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Full-Text Articles in Pediatrics

Advisory Committee On Immunization Practices Recommended Immunization Schedule For Children And Adolescents Aged 18 Years Or Younger - United States, 2019, C. L. Robinson, H. Bernstein, J. R. Romero, P. Szilagyi Jan 2019

Advisory Committee On Immunization Practices Recommended Immunization Schedule For Children And Adolescents Aged 18 Years Or Younger - United States, 2019, C. L. Robinson, H. Bernstein, J. R. Romero, P. Szilagyi

Journal Articles

No abstract provided.


Somatic Mutations And Clonal Hematopoiesis In Congenital Neutropenia, J. Xia, C. A. Miller, J. Baty, A. Ramesh, M. R. Jotte, R. S. Fulton, T. P. Vogel, M. A. Cooper, A. Vlachos, D. C. Link, +11 Additional Authors Jan 2018

Somatic Mutations And Clonal Hematopoiesis In Congenital Neutropenia, J. Xia, C. A. Miller, J. Baty, A. Ramesh, M. R. Jotte, R. S. Fulton, T. P. Vogel, M. A. Cooper, A. Vlachos, D. C. Link, +11 Additional Authors

Journal Articles

© 2018 by The American Society of Hematology. Severe congenital neutropenia (SCN) and Shwachman-Diamond syndrome (SDS) are congenital neutropenia syndromes with a high rate of leukemic transformation. Hematopoietic stressors may contribute to leukemic transformation by increasing the mutation rate in hematopoietic stem/progenitor cells (HSPCs) and/or by promoting clonal hematopoiesis. We sequenced the exome of individual hematopoietic colonies derived from 13 patients with congenital neutropenia to measure total mutation burden and performed error-corrected sequencing on a panel of 46 genes on 80 patients with congenital neutropenia to assess for clonal hematopoiesis. An average of 3.6 ± 1.2 somatic mutations per exome …


High Levels Of Dek Autoantibodies In Sera Of Patients With Polyarticular Juvenile Idiopathic Arthritis And With Early Disease Flares Following Cessation Of Anti–Tumor Necrosis Factor Therapy, N. Mor-Vaknin, M. Rivas, M. Legendre, S. Mohan, Y. Yuanfan, T. Mau, A. Johnson, B. Huang, B. S. Gottlieb, D. M. Markovitz, +17 Additional Authors Jan 2018

High Levels Of Dek Autoantibodies In Sera Of Patients With Polyarticular Juvenile Idiopathic Arthritis And With Early Disease Flares Following Cessation Of Anti–Tumor Necrosis Factor Therapy, N. Mor-Vaknin, M. Rivas, M. Legendre, S. Mohan, Y. Yuanfan, T. Mau, A. Johnson, B. Huang, B. S. Gottlieb, D. M. Markovitz, +17 Additional Authors

Journal Articles

© 2017 The Authors. Arthritis & Rheumatology published by Wiley Periodicals, Inc. on behalf of American College of Rheumatology. Objective: The nuclear oncoprotein DEK is an autoantigen associated with juvenile idiopathic arthritis (JIA), especially the oligoarticular subtype. DEK is a secreted chemotactic factor. Abundant levels of DEK and DEK autoantibodies are found in inflamed synovium in JIA. We undertook this study to further characterize the nature of DEK autoantibodies in screening serum samples from 2 different cohorts that consisted mostly of patients with JIA. Methods: DEK autoantibody levels were analyzed in sera from 33 JIA patients, 13 patients with other …


Long Ncrna Landscape In The Ileum Of Treatment-Naive Early-Onset Crohn Disease., Y. Haberman, M. Benshoshan, A. Di Segni, P. J. Dexheimer, T. Braun, B. Weiss, T. D. Walters, R. N. Baldassano, J. Markowitz, L. A. Denson, +27 Additional Authors Jan 2018

Long Ncrna Landscape In The Ileum Of Treatment-Naive Early-Onset Crohn Disease., Y. Haberman, M. Benshoshan, A. Di Segni, P. J. Dexheimer, T. Braun, B. Weiss, T. D. Walters, R. N. Baldassano, J. Markowitz, L. A. Denson, +27 Additional Authors

Journal Articles

Long noncoding RNAs (lncRNA) are key regulators of gene transcription and many show tissue-specific expression. We previously defined a novel inflammatory and metabolic ileal gene signature in treatment-naive pediatric Crohn disease (CD). We now extend our analyses to include potential regulatory lncRNA.Using RNAseq, we systematically profiled lncRNAs and protein-coding gene expression in 177 ileal biopsies. Co-expression analysis was used to identify functions and tissue-specific expression. RNA in situ hybridization was used to validate expression. Real-time polymerase chain reaction was used to test lncRNA regulation by IL-1β in Caco-2 enterocytes.We characterize widespread dysregulation of 459 lncRNAs in the ileum of CD …


Association Of Early Postresuscitation Hypotension With Survival To Discharge After Targeted Temperature Management For Pediatric Out-Of-Hospital Cardiac Arrest Secondary Analysis Of A Randomized Clinical Trial, A. A. Topjian, F. W. Moler, R. Telford, R. Holubkov, V. M. Nadkarni, R. A. Berg, J. M. Dean, K. L. Meert, C. L. Schleien, B. Browning, +34 Additional Authors Jan 2018

Association Of Early Postresuscitation Hypotension With Survival To Discharge After Targeted Temperature Management For Pediatric Out-Of-Hospital Cardiac Arrest Secondary Analysis Of A Randomized Clinical Trial, A. A. Topjian, F. W. Moler, R. Telford, R. Holubkov, V. M. Nadkarni, R. A. Berg, J. M. Dean, K. L. Meert, C. L. Schleien, B. Browning, +34 Additional Authors

Journal Articles

© 2017 American Medical Association. All rights reserved. IMPORTANCE Out-of-hospital cardiac arrest (OHCA) occurs in more than 6000 children each year in the United States, with survival rates of less than 10% and severe neurologic morbidity in many survivors. Post-cardiac arrest hypotension can occur, but its frequency and association with survival have not been well described duringtargeted temperature management. OBJECTIVE To determine whether hypotension is associated with survival to discharge in children and adolescents after resuscitation from OHCA. DESIGN, SETTING, AND PARTICIPANTS This post hoc secondary analysis of the Therapeutic Hypothermia After Pediatric Cardiac Arrest (THAPCA) trial included 292 pediatric …


The New Mobile Universal Lexicon Evaluation System (Mules): A Test Of Rapid Picture Naming For Concussion Sized For The Sidelines, O. Akhand, M. S. Galetta, L. Cobbs, L. Hasanaj, N. Webb, J. Drattell, P. Amorapanth, J. R. Rizzo, A. Silverio, L. J. Balcer, +6 Additional Authors Jan 2018

The New Mobile Universal Lexicon Evaluation System (Mules): A Test Of Rapid Picture Naming For Concussion Sized For The Sidelines, O. Akhand, M. S. Galetta, L. Cobbs, L. Hasanaj, N. Webb, J. Drattell, P. Amorapanth, J. R. Rizzo, A. Silverio, L. J. Balcer, +6 Additional Authors

Journal Articles

© 2018 Objective: Measures of rapid automatized naming (RAN) have been used for over 50 years to capture vision-based aspects of cognition. The Mobile Universal Lexicon Evaluation System (MULES) is a test of rapid picture naming under investigation for detection of concussion and other neurological disorders. MULES was designed as a series of 54 grouped color photographs (fruits, random objects, animals) that integrates saccades, color perception and contextual object identification. Recent changes to the MULES test have been made to improve ease of use on the athletic sidelines. Originally an 11 × 17-inch single-sided paper, the test has been reduced …


Effects Of Soybean Lipid Infusion On Triglyceride And Unbound Free Fatty Acid Levels In Preterm Infants, T. Hegyi, A. Kleinfeld, A. Huber, B. Weinberger, N. Memon, W. Joe Shih, M. Carayannopoulos, W. Oh Jan 2018

Effects Of Soybean Lipid Infusion On Triglyceride And Unbound Free Fatty Acid Levels In Preterm Infants, T. Hegyi, A. Kleinfeld, A. Huber, B. Weinberger, N. Memon, W. Joe Shih, M. Carayannopoulos, W. Oh

Journal Articles

© 2018 Informa UK Limited, trading as Taylor & Francis Group Objective: To determine the plasma triglyceride (TG) and unbound free fatty acid (FFAu) levels in infants treated with increasing dosages of soybean lipid, intralipid (IL), infusion. Study design: TG and FFAu levels were measured in 78 preterm infants (BW 500–2000 g; GA 23–34 weeks) using the fluorescent probe ADIFAB2 and enzymatic method. Results: The infants’ BW was 1266.2 ± 440.7 g and GA 28.8 ± 3.1 weeks. TG levels were 77.4 ± 50 mg/dL, 140.2 ± 188 mg/dL (p < .04 compared to levels during low dose IL infusion) and 135.6 ± 118 mg/dL (p < .004), respectively during increased IL rates. FFAu levels were 17.7 ± 13 nM, 47.3 ± 102.8 nM (p = .07) and 98 ± 234 nM (p = .03). TG levels correlated with IL dose, the rate of IL administration, and FFAu levels. TG and FFAu levels were higher in infants below 28 weeks’ gestation Conclusions: Increasing dosage of IL is associated with increasing levels of TG and FFAu, especially in infants below 29 weeks of gestation. The increased level of FFAu suggests inefficient cellular utilization.


New-Onset Isolated Asymptomatic Papilledema In Two Patients Treated With Recombinant Growth Hormone, L. A. Kanner, J. Klein, M. Gaffar, H. Pomeranz, G. Frank Jan 2018

New-Onset Isolated Asymptomatic Papilledema In Two Patients Treated With Recombinant Growth Hormone, L. A. Kanner, J. Klein, M. Gaffar, H. Pomeranz, G. Frank

Journal Articles

No abstract provided.


Interindividual Regulation Of The Breast Cancer Resistance Protein/Abcg2 Transporter In Term Human Placentas, K. M. Bircsak, J. E. Moscovitz, X. Wen, F. Archer, P. Y. Yuen, M. Mohammed, N. Memon, B. I. Weinberger, L. M. Saba, L. M. Aleksunes, +1 Additional Author Jan 2018

Interindividual Regulation Of The Breast Cancer Resistance Protein/Abcg2 Transporter In Term Human Placentas, K. M. Bircsak, J. E. Moscovitz, X. Wen, F. Archer, P. Y. Yuen, M. Mohammed, N. Memon, B. I. Weinberger, L. M. Saba, L. M. Aleksunes, +1 Additional Author

Journal Articles

© 2018 by The American Society for Pharmacology and Experimental Therapeutics The breast cancer resistance protein (BCRP/ABCG2) is a maternally-facing efflux transporter that regulates the placental disposition of chemicals. Transcription factors and gene variants are important regulatory factors that influence transporter expression. In this study, we sought to identify the genetic and transcriptional mechanisms underlying the interindividual expression of BCRP mRNA and protein across 137 term placentas from uncomplicated pregnancies. Placental expression of BCRP and regulatory transcription factor mRNAs was measured using multiplex-branched DNA analysis. BCRP expression and ABCG2 genotypes were determined using Western blot and Fluidigm Biomark genetic analysis, …


Serologic Reactivity Reflects Clinical Expression Of Ulcerative Colitis In Children, E. A. Spencer, S. M. Davis, D. R. Mack, B. M. Boyle, A. M. Griffiths, N. S. Leleiko, C. G. Sauer, D. J. Keljo, J. F. Markowitz, M. C. Dubinsky, +26 Additional Authors Jan 2018

Serologic Reactivity Reflects Clinical Expression Of Ulcerative Colitis In Children, E. A. Spencer, S. M. Davis, D. R. Mack, B. M. Boyle, A. M. Griffiths, N. S. Leleiko, C. G. Sauer, D. J. Keljo, J. F. Markowitz, M. C. Dubinsky, +26 Additional Authors

Journal Articles

© 2018 Crohn's & Colitis Foundation. Published by Oxford University Press. All rights reserved. Background In contrast to pediatric Crohn's disease (CD), little is known in pediatric ulcerative colitis (UC) about the relationship between disease phenotype and serologic reactivity to microbial and other antigens. Aim The aim of this study was to examine disease phenotype and serology in a well-characterized inception cohort of children newly diagnosed with UC during the PROTECT Study (Predicting Response to Standardized Pediatric Colitis Therapy). Methods Patients were recruited from 29 participating centers. Demographic, clinical, laboratory, and serologic (pANCA, ASCA IgA/IgG, Anti-CBir1, and Anti-OmpC) data were …


Vaginal Discharge In A Pre-Pubertal Girl Posing A Diagnostic Challenge., S. Ganguli, Q. Liu, A. Tsoumpariotis, S. Rapaport, E. Fakioglu Jan 2018

Vaginal Discharge In A Pre-Pubertal Girl Posing A Diagnostic Challenge., S. Ganguli, Q. Liu, A. Tsoumpariotis, S. Rapaport, E. Fakioglu

Journal Articles

Vaginal discharge in prepubescent girls is not an uncommon problem in pediatric outpatient practice. Among its various etiologies, foreign body lodgement is quite frequent in this age group. Diagnosis is sometimes forthcoming after history and physical exam, and the removal of the foreign object is followed by a prompt resolution of symptoms. However, in rare circumstances, an intravaginal foreign body may mimic other pathologies, including infections and neoplasms, as well as raising suspicion for child abuse. In such cases, diagnosis may remain unclear even after laboratory tests and imaging studies. We describe a seven-year-old girl with vaginal discharge, who needed …


Exome Chip Analyses Identify Genes Affecting Mortality After Hla-Matched Unrelated-Donor Blood And Marrow Transplantation, Q. Zhu, L. Yan, Q. Liu, C. Zhang, L. Wei, Q. Hu, L. Preus, A. I. Clay-Gilmour, K. Onel, L. E. Sucheston-Campbell, +10 Additional Authors Jan 2018

Exome Chip Analyses Identify Genes Affecting Mortality After Hla-Matched Unrelated-Donor Blood And Marrow Transplantation, Q. Zhu, L. Yan, Q. Liu, C. Zhang, L. Wei, Q. Hu, L. Preus, A. I. Clay-Gilmour, K. Onel, L. E. Sucheston-Campbell, +10 Additional Authors

Journal Articles

© 2018 by The American Society of Hematology Although survival outcomes have significantly improved, up to 40% of patients die within 1 year of HLA-matched unrelated-donor blood and marrow transplantation (BMT). To identify non-HLA genetic contributors to mortality after BMT, we performed the first exome-wide association study in the DISCOVeRY-BMT cohorts using the Illumina HumanExome BeadChip. This study includes 2473 patients with acute myeloid leukemia, acute lymphoblastic leukemia, or myelodysplastic syndrome and 2221 10/10 HLA-matched donors treated from 2000 to 2011. Single-variant and gene-level analyses were performed on overall survival (OS), transplantation-related mortality (TRM), and disease-related mortality (DRM). Genotype mismatches …


A 6-Month, Multicenter, Open-Label Study Of Fixed Dose Naproxen/Esomeprazole In Adolescent Patients With Juvenile Idiopathic Arthritis, D. J. Lovell, J. A. Dare, M. Francis-Sedlak, J. Ball, B. D. Lamoreaux, E. Von Scheven, A. Reinhardt, R. Jerath, B. Gottlieb, R. J. Holt, +6 Additional Authors Jan 2018

A 6-Month, Multicenter, Open-Label Study Of Fixed Dose Naproxen/Esomeprazole In Adolescent Patients With Juvenile Idiopathic Arthritis, D. J. Lovell, J. A. Dare, M. Francis-Sedlak, J. Ball, B. D. Lamoreaux, E. Von Scheven, A. Reinhardt, R. Jerath, B. Gottlieb, R. J. Holt, +6 Additional Authors

Journal Articles

© 2018 The Author(s). Background: Juvenile idiopathic arthritis (JIA) is an inflammatory arthritis of unknown etiology, which lasts for greater than 6 weeks with onset before 16 years of age. JIA is the most common chronic rheumatic disease in children. NSAIDs have been the mainstay of initial management with naproxen (NAP) being commonly used, but they may cause serious side effects such as gastric ulcers which can be reduced by concomitant administration of proton pump inhibitors, such as esomeprazole (ESO). Methods: Primary objective was to evaluate the safety and tolerability of 3 fixed doses of NAP/ESO in JIA patients aged …


Design And Preliminary Validation Of A Mobile Application-Based Expert System To Facilitate Repair Of Medical Equipment In Resource-Limited Health Settings, A. L. Wong, K. M. Lacob, M. G. Wilson, S. M. Zwolski, S. Acharya Jan 2018

Design And Preliminary Validation Of A Mobile Application-Based Expert System To Facilitate Repair Of Medical Equipment In Resource-Limited Health Settings, A. L. Wong, K. M. Lacob, M. G. Wilson, S. M. Zwolski, S. Acharya

Journal Articles

© 2018 Wong et al. Background: One of the greatest barriers to safe surgery is the availability of functional biomedical equipment. Biomedical technicians play a major role in ensuring that equipment is functional. Following in-field observations and an online survey, a mobile application was developed to aid technicians in troubleshooting biomedical equipment. It was hypothesized that this application could be used to aid technicians in equipment repair, as modeled by repair of a pulse oximeter. Methods: To identify specific barriers to equipment repair and maintenance for biomedical technicians, an online survey was conducted to determine current practices and challenges. These …


Free And Bioavailable 25-Hydroxyvitamin D Concentrations Are Associated With Disease Activity In Pediatric Patients With Newly Diagnosed Treatment Naive Ulcerative Colitis, C. G. Sauer, M. S. Loop, S. Venkateswaran, V. Tangpricha, T. R. Ziegler, A. Dhawan, C. Mccall, E. Bonkowski, J. Markowitz, L. A. Denson, +16 Additional Authors Jan 2018

Free And Bioavailable 25-Hydroxyvitamin D Concentrations Are Associated With Disease Activity In Pediatric Patients With Newly Diagnosed Treatment Naive Ulcerative Colitis, C. G. Sauer, M. S. Loop, S. Venkateswaran, V. Tangpricha, T. R. Ziegler, A. Dhawan, C. Mccall, E. Bonkowski, J. Markowitz, L. A. Denson, +16 Additional Authors

Journal Articles

© 2018 Crohn's & Colitis Foundation. Published by Oxford University Press. All rights reserved. Background Vitamin D regulates intestinal epithelial and immune functions, and vitamin D receptor deficiency increases the severity of murine colitis. Bioavailable 25-hydroxyvitamin D (25(OH)D) is available to target tissues and may be a driver of immune function. The aim is to evaluate the relationship of bioavailable 25(OH)D to the clinical expression of treatment naive pediatric ulcerative colitis (UC). Methods The PROTECT (Predicting Response to Standardized Pediatric Colitis Therapy) study enrolled children ≤17 years newly diagnosed with UC. Free and total 25(OH)D were directly measured and 25(OH)D …


Risk, Timing, And Predictors Of Disease Flare After Discontinuation Of Anti–Tumor Necrosis Factor Therapy In Children With Polyarticular Forms Of Juvenile Idiopathic Arthritis With Clinically Inactive Disease, D. J. Lovell, A. L. Johnson, B. Huang, B. S. Gottlieb, P. W. Morris, Y. Kimura, K. Onel, S. C. Li, B. A. Eberhard, E. H. Giannini, +34 Additional Authors Jan 2018

Risk, Timing, And Predictors Of Disease Flare After Discontinuation Of Anti–Tumor Necrosis Factor Therapy In Children With Polyarticular Forms Of Juvenile Idiopathic Arthritis With Clinically Inactive Disease, D. J. Lovell, A. L. Johnson, B. Huang, B. S. Gottlieb, P. W. Morris, Y. Kimura, K. Onel, S. C. Li, B. A. Eberhard, E. H. Giannini, +34 Additional Authors

Journal Articles

© 2018, American College of Rheumatology Objective: To determine the frequency, time to flare, and predictors of disease flare upon withdrawal of anti–tumor necrosis factor (anti-TNF) therapy in children with polyarticular forms of juvenile idiopathic arthritis (JIA) who demonstrated ≥6 months of continuous clinically inactive disease. Methods: In 16 centers 137 patients with clinically inactive JIA who were receiving anti-TNF therapy (42% of whom were also receiving methotrexate [MTX]) were prospectively followed up. If the disease remained clinically inactive for the initial 6 months of the study, anti-TNF was stopped and patients were assessed for flare at 1, 2, 3, …


Blood-Induced Bone Loss In Murine Hemophilic Arthropathy Is Prevented By Blocking The Irhom2/Adam17/Tnf-A Pathway, C. Haxaire, N. Hakobyan, T. Pannellini, C. Carballo, D. Mcilwain, T. W. Mak, S. Rodeo, S. Acharya, D. Li, C. P. Blobel, +5 Additional Authors Jan 2018

Blood-Induced Bone Loss In Murine Hemophilic Arthropathy Is Prevented By Blocking The Irhom2/Adam17/Tnf-A Pathway, C. Haxaire, N. Hakobyan, T. Pannellini, C. Carballo, D. Mcilwain, T. W. Mak, S. Rodeo, S. Acharya, D. Li, C. P. Blobel, +5 Additional Authors

Journal Articles

© 2018 by The American Society of Hematology. Hemophilic arthropathy (HA) is a debilitating degenerative joint disease that is a major manifestation of the bleeding disorder hemophilia A. HA typically begins with hemophilic synovitis that resembles inflammatory arthritides, such as rheumatoid arthritis, and frequently results in bone loss in patients. A major cause of rheumatoid arthritis is inappropriate release of the proinflammatory cytokine tumor necrosis factor-a (TNF-a) by the TNF-a convertase (TACE; also referred to as ADAM17) and its regulator, iRhom2. Therefore, we hypothesized that iRhom2/ADAM17-dependent shedding of TNF-a also has a pivotal role in mediating HA. Here, we show …


Macrophage Migration Inhibitory Factor (Mif) In The Development And Progression Of Pulmonary Arterial Hypertension., M. Ahmed, E. Miller Jan 2018

Macrophage Migration Inhibitory Factor (Mif) In The Development And Progression Of Pulmonary Arterial Hypertension., M. Ahmed, E. Miller

Journal Articles

Macrophage migration inhibitory factor (MIF) has been described as a pro-inflammatory cytokine and regulator of neuro-endocrine function. It plays an important upstream role in the inflammatory cascade by promoting the release of other inflammatory cytokines such as TNF-alpha and IL-6, ultimately triggering a chronic inflammatory immune response. As lungs can synthesize and release MIF, many studies have investigated the potential role of MIF as a biomarker in assessment of patients with pulmonary arterial hypertension (PAH) and using anti-MIFs as a new therapeutic modality for PAH.


Microbiota-Sensitive Epigenetic Signature Predicts Inflammation In Crohn's Disease., D. Kelly, M. Kotliar, V. Woo, S. Jagannathan, J. Whitt, J. Moncivaiz, B. J. Aronow, M. C. Dubinsky, J. F. Markowitz, T. Alenghat, +12 Additional Authors Jan 2018

Microbiota-Sensitive Epigenetic Signature Predicts Inflammation In Crohn's Disease., D. Kelly, M. Kotliar, V. Woo, S. Jagannathan, J. Whitt, J. Moncivaiz, B. J. Aronow, M. C. Dubinsky, J. F. Markowitz, T. Alenghat, +12 Additional Authors

Journal Articles

Altered response to the intestinal microbiota strongly associates with inflammatory bowel disease (IBD); however, how commensal microbial cues are integrated by the host during the pathogenesis of IBD is not understood. Epigenetics represents a potential mechanism that could enable intestinal microbes to modulate transcriptional output during the development of IBD. Here, we reveal a histone methylation signature of intestinal epithelial cells isolated from the terminal ilea of newly diagnosed pediatric IBD patients. Genes characterized by significant alterations in histone H3-lysine 4 trimethylation (H3K4me3) showed differential enrichment in pathways involving immunoregulation, cell survival and signaling, and metabolism. Interestingly, a large subset …


Expanding The Clinical And Molecular Findings In Rasa1 Capillary Malformation-Arteriovenous Malformation, W. L. Wooderchak-Donahue, P. Johnson, J. Mcdonald, F. Blei, A. Berenstein, M. Sorscher, J. Mayer, A. E. Scheuerle, T. Lewis, P. Bayrak-Toydemir, +5 Additional Authors Jan 2018

Expanding The Clinical And Molecular Findings In Rasa1 Capillary Malformation-Arteriovenous Malformation, W. L. Wooderchak-Donahue, P. Johnson, J. Mcdonald, F. Blei, A. Berenstein, M. Sorscher, J. Mayer, A. E. Scheuerle, T. Lewis, P. Bayrak-Toydemir, +5 Additional Authors

Journal Articles

No abstract provided.


Correction To: Minimum Quality Threshold In Pre-Clinical Sepsis Studies (Mqtipss): An International Expert Consensus Initiative For Improvement Of Animal Modeling In Sepsis (Infection, (2018), 10.1007/S15010-018-1183-8), M. F. Osuchowski, A. Ayala, S. Bahrami, M. Bauer, M. Boros, J. M. Cavaillon, I. H. Chaudry, C. Deutschman, P. Wang, B. Zingarelli, +21 Additional Authors Jan 2018

Correction To: Minimum Quality Threshold In Pre-Clinical Sepsis Studies (Mqtipss): An International Expert Consensus Initiative For Improvement Of Animal Modeling In Sepsis (Infection, (2018), 10.1007/S15010-018-1183-8), M. F. Osuchowski, A. Ayala, S. Bahrami, M. Bauer, M. Boros, J. M. Cavaillon, I. H. Chaudry, C. Deutschman, P. Wang, B. Zingarelli, +21 Additional Authors

Journal Articles

© 2018, The Author(s). The original version of this article unfortunately contained mistakes.


Translational Research: The Model Matters, C. S. Deutschman Jan 2018

Translational Research: The Model Matters, C. S. Deutschman

Journal Articles

No abstract provided.


An Eqtl Landscape Of Kidney Tissue In Human Nephrotic Syndrome, C. E. Gillies, R. Putler, R. Menon, E. Otto, K. Yasutake, V. Nair, P. Hoover, D. Lieb, C. Sethna, K. Abbott, +89 Additional Authors Jan 2018

An Eqtl Landscape Of Kidney Tissue In Human Nephrotic Syndrome, C. E. Gillies, R. Putler, R. Menon, E. Otto, K. Yasutake, V. Nair, P. Hoover, D. Lieb, C. Sethna, K. Abbott, +89 Additional Authors

Journal Articles

© 2018 American Society of Human Genetics Expression quantitative trait loci (eQTL) studies illuminate the genetics of gene expression and, in disease research, can be particularly illuminating when using the tissues directly impacted by the condition. In nephrology, there is a paucity of eQTL studies of human kidney. Here, we used whole-genome sequencing (WGS) and microdissected glomerular (GLOM) and tubulointerstitial (TI) transcriptomes from 187 individuals with nephrotic syndrome (NS) to describe the eQTL landscape in these functionally distinct kidney structures. Using MatrixEQTL, we performed cis-eQTL analysis on GLOM (n = 136) and TI (n = 166). We used the Bayesian …


Pilot Study Comparing The Childhood Arthritis And Rheumatology Research Alliance Consensus Treatment Plans For Induction Therapy Of Juvenile Proliferative Lupus Nephritis, J. C. Cooper, K. Rouster-Stevens, T. B. Wright, J. J. Hsu, M. S. Klein-Gitelman, S. P. Ardoin, L. E. Schanberg, H. I. Brunner, B. A. Eberhard, E. Von Scheven, +6 Additional Authors Jan 2018

Pilot Study Comparing The Childhood Arthritis And Rheumatology Research Alliance Consensus Treatment Plans For Induction Therapy Of Juvenile Proliferative Lupus Nephritis, J. C. Cooper, K. Rouster-Stevens, T. B. Wright, J. J. Hsu, M. S. Klein-Gitelman, S. P. Ardoin, L. E. Schanberg, H. I. Brunner, B. A. Eberhard, E. Von Scheven, +6 Additional Authors

Journal Articles

BACKGROUND: To reduce treatment variability and facilitate comparative effectiveness studies, the Childhood Arthritis and Rheumatology Research Alliance (CARRA) published consensus treatment plans (CTPs) including one for juvenile proliferative lupus nephritis (LN). Induction immunosuppression CTPs outline treatment with either monthly intravenous (IV) cyclophosphamide (CYC) or mycophenolate mofetil (MMF) in conjunction with one of three corticosteroid (steroid) CTPs: primarily oral, primarily IV or mixed oral/IV. The acceptability and in-practice use of these CTPs are unknown. Therefore, the primary aims of the pilot study were to demonstrate feasibility of adhering to the LN CTPs and delineate barriers to implementation in clinical care in …


Age Restriction Warning Label Efficacy And High School Student Consumption Of Highly-Caffeinated Products., J. Goldman, M. Zhu, T. B. Pham, R. Milanaik Jan 2018

Age Restriction Warning Label Efficacy And High School Student Consumption Of Highly-Caffeinated Products., J. Goldman, M. Zhu, T. B. Pham, R. Milanaik

Journal Articles

Age restriction warning labels (ARLs) are placed on highly-caffeinated food and drinks, such as Red Bull, to deter consumption by minors who are especially vulnerable to the risks of excessive caffeine consumption. Previous studies have shown that ARLs on media like TV programs and video games fail to discourage minors. However, it is unclear how ARLs on food and drink packages affect minors' purchasing behavior. High school students aged 14 to 17 years (n = 150, M


Realizing Effectiveness Across Continents With Hydroxyurea: Enrollment And Baseline Characteristics Of The Multicenter Reach Study In Sub-Saharan Africa., P. T. Mcgann, T. N. Williams, P. Olupot-Olupot, G. A. Tomlinson, A. Lane, J. Luís Reis Da Fonseca, R. Kitenge, G. Mochamah, B. Aygun, R. E. Ware, +7 Additional Authors Jan 2018

Realizing Effectiveness Across Continents With Hydroxyurea: Enrollment And Baseline Characteristics Of The Multicenter Reach Study In Sub-Saharan Africa., P. T. Mcgann, T. N. Williams, P. Olupot-Olupot, G. A. Tomlinson, A. Lane, J. Luís Reis Da Fonseca, R. Kitenge, G. Mochamah, B. Aygun, R. E. Ware, +7 Additional Authors

Journal Articles

Despite its well-described safety and efficacy in the treatment of sickle cell anemia (SCA) in high-income settings, hydroxyurea remains largely unavailable in sub-Saharan Africa, where more than 75% of annual SCA births occur and many comorbidities exist. Realizing Effectiveness Across Continents with Hydroxyurea (REACH, ClinicalTrials.gov NCT01966731) is a prospective, Phase I/II open-label trial of hydroxyurea designed to evaluate the feasibility, safety, and benefits of hydroxyurea treatment for children with SCA in four sub-Saharan African countries. Following comprehensive training of local research teams, REACH was approved by local Ethics Committees and achieved full enrollment ahead of projections with 635 participants enrolled …


Assessment Of A Renal Angina Index For Prediction Of Severe Acute Kidney Injury In Critically Ill Children: A Multicentre, Multinational, Prospective Observational Study., R. K. Basu, A. Kaddourah, S. L. Goldstein, A. Akcan-Arikan, M. Arnold, C. Cruz, J. Schneider, T. Sweberg, A. Kessel, J. Zaritsky, +73 Additional Authors Jan 2018

Assessment Of A Renal Angina Index For Prediction Of Severe Acute Kidney Injury In Critically Ill Children: A Multicentre, Multinational, Prospective Observational Study., R. K. Basu, A. Kaddourah, S. L. Goldstein, A. Akcan-Arikan, M. Arnold, C. Cruz, J. Schneider, T. Sweberg, A. Kessel, J. Zaritsky, +73 Additional Authors

Journal Articles

Background: Acute kidney injury (AKI) occurs in one in four children admitted to the intensive care unit (ICU) and AKI severity is independently associated with increased patient morbidity and mortality. Early prediction of AKI has the potential to improve outcomes. In smaller, single center populations, we have previously derived and validated the performance of the renal angina index (RAI), a context driven risk stratification system, to predict severe AKI.

Methods: A prospective, observational study (AWARE

Findings: The 1590 patients studied were 55% male and had median age of 54.5 months. 286 patients (17.9%) were RA+. AKI

Interpretation: Earlier, better prediction …


Congenital Adrenal Hyperplasia Due To Steroid 21-Hydroxylase Deficiency: An Endocrine Society Clinical Practice Guideline, P. W. Speiser, W. Arlt, R. J. Auchus, L. S. Baskin, G. S. Conway, D. P. Merke, H. F. Meyer-Bahlburg, W. L. Miller, M. H. Murad, S. E. Oberfield, P. C. White Jan 2018

Congenital Adrenal Hyperplasia Due To Steroid 21-Hydroxylase Deficiency: An Endocrine Society Clinical Practice Guideline, P. W. Speiser, W. Arlt, R. J. Auchus, L. S. Baskin, G. S. Conway, D. P. Merke, H. F. Meyer-Bahlburg, W. L. Miller, M. H. Murad, S. E. Oberfield, P. C. White

Journal Articles

Objective: To update the congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency clinical practice guideline published by the Endocrine Society in 2010.Conclusions: The writing committee presents updated best practice guidelines for the clinical management of congenital adrenal hyperplasia based on published evidence and expert opinion with added considerations for patient safety, quality of life, cost, and utilization.


Management Of Acute Pancreatitis In The Pediatric Population: A Clinical Report From The North American Society For Pediatric Gastroenterology, Hepatology And Nutrition Pancreas Committee., M. Abu-El-Haija, S. Kumar, J. A. Quiros, K. Balakrishnan, B. Barth, S. Bitton, J. F. Eisses, E. J. Foglio, V. Fox, V. D. Morinville, +10 Additional Authors Jan 2018

Management Of Acute Pancreatitis In The Pediatric Population: A Clinical Report From The North American Society For Pediatric Gastroenterology, Hepatology And Nutrition Pancreas Committee., M. Abu-El-Haija, S. Kumar, J. A. Quiros, K. Balakrishnan, B. Barth, S. Bitton, J. F. Eisses, E. J. Foglio, V. Fox, V. D. Morinville, +10 Additional Authors

Journal Articles

BACKGROUND:Although the incidence of acute pancreatitis (AP) in children is increasing, management recommendations rely on adult published guidelines. Pediatric-specific recommendations are needed. METHODS:The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition Pancreas committee performed a MEDLINE review using several preselected key terms relating to management considerations in adult and pediatric AP. The literature was summarized, quality of evidence reviewed, and statements of recommendations developed. The authorship met to discuss the evidence, statements, and voted on recommendations. A consensus of at least 75% was required to approve a recommendation. RESULTS:The diagnosis of pediatric AP should follow the published INternational Study …


Management Of Acute Pancreatitis In The Pediatric Population: A Clinical Report From The North American Society For Pediatric Gastroenterology, Hepatology And Nutrition Pancreas Committee, M. Abu-El-Haija, S. Kumar, J. A. Quiros, K. Balakrishnan, B. Barth, S. Bitton, J. F. Eisses, E. J. Foglio, V. Fox, V. D. Morinville, +14 Additional Authors Jan 2018

Management Of Acute Pancreatitis In The Pediatric Population: A Clinical Report From The North American Society For Pediatric Gastroenterology, Hepatology And Nutrition Pancreas Committee, M. Abu-El-Haija, S. Kumar, J. A. Quiros, K. Balakrishnan, B. Barth, S. Bitton, J. F. Eisses, E. J. Foglio, V. Fox, V. D. Morinville, +14 Additional Authors

Journal Articles

No abstract provided.