adult healthy donors. Here LIN28A effects were studied further using erythrocytes cultured from peripheral blood progenitor cells of pediatric subjects with SCD. Transgenic expression of LIN28A was accomplished by lentiviral transduction in CD34(+) sickle cells cultivated ex vivo in serum-free medium. LIN28A over-expression (LIN28A-OE) increased HbF, reduced beta (sickle)-globin, and strongly suppressed …


Epidemiology Of Patients Presenting To The Emergency Centre Of Princess Marina Hospital In Gaborone, Botswana, Amit Chandra, Paul C. Mullan, Ari Ho-Foster, Antanoid Langeveldt, Ngaire Caruso, Joseph Motsumi, Andrew Kestler Sep 2014

Epidemiology Of Patients Presenting To The Emergency Centre Of Princess Marina Hospital In Gaborone, Botswana, Amit Chandra, Paul C. Mullan, Ari Ho-Foster, Antanoid Langeveldt, Ngaire Caruso, Joseph Motsumi, Andrew Kestler

Pediatrics Faculty Publications

No abstract provided.


Somatic Intronic Microsatellite Loci Differentiate Glioblastoma From Lower-Grade Gliomas, Enusha Karunasena, Lauren J. Mciver, Brian R. Rood, Xiaowei Wu, Hongxiao Zhu, Jasmin H. Bavarva, Harold R. Garner Aug 2014

Somatic Intronic Microsatellite Loci Differentiate Glioblastoma From Lower-Grade Gliomas, Enusha Karunasena, Lauren J. Mciver, Brian R. Rood, Xiaowei Wu, Hongxiao Zhu, Jasmin H. Bavarva, Harold R. Garner

Pediatrics Faculty Publications

Genomic studies of glioma sub-types have amassed new disease specific mutations, yet these only partially explain how mutations are linked to predisposition or progression. We hypothesized that microsatellite variation could expand the understanding of glioma etiology. Furthermore, germline markers for gliomas are typically undetectable; therefore we also hypothesize that the predictability of cancer-associated microsatellite loci in germline DNA may support the current hypothesis of a glioma cell of origin.

In this study, “normal” germline exome sequenced DNA from the 1000 Genomes Project (n=390) were compared with exome sequences from germlines of subjects with WHO grade II and III lower-grade glioma …


Sleep Disturbance, Depression And Pain In Adults With Sickle Cell Disease, Gwenyth R. Wallen, Caterina P. Minniti, Michael Krumlauf, Ellen Eckes, Darlene Allen, Anna Oguhebe, Cassie Seamon, Deepika Darbari Jul 2014

Sleep Disturbance, Depression And Pain In Adults With Sickle Cell Disease, Gwenyth R. Wallen, Caterina P. Minniti, Michael Krumlauf, Ellen Eckes, Darlene Allen, Anna Oguhebe, Cassie Seamon, Deepika Darbari

Pediatrics Faculty Publications

Background

Sleep disturbance and depression are commonly encountered in primary care. In sickle cell disease, depression is associated with pain, poor treatment compliance, and lower quality of life. The prevalence of sleep disturbance and its effect upon quality of life in adults with sickle cell disease is unknown. The goal of this study was to determine the prevalence of sleep disturbance and if it is associated with pain and depression in sickle cell disease.

Methods

Three hundred twenty eight adults with sickle cell disease enrolled on the Bethesda Sickle Cell Cohort Study were assessed using the Pittsburgh Sleep Quality Index …


Safety Of Celecoxib And Nonselective Nonsteroidal Anti-Inflammatory Drugs In Juvenile Idiopathic Arthritis: Results Of The Phase 4 Registry, Rachel E. Sobel, Daniel J. Lovell, Hermine I. Brunner, Jennifer E. Weiss, Paula W. Morris, Beth S. Gottlieb, Elizabeth C. Chalom, Lawrence K. Jung, Karen B. Onel, Lisa Petiniot, Donald P. Goldsmith Jul 2014

Safety Of Celecoxib And Nonselective Nonsteroidal Anti-Inflammatory Drugs In Juvenile Idiopathic Arthritis: Results Of The Phase 4 Registry, Rachel E. Sobel, Daniel J. Lovell, Hermine I. Brunner, Jennifer E. Weiss, Paula W. Morris, Beth S. Gottlieb, Elizabeth C. Chalom, Lawrence K. Jung, Karen B. Onel, Lisa Petiniot, Donald P. Goldsmith

Pediatrics Faculty Publications

Background

This study aimed to assess long-term safety and developmental data on juvenile idiopathic arthritis (JIA) patients treated in routine clinical practice with celecoxib or nonselective nonsteroidal anti-inflammatory drugs (nsNSAIDs).

Methods

Children aged ≥2 to <18 years with rheumatoid-factor–positive or –negative polyarthritis, persistent or extended oligoarthritis, or systemic arthritis were enrolled into this prospective, observational, multicenter standard-of-care registry. Eligible patients were newly or recently prescribed (≤6 months) an nsNSAID or celecoxib. Enrolled patients were followed to the end of the study, whether they remained on the original NSAID, switched, or discontinued therapy altogether. All adverse events (AEs) regardless of severity were captured in the database.

Results

A total of 274 patients (nsNSAID, n = 219; celecoxib, n = 55) were observed for 410 patient-years of observation. Naproxen, meloxicam, and nabumetone were the most frequently used nsNSAIDs. At baseline, the celecoxib group was older, had a numerically longer median time since diagnosis, and a numerically higher proportion of patients with a history of gastrointestinal-related NSAID intolerance. AEs reported were those frequently …


Controlling Cytomegalovirus: Helping The Immune System Take The Lead, Patrick J. Hanley, Catherine M. Bollard May 2014

Controlling Cytomegalovirus: Helping The Immune System Take The Lead, Patrick J. Hanley, Catherine M. Bollard

Pediatrics Faculty Publications

Cytomegalovirus, of the Herpesviridae family, has evolved alongside humans for thousands of years with an intricate balance of latency, immune evasion, and transmission. While upwards of 70% of humans have evidence of CMV infection, the majority of healthy people show little to no clinical symptoms of primary infection and CMV disease is rarely observed during persistent infection in immunocompetent hosts. Despite the fact that the majority of infected individuals are asymptomatic, immunologically, CMV hijacks the immune system by infecting and remaining latent in antigen-presenting cells that occasionally reactivate subclinically and present antigen to T cells, eventually causing the inflation of …


Response To Androgen Therapy In Patients With Dyskeratosis Congenita, Payal P. Khincha, Ingrid M. Wentzensen, Neelam Giri, Blanche P. Alter, Sharon A. Savage May 2014

Response To Androgen Therapy In Patients With Dyskeratosis Congenita, Payal P. Khincha, Ingrid M. Wentzensen, Neelam Giri, Blanche P. Alter, Sharon A. Savage

Pediatrics Faculty Publications

Dyskeratosis congenita (DC) is an inherited bone marrow failure syndrome and telomere biology disorder characterized by dysplastic nails, reticular skin pigmentation and oral leucoplakia. Androgens are a standard therapeutic option for bone marrow failure in those patients with DC who are unable to undergo haematopoietic stem cell transplantation, but there are no systematic data on its use in those patients. We evaluated haematological response and side effects of androgen therapy in 16 patients with DC in our observational cohort study. Untreated DC patients served as controls. Seventy percent of treated DC patients had a haematological response with red blood cell …


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 Mar 2014

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.


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 Mar 2014

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. …


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 Mar 2014

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 …


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 Feb 2014

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 …


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 Jan 2014

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 …


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 Jan 2014

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 Jan 2014

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 Jan 2014

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.


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 Jan 2014

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 …


High Levels Of Dek Autoantibodies May Predict Early Flare Following Cessation Of Anti-Tnf Therapy In Juvenile Idiopathic Arthritis, Nirit Mor-Vaknin, Miguel Rivas, Maureen Legendre, Y. Yuanfang, Anne Johnson, Lawrence K. Jung, +12 Additional Authors Jan 2014

High Levels Of Dek Autoantibodies May Predict Early Flare Following Cessation Of Anti-Tnf Therapy In Juvenile Idiopathic Arthritis, Nirit Mor-Vaknin, Miguel Rivas, Maureen Legendre, Y. Yuanfang, Anne Johnson, Lawrence K. Jung, +12 Additional Authors

Pediatrics Faculty Publications

Introduction The nuclear oncoprotein DEK is a biochemically distinct, pro-inflammatory protein that is a chemoattractant for neutrophils and T-cells. High levels of DEK autoantibodies have been found in several autoimmune diseases including juvenile idiopathic arthritis (JIA), but their role in disease pathogenesis is unclear.

Objectives Since DEK and DEK autoantibodies can contribute to the development of immune complexes and joint inflammation, we suggest that DEK antibody levels can predict disease flare with discontinuation of anti-TNF therapy. Methods In 16 pediatric rheumatology centers, sera samples were collected from 137 children with polyarticular JIA on anti-TNF therapy. Therapy was stopped after 6 …


A Pilot Academic-Community Partnership To Advance The D.C. Healthy Schools Act, Nisha Narayanan, Jonathan Tatum, Margarita Ramos, Jessica Kim, Nisha Punatar, Michele L. Mietus-Snyder Jan 2014

A Pilot Academic-Community Partnership To Advance The D.C. Healthy Schools Act, Nisha Narayanan, Jonathan Tatum, Margarita Ramos, Jessica Kim, Nisha Punatar, Michele L. Mietus-Snyder

GW Research Days 2014

No abstract provided.


Mucopolysaccharidosis Iva (Morquio A Syndrome) And Vi (Maroteaux-Lamy Syndrome): Under-Recognized And Challenging To Diagnose, Ralph S. Lachman, Barbara K. Burton, Lorne A. Clarke, Scott Hoffinger, Shiro Ikegawa, Dong-Kyu Jin, Hiroki Kano, Ok-Hwa Kim, Christina Lampe, Nancy J. Mendelsohn, Renee Shediac, Pranoot Tanpaiboon, Klane K. White Jan 2014

Mucopolysaccharidosis Iva (Morquio A Syndrome) And Vi (Maroteaux-Lamy Syndrome): Under-Recognized And Challenging To Diagnose, Ralph S. Lachman, Barbara K. Burton, Lorne A. Clarke, Scott Hoffinger, Shiro Ikegawa, Dong-Kyu Jin, Hiroki Kano, Ok-Hwa Kim, Christina Lampe, Nancy J. Mendelsohn, Renee Shediac, Pranoot Tanpaiboon, Klane K. White

Pediatrics Faculty Publications

OBJECTIVE:

Mucopolysaccharidosis IVA (MPS IVA, or Morquio A syndrome) and VI (MPS VI, or Maroteaux-Lamy syndrome) are autosomal recessive lysosomal storage disorders. Skeletal abnormalities are common initial presenting symptoms and, when recognized early, may facilitate timely diagnosis and intervention, leading to improved patient outcomes. Patients with slowly progressing disease and nonclassic phenotypes can be particularly challenging to diagnose. The objective was to describe the radiographic features of patients with a delayed diagnosis of MPS IVA or VI.

MATERIALS AND METHODS:

This was a retrospective study. The records of 5 MPS IVA and 3 MPS VI patients with delayed diagnosis were …


Gauging Regional Differences In The Hiv Prevalence Rate Among Injection Drug Users In The U.S., Jichuan Wang, Brian Kelly Jan 2014

Gauging Regional Differences In The Hiv Prevalence Rate Among Injection Drug Users In The U.S., Jichuan Wang, Brian Kelly

Pediatrics Faculty Publications

This article aims to introduce and demonstrate the application of the standardization and decomposition analysis (SDA) method to gauge differences in HIV prevalence rates among injection drug users (IDUs) across regions (Northeast, South, Midwest, and West) in the U.S. Using the SDA, the regional HIV prevalence rates were standardized and a rate difference between regions was decomposed into component effects, such as the “real” rate difference, and component effects attributed to differences in specific compositions of confounding factors. A total of 9,824 injection drug users (IDUs) retrieved from the national database of the National Institute on Drug Abuse’s Cooperative Agreement …


Superresolution Imaging Of Human Cytomegalovirus Vmia Localization In Sub-Mitochondrial Compartments, Shivaprasad Bhuvanendran, Kyle Salka, Kristen Rainey, Sen Chandra Sreetama, Elizabeth Williams, Margretha Leeker, Vidhya Prasad, Jonathan Boyd, George H. Patterson, Jyoti K. Jaiswal, Anamaris M. Colberg-Poley Jan 2014

Superresolution Imaging Of Human Cytomegalovirus Vmia Localization In Sub-Mitochondrial Compartments, Shivaprasad Bhuvanendran, Kyle Salka, Kristen Rainey, Sen Chandra Sreetama, Elizabeth Williams, Margretha Leeker, Vidhya Prasad, Jonathan Boyd, George H. Patterson, Jyoti K. Jaiswal, Anamaris M. Colberg-Poley

Pediatrics Faculty Publications

The human cytomegalovirus (HCMV) viral mitochondria-localized inhibitor of apoptosis (vMIA) protein, traffics to mitochondria-associated membranes (MAM), where the endoplasmic reticulum (ER) contacts the outer mitochondrial membrane (OMM). vMIA association with the MAM has not been visualized by imaging. Here, we have visualized this by using a combination of confocal and superresolution imaging. Deconvolution of confocal microscopy images shows vMIA localizes away from mitochondrial matrix at the Mitochondria-ER interface. By gated stimulated emission depletion (GSTED) imaging, we show that along this interface vMIA is distributed in clusters. Through multicolor, multifocal structured illumination microscopy (MSIM), we find vMIA clusters localize away from …