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Common variable immune deficiency

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https://www.readbyqxmd.com/read/28095323/pattern-recognitions-receptors-in-immunodeficiency-disorders
#1
Esameil Mortaz, Ian M Adcock, Payam Tabarsi, Ilad Alavi Darazam, Masoud Movassaghi, Johan Garssen, Hamidreza Jamaati, Aliakbar Velayati
Pattern recognition receptors (PRRs) recognize common microbial or host-derived macromolecules and have important roles in early activation and response of the immune system. Initiation of the innate immune response starts with the recognition of microbial structures called pathogen associated molecular patterns (PAMPs). Recognition of PAMPs is performed by germline-encoded receptors expressed mainly on immune cells termed pattern recognition receptors (PRRs). Several classes of pattern recognition receptors (PRRs) are involved in the pathogenesis of diseases, including Toll-like receptors (TLRs), C-type lectin receptors (CLRs), and Nod-like receptors (NLRs)...
January 14, 2017: European Journal of Pharmacology
https://www.readbyqxmd.com/read/28093361/neil1-is-a-candidate-gene-associated-with-common-variable-immunodeficiency-in-a-patient-with-a-chromosome-15q24-deletion
#2
Rosa Romano, Apostolos Zaravinos, Kyriaki Liadaki, Rozina Caridha, Johanna Lundin, Göran Carlsson, Jacek Winiarski, Qiang Pan-Hammarström, Lennart Hammarström
We report the first patient with an interstitial deletion of chromosome 15q24.1-q24.3 associated with common variable immunodeficiency (CVID). The 18-year old female patient's clinical and immunological phenotype was compared with 8 additional previously published patients with chr15q24 deletions. A CGH analysis estimated the deletion to be 3.767Mb in size (chr15: 74,410,916-78,178,418) and the result was confirmed using qRT-PCR. We defined an immune-related commonly deleted region (ICDR) within the chromosomal band 15q24...
January 13, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28092390/asthma-comparing-the-impact-of-vitamin-d-versus-uvr-on-clinical-and-immune-parameters
#3
Kylie A Morgan, Elizabeth H Mann, Antony R Young, Catherine M Hawrylowicz
The incidence of asthma has increased markedly since the 1960s and is currently estimated to affect more than 300 million individuals worldwide. A number of environmental factors are implicated in asthma pathogenesis, one of which is vitamin D. Vitamin D deficiency is a global health concern and has increased in parallel with asthma incidence. Epidemiological studies report associations between low vitamin D status, assessed as circulating levels of 25-hydroxyvitamin D, with asthma incidence, severity, exacerbations and responses to treatment...
January 16, 2017: Photochemical & Photobiological Sciences
https://www.readbyqxmd.com/read/28068853/chronic-lung-disease-in-common-variable-immune-deficiency-cvid-a-pathophysiological-role-for-microbial-and-non-b-cell-immune-factors
#4
Denver Mooney, David Edgar, Gisli Einarsson, Damian Downey, Stuart Elborn, Michael Tunney
One of the most common and most severe forms of primary antibody deficiency encountered in the clinical setting is a heterogeneous group of syndromes termed common variable immune deficiency (CVID). This disorder is characterized by reduced immunoglobulin production and increased susceptibility to infection, particularly of the respiratory tract. Infection and subsequent immunological/inflammatory processes may contribute to the development of pulmonary complications such as bronchiectasis and interstitial lung disease...
January 10, 2017: Critical Reviews in Microbiology
https://www.readbyqxmd.com/read/28017520/microbial-dysbiosis-in-common-variable-immune-deficiencies-evidence-causes-and-consequences
#5
REVIEW
Roos-Marijn Berbers, Stefan Nierkens, Jacob M van Laar, Debby Bogaert, Helen L Leavis
Common variable immunodeficiency (CVID) is an immune disorder that not only causes increased susceptibility to infection, but also to inflammatory complications such as autoimmunity, lymphoid proliferation, malignancy, and granulomatous disease. Recent findings implicate the microbiome as a driver of this systemic immune dysregulation. Here, we critically review the current evidence for a role of the microbiome in the pathogenesis of CVID immune dysregulation, and describe the possible immunologic mechanisms behind causes and consequences of microbial dysbiosis in CVID...
December 22, 2016: Trends in Immunology
https://www.readbyqxmd.com/read/28005038/-common-variable-immunodeficiency-in-adults
#6
N V Shabashova, L V Filippova, A E Uchevatkina, E V Frolova
The paper analyzes 7 cases of common variable immune deficiency (CVID), a primary immunodeficiency disease. All the cases were detected in outpatients over the age of 40 years. The diagnosis was based on their history data and general clinical findings with due regard for the results of previously conducted functional studies, expert opinions, and the results of immunological studies including the quantitative and functional indices of T and B cells, phagocytes and the levels of immunoglobulins. The analysis showed that the early signs of impaired immunity in all the patients were seen by physicians of various specialties in both outpatient and inpatient settings...
2016: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/27942026/characterisation-of-the-immunophenotype-of-dogs-with-primary-immune-mediated-haemolytic-anaemia
#7
James W Swann, Kelly Woods, Ying Wu, Barbara Glanemann, Oliver A Garden
BACKGROUND: Immune-mediated haemolytic anaemia (IMHA) is reported to be the most common autoimmune disease of dogs, resulting in significant morbidity and mortality in affected animals. Haemolysis is caused by the action of autoantibodies, but the immunological changes that result in their production have not been elucidated. AIMS: To investigate the frequency of regulatory T cells (Tregs) and other lymphocyte subsets and to measure serum concentrations of cytokines and peripheral blood mononuclear cell expression of cytokine genes in dogs with IMHA, healthy dogs and dogs with inflammatory diseases...
2016: PloS One
https://www.readbyqxmd.com/read/27931306/idiopathic-cd4-lymphocytopenia
#8
Joel P Brooks, Gisoo Ghaffari
BACKGROUND: Idiopathic CD4 lymphocytopenia (ICL) is a rare disorder of unknown etiology. Diagnostic criteria include a persistent CD4 T-cell lymphopenia with no underlying primary or secondary immune deficiencies and a CD4 T-cell count of 300 cells/mL or 20% total lymphocyte on multiple occasions. OBJECTIVE: To increase awareness of ICL and to provide a review of the clinical characteristics, diagnosis, and management of this disease process. Presently, many of these patients receive prophylactic treatment similar to other T-cell deficient conditions, most notably patients with human immunodeficiency virus (HIV) or acquired immunodeficiency syndrome; however, the same indications may not necessarily apply to this patient population because the T cells are not affected by a virus as in HIV...
November 2016: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/27865716/common-variable-immune-deficiency-associated-with-pemphigoid
#9
Rayna Doll, John Johnson, Haig Tcheurekdjian, Robert Hostoffer
No abstract text is available yet for this article.
January 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/27836055/lung-disease-in-primary-antibody-deficiencies
#10
REVIEW
Edith Schussler, Mary B Beasley, Paul J Maglione
Primary antibody deficiencies (PADs) are the most common form of primary immunodeficiency and predispose to severe and recurrent pulmonary infections, which can result in chronic lung disease including bronchiectasis. Chronic lung disease is among the most common complications of PAD and a significant source of morbidity and mortality for these patients. However, the development of lung disease in PAD may not be solely the result of recurrent bacterial infection or a consequence of bronchiectasis. Recent characterization of monogenic immune dysregulation disorders and more extensive study of common variable immunodeficiency have demonstrated that interstitial lung disease (ILD) in PAD can result from generalized immune dysregulation and frequently occurs in the absence of pneumonia history or bronchiectasis...
November 2016: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/27821991/a-study-to-assess-empowerment-of-adolescent-girls-in-terms-of-knowledge-based-life-skills-education-about-child-survival-and-safe-motherhood-practices
#11
A K Choudhary, D M Saxena, Rituja Kaushal
OBJECTIVE: This study has been designed with the intention to bring a substantial change in the mindsets or life skills of adolescent girls at secondary school level regarding their concept of child survival and safe motherhood practices in order to deal effectively with real-life situations. METHODOLOGY: A cross-sectional analytical study was carried out with 538 adolescent girls regarding assessment of their knowledge on variables like correct nutrition, iron-deficiency anemia and its prevention, care during pregnancy, safe birth, skilled birth attendance, breast feeding and complementary feeding practices, child's growth and development issues, immunization, management of common childhood illnesses...
December 2016: Journal of Obstetrics and Gynaecology of India
https://www.readbyqxmd.com/read/27795213/-clinic-of-humoral-primary-immunodeficiencies-in-adults-experience-in-a-tertiary-hospital
#12
Julio César Cambray-Gutiérrez, Diana Andrea Herrera-Sánchez, Lizbeth Blancas-Galicia, Patricia María O'Farrill-Romanillos
BACKGROUND: Primary immunodeficiencies (PID) are characterized by alteration of the components of the immune system. Humoral deficiencies represent 50%. The most common are selective IgA deficiency, Bruton agammaglobulinemia, and common variable immunodeficiency (CVID). OBJECTIVE: To describe the epidemiological and clinical characteristics of adults with humoral PID, cared for in a Primary Humoral Immunodeficiencies Clinic. METHODS: A descriptive cross-sectional study that included a year of analysis, including 35 patients with humoral PID, 31 with CVID, and 4 with Bruton agammaglobulinemia...
October 2016: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/27665490/killer-cell-immunoglobulin-like-receptors-are-associated-with-common-variable-immune-deficiency-pathogenesis
#13
Yuge Wang, Tracy Hwangpo, Maureen P Martin, Nicolas Vince, Ying Qi, Richard J Reynolds, Devin Absher, Xiaojiang Gao, Carol A Ballinger, Peter D Burrows, T Prescott Atkinson, Elizabeth E Brown, Ada Elgavish, Cunren Liu, Mary Carrington, Harry W Schroeder
No abstract text is available yet for this article.
November 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27665385/development-and-initial-validation-of-a-questionnaire-to-measure-health-related-quality-of-life-of-adults-with-common-variable-immune-deficiency-the-cvid_qol-questionnaire
#14
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
https://www.readbyqxmd.com/read/27636936/-pulmonary-manifestations-in-adult-patients-with-a-defect-in-humoral-immunity
#15
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
https://www.readbyqxmd.com/read/27636919/-malabsorption-is-a-leading-clinical-sign-of-small-bowel-disease
#16
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
https://www.readbyqxmd.com/read/27636680/autoimmunity-in-common-variable-immunodeficiency-epidemiology-pathophysiology-and-management
#17
Gholamreza Azizi, Hassan Abolhassani, Mohammad Hosein Asgardoon, Tina Alinia, Reza Yazdani, Javad Mohammadi, Nima Rezaei, Hans D Ochs, Asghar Aghamohammadi
Common variable immunodeficiency (CVID) comprises a large heterogeneous group of patients with primary antibody deficiency. Areas covered: The affected patients are characterized by increased susceptibility to infections and low levels of serum immunoglobulin. However, enteropathy, granulomatous organ infiltrates, malignancy, inflammatory and autoimmune conditions are also prevalent. The concomitance of immunodeficiency and autoimmunity appears to be paradoxical and creates difficulties in the management of autoimmune complications affecting these patients...
February 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/27614471/clonal-lymphoproliferations-in-a-patient-with-common-variable-immunodeficiency
#18
Sarah A Williams, Laura E Moench, Fatima Khan, Gregory Vercellotti, Michael A Linden
Common variable immunodeficiency (CVID) comprises a heterogeneous group of disorders of humoral immunity, characterized by marked deficiencies in serum immunoglobulins. Immune dysregulation causes susceptibility to recurrent bacterial infections, as well as autoimmune and lymphoproliferative disorders. Although the lymphoid cells comprising the atypical proliferations are often clonally related, their malignant potential and clinical significance differ from similar lesions in individuals with immunocompetence...
November 2016: Laboratory Medicine
https://www.readbyqxmd.com/read/27594285/incidence-of-parasitic-diarrhea-in-patients-with-common-variable-immune-deficiency
#19
Serhat Uysal, Varol Tunalı, Eylem Akdur Öztürk, Ömür Ardeniz, Meltem Işıkgöz Taşbakan, Hüsnü Pullukçu, Seray Özensoy Töz, Nevin Turgay, Bilgin Arda
OBJECTIVE: Parasites might cause atypical and severe infections in immunocompromised hosts. The prevalence of diarrhea among common variable immune deficiency (CVID) syndrome patients varies between 20% and 94%, which indicates that diarrhea and gastrointestinal system (GIS) complaints could be the second leading cause of morbidity in CVID patients after respiratory tract infections. This study aimed to assess the prevalence of intestinal parasites in CVID patients with GIS complaints and diarrhea...
June 2016: Türkiye Parazitolojii Dergisi
https://www.readbyqxmd.com/read/27577557/vitamin-d-levels-and-influenza-vaccine-immunogenicity-among-hiv-infected-and-hiv-uninfected-adults
#20
Nancy F Crum-Cianflone, Seunghyun Won, Rachel Lee, Tahaniyat Lalani, Anuradha Ganesan, Timothy Burgess, Brian K Agan
BACKGROUND: Vaccination is the most important preventive strategy against influenza, however post-vaccination antibody responses are often inadequate especially among HIV-infected persons. Vitamin D deficiency has been suggested to adversely influence immune responses and is highly prevalent among HIV-infected adults. Therefore, we evaluated the association between 25-hydroxyvitamin D [25(OH)D] levels and post-influenza vaccination responses. METHODS: We conducted a prospective cohort study evaluating the immunogenicity of monovalent influenza A (H1N1) vaccination among both HIV-infected and HIV-uninfected adults (18-50years of age) during the 2009-2010 influenza season...
September 22, 2016: Vaccine
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