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Common variable immune deficiency

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.
September 21, 2016: Journal of Allergy and Clinical Immunology
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
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
Gholamreza Azizi, Hassan Abolhassani, Mohammad Hosein Asgardoon, Tina Alinia, Reza Yazdani, Javad Mohammadi, Nima Rezaei, Hans D Ochs, Asghar Aghamohammadi
INTRODUCTION: 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...
September 16, 2016: Expert Review of Clinical Immunology
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
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
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
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
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
Daan J Aan de Kerk, Machiel H Jansen, Stephen Jolles, Klaus Warnatz, Suranjith L Seneviratne, Ineke J M Ten Berge, Ester M M van Leeuwen, Taco W Kuijpers
Primary antibody deficiencies (PADs) are the most common immunodeficiency in humans, characterized by low levels of immunoglobulins and inadequate antibody responses upon immunization. These PADs may result from an early block in B cell development with a complete absence of peripheral B cells and lack of immunoglobulins. In the presence of circulating B cells, some PADs are genetically caused by a class switch recombination (CSR) defect, but in the most common PAD, common variable immunodeficiency (CVID), very few gene defects have as yet been characterized despite various phenotypic classifications...
October 2016: Journal of Clinical Immunology
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
Baerbel Keller, Zoltan Cseresnyes, Ina Stumpf, Claudia Wehr, Manfred Fliegauf, Alla Bulashevska, Susanne Usadel, Bodo Grimbacher, Marta Rizzi, Hermann Eibel, Raluca Niesner, Klaus Warnatz
BACKGROUND: Most patients with common variable immunodeficiency (CVID) present with severely reduced switched memory B-cell counts, and some display an increase of CD21(low) B-cell counts (CVID 21low), whereas others do not (CVID 21norm). Altered B-cell receptor (BCR) signaling might contribute to the defective memory formation observed in patients with CVID. OBJECTIVE: We sought to investigate canonical nuclear factor of κ light chain (NF-κB) signaling in B cells from patients with CVID as a central pathway in B-cell differentiation...
June 14, 2016: Journal of Allergy and Clinical Immunology
Aleksandra Szczawinska-Poplonyk, Katarzyna Tapolska-Jozwiak, Husam Samara
BACKGROUND: Hypogammaglobulinemia in early childhood is a common feature characterized by distinct intrinsic and extrinsic factors leading to disturbed peripheral blood lymphocyte homeostasis. Detailed flow cytometric immunophenotyping of the peripheral blood B cell compartment is an informative tool for delineating disturbed generation of B cell subpopulations crucial for the diagnosis of hypogammaglobulinemia in young children. METHODS: We analyzed by flow cytometry the proportions and absolute values of total, naïve, memory - non-switched and switched, transitional and immature B lymph cells as well as plasmablasts in the peripheral blood of 50 hypogammaglobulinemic children aged from 3 to 50 months...
2016: Italian Journal of Pediatrics
Alessia Calgani, Marco Iarlori, Vincenzo Rizi, Gianna Pace, Mauro Bologna, Carlo Vicentini, Adriano Angelucci
Hypovitaminosis D is increasingly recognized as a cofactor in several diseases. In addition to bone homeostasis, vitamin D status influences immune system, muscle activity and cell differentiation in different tissues. Vitamin D is produced in the skin upon exposure to UVB rays, and sufficient levels of serum 25(OH)D are dependent mostly on adequate sun exposure, and then on specific physiologic variables, including skin type, age and Body Mass Index (BMI). In contrast with common belief, epidemiologic data are demonstrating that hypovitaminosis D must be a clinical concern not only in northern Countries...
September 2016: Journal of Photochemistry and Photobiology. B, Biology
Camila de Lollo, Dewton de Moraes Vasconcelos, Luanda Mara da Silva Oliveira, Rosana Domingues, Gabriel Costa de Carvalho, Alberto José da Silva Duarte, Maria Notomi Sato
Common variable immunodeficiency (CVID) is the most common symptomatic primary antibody deficiency and is associated with recurrent infections and chronic inflammatory diseases. We evaluated the ability of Toll-like receptor (TLR) ligands to induce secretion of chemokines, cytokines and type I interferons by peripheral blood mononuclear cells (PBMCs) from CVID patients. High levels of CXCL10, CCL2, CXCL9, CCL5, CXCL8, and IL-6 were detected in sera of CVID patients compared with healthy controls. Increased chemokine levels were observed in unstimulated PBMCs, but after stimulation with TLR2 and TLR4 agonists, equivalent chemokine and pro-inflammatory cytokine secretion, as in healthy controls, was observed, whereas TLR4 agonist induced a decreased secretion of CCL2 and CXCL8 and increased secretion of TNF...
August 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
Lamia Sfaihi, Dominique Stoppa Lyonnet, Salma Ben Ameur, Catherine Dubois D'enghien, Thouraya Kamoun, Mohamed Ridha Barbouch, Mongia Hachicha
BACKGROUND: Ataxia-telangiectasia (A-T) is a multisystem disorder characterized by progressive neurologic impairment, variable immunodeficiency, impaired organ maturation, X-ray hypersensitivity, oculocutaneous telangiectasia, and a predisposition to malignancy. AIM: We performed this study in order to describe clinical, immunological and molecular features of patients with AT followed in the south of Tunisia Methods: we performed a retrospective study (1996-2012) in the south of Tunisia about all cases of A-T in order to describe their clinical, immunological and molecular features...
August 2015: La Tunisie Médicale
Heidrun Boztug, Tatjana Hirschmugl, Wolfgang Holter, Karoly Lakatos, Leo Kager, Doris Trapin, Winfried Pickl, Elisabeth Förster-Waldl, Kaan Boztug
PURPOSE: NF-κB signaling is critically important for regulation of both innate and adaptive immune responses. While activation of NF-κB has been implicated in malignancies such as leukemia and lymphoma, loss-of-function mutations affecting different NF-κB pathway components have been shown to cause primary immunodeficiency disorders. Recently, haploinsufficiency of NF-κB1 has been described in three families with common variable immunodeficiency (CVID). METHODS AND RESULTS: We studied a patient with recurrent respiratory infections and bacterial parapharyngeal abscess...
August 2016: Journal of Clinical Immunology
Mohini Pathria, Daniel Urbine, Marc Stuart Zumberg, Juan Guarderas
A 61-year-old woman presented with longstanding cough and progressive dyspnoea. She underwent an extensive evaluation and was diagnosed with common variable immunodeficiency (CVID) with granulomatous lymphocytic interstitial lung disease (GLILD). She was initially treated with subcutaneous immunoglobulin therapy, having declined intravenous immunoglobulin (IVIG) therapy. She also declined treatment with oral glucocorticoids. Over several months, she became increasingly symptomatic and developed increased pulmonary infiltrates, pleural effusions, mediastinal adenopathy, splenomegaly, pancytopenia and ascites...
2016: BMJ Case Reports
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