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Articles 1 - 30 of 35
Full-Text Articles in Medicine and Health Sciences
Decreased Langerhans Cell Responses To Il-36 Gamma: Altered Innate Immunity In Patients With Recurrent Respiratory Papillomatosis, J. Devoti, L. Hatam, A. Lucs, A. Afzal, A. Abramson, B. M. Steinberg, V. Bonagura
Decreased Langerhans Cell Responses To Il-36 Gamma: Altered Innate Immunity In Patients With Recurrent Respiratory Papillomatosis, J. Devoti, L. Hatam, A. Lucs, A. Afzal, A. Abramson, B. M. Steinberg, V. Bonagura
Journal Articles
Recurrent respiratory papillomatosis (RRP) is a rare, chronic disease caused by human papillomaviruses (HPVs) types 6 and 11 that is characterized by the polarization of adaptive immune responses that support persistent HPV infection. Respiratory papillomas express elevated mRNA levels of IL-36 gamma, a proinflammatory cytokine in comparison to autologous clinically normal laryngeal tissues; however there is no evidence of inflammation in these lesions. Consistent with this, respiratory papillomas do not contain T(H)1-like CD4(+) T-cells or cytotoxic CD8(+) T-cells, but instead contain a predominance of T(H)2-like and T regulatory cells (Tregs). In addition, papillomas also are infiltrated with immature Langerhans cells …
X-Linked Agammaglobulinemia Presenting As Polymicrobial Pneumonia, Including Pneumocystis Jirovecii, A. M. Jongco, J. D. Gough, K. Sarnataro, D. W. Rosenthal, J. Moreau, P. Ponda, V. R. Bonagura
X-Linked Agammaglobulinemia Presenting As Polymicrobial Pneumonia, Including Pneumocystis Jirovecii, A. M. Jongco, J. D. Gough, K. Sarnataro, D. W. Rosenthal, J. Moreau, P. Ponda, V. R. Bonagura
Journal Articles
No abstract provided.
Blood Pressure Variability In Children With Primary Vs Secondary Hypertension, D. Leisman, M. Meyers, J. Schnall, N. Chorny, R. Frank, L. Infante, C. B. Sethna
Blood Pressure Variability In Children With Primary Vs Secondary Hypertension, D. Leisman, M. Meyers, J. Schnall, N. Chorny, R. Frank, L. Infante, C. B. Sethna
Journal Articles
Increased blood pressure variability (BPV) is correlated with adverse cardiovascular (CV) events in adults. However, there has been limited research on its effect in the pediatric population. Additionally, BPV differences between primary and secondary hypertension (HTN) are not known. Children with primary and secondary HTN underwent 24-hour ambulatory blood pressure monitoring and echocardiography studies. BPV measures of standard deviation (SD), average real variability (ARV), and range were calculated for the 24-hour, daytime, and nighttime periods. Seventy-four patients (median age, 13.5 years; 74% boys) were examined, 40 of whom had primary HTN. Body mass index z score and age were independent …
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 …
Characteristics Of Avoidant/Restrictive Food Intake Disorder In Children And Adolescents: A "New Disorder" In Dsm-5, M. M. Fisher, D. S. Rosen, R. M. Ornstein, K. A. Mammel, D. K. Katzman, E. S. Rome, S. T. Callahan, J. Malizio, S. Kearney, B. T. Walsh
Characteristics Of Avoidant/Restrictive Food Intake Disorder In Children And Adolescents: A "New Disorder" In Dsm-5, M. M. Fisher, D. S. Rosen, R. M. Ornstein, K. A. Mammel, D. K. Katzman, E. S. Rome, S. T. Callahan, J. Malizio, S. Kearney, B. T. Walsh
Journal Articles
Purpose: To evaluate the DSM-5 diagnosis of Avoidant/Restrictive Food Intake Disorder (ARFID) in children and adolescents with poor eating not associated with body image concerns. Methods: A retrospective case-control study of 8e18-year-olds, using a diagnostic algorithm, compared all cases with ARFID presenting to seven adolescent-medicine eating disorder programs in 2010 to a randomly selected sample with anorexia nervosa (AN) and bulimia nervosa (BN). Demographic and clinical information were recorded. Results: Of 712 individuals studied, 98 (13.8%) met ARFID criteria. Patients with ARFID were younger than those with AN (n = 98) or BN (n = 66), (12.9 vs. 15.6 vs. …
Anti-Inflammatory Actions Of Endogenous And Exogenous Interleukin-10 Versus Glucocorticoids On Macrophage Functions Of The Newly Born, K. Kasat, H. Patel, O. Predtechenska, I. Vancurova, D. Davidson
Anti-Inflammatory Actions Of Endogenous And Exogenous Interleukin-10 Versus Glucocorticoids On Macrophage Functions Of The Newly Born, K. Kasat, H. Patel, O. Predtechenska, I. Vancurova, D. Davidson
Journal Articles
OBJECTIVE: To determine whether specific macrophage immune functions of the newly born are insensitive to the actions of therapeutic levels of dexamethasone (DEX), previously measured in infants with bronchopulmonary dysplasia (BPD), compared with betamethasone (BETA) and exogenous or endogenous interleukin-10 (IL-10). STUDY DESIGN: Macrophages were differentiated from cord blood monocytes (N=18). A serial dose-response (around 10(-8)M), in vitro study was used to examine the effect of DEX, BETA and IL-10, on proinflammatory (PI) cytokine release, phagocytosis and respiratory burst. RESULT: Exogenous IL-10 (10(-8)M) significantly (P < 0.05) inhibited the endotoxin-stimulated release of IL-6, IL-8 and tumor necrosis factor by 63 to 82% with no significant effect by DEX and BETA. There was no inhibition by these three agents at 10(-8)M on phagocytosis and respiratory burst. Inhibition of endogenous IL-10 with a monoclonal antibody significantly increased endotoxin-stimulated cytokine release by at least fourfold. CONCLUSION: Macrophages were relatively insensitive to therapeutic levels of DEX and BETA with regard to PI cytokine release. This study provides rationale for translational and preclinical research using airway instillation of IL-10 for the treatment of BPD.
Bisphenol A Exposure Is Associated With Decreased Lung Function, A. J. Spanier, E. K. Fiorino, L. Trasande
Bisphenol A Exposure Is Associated With Decreased Lung Function, A. J. Spanier, E. K. Fiorino, L. Trasande
Journal Articles
Objective To examine the associations of bisphenol A (BPA) exposure with lung function measures and exhaled nitric oxide (FeNO) in children. Study design We performed a cross-sectional analysis of a subsample of US children age 6-19 years who participated in the 2007-2010 National Health and Nutrition Examination Survey. We assessed univariate and multivariable associations of urinary BPA concentration with the predicted pulmonary function measures for age, sex, race/ethnicity and height (forced expiratory volume in 1 second [FEV1], forced vital capacity [FVC], forced expiratory flow 25%-75%, and FEV1 divided by FVC) and with FeNO. Results Exposure and outcome data were available …
Compliance With Referrals For Non-Acute Child Health Conditions: Evidence From The Longitudinal Asenze Study In Kwazulu Natal, South Africa, O. T. Uwemedimo, S. M. Arpadi, M. K. Chhagan, S. Kauchali, M. H. Craib, F. Bah, L. L. Davidson
Compliance With Referrals For Non-Acute Child Health Conditions: Evidence From The Longitudinal Asenze Study In Kwazulu Natal, South Africa, O. T. Uwemedimo, S. M. Arpadi, M. K. Chhagan, S. Kauchali, M. H. Craib, F. Bah, L. L. Davidson
Journal Articles
BACKGROUND: Caregiver compliance with referrals for child health services is essential to child health outcomes. Many studies in sub-Saharan Africa have examined compliance patterns for children referred for acute, life-threatening conditions but few for children referred for non-acute conditions. The aims of this analysis were to determine the rate of referral compliance and investigate factors associated with referral compliance in KwaZulu Natal, South Africa. METHODS: From September 2008-2010, a door-to-door household survey was conducted to identify children aged 4-6 years in outer-west eThekwini District, KwaZulu-Natal, South Africa. Of 2,049 identified, informed consent was obtained for 1787 (89%) children who were …
Diagnosis And Treatment Of Pediatric Acquired Aplastic Anemia (Aaa): An Initial Survey Of The North American Pediatric Aplastic Anemia Consortium (Napaac), D. A. Williams, C. Bennett, A. Bertuch, M. Bessler, T. Coates, S. Corey, Y. Dror, J. Lipton, A. Vlachos, A. Shimamura, +7 Additional Authors
Diagnosis And Treatment Of Pediatric Acquired Aplastic Anemia (Aaa): An Initial Survey Of The North American Pediatric Aplastic Anemia Consortium (Napaac), D. A. Williams, C. Bennett, A. Bertuch, M. Bessler, T. Coates, S. Corey, Y. Dror, J. Lipton, A. Vlachos, A. Shimamura, +7 Additional Authors
Journal Articles
BackgroundRandomized clinical trials in pediatric aplastic anemia (AA) are rare and data to guide standards of care are scarce. ProcedureEighteen pediatric institutions formed the North American Pediatric Aplastic Anemia Consortium to foster collaborative studies in AA. The initial goal of NAPAAC was to survey the diagnostic studies and therapies utilized in AA. ResultsOur survey indicates considerable variability among institutions in the diagnosis and treatment of AA. There were areas of general consensus, including the need for a bone marrow evaluation, cytogenetic and specific fluorescent in situ hybridization assays to establish diagnosis and exclude genetic etiologies with many institutions requiring results …
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, …
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.
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 …
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 …
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 …
A New System For Naming Ribosomal Proteins, N. Ban, R. Beckmann, J. H. D. Cate, J. D. Dinman, F. Dragon, S. R. Ellis, D. L. J. Lafontaine, L. Lindahl, J. M. Lipton, M. Yusupov, +15 Additional Authors
A New System For Naming Ribosomal Proteins, N. Ban, R. Beckmann, J. H. D. Cate, J. D. Dinman, F. Dragon, S. R. Ellis, D. L. J. Lafontaine, L. Lindahl, J. M. Lipton, M. Yusupov, +15 Additional Authors
Journal Articles
A system for naming ribosomal proteins is described that the authors intend to use in the future. They urge others to adopt it. The objective is to eliminate the confusion caused by the assignment of identical names to ribosomal proteins from different species that are unrelated in structure and function. In the system proposed here, homologous ribosomal proteins are assigned the same name, regardless of species. It is designed so that new names are similar enough to old names to be easily recognized, but are written in a format that unambiguously identifies them as 'new system' names.
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 …
In Utero Exposure To A Maternal High-Fat Diet Alters The Epigenetic Histone Code In A Murine Model, M. A. Suter, J. Ma, P. M. Vuguin, K. Hartil, A. Fiallo, R. A. Harris, M. J. Charron, K. M. Aagaard
In Utero Exposure To A Maternal High-Fat Diet Alters The Epigenetic Histone Code In A Murine Model, M. A. Suter, J. Ma, P. M. Vuguin, K. Hartil, A. Fiallo, R. A. Harris, M. J. Charron, K. M. Aagaard
Journal Articles
OBJECTIVE: Data from animal models show that in utero exposure to a maternal high-fat diet (HFD) renders susceptibility of these offspring to the adult onset of metabolic syndrome. We and others have previously shown that epigenetic modifications to histones may serve as a molecular memory of the in utero exposure, rendering the risk of adult disease. Because mice heterozygous for the Glut4 gene (insulin sensitive glucose transporter) born to wild-type (WT) mothers demonstrate exacterbated metabolic syndrome when exposed to an HFD in utero, we sought to analyze the genome-wide epigenetic changes that occur in the fetal liver in susceptible offspring. …
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 …
Newborn Screening For Severe Combined Immunodeficiency In 11 Screening Programs In The United States, A. Kwan, R. S. Abraham, R. Currier, A. Brower, K. Andruszewski, J. K. Abbott, M. Baker, M. Ballow, L. E. Bartoshesky, V. R. Bonagura, +70 Additional Authors
Newborn Screening For Severe Combined Immunodeficiency In 11 Screening Programs In The United States, A. Kwan, R. S. Abraham, R. Currier, A. Brower, K. Andruszewski, J. K. Abbott, M. Baker, M. Ballow, L. E. Bartoshesky, V. R. Bonagura, +70 Additional Authors
Journal Articles
IMPORTANCE: Newborn screening for severe combined immunodeficiency (SCID) using assays to detect T-cell receptor excision circles (TRECs) began in Wisconsin in 2008, and SCID was added to the national recommended uniform panel for newborn screened disorders in 2010. Currently 23 states, the District of Columbia, and the Navajo Nation conduct population-wide newborn screening for SCID. The incidence of SCID is estimated at 1 in 100,000 births. OBJECTIVES: To present data from a spectrum of SCID newborn screening programs, establish population-based incidence for SCID and other conditions with T-cell lymphopenia, and document early institution of effective treatments. DESIGN: Epidemiological and retrospective …
Newborn Screening For Scid In New York State: Experience From The First Two Years, B. H. Vogel, V. Bonagura, G. A. Weinberg, M. Ballow, J. Isabelle, L. Diantonio, A. Parker, A. Young, C. Cunningham-Rundles, M. Caggana, +8 Additional Authors
Newborn Screening For Scid In New York State: Experience From The First Two Years, B. H. Vogel, V. Bonagura, G. A. Weinberg, M. Ballow, J. Isabelle, L. Diantonio, A. Parker, A. Young, C. Cunningham-Rundles, M. Caggana, +8 Additional Authors
Journal Articles
PURPOSE: To describe the process and assess outcomes for the first 2 years of newborn screening for severe combined immunodeficiency (SCID NBS) in New York State (NYS). METHODS: The NYS algorithm utilizes a first-tier molecular screen for TRECs (T-cell receptor excision circles), the absence of which is indicative of increased risk of immunodeficiency. RESULTS: During the first 2 years, 485,912 infants were screened for SCID. Repeat specimens were requested from 561 premature and 746 non-premature infants with low or borderline TRECs. A total of 531 infants were referred for diagnostic evaluation leading to identification of 10 infants with SCID and …
Magnetic Resonance Imaging/Angiography And Transcranial Doppler Velocities In Sickle Cell Anemia: Results From The Switch Trial, K. J. Helton, R. J. Adams, K. L. Kesler, A. Lockhart, B. Aygun, C. Driscoll, M. M. Heeney, S. M. Jackson, L. Krishnamurti, R. E. Ware, +3 Additional Authors
Magnetic Resonance Imaging/Angiography And Transcranial Doppler Velocities In Sickle Cell Anemia: Results From The Switch Trial, K. J. Helton, R. J. Adams, K. L. Kesler, A. Lockhart, B. Aygun, C. Driscoll, M. M. Heeney, S. M. Jackson, L. Krishnamurti, R. E. Ware, +3 Additional Authors
Journal Articles
The Stroke With Transfusions Changing to Hydroxyurea (SWiTCH) trial compared standard (transfusions/chelation) to alternative (hydroxyurea/phlebotomy) treatment to prevent recurrent stroke and manage iron overload in children chronically transfused over 7 years before enrollment. Standardized brain magnetic resonance imaging/magnetic resonance angiography (MRA) and transcranial Doppler (TCD) exams were performed at entry and exit, with a central blinded review. A novel MRA vasculopathy grading scale demonstrated frequent severe baseline left/right vessel stenosis (53%/41% >/=Grade 4); 31% had no vessel stenosis on either side. Baseline parenchymal injury was prevalent (85%/79% subcortical, 53%/37% cortical, 50%/35% subcortical and cortical). Most children had low or uninterpretable …
P53-Independent Cell Cycle And Erythroid Differentiation Defects In Murine Embryonic Stem Cells Haploinsufficient For Diamond Blackfan Anemia-Proteins: Rps19 Versus Rpl5, S. A. Singh, T. A. Goldberg, A. L. Henson, S. Husain-Krautter, A. Nihrane, L. Blanc, S. R. Ellis, J. M. Lipton, J. M. Liu
P53-Independent Cell Cycle And Erythroid Differentiation Defects In Murine Embryonic Stem Cells Haploinsufficient For Diamond Blackfan Anemia-Proteins: Rps19 Versus Rpl5, S. A. Singh, T. A. Goldberg, A. L. Henson, S. Husain-Krautter, A. Nihrane, L. Blanc, S. R. Ellis, J. M. Lipton, J. M. Liu
Journal Articles
Diamond Blackfan anemia (DBA) is a rare inherited bone marrow failure syndrome caused by ribosomal protein haploinsufficiency. DBA exhibits marked phenotypic variability, commonly presenting with erythroid hypoplasia, less consistently with non-erythroid features. The p53 pathway, activated by abortive ribosome assembly, is hypothesized to contribute to the erythroid failure of DBA. We studied murine embryonic stem (ES) cell lines harboring a gene trap mutation in a ribosomal protein gene, either Rps19 or Rpl5. Both mutants exhibited ribosomal protein haploinsufficiency and polysome defects. Rps19 mutant ES cells showed significant increase in p53 protein expression, however, there was no similar increase in the …
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. …
The Treatment-Naive Microbiome In New-Onset Crohn's Disease, D. Gevers, S. Kugathasan, L. A. Denson, Y. Vazquez-Baeza, W. Van Treuren, B. Y. Ren, E. Schwager, D. Knights, J. Markowitz, R. J. Xavier, +22 Additional Authors
The Treatment-Naive Microbiome In New-Onset Crohn's Disease, D. Gevers, S. Kugathasan, L. A. Denson, Y. Vazquez-Baeza, W. Van Treuren, B. Y. Ren, E. Schwager, D. Knights, J. Markowitz, R. J. Xavier, +22 Additional Authors
Journal Articles
Inflammatory bowel diseases (IBDs), including Crohn's disease (CD), are genetically linked to host pathways that implicate an underlying role for aberrant immune responses to intestinal microbiota. However, patterns of gut microbiome dysbiosis in IBD patients are inconsistent among published studies. Using samples from multiple gastrointestinal locations collected prior to treatment in new-onset cases, we studied the microbiome in the largest pediatric CD cohort to date. An axis defined by an increased abundance in bacteria which include Enterobacteriaceae, Pasteurellacaea, Veillonellaceae, and Fusobacteriaceae, and decreased abundance in Erysipelotrichales, Bacteroidales, and Clostridiales, correlates strongly with disease status. Microbiome comparison between CD patients with …
Randomized, Double-Blind, Placebo-Controlled Trial Of The Efficacy And Safety Of Rilonacept In The Treatment Of Systemic Juvenile Idiopathic Arthritis, N. T. Ilowite, K. Prather, Y. Lokhnygina, L. E. Schanberg, M. Elder, D. Milojevic, J. W. Verbsky, S. J. Spalding, B. S. Gottlieb, C. I. Sandborg, +12 Additional Authors
Randomized, Double-Blind, Placebo-Controlled Trial Of The Efficacy And Safety Of Rilonacept In The Treatment Of Systemic Juvenile Idiopathic Arthritis, N. T. Ilowite, K. Prather, Y. Lokhnygina, L. E. Schanberg, M. Elder, D. Milojevic, J. W. Verbsky, S. J. Spalding, B. S. Gottlieb, C. I. Sandborg, +12 Additional Authors
Journal Articles
OBJECTIVE: To assess the efficacy and safety of rilonacept, an interleukin-1 inhibitor, in a randomized, double-blind, placebo-controlled trial. METHODS: An initial 4-week double-blind placebo phase was incorporated into a 24-week randomized multicenter design, followed by an open-label phase. Seventy-one children who had active arthritis in >/=2 joints were randomized (1:1) to the 2 arms of the study. Patients in the rilonacept arm received rilonacept (loading dose 4.4 mg/kg followed by 2.2 mg/kg weekly, subcutaneously) beginning on day 0. Patients in the placebo arm received placebo for 4 weeks followed by a loading dose of rilonacept at week 4 followed by …
Second-Line Immunosuppressive Treatment Of Childhood Nephrotic Syndrome: A Single-Center Experience, J. Kim, N. Patnaik, N. Chorny, R. Frank, L. Infante, C. Sethna
Second-Line Immunosuppressive Treatment Of Childhood Nephrotic Syndrome: A Single-Center Experience, J. Kim, N. Patnaik, N. Chorny, R. Frank, L. Infante, C. Sethna
Journal Articles
OBJECTIVE: Most cases of idiopathic nephrotic syndrome in childhood are responsive to corticosteroids. However, there is a small group of children that demonstrate steroid resistance (steroid-resistant nephrotic syndrome; SRNS), steroid dependence, or that frequently relapse (frequent-relapse steroid-sensitive nephrotic syndrome; FR-SSNS) which are more clinically difficult to treat. Therefore, second-line immunosuppressants, such as alkylating agents, calcineurin inhibitors, antimetabolites and, more recently, rituximab, have been used with varying success. The objective was to evaluate the response rates of various second-line therapies in the treatment of childhood nephrotic syndrome. STUDY DESIGN: A retrospective chart review of pediatric subjects with idiopathic nephrotic syndrome was …
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 …