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Articles 1 - 30 of 43
Full-Text Articles in Medicine and Health Sciences
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
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
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 …
Somatic Mosaicism Of An Intragenic Fancb Duplication In Both Fibroblast And Peripheral Blood Cells Observed In A Fanconi Anemia Patient Leads To Milder Phenotype, R. S. Asur, D. C. Kimble, F. P. Lach, M. Jung, F. X. Donovan, A. Kamat, R. J. Noonan, J. W. Thomas, A. Vlachos, S. C. Chandrasekharappa, +4 Additional Authors
Somatic Mosaicism Of An Intragenic Fancb Duplication In Both Fibroblast And Peripheral Blood Cells Observed In A Fanconi Anemia Patient Leads To Milder Phenotype, R. S. Asur, D. C. Kimble, F. P. Lach, M. Jung, F. X. Donovan, A. Kamat, R. J. Noonan, J. W. Thomas, A. Vlachos, S. C. Chandrasekharappa, +4 Additional Authors
Journal Articles
© 2017 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc. Background: Fanconi anemia (FA) is a rare disorder characterized by congenital malformations, progressive bone marrow failure, and predisposition to cancer. Patients harboring X-linked FANCB pathogenic variants usually present with severe congenital malformations resembling VACTERL syndrome with hydrocephalus. Methods: We employed the diepoxybutane (DEB) test for FA diagnosis, arrayCGH for detection of duplication, targeted capture and next-gen sequencing for defining the duplication breakpoint, PacBio sequencing of full-length FANCB aberrant transcript, FANCD2 ubiquitination and foci formation assays for the evaluation of FANCB protein function by viral transduction of …
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
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 …
Hyposialylated Igg Activates Endothelial Igg Receptor Fcγriib To Promote Obesity-Induced Insulin Resistance, K. Tanigaki, A. Sacharidou, J. Peng, K. L. Chambliss, I. S. Yuhanna, D. Ghosh, M. Ahmed, A. J. Szalai, W. Vongpatanasin, C. Mineo, +10 Additional Authors
Hyposialylated Igg Activates Endothelial Igg Receptor Fcγriib To Promote Obesity-Induced Insulin Resistance, K. Tanigaki, A. Sacharidou, J. Peng, K. L. Chambliss, I. S. Yuhanna, D. Ghosh, M. Ahmed, A. J. Szalai, W. Vongpatanasin, C. Mineo, +10 Additional Authors
Journal Articles
Type 2 diabetes mellitus (T2DM) is a common complication of obesity. Here, we have shown that activation of the IgG receptor FcγRIIB in endothelium by hyposialylated IgG plays an important role in obesity-induced insulin resistance. Despite becoming obese on a high-fat diet (HFD), mice lacking FcγRIIB globally or selectively in endothelium were protected from insulin resistance as a result of the preservation of insulin delivery to skeletal muscle and resulting maintenance of muscle glucose disposal. IgG transfer in IgG-deficient mice implicated IgG as the pathogenetic ligand for endothelial FcγRIIB in obesity-induced insulin resistance. Moreover, IgG transferred from patients with T2DM …
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
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
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 …
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
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.
Business Travel-Associated Illness: A Geosentinel Analysis, L. H. Chen, K. Leder, K. A. Barbre, P. Schlagenhauf, M. Libman, J. Keystone, M. Mendelson, P. Gautret, S. Hagmann, S. Kuhn, +81 Additional Authors
Business Travel-Associated Illness: A Geosentinel Analysis, L. H. Chen, K. Leder, K. A. Barbre, P. Schlagenhauf, M. Libman, J. Keystone, M. Mendelson, P. Gautret, S. Hagmann, S. Kuhn, +81 Additional Authors
Journal Articles
© International Society of Travel Medicine, 2017. Published by Oxford University Press. All rights reserved. Background: Analysis of a large cohort of business travelers will help clinicians focus on frequent and serious illnesses. We aimed to describe travel-related health problems in business travelers. Methods: GeoSentinel Surveillance Network consists of 64 travel and tropical medicine clinics in 29 countries; descriptive analysis was performed on ill business travelers, defined as persons traveling for work, evaluated after international travel 1 January 1997 through 31 December 2014. Results: Among 12 203 business travelers seen 1997-2014 (14 045 eligible diagnoses), the majority (97%) were adults …
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
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.
Enhanced Contribution Of Hla In Pediatric Onset Ulcerative Colitis, S. Venkateswaran, J. Prince, D. J. Cutler, U. M. Marigorta, D. T. Okou, S. Prahalad, D. Mack, B. Boyle, J. Markowitz, S. Kugathasan, +20 Additional Authors
Enhanced Contribution Of Hla In Pediatric Onset Ulcerative Colitis, S. Venkateswaran, J. Prince, D. J. Cutler, U. M. Marigorta, D. T. Okou, S. Prahalad, D. Mack, B. Boyle, J. Markowitz, S. Kugathasan, +20 Additional Authors
Journal Articles
© 2018 Crohn's & Colitis Foundation. Published by Oxford University Press on behalf of Crohn's & Colitis Foundation. Background The genetic contributions to pediatric onset ulcerative colitis (UC), characterized by severe disease and extensive colonic involvement, are largely unknown. In adult onset UC, Genome Wide Association Study (GWAS) has identified numerous loci, most of which have a modest susceptibility risk (OR 0.84-1.14), with the exception of the human leukocyte antigen (HLA) region on Chromosome 6 (OR 3.59). Method To study the genetic contribution to exclusive pediatric onset UC, a GWAS was performed on 466 cases with 2099 healthy controls using …
New-Onset Isolated Asymptomatic Papilledema In Two Patients Treated With Recombinant Growth Hormone, L. A. Kanner, J. Klein, M. Gaffar, H. Pomeranz, G. Frank
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.
Increased Prevalence Of Congenital Heart Disease In Children With Diamond Blackfan Anemia Suggests Unrecognized Diamond Blackfan Anemia As A Cause Of Congenital Heart Disease In The General Population: A Report Of The Diamond Blackfan Anemia Registry., A. Vlachos, D. S. Osorio, E. Atsidaftos, J. Kang, M. L. Lababidi, H. S. Seiden, D. Gruber, B. E. Glader, K. Onel, J. M. Lipton, +6 Additional Authors
Increased Prevalence Of Congenital Heart Disease In Children With Diamond Blackfan Anemia Suggests Unrecognized Diamond Blackfan Anemia As A Cause Of Congenital Heart Disease In The General Population: A Report Of The Diamond Blackfan Anemia Registry., A. Vlachos, D. S. Osorio, E. Atsidaftos, J. Kang, M. L. Lababidi, H. S. Seiden, D. Gruber, B. E. Glader, K. Onel, J. M. Lipton, +6 Additional Authors
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
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, …
Barriers To Adherence In Juvenile Idiopathic Arthritis: A Multicenter Collaborative Experience And Preliminary Results, L. A. Favier, J. Taylor, K. L. Rich, K. B. Jones, S. S. Vora, J. G. Harris, B. S. Gottlieb, L. Robbins, J. T. Lai, A. C. Modi, +7 Additional Authors
Barriers To Adherence In Juvenile Idiopathic Arthritis: A Multicenter Collaborative Experience And Preliminary Results, L. A. Favier, J. Taylor, K. L. Rich, K. B. Jones, S. S. Vora, J. G. Harris, B. S. Gottlieb, L. Robbins, J. T. Lai, A. C. Modi, +7 Additional Authors
Journal Articles
© 2018. All rights reserved. Objective. Nonadherence is currently an underrecognized and potentially modifiable obstacle to care in juvenile idiopathic arthritis (JIA). The purpose of our study was to design and implement a standardized approach to identifying adherence barriers for youth with JIA across 7 pediatric rheumatology clinics through the Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) and to assess the frequency of adherence barriers in patients and their caregivers across treatment modalities. Methods. An iterative process using coproduction among parents and providers of patients with JIA was used to design the Barriers Assessment Tool to screen for adherence …
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
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 …
Microarray Analysis Of Human Keratinocytes From Different Anatomic Sites Reveals Site-Specific Immune Signaling And Responses To Human Papillomavirus Type 16 Transfection, M. Israr, D. Rosenthal, L. Frejo-Navarro, J. Devoti, C. Meyers, V. R. Bonagura
Microarray Analysis Of Human Keratinocytes From Different Anatomic Sites Reveals Site-Specific Immune Signaling And Responses To Human Papillomavirus Type 16 Transfection, M. Israr, D. Rosenthal, L. Frejo-Navarro, J. Devoti, C. Meyers, V. R. Bonagura
Journal Articles
No abstract provided.
Vaginal Discharge In A Pre-Pubertal Girl Posing A Diagnostic Challenge., S. Ganguli, Q. Liu, A. Tsoumpariotis, S. Rapaport, E. Fakioglu
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
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 Functional Assay For The Clinical Annotation Of Genetic Variants Of Uncertain Significance In Diamond–Blackfan Anemia, A. Aspesi, M. Betti, M. Sculco, C. Actis, C. Olgasi, M. W. Wlodarski, A. Vlachos, J. M. Lipton, U. Ramenghi, I. Dianzani, +3 Additional Authors
A Functional Assay For The Clinical Annotation Of Genetic Variants Of Uncertain Significance In Diamond–Blackfan Anemia, A. Aspesi, M. Betti, M. Sculco, C. Actis, C. Olgasi, M. W. Wlodarski, A. Vlachos, J. M. Lipton, U. Ramenghi, I. Dianzani, +3 Additional Authors
Journal Articles
© 2018 The Authors. Human Mutation published by Wiley Periodicals, Inc. Diamond–Blackfan anemia (DBA) is a rare genetic hypoplasia of erythroid progenitors characterized by mild to severe anemia and associated with congenital malformations. Clinical manifestations in DBA patients are quite variable and genetic testing has become a critical factor in establishing a diagnosis of DBA. The majority of DBA cases are due to heterozygous loss-of-function mutations in ribosomal protein (RP) genes. Causative mutations are fairly straightforward to identify in the case of large deletions and frameshift and nonsense mutations found early in a protein coding sequence, but diagnosis becomes more …
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
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
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 …
Disparities In Utilization Of Social Determinants Of Health Referrals Among Children In Immigrant Families, O. T. Uwemedimo, H. May
Disparities In Utilization Of Social Determinants Of Health Referrals Among Children In Immigrant Families, O. T. Uwemedimo, H. May
Journal Articles
© 2018 Uwemedimo and May. Objective: Children in immigrant families (CIF) are at elevated risk of experiencing adverse social determinants of health (SDH), particularly material hardship, which contribute to disparate health outcomes. Previous studies have found that SDH screening programs integrated into pediatric practices have increased receipt of social service resources. Few studies have examined use of social services in these programs among ethnically-diverse patient populations and associations with caregiver immigrant status or limited English proficiency (LEP). Methods: Caregivers of children (<18 >years) were routinely screened in a practice-based, SDH screening program offering referral, assisted navigation and follow-up support. Information …
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
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
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
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 …
Minimum Quality Threshold In Pre-Clinical Sepsis Studies (Mqtipss): An International Expert Consensus Initiative For Improvement Of Animal Modeling In Sepsis., M. F. Osuchowski, A. Ayala, S. Bahrami, M. Bauer, M. Boros, J. Cavaillon, I. H. Chaudry, C. S. Deutschman, P. Wang, B. Zingarelli, +21 Additional Authors
Minimum Quality Threshold In Pre-Clinical Sepsis Studies (Mqtipss): An International Expert Consensus Initiative For Improvement Of Animal Modeling In Sepsis., M. F. Osuchowski, A. Ayala, S. Bahrami, M. Bauer, M. Boros, J. Cavaillon, I. H. Chaudry, C. S. Deutschman, P. Wang, B. Zingarelli, +21 Additional Authors
Journal Articles
Preclinical animal studies precede the majority of clinical trials. While the clinical definitions of sepsis and recommended treatments are regularly updated, a systematic review of preclinical models of sepsis has not been done and clear modeling guidelines are lacking. To address this deficit, a Wiggers-Bernard Conference on preclinical sepsis modeling was held in Vienna in May, 2017. The goal of the conference was to identify limitations of preclinical sepsis models and to propose a set of guidelines, defined as the "Minimum Quality Threshold in Preclinical Sepsis Studies" (MQTiPSS), to enhance translational value of these models. A total of 31 experts …
Minimum Quality Threshold In Pre-Clinical Sepsis Studies (Mqtipss): An International Expert Consensus Initiative For Improvement Of Animal Modeling In Sepsis, 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
Minimum Quality Threshold In Pre-Clinical Sepsis Studies (Mqtipss): An International Expert Consensus Initiative For Improvement Of Animal Modeling In Sepsis, 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). Purpose: Pre-clinical animal studies precede the majority of clinical trials. While the clinical sepsis definitions and recommended treatments are regularly updated, a systematic review of pre-clinical models of sepsis has not been done and clear modeling guidelines are lacking. To address this deficit, a Wiggers-Bernard Conference on pre-clinical sepsis modeling was held in Vienna in May, 2017. The conference goal was to identify limitations of pre-clinical sepsis models and to propose a set of guidelines, defined as the “Minimum Quality Threshold in Pre-Clinical Sepsis Studies” (MQTiPSS), to enhance translational value of these models. Methods: 31 experts …
Identification, Management, And Transition Of Care For Patients With Opioid Use Disorder In The Emergency Department, H. C. Duber, I. A. Barata, E. Cioe-Pena, S. Y. Liang, E. Ketcham, W. Macias-Konstantopoulos, S. A. Ryan, M. Stavros, L. K. Whiteside
Identification, Management, And Transition Of Care For Patients With Opioid Use Disorder In The Emergency Department, H. C. Duber, I. A. Barata, E. Cioe-Pena, S. Y. Liang, E. Ketcham, W. Macias-Konstantopoulos, S. A. Ryan, M. Stavros, L. K. Whiteside
Journal Articles
No abstract provided.
Macrophage Migration Inhibitory Factor (Mif) In The Development And Progression Of Pulmonary Arterial Hypertension., M. Ahmed, E. Miller
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.