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Common Variable Immune disease

J A DE Fuentes-Vicente, M Cabrera-Bravo, J N Enríquez-Vara, M I Bucio-Torres, A E Gutiérrez-Cabrera, D G Vidal-López, J A Martínez-Ibarra, P M Salazar-Schettino, A Córdoba-Aguilar
Little is known about how the virulence of a human pathogen varies in the environment it shares with its vector. This study focused on whether the virulence of Trypanosoma cruzi (Trypanosomatida: Trypanosomatidae), the causal agent of Chagas' disease, is related to altitude. Accordingly, Triatoma dimidiata (Hemiptera: Reduviidae) specimens were collected at three different altitudes (300, 700 and 1400 m a.s.l.) in Chiapas, Mexico. The parasite was then isolated to infect uninfected T. dimidiata from the same altitudes, as well as female CD-1 mice...
October 18, 2016: Medical and Veterinary Entomology
Esmaeil Mortaz, Payam Tabarsi, Davod Mansouri, Adnan Khosravi, Johan Garssen, Aliakbar Velayati, Ian M Adcock
The life span of patients with primary and secondary immunodeficiency is increasing due to recent improvements in therapeutic strategies. While the incidence of primary immunodeficiencies (PIDs) is 1:10,000 births, that of secondary immunodeficiencies are more common and are associated with posttransplantation immune dysfunction, with immunosuppressive medication for human immunodeficiency virus or with human T-cell lymphotropic virus infection. After infection, malignancy is the most prevalent cause of death in both children and adults with (PIDs)...
2016: Frontiers in Immunology
Diana Gassó, Joaquín Vicente, Gregorio Mentaberre, Ramón Soriguer, Rocío Jiménez Rodríguez, Nora Navarro-González, Asta Tvarijonaviciute, Santiago Lavín, Pedro Fernández-Llario, Joaquim Segalés, Emmanuel Serrano
Reactive oxygen and nitrogen species (ROS-RNS) are important defence substances involved in the immune response against pathogens. An excessive increase in ROS-RNS, however, can damage the organism causing oxidative stress (OS). The organism is able to neutralise OS by the production of antioxidant enzymes (AE); hence, tissue damage is the result of an imbalance between oxidant and antioxidant status. Though some work has been carried out in humans, there is a lack of information about the oxidant/antioxidant status in the presence of tuberculosis (TB) in wild reservoirs...
2016: PloS One
Daniel Peckham, Thomas Scambler, Sinisa Savic, Michael F McDermott
Immune-mediated autoinflammatory diseases are occupying an increasingly prominent position among the pantheon of debilitating conditions that afflict mankind. This review focuses on some of the key developments which have occurred since the original description of autoinflammatory disease in 1999, and focuses on underlying mechanisms that trigger autoinflammation. The monogenic autoinflammatory disease range has expanded considerably during that time, and now includes a broad spectrum of disorders, including relatively common conditions such as cystic fibrosis and subsets of systemic lupus erythematosus...
September 29, 2016: Journal of Pathology
Isabella Quinti, Federica Pulvirenti, Patrizia Giannantoni, Joud Hajjar, Debra L Canter, Cinzia Milito, Damiano Abeni, Jordan S Orange, Stefano Tabolli
BACKGROUND: Generic health status quality of life (QoL) instruments have been used in patients with common variable immune deficiency (CVID). However, by their nature, these tools may over- or underestimate the impact of diseases on an individual's QoL. OBJECTIVE: The objective of this study was to develop and validate a questionnaire to measure specific-health-related QoL for adults with CVID (CVID_QoL). METHODS: The 32-item content of the CVID_QoL questionnaire was developed using focus groups and individual patient interviews...
September 21, 2016: Journal of Allergy and Clinical Immunology in Practice
Abdelrahim Abdrabou Sadek, Mostafa Ashry Mohamed, Ashraf Abou-Taleb, Marwa Ibrahim Mohammed
INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is an immune mediated disease of the brain. Although it occurs in all ages, most reported cases are in children and adolescents. The aims of this study were to study the clinical pattern and outcome of ADEM in children in a tertiary center in Upper Egypt and to determine the effect of combined use of steroids and IVIg on outcome. METHODS: This observational study was carried out from January 2014 through December 2014 in the Pediatric Department of Sohag University Hospital (Egypt)...
July 2016: Electronic Physician
T V Latysheva, E A Latysheva, I A Martynova, G E Aminova
Primary immunodeficiencies (PIDs) are a group of congenital diseases of the immune system, which numbers more than 230 nosological entities associated with lost, decreased, or wrong function of its one or several components. Due to the common misconception that these are extremely rare diseases that occur only in children and lead to their death at an early age, PIDs are frequently ruled out by physicians of related specialties from the range of differential diagnosis. The most common forms of PIDs, such as humoral immunity defects, common variable immune deficiency, X-linked agammaglobulinemia, selective IgA deficiency, etc...
2016: Terapevticheskiĭ Arkhiv
A I Parfenov, L M Krums
The paper presents a variety of clinical manifestations of malabsorption syndrome (MAS) in celiac disease, collagenous sprue, Whipple's disease, Crohn's disease, intestinal lymphangiectasia, amyloidosis, common variable immune deficiency, and treatment of short bowel syndrome. It shows the specific features of the pathophysiology, diagnosis, and treatment of MAS in small bowel diseases.
2016: Terapevticheskiĭ Arkhiv
Arnald Alonso, Antonio Julià, Maria Vinaixa, Eugeni Domènech, Antonio Fernández-Nebro, Juan D Cañete, Carlos Ferrándiz, Jesús Tornero, Javier P Gisbert, Pilar Nos, Ana Gutiérrez Casbas, Lluís Puig, Isidoro González-Álvaro, José A Pinto-Tasende, Ricardo Blanco, Miguel A Rodríguez, Antoni Beltran, Xavier Correig, Sara Marsal
BACKGROUND: Immune-mediated inflammatory diseases (IMIDs) are a group of complex and prevalent diseases where disease diagnostic and activity monitoring is highly challenging. The determination of the metabolite profiles of biological samples is becoming a powerful approach to identify new biomarkers of clinical utility. In order to identify new metabolite biomarkers of diagnosis and disease activity, we have performed the first large-scale profiling of the urine metabolome of the six most prevalent IMIDs: rheumatoid arthritis, psoriatic arthritis, psoriasis, systemic lupus erythematosus, Crohn's disease, and ulcerative colitis...
2016: BMC Medicine
Magdalena Frączyk, Grzegorz Woźniakowski, Andrzej Kowalczyk, Łukasz Bocian, Edyta Kozak, Krzysztof Niemczuk, Zygmunt Pejsak
African swine fever (ASF) is a notifiable and one of the most complex and devastating infectious disease of pigs, wild boars and other representatives of Suidae family. African swine fever virus (ASFV) developed various molecular mechanisms to evade host immune response including alteration of interferon production by multigene family protein (MGF505-2R), inhibition of NF-κB and nuclear activating factor in T-cells by the A238L protein, or modulation of host defense by CD2v lectin-like protein encoded by EP402R and EP153R genes...
September 25, 2016: Veterinary Microbiology
Pascal I Hablützel, Martha Brown, Ida M Friberg, Joseph A Jackson
BACKGROUND: The effect of anthropogenic environments on the function of the vertebrate immune system is a problem of general importance. For example, it relates to the increasing rates of immunologically-based disease in modern human populations and to the desirability of identifying optimal immune function in domesticated animals. Despite this importance, our present understanding is compromised by a deficit of experimental studies that make adequately matched comparisons between wild and captive vertebrates...
2016: BMC Evolutionary Biology
Xiaoying Xu, Hao Song, Jianxun Qi, Yuqian Liu, Haiyuan Wang, Chao Su, Yi Shi, George F Gao
The association of Zika virus (ZIKV) infections with microcephaly and neurological diseases has highlighted an emerging public health concern. Here, we report the crystal structure of the full-length ZIKV nonstructural protein 1 (NS1), a major host-interaction molecule that functions in flaviviral replication, pathogenesis, and immune evasion. Of note, a long intertwined loop is observed in the wing domain of ZIKV NS1, and forms a hydrophobic "spike", which can contribute to cellular membrane association. For different flaviviruses, the amino acid sequences of the "spike" are variable but their common characteristic is either hydrophobic or positively charged, which is a beneficial feature for membrane binding...
August 30, 2016: EMBO Journal
Jarrett E Walsh, Jose G Gurrola, Scott M Graham, Sarah L Mott, Zuhair K Ballas
BACKGROUND: Patients with primary antibody deficiencies have an increased frequency of sinonasal and pulmonary infections. Immunoglobulin (Ig) replacement is a standard therapy for common variable immunodeficiency (CVID) and other antibody deficiency diseases. Although there is convincing evidence that Ig replacement reduces pulmonary infections, there is little evidence that it reduces sinus infections or abates chronic rhinosinusitis (CRS). This study aims to identify the impact of Ig replacement on CRS in antibody deficiencies...
August 23, 2016: International Forum of Allergy & Rhinology
Mahisa Mokhtari, Alireza Shakeri, Babak Mirminachi, Hassan Abolhassani, Reza Yazdani, Bodo Grimbacher, Asghar Aghamohammadi
BACKGROUND: Common variable immunodeficiency (CVID) is a primary immune deficiency with heterogeneous complications. The purpose of this study is to determine disease severity in a cohort of CVID patients based on the suggested scoring system and investigate predisposing factors which would be helpful to predict the severity of the disease. METHODS: The study population comprised 113 CVID patients (69 males and 44 females) who were visited at Children's Medical Center (Pediatrics Center of Excellence affiliated to Tehran University of Medical Sciences, Tehran, Iran) during the last 30 years (from 1984-2014)...
August 2016: Archives of Iranian Medicine
Jeremy Honaker, Christie Ammons, Teresa Kelechi
PURPOSE: To provide information about initiating interdisciplinary research related to microbiomes and their role in human immunity, disease, and metabolic processes. TARGET AUDIENCE: This continuing education activity is intended for physicians and nurses with an interest in skin and wound care. OBJECTIVES: After participating in this educational activity, the participant should be better able to:1. Describe techniques to identify and characterize bacterial bioburden...
September 2016: Advances in Skin & Wound Care
Silje F Jørgensen, Henrik M Reims, Didrik Frydenlund, Kristian Holm, Vemund Paulsen, Annika E Michelsen, Kristin K Jørgensen, Liv T Osnes, Jorunn Bratlie, Tor J Eide, Christen P Dahl, Ellen Holter, Rune R Tronstad, Kurt Hanevik, Hans-Richard Brattbakk, Fatemeh Kaveh, Torunn Fiskerstrand, Anne-Marte B Kran, Thor Ueland, Tom H Karlsen, Pål Aukrust, Knut E A Lundin, Børre Fevang
OBJECTIVES: The objective of this study was to study the prevalence of gastrointestinal (GI) symptoms and histopathology in patients with common variable immunodeficiency (CVID) as well as linking the findings to GI infections and markers of systemic immune activation. METHODS: In this cross-sectional study, we addressed GI symptoms in 103 patients and GI histopathological findings in 53 patients who underwent upper and lower endoscopic examination. The most frequent histopathological findings were linked to GI symptoms, B-cell phenotype, and markers of systemic immune activation (soluble (s)CD14, sCD25, and sCD163)...
October 2016: American Journal of Gastroenterology
Rémi Bertinchamp, Laurence Gérard, David Boutboul, Marion Malphettes, Claire Fieschi, Eric Oksenhendler
BACKGROUND: In 2014, the European Society for Immune Deficiencies (ESID) revised the common variable immunodeficiency (CVID) diagnosis criteria by incorporating new clinical and biological markers. The new definition appeared more restrictive but had not yet been evaluated in a large cohort of patients. OBJECTIVE: The objective of this study was to evaluate the impact of this new definition in a large cohort of patients with primary hypogammaglobulinemia. METHODS: Evaluation of 3 different CVID definitions (ESID/Pan-American Group for Immunodeficiency [PAGID] 1999, ESID 2014, DEFI 2015) in 521 patients included in the French DEFI study with a diagnosis of primary hypogammaglobulinemia...
August 10, 2016: Journal of Allergy and Clinical Immunology in Practice
Ralph D Levinson, Madeline Yung, Akira Meguro, Elham Ashouri, Fei Yu, Nobuhisa Mizuki, Shigeaki Ohno, Raja Rajalingam
Cytotoxic T lymphocytes (CTL) and natural killer (NK) cells are killer lymphocytes that provide defense against viral infections and tumor transformation. Analogous to that of CTL, interactions of killer-cell immunoglobulin-like receptors (KIR) with specific human leukocyte antigen (HLA) class I ligands calibrate NK cell education and response. Gene families encoding KIRs and HLA ligands are located on different chromosomes, and feature variation in the number and type of genes. The independent segregation of KIR and HLA genes results in variable KIR-HLA interactions in individuals, which may impact disease susceptibility...
2016: PloS One
William Rae
Primary immunodeficiencies (PIDs) are a group of rare genetic diseases resulting in the impairment of one or more functions of the human immune system. Common variable immunodeficiency (CVID) is one of the most prevalent PIDs, yet despite extensive genetic analysis, most patients do not have a monogenetic diagnosis. This has led to the theory that CVID must be a polygenetic condition. An alternative theory to a monogenetic or polygenetic underlying cause of CVID is that it is epigenetic phenomena that are causal in the majority of CVID patients...
August 2, 2016: Archivum Immunologiae et Therapiae Experimentalis
Lina Gölz, Benedikt C Buerfent, Andrea Hofmann, Heiko Rühl, Nadine Fricker, Wiebke Stamminger, Johannes Oldenburg, James Deschner, Achim Hoerauf, Markus M Nöthen, Johannes Schumacher, Marc P Hübner, Andreas Jäger
UNLABELLED: Nickel-containing alloys are frequently used in the biomedical field, although, owing to corrosive processes metal ion leaching is inevitable. Due to nickel ion (Ni(2+)) leaching several adverse effects are described in the literature. However, only a few studies evaluated the genetic profile of Ni(2+) in human cells which is of great importance since nickel-induced effects differ between humans and mice as a result of species-specific receptor variability. Thus, we investigated gene expression induced by Ni(2+)in human monocytes using a transcriptome-wide approach determining new target genes implicated in nickel-induced pathologies...
October 1, 2016: Acta Biomaterialia
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