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Articles 181 - 210 of 227
Full-Text Articles in Pediatrics
Presurgical Ct Evaluation Of Congenital Aural Atresia, V. T. Nguyen, G. Paek, J. Hu, L. P. Smith
Presurgical Ct Evaluation Of Congenital Aural Atresia, V. T. Nguyen, G. Paek, J. Hu, L. P. Smith
Journal Articles
Congenital aural atresia occurs in approximately 1 in 10,000‐20,000 births and may be surgically repaired if the middle ear malformation is limited in character. External auditory canal atresia is difficult to repair surgically, with significant risks and complications. Surgical candidacy in congenital aural atresia is based on multiple factors, central to which are the anatomy of the temporal bone and audiometric findings. High-resolution multidetector CT is the imaging technique of choice for anatomy delineation, although there are some specific indications for MR imaging in presurgical assessment. Various CT grading systems have been developed to determine surgical candidacy and are described …
Poly(I:C) Induces Controlled Release Of Il-36gamma From Keratinocytes In The Absence Of Cell Death, A. A. Rana, A. V. Lucs, J. Devoti, L. Blanc, J. Papoin, R. Wu, C. J. Papayannakos, A. Abramson, V. R. Bonagura, B. M. Steinberg
Poly(I:C) Induces Controlled Release Of Il-36gamma From Keratinocytes In The Absence Of Cell Death, A. A. Rana, A. V. Lucs, J. Devoti, L. Blanc, J. Papoin, R. Wu, C. J. Papayannakos, A. Abramson, V. R. Bonagura, B. M. Steinberg
Journal Articles
The epithelium is part of an integrated immune system where cytokines, toll-like receptors and their ligands, and extracellular vesicles play a crucial role in initiating an innate immune response. IL-36gamma is a pro-inflammatory member of the IL-1 family that is mainly expressed by epithelial cells, but regulation of its expression and release are only beginning to be understood. Previous studies reported that IL-36gamma is abundant in recurrent respiratory papillomatosis, a rare but devastating disease caused by human papillomaviruses (HPV) types 6 and 11, in which papillomas recurrently grow in and block the airway. Despite the overexpression of IL-36gamma, papilloma tissues …
Effects Of Obesity On Health-Related Quality Of Life In Juvenile-Onset Systemic Lupus Erythematosus, R. Mina, M. S. Klein-Gitelman, S. Nelson, B. A. Eberhard, G. Higgins, N. G. Singer, K. Onel, L. Tucker, K. M. O'Neil, H. I. Brunner, +4 Additional Authors
Effects Of Obesity On Health-Related Quality Of Life In Juvenile-Onset Systemic Lupus Erythematosus, R. Mina, M. S. Klein-Gitelman, S. Nelson, B. A. Eberhard, G. Higgins, N. G. Singer, K. Onel, L. Tucker, K. M. O'Neil, H. I. Brunner, +4 Additional Authors
Journal Articles
OBJECTIVE: This study evaluated the effects of obesity on health-related quality of life (HRQOL) measures in juvenile-onset systemic lupus erythematosus (jSLE). METHODS: Obesity was defined as a body mass index (BMI) >/=95th percentile according to the Sex-specific Center for Disease Control BMI-For-Age Charts and determined in a multicenter cohort of jSLE patients. In this secondary analysis, the domain and summary scores of the Pediatric Quality of Life (PedsQL) Inventory and the Child Health Questionnaire (CHQ) of obese jSLE patients were compared to those of non-obese jSLE patients as well as historical obese and non-obese healthy controls. Mixed-effects modeling was performed …
Clinical And Genomic Heterogeneity Of Diamond Blackfan Anemia In The Russian Federation, N. S. Smetanina, I. V. Mersiyanova, M. A. Kurnikova, G. S. Ovsyannikova, L. A. Hachatryan, V. O. Bobrynina, M. A. Maschan, G. A. Novichkova, J. M. Lipton, A. A. Maschan
Clinical And Genomic Heterogeneity Of Diamond Blackfan Anemia In The Russian Federation, N. S. Smetanina, I. V. Mersiyanova, M. A. Kurnikova, G. S. Ovsyannikova, L. A. Hachatryan, V. O. Bobrynina, M. A. Maschan, G. A. Novichkova, J. M. Lipton, A. A. Maschan
Journal Articles
BackgroundDiamond Blackfan anemia (DBA) is a genetically and clinically heterogeneous ribosomopathy and inherited bone marrow failure syndrome characterized by anemia, reticulocytopenia, and decreased erythroid precursors in the bone marrow with an increased risk of malignancy and, in approximately 50%, physical abnormalities. MethodsWe retrospectively analyzed clinical data from 77 patients with DBA born in the Russian Federation from 1993 to 2014. In 74 families there was one clinically affected individual; in only three instances a multiplex family was identified. Genomic DNA from 57 DBA patients and their first-degree relatives was sequenced for mutations in RPS19, RPS10, RPS24, RPS26, RPS7, RPS17, RPL5, …
Dissecting Allele Architecture Of Early Onset Ibd Using High-Density Genotyping, D. J. Cutler, M. E. Zwick, D. T. Okou, S. Prahalad, T. Walters, S. L. Guthery, M. Dubinsky, R. Baldassano, J. Markowitz, S. Kugathasan, +24 Additional Authors
Dissecting Allele Architecture Of Early Onset Ibd Using High-Density Genotyping, D. J. Cutler, M. E. Zwick, D. T. Okou, S. Prahalad, T. Walters, S. L. Guthery, M. Dubinsky, R. Baldassano, J. Markowitz, S. Kugathasan, +24 Additional Authors
Journal Articles
BACKGROUND: The inflammatory bowel diseases (IBD) are common, complex disorders in which genetic and environmental factors are believed to interact leading to chronic inflammatory responses against the gut microbiota. Earlier genetic studies performed in mostly adult population of European descent identified 163 loci affecting IBD risk, but most have relatively modest effect sizes, and altogether explain only ~20% of the genetic susceptibility. Pediatric onset represents about 25% of overall incident cases in IBD, characterized by distinct disease physiology, course and risks. The goal of this study is to compare the allelic architecture of early onset IBD with adult onset in …
The Impact Of Disease Duration On Quality Of Life In Children With Nephrotic Syndrome: A Midwest Pediatric Nephrology Consortium Study, D. T. Selewski, J. P. Troost, S. F. Massengill, R. A. Gbadegesin, L. A. Greenbaum, I. F. Shatat, Y. Cai, G. Kapur, C. B. Sethna, D. S. Gipson, +13 Additional Authors
The Impact Of Disease Duration On Quality Of Life In Children With Nephrotic Syndrome: A Midwest Pediatric Nephrology Consortium Study, D. T. Selewski, J. P. Troost, S. F. Massengill, R. A. Gbadegesin, L. A. Greenbaum, I. F. Shatat, Y. Cai, G. Kapur, C. B. Sethna, D. S. Gipson, +13 Additional Authors
Journal Articles
BACKGROUND: The Patient Reported Outcomes Measurement Information System (PROMIS) II is a prospective study that evaluates patient reported outcomes in pediatric chronic diseases as a measure of health-related quality of life (HRQOL). We have evaluated the influence of disease duration on HRQOL and, for the first time, compared the findings of the PROMIS measures to those of the PedsQL 4.0 Generic Scales (PedsQL) from the PROMIS II nephrotic syndrome (NS) longitudinal cohort. METHODS: This was a prospective study in which 127 children (age range 8-17 years) with active NS from 14 centers were enrolled. Children with active NS defined as …
Mitochondrial Dysfunction In Peripheral Blood Mononuclear Cells In Pediatric Septic Shock, S. L. Weiss, M. A. Selak, F. Tuluc, J. Perales Villarroel, V. M. Nadkarni, C. S. Deutschman, L. B. Becker
Mitochondrial Dysfunction In Peripheral Blood Mononuclear Cells In Pediatric Septic Shock, S. L. Weiss, M. A. Selak, F. Tuluc, J. Perales Villarroel, V. M. Nadkarni, C. S. Deutschman, L. B. Becker
Journal Articles
OBJECTIVES: Mitochondrial dysfunction in peripheral blood mononuclear cells has been linked to immune dysregulation and organ failure in adult sepsis, but pediatric data are limited. We hypothesized that pediatric septic shock patients exhibit mitochondrial dysfunction within peripheral blood mononuclear cells which in turn correlates with global organ injury. DESIGN: Prospective observational study. SETTING: Academic PICU. PATIENTS: Thirteen pediatric patients with septic shock and greater than or equal to two organ failures and 11 PICU controls without sepsis or organ failure. INTERVENTIONS: Ex vivo measurements of mitochondrial oxygen consumption and membrane potential (DeltaPsim) were performed in intact peripheral blood mononuclear cells …
Non Alcoholic Fatty Liver Disease In A Case Of Kabuki Syndrome, A. Jain, M. See
Non Alcoholic Fatty Liver Disease In A Case Of Kabuki Syndrome, A. Jain, M. See
Journal Articles
No abstract provided.
Population Pharmacokinetics Of Darbepoetin Alfa In Conjunction With Hypothermia For The Treatment Of Neonatal Hypoxic-Ischemic Encephalopathy, J. K. Roberts, C. Stockmann, R. M. Ward, J. Beachy, M. C. Baserga, M. G. Spigarelli, C. M. Sherwin
Population Pharmacokinetics Of Darbepoetin Alfa In Conjunction With Hypothermia For The Treatment Of Neonatal Hypoxic-Ischemic Encephalopathy, J. K. Roberts, C. Stockmann, R. M. Ward, J. Beachy, M. C. Baserga, M. G. Spigarelli, C. M. Sherwin
Journal Articles
AIM: The aim of this study was to determine the population pharmacokinetics of darbepoetin alfa in hypothermic neonates with hypoxic-ischemic encephalopathy treated with hypothermia. METHODS: Neonates >/=36 weeks gestation andrandomized, multicenter, investigational, new drug pharmacokinetic study. Two intravenous darbepoetin alfa treatment groups were evaluated: 2 and 10 microg/kg. Serum erythropoietin concentrations were measured using an enzyme-linked immunosorbent assay. Monolix 4.3.1 was used to estimate darbepoetin alfa clearance and volume of distribution. Covariates tested included: birthweight, gestational age, postnatal age, postmenstrual age, sex, Sarnat score, and study site. RESULTS: Darbepoetin alfa pharmacokinetics were well described by a one-compartment model with exponential …
Protocolized Sedation Vs Usual Care In Pediatric Patients Mechanically Ventilated For Acute Respiratory Failure: A Randomized Clinical Trial, M. A. Curley, D. Wypij, R. S. Watson, M. J. Grant, L. A. Asaro, I. M. Cheifetz, B. L. Dodson, L. S. Franck, R. G. Gedeit, J. Schneider, +2 Additional Authors
Protocolized Sedation Vs Usual Care In Pediatric Patients Mechanically Ventilated For Acute Respiratory Failure: A Randomized Clinical Trial, M. A. Curley, D. Wypij, R. S. Watson, M. J. Grant, L. A. Asaro, I. M. Cheifetz, B. L. Dodson, L. S. Franck, R. G. Gedeit, J. Schneider, +2 Additional Authors
Journal Articles
IMPORTANCE: Protocolized sedation improves clinical outcomes in critically ill adults, but its effect in children is unknown. OBJECTIVE: To determine whether critically ill children managed with a nurse-implemented, goal-directed sedation protocol experience fewer days of mechanical ventilation than patients receiving usual care. DESIGN, SETTING, AND PARTICIPANTS: Cluster randomized trial conducted in 31 US pediatric intensive care units (PICUs). A total of 2449 children (mean age, 4.7 years; range, 2 weeks to 17 years) mechanically ventilated for acute respiratory failure were enrolled in 2009-2013 and followed up until 72 hours after opioids were discontinued, 28 days, or hospital discharge. INTERVENTION: Intervention …
Use Of Serogroup B Meningococcal Vaccines In Adolescents And Young Adults: Recommendations Of The Advisory Committee On Immunization Practices, 2015, J. R. Macneil, L. Rubin, T. Folaranmi, I. R. Ortega-Sanchez, M. Patel, S. W. Martin
Use Of Serogroup B Meningococcal Vaccines In Adolescents And Young Adults: Recommendations Of The Advisory Committee On Immunization Practices, 2015, J. R. Macneil, L. Rubin, T. Folaranmi, I. R. Ortega-Sanchez, M. Patel, S. W. Martin
Journal Articles
At its June 2015 meeting, the Advisory Committee on Immunization Practices (ACIP) recommended that adolescents and young adults aged 16-23 years may be vaccinated with a serogroup B meningococcal (MenB) vaccine to provide short-term protection against most strains of serogroup B meningococcal disease. This report summarizes the deliberations of ACIP, the rationale for its decision, and recommendations for use of MenB vaccines in adolescents and young adults. Two MenB vaccines have recently been licensed by the Food and Drug Administration (FDA) for use in the United States and approved for use in persons aged 10-25 years: MenB-FHbp (Trumenba, Wyeth Pharmaceuticals, …
Exosomes In Cancer Research, S. R. Vaiselbuh
Animal Models Of In Utero Exposure To A High Fat Diet: A Review, L. Williams, Y. Seki, P. M. Vuguin, M. J. Charron
Animal Models Of In Utero Exposure To A High Fat Diet: A Review, L. Williams, Y. Seki, P. M. Vuguin, M. J. Charron
Journal Articles
The incidence of metabolic disease, including type 2 diabetes and obesity, has increased to epidemic levels in recent years. A growing body of evidence suggests that the intrauterine environment plays a key role in the development of metabolic disease in offspring. Among other perturbations in early life, alteration in the provision of nutrients has profound and lasting effects on the long term health and well being of offspring. Rodent and non-human primate models provide a means to understand the underlying mechanisms of this programming effect. These different models demonstrate converging effects of a maternal high fat diet on insulin and …
Greater Mortality And Morbidity In Extremely Preterm Infants Fed A Diet Containing Cow Milk Protein Products, S. A. Abrams, R. J. Schanler, M. L. Lee, D. J. Rechtman
Greater Mortality And Morbidity In Extremely Preterm Infants Fed A Diet Containing Cow Milk Protein Products, S. A. Abrams, R. J. Schanler, M. L. Lee, D. J. Rechtman
Journal Articles
Background: Provision of human milk has important implications for the health and outcomes of extremely preterm (EP) infants. This study evaluated the effects of an exclusive human milk diet on the health of EP infants during their stay in the neonatal intensive care unit. Subjects and Methods: EP infants= 10% CM (p < 0.02). Conclusions: An exclusive human milk diet, devoid of CM-containing products, was associated with lower mortality and morbidity in EP infants without compromising growth and should be considered as an approach to nutritional care of these infants.
Effects Of Genetics And In Utero Diet On Murine Pancreatic Development, C. L. Lin, L. Williams, Y. Seki, H. Kaur, K. Hartil, A. Fiallo, A. S. Glenn, E. B. Katz, M. J. Charron, P. M. Vuguin
Effects Of Genetics And In Utero Diet On Murine Pancreatic Development, C. L. Lin, L. Williams, Y. Seki, H. Kaur, K. Hartil, A. Fiallo, A. S. Glenn, E. B. Katz, M. J. Charron, P. M. Vuguin
Journal Articles
Intrauterine (IU) malnutrition could alter pancreatic development. In this study, we describe the effects of high-fat diet (HFD) during pregnancy on fetal growth and pancreatic morphology in an 'at risk' animal model of metabolic disease, the glucose transporter 4 (GLUT4) heterozygous mouse (G4+/-). WT female mice mated with G4+/- males were fed HFD or control diet (CD) for 2 weeks before mating and throughout pregnancy. At embryonic day 18.5, fetuses were killed and pancreata isolated for analysis of morphology and expression of genes involved in insulin (INS) cell development, proliferation, apoptosis, glucose transport and function. Compared with WT CD, WT …
Dose And Outcomes In Primary Immunodeficiency Disorders, V. R. Bonagura
Dose And Outcomes In Primary Immunodeficiency Disorders, V. R. Bonagura
Journal Articles
No abstract provided.
From Infancy To Adolescence: Fifteen Years Of Continuous Treatment With Hydroxyurea In Sickle Cell Anemia, J. S. Hankins, B. Aygun, K. Nottage, C. Thornburg, M. P. Smeltzer, R. E. Ware, W. C. Wang
From Infancy To Adolescence: Fifteen Years Of Continuous Treatment With Hydroxyurea In Sickle Cell Anemia, J. S. Hankins, B. Aygun, K. Nottage, C. Thornburg, M. P. Smeltzer, R. E. Ware, W. C. Wang
Journal Articles
Despite documented laboratory and clinical benefits of hydroxyurea for children with sickle cell anemia (SCA), the drug's long-term safety and efficacy remains poorly defined. The HUSOFT trial and extension study examined feasibility, toxicity, and hematological efficacy of hydroxyurea in infants with SCA.This report describes HUSOFT participants who have continued hydroxyurea therapy for 15 years. With IRB approval, medical records were reviewed for clinical, laboratory, and growth parameters.Twenty-eight infants enrolled in the original 2-year HUSOFT study received open-label liquid hydroxyurea at 20 mg/kg/day; 17 completed the extension study with dose escalation to 30 mg/kg/day. Eight of these 17 (6 girls and …
Immunizations In Children With Inflammatory Bowel Disease Treated With Immunosuppressive Therapy, Y. Lu, A. Bousvaros
Immunizations In Children With Inflammatory Bowel Disease Treated With Immunosuppressive Therapy, Y. Lu, A. Bousvaros
Journal Articles
The vast majority of patients with inflammatory bowel disease (IBD) will receive immunosuppressive therapy at some point for their disease, whether for the short term (such as a course of corticosteroids) or long term (such as maintenance therapy with immunomodulators or biologics). The systemic immunosuppression places patients at increased risk for infections. Therefore, it is important that patients are up-to-date with immunizations to minimize vaccine-preventable infections. However, the literature shows that the rate of immunization in patients with IBD is low. Ideally, the vaccination status is checked at diagnosis, and patients are immunized with the vaccines they need. Drawing titers …
Outcomes After Hematopoietic Stem Cell Transplantation For Children With I-Cell Disease, T. C. Lund, S. S. Cathey, W. P. Miller, M. Eapen, M. Andreansky, C. C. Dvorak, J. H. Davis, J. D. Dalal, I. Sahdev, P. J. Orchard, +9 Additional Authors
Outcomes After Hematopoietic Stem Cell Transplantation For Children With I-Cell Disease, T. C. Lund, S. S. Cathey, W. P. Miller, M. Eapen, M. Andreansky, C. C. Dvorak, J. H. Davis, J. D. Dalal, I. Sahdev, P. J. Orchard, +9 Additional Authors
Journal Articles
Mucolipidosis type II (MLII), or I-cell disease, is a rare but severe disorder affecting localization of enzymes to the lysosome, generally resulting in death before the 10th birthday. Although hematopoietic stem cell transplantation (HSCT) has been used to successfully treat some lysosomal storage diseases, only 2 cases have been reported on the use of HSCT to treat MLII. For the first time, we describe the combined international experience in the use of HSCT for MLII in 22 patients. Although 95% of the patients engrafted, overall survival was low, with only 6 patients (27%) alive at last follow-up. The most common …
Pediatric Crohn Disease Patients Exhibit Specific Ileal Transcriptome And Microbiome Signature, Y. Haberman, T. L. Tickle, P. J. Dexheimer, M. O. Kim, D. Tang, R. Karns, R. N. Baldassano, J. D. Noe, J. Markowitz, L. A. Denson, +14 Additional Authors
Pediatric Crohn Disease Patients Exhibit Specific Ileal Transcriptome And Microbiome Signature, Y. Haberman, T. L. Tickle, P. J. Dexheimer, M. O. Kim, D. Tang, R. Karns, R. N. Baldassano, J. D. Noe, J. Markowitz, L. A. Denson, +14 Additional Authors
Journal Articles
Interactions between the host and gut microbial community likely contribute to Crohn disease (CD) pathogenesis; however, direct evidence for these interactions at the onset of disease is lacking. Here, we characterized the global pattern of ileal gene expression and the ileal microbial community in 359 treatment-naive pediatric patients with CD, patients with ulcerative colitis (UC), and control individuals. We identified core gene expression profiles and microbial communities in the affected CD ilea that are preserved in the unaffected ilea of patients with colon-only CD but not present in those with UC or control individuals; therefore, this signature is specific to …
Prevention And Control Of Haemophilus Influenzae Type B Disease: Recommendations Of The Advisory Committee On Immunization Practices (Acip), E. C. Briere, L. Rubin, P. L. Moro, A. Cohn, T. Clark, N. Messonnier
Prevention And Control Of Haemophilus Influenzae Type B Disease: Recommendations Of The Advisory Committee On Immunization Practices (Acip), E. C. Briere, L. Rubin, P. L. Moro, A. Cohn, T. Clark, N. Messonnier
Journal Articles
This report compiles and summarizes all recommendations from CDC's Advisory Committee on Immunization Practices (ACIP) regarding prevention and control of Haemophilus influenzae type b (Hib) disease in the United States. As a comprehensive summary of previously published recommendations, this report does not contain any new recommendations; it is intended for use by clinicians, public health officials, vaccination providers, and immunization program personnel as a resource. ACIP recommends routine vaccination with a licensed conjugate Hib vaccine for infants aged 2 through 6 months (2 or 3 doses, depending on vaccine product) with a booster dose at age 12 through 15 months. …
Pediatric Trainees' Engagement In The Online Nutrition Curriculum: Preliminary Results, K. O. Lewis, G. R. Frank, R. Nagel, T. L. Turner, C. L. Ferrell, S. G. Sangvai, R. Donthi, J. D. Mahan
Pediatric Trainees' Engagement In The Online Nutrition Curriculum: Preliminary Results, K. O. Lewis, G. R. Frank, R. Nagel, T. L. Turner, C. L. Ferrell, S. G. Sangvai, R. Donthi, J. D. Mahan
Journal Articles
Background: The Pediatric Nutrition Series (PNS) consists of ten online, interactive modules and supplementary educational materials that have utilized web-based multimedia technologies to offer nutrition education for pediatric trainees and practicing physicians. The purpose of the study was to evaluate pediatric trainees' engagement, knowledge acquisition, and satisfaction with nutrition modules delivered online in interactive and non-interactive formats. Methods: From December 2010 through August 2011, pediatric trainees from seventy-three (73) different U. S. programs completed online nutrition modules designed to develop residents' knowledge of counseling around and management of nutritional issues in children. Data were analyzed using SPSS version 19. Both …
Use Of Menacwy-Crm Vaccine In Children Aged 2 Through 23 Months At Increased Risk For Meningococcal Disease: Recommendations Of The Advisory Committee On Immunization Practices, 2013, J. R. Macneil, L. Rubin, L. Mcnamara, E. C. Briere, T. A. Clark, A. C. Cohn
Use Of Menacwy-Crm Vaccine In Children Aged 2 Through 23 Months At Increased Risk For Meningococcal Disease: Recommendations Of The Advisory Committee On Immunization Practices, 2013, J. R. Macneil, L. Rubin, L. Mcnamara, E. C. Briere, T. A. Clark, A. C. Cohn
Journal Articles
During its October 2013 meeting, the Advisory Committee on Immunization Practices (ACIP) recommended use of a third meningococcal conjugate vaccine, MenACWY-CRM (Menveo, Novartis), as an additional option for vaccinating infants aged 2 through 23 months at increased risk for meningococcal disease. MenACWY-CRM is the first quadrivalent meningococcal conjugate vaccine licensed for use in children aged 2 through 8 months. MenACWY-D (Menactra, Sanofi Pasteur) is recommended for use in children aged 9 through 23 months who are at increased risk for meningococcal disease, and Hib-MenCY-TT (MenHibrix, GlaxoSmithKline) is recommended for use in children aged 6 weeks through 18 months at increased …
Safety Of Celecoxib And Nonselective Nonsteroidal Anti-Inflammatory Drugs In Juvenile Idiopathic Arthritis: Results Of The Phase 4 Registry, R. E. Sobel, D. J. Lovell, H. I. Brunner, J. E. Weiss, P. W. Morris, B. S. Gottlieb, E. C. Chalom, L. K. Jung, K. B. Onel, E. H. Giannini, +6 Additional Authors
Safety Of Celecoxib And Nonselective Nonsteroidal Anti-Inflammatory Drugs In Juvenile Idiopathic Arthritis: Results Of The Phase 4 Registry, R. E. Sobel, D. J. Lovell, H. I. Brunner, J. E. Weiss, P. W. Morris, B. S. Gottlieb, E. C. Chalom, L. K. Jung, K. B. Onel, E. H. Giannini, +6 Additional Authors
Journal Articles
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-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 (
Utility Of Neutrophil Fcgamma Receptor I (Cd64) Index As A Biomarker For Mucosal Inflammation In Pediatric Crohn's Disease, P. Minar, Y. Haberman, I. Jurickova, T. Wen, M. E. Rothenberg, M. O. Kim, S. A. Saeed, R. N. Baldassano, J. Markowitz, L. A. Denson, +9 Additional Authors
Utility Of Neutrophil Fcgamma Receptor I (Cd64) Index As A Biomarker For Mucosal Inflammation In Pediatric Crohn's Disease, P. Minar, Y. Haberman, I. Jurickova, T. Wen, M. E. Rothenberg, M. O. Kim, S. A. Saeed, R. N. Baldassano, J. Markowitz, L. A. Denson, +9 Additional Authors
Journal Articles
BACKGROUND: Neutrophil expression of the Fcgamma receptor I (CD64) is upregulated in adult patients with clinically active inflammatory bowel disease (IBD). We tested the relationship of CD64 with mucosal inflammation and clinical relapse in pediatric Crohn's disease (CD). METHODS: In a cohort of 208 newly diagnosed CD and 43 non-IBD controls, ileal expression of FcgammaRI/S100A9 was determined by RNA sequencing from biopsies obtained at ileocolonoscopy. In a second cohort, we tested for the peripheral blood polymorphonuclear neutrophil (PMN) CD64 index from 26 newly diagnosed CD, 30 non-IBD controls, and 83 children with established CD. RESULTS: Ileal FcgammaRIA mRNA expression was …
Pulse Oximetry: Fundamentals And Technology Update, M. Nitzan, A. Romem, R. Koppel
Pulse Oximetry: Fundamentals And Technology Update, M. Nitzan, A. Romem, R. Koppel
Journal Articles
Oxygen saturation in the arterial blood (SaO2) provides information on the adequacy of respiratory function. SaO2 can be assessed noninvasively by pulse oximetry, which is based on photoplethysmographic pulses in two wavelengths, generally in the red and infrared regions. The calibration of the measured photoplethysmographic signals is performed empirically for each type of commercial pulse-oximeter sensor, utilizing in vitro measurement of SaO2 in extracted arterial blood by means of co-oximetry. Due to the discrepancy between the measurement of SaO2 by pulse oximetry and the invasive technique, the former is denoted as SpO2. Manufacturers of pulse oximeters generally claim an accuracy …
Whole Exome Sequencing Identifies Novel Genes For Fetal Hemoglobin Response To Hydroxyurea In Children With Sickle Cell Anemia., V. A. Sheehan, J. R. Crosby, A. Sabo, N. A. Mortier, T. A. Howard, D. M. Muzny, S. Dugan-Perez, Banu Aygun, K. A. Nottage, J. M. Flanagan, +3 Additional Authors
Whole Exome Sequencing Identifies Novel Genes For Fetal Hemoglobin Response To Hydroxyurea In Children With Sickle Cell Anemia., V. A. Sheehan, J. R. Crosby, A. Sabo, N. A. Mortier, T. A. Howard, D. M. Muzny, S. Dugan-Perez, Banu Aygun, K. A. Nottage, J. M. Flanagan, +3 Additional Authors
Journal Articles
Hydroxyurea has proven efficacy in children and adults with sickle cell anemia (SCA), but with considerable inter-individual variability in the amount of fetal hemoglobin (HbF) produced. Sibling and twin studies indicate that some of that drug response variation is heritable. To test the hypothesis that genetic modifiers influence pharmacological induction of HbF, we investigated phenotype-genotype associations using whole exome sequencing of children with SCA treated prospectively with hydroxyurea to maximum tolerated dose (MTD). We analyzed 171 unrelated patients enrolled in two prospective clinical trials, all treated with dose escalation to MTD. We examined two MTD drug response phenotypes: HbF (final …
Critical Periods Of Increased Fetal Vulnerability To A Maternal High Fat Diet, M. D. Plata, L. Williams, Y. Seki, K. Hartil, H. Kaur, C. L. Lin, A. Fiallo, A. S. Glenn, E. B. Katz, P. M. Vuguin, +2 Additional Authors
Critical Periods Of Increased Fetal Vulnerability To A Maternal High Fat Diet, M. D. Plata, L. Williams, Y. Seki, K. Hartil, H. Kaur, C. L. Lin, A. Fiallo, A. S. Glenn, E. B. Katz, P. M. Vuguin, +2 Additional Authors
Journal Articles
Background: Fetal adaptations to high fat (HF) diet in utero (IU) that may predispose to Metabolic Syndrome (MetS) in adulthood include changes in fetal hepatic gene expression. Studies were performed to determine whether maternal exposure to HF diet at different stages during pregnancy had different effects on the fetus, including hepatic gene expression. Methods: Female wild type mice were fed either a HF or breeding chow (C) for 2 wks prior to mating. The experimental groups were composed of embryonic day (e) 18.5 fetuses obtained from WT female mice that were fed HF (HF, 35.5% fat) or breeding chow (C, …
Evpedia: A Community Web Portal For Extracellular Vesicles Research, D. K. Kim, J. Lee, S. R. Kim, D. S. Choi, Y. J. Yoon, J. H. Kim, G. Go, L. Blanc, S. Sahoo, Y. S. Gho, +85 Additional Authors
Evpedia: A Community Web Portal For Extracellular Vesicles Research, D. K. Kim, J. Lee, S. R. Kim, D. S. Choi, Y. J. Yoon, J. H. Kim, G. Go, L. Blanc, S. Sahoo, Y. S. Gho, +85 Additional Authors
Journal Articles
MOTIVATION: Extracellular vesicles (EVs) are spherical bilayered proteolipids, harboring various bioactive molecules. Due to the complexity of the vesicular nomenclatures and components, online searches for EV-related publications and vesicular components are currently challenging. RESULTS: We present an improved version of EVpedia, a public database for EVs research. This community web portal contains a database of publications and vesicular components, identification of orthologous vesicular components, bioinformatic tools and a personalized function. EVpedia includes 6879 publications, 172 080 vesicular components from 263 high-throughput datasets, and has been accessed more than 65 000 times from more than 750 cities. In addition, about 350 …
Exploiting Pre-Rrna Processing In Diamond Blackfan Anemia Gene Discovery And Diagnosis, J. E. Farrar, P. Quarello, R. Fisher, K. A. O'Brien, A. Aspesi, S. Parrella, E. Atsidaftos, A. Vlachos, J. M. Lipton, S. R. Ellis, +9 Additional Authors
Exploiting Pre-Rrna Processing In Diamond Blackfan Anemia Gene Discovery And Diagnosis, J. E. Farrar, P. Quarello, R. Fisher, K. A. O'Brien, A. Aspesi, S. Parrella, E. Atsidaftos, A. Vlachos, J. M. Lipton, S. R. Ellis, +9 Additional Authors
Journal Articles
Diamond Blackfan anemia (DBA), a syndrome primarily characterized by anemia and physical abnormalities, is one among a group of related inherited bone marrow failure syndromes (IBMFS) which share overlapping clinical features. Heterozygous mutations or single-copy deletions have been identified in 12 ribosomal protein genes in approximately 60% of DBA cases, with the genetic etiology unexplained in most remaining patients. Unlike many IBMFS, for which functional screening assays complement clinical and genetic findings, suspected DBA in the absence of typical alterations of the known genes must frequently be diagnosed after exclusion of other IBMFS. We report here a novel deletion in …