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https://www.readbyqxmd.com/read/28805315/a-comparison-of-clinical-and-immunologic-phenotypes-in-familial-and-sporadic-forms-of-common-variable-immunodeficiency
#1
Amir Valizadeh, Reza Yazdani, Gholamreza Azizi, Hassan Abolhassani, Asghar Aghamohammadi
Common variable immunodeficiency (CVID) is the most frequent symptomatic primary immunodeficiency disease and its prevalence varies significantly among different population. Minority of CVID patients present a familial aggregation suggesting a higher probability of heritable genetic defects. A total of 235 registered CVID patients were evaluated in this cohort study. Familial and sporadic patients were stratified and demographic information, clinical records, laboratory and molecular data were compared among these two groups of patients...
August 11, 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/28781847/management-of-common-variable-immunodeficiency-by-subcutaneous-igg-self-administration-during-pregnancy-a-case-report
#2
Carolina Marasco, Alberta Venturelli, Luigia Rao, Angelo Vacca, Maria Rosaria Carratù
Patients with common variable immunodeficiency are prone to infections, and this poses a particular challenge during pregnancy, when the requirement for immunoglobulin (Ig) replacement therapy is even more demanding so as to achieve an effective protection also of the fetus. This case report highlights the benefits observed with subcutaneous IgG self-administration in the management of common variable immunodeficiency (CVID) during pregnancy, in terms of efficacy and safety.
August 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28755066/two-sides-of-the-same-coin-pediatric-onset-and-adult-onset-common-variable-immune-deficiency
#3
Lauren A Sanchez, Solrun Melkorka Maggadottir, Matthew S Pantell, Patricia Lugar, Charlotte Cunningham Rundles, Kathleen E Sullivan
PURPOSE: Common variable immunodeficiency (CVID) is a complex, heterogeneous immunodeficiency characterized by hypogammaglobulinemia, recurrent infections, and poor antibody response to vaccination. While antibiotics and immunoglobulin prophylaxis have significantly reduced infectious complications, non-infectious complications of autoimmunity, inflammatory lung disease, enteropathy, and malignancy remain of great concern. Previous studies have suggested that CVID patients diagnosed in childhood are more severely affected by these complications than adults diagnosed later in life...
July 28, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28735808/autoimmunity-and-its-association-with-regulatory-t-cells-and-b-cell-subsets-in-patients-with-common-variable-immunodeficiency
#4
G Azizi, H Abolhassani, F Kiaee, N Tavakolinia, H Rafiemanesh, R Yazdani, S A Mahdaviani, S Mohammadikhajehdehi, M Tavakol, V Ziaee, B Negahdari, J Mohammadi, A Mirshafiey, A Aghamohammadi
BACKGROUND: Common variable immunodeficiency (CVID) is one of the most prevalent symptomatic primary immunodeficiencies (PIDs), which manifests a wide clinical variability such as autoimmunity, as well as T cell and B cell abnormalities. METHODS: A total of 72 patients with CVID were enrolled in this study. Patients were evaluated for clinical manifestations and classified according to the presence or absence of autoimmune disease. We measured regulatory T cells (Tregs) and B-cell subsets using flow cytometry, as well as specific antibody response (SAR) to pneumococcal vaccine, autoantibodies and anti-IgA in patients...
July 20, 2017: Allergologia et Immunopathologia
https://www.readbyqxmd.com/read/28734862/respiratory-infections-and-antibiotic-usage-in-common-variable-immunodeficiency
#5
Johannes M Sperlich, Bodo Grimbacher, Sarita Workman, Tanzina Haque, Suranjith L Seneviratne, Siobhan O Burns, Veronika Reiser, Werner Vach, John R Hurst, David M Lowe
BACKGROUND: Patients with common variable immunodeficiency (CVID) suffer frequent respiratory tract infections despite immunoglobulin replacement and are prescribed significant quantities of antibiotics. The clinical and microbiological nature of these exacerbations, the symptomatic triggers to take antibiotics, and the response to treatment have not been previously investigated. OBJECTIVES: To describe the nature, frequency, treatment, and clinical course of respiratory tract exacerbations in patients with CVID and to describe pathogens isolated during respiratory tract exacerbations...
July 19, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28722362/-transient-hypogammaglobulinemia-of-infancy
#6
Felicitas Bellutti Enders, Francesca Conti, Fabio Candotti, Federica Angelini
Transient hypogammaglobulinemia of infancy is characterized as a reduction of one or more classes of immunoglobulins with a response to vaccines and normal subpopulations of lymphocytes B presenting in the first years of life. The diagnosis is made a posteriori, once the levels of immunoglobulins are normalized, in general between 2 and 4 years of age. Clinical presentation varies : the child may be either asymptomatic or present with recurrent infections, atopy and / or auto-immunity. There are no clinical or immunological features that distinguish this condition from a common variable immunodeficiency (CVID)...
April 5, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28721392/the-response-to-typhi-vi-vaccination-is-compromised-in-individuals-with-primary-immunodeficiency
#7
Jeevani Kumarage, Suranjith L Seneviratne, Vijitha Senaratne, Amitha Fernando, Kirthi Gunasekera, Bandu Gunasena, Padmalal Gurugama, Sudath Peiris, Antony R Parker, Stephen Harding, Nilhan Rajiva de Silva
Measurement of an individuals ability to respond to polysaccharide antigens is a crucial test to determine adaptive immunity. Currently the response to Pneumovax(®) is utilized but with the success of Prevnar(®), measurement of the response to Pneumovax may be challenging. The aim of the study was to assess the response to Typhi Vi vaccination in both children and adult control groups and patients with primary immunodeficiency (PID). In the control groups, >95% of the individuals had pre Typhi Vi vaccination concentrations <100 U/mL and there was significant increase in concentration post Typhi Vi vaccination (p<0...
June 2017: Heliyon
https://www.readbyqxmd.com/read/28718914/reduced-numbers-of-circulating-group-2-innate-lymphoid-cells-in-patients-with-common-variable-immunodeficiency
#8
Christoph B Geier, Sophie Kraupp, David Bra, Martha M Eibl, Jocelyn R Farmer, Krisztian Csomos, Jolan E Walter, Hermann M Wolf
Recent studies identified an emerging role of group 2 and 3 innate lymphoid cells (ILCs) as key players in the generation of T-dependent and T-independent antibody production. In this retrospective case-control study CD117(+) ILCs (including the majority of ILC2 and ILC3) were reduced in patients with common variable immunodeficiency (CVID). The reduction in CD117(+) ILCs was distinctive to CVID and could not be observed in patients with X linked agammaglobulinemia (XLA). Patients with a more pronounced reduction in CD117(+) ILC numbers showed significantly lower numbers of peripheral MZ-like B cells and an increased prevalence of chronic, non-infectious enteropathy...
July 18, 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/28718689/orbital-diffuse-large-b-cell-lymphoma-with-combined-variable-immunodeficiency
#9
Vishal S Parikh, Deepa Jagadeesh, James M Fernandez, Eric D Hsi, Arun D Singh
Common variable immunodeficiency (CVID) is a primary immunodeficiency manifesting as a reduction in the level of total immunoglobulin (Ig) G, a reduction in the level of either IgA or IgM, poor response to polysaccharide vaccine, and usually frequent infections. The association of CVID with an increased risk of malignancy, specifically lymphoma, is well known. A 63-year-old female with a past medical history significant for CVID presented with a 1-month history of dull, left eye pain with proptosis, hypoglobus, and left upper lid fullness without a discrete palpable mass...
July 18, 2017: Orbit
https://www.readbyqxmd.com/read/28716658/stool-antigen-immunodetection-for-diagnosis-of-giardia-duodenalis-infection-in-human-subjects-with-hiv-and-cancer
#10
Maryam Nooshadokht, Behjat Kalantari-Khandani, Iraj Sharifi, Hossein Kamyabi, Namal P M Liyanage, Laurel A Lagenaur, Martin F Kagnoff, Steven M Singer, Zahra Babaei, Shahram Solaymani-Mohammadi
Human infection with the protozoan parasite Giardia duodenalis is one the most common parasitic diseases worldwide. Higher incidence rates of giardiasis have been reported from human subjects with multiple debilitating chronic conditions, including hypogammaglobulinemia and common variable immunodeficiency (CVID). In the current study, stool specimens were collected from 199 individuals diagnosed with HIV or cancer and immunocompetent subjects. The sensitivity of microscopy-based detection on fresh stool preparations, trichrome staining and stool antigen immunodetection for the diagnosis of G...
July 14, 2017: Journal of Microbiological Methods
https://www.readbyqxmd.com/read/28672090/efficacy-of-mycophenolate-on-lung-disease-and-autoimmunity-in-children-with-immunodeficiency
#11
Giorgia Bucciol, Angelamaria Petrone, Maria Caterina Putti
The autoimmune manifestations of primary immunodeficiencies, such as autoimmune lymphoproliferative syndrome (ALPS) and common variable immunodeficiency (CVID), often constitute a great therapeutic challenge and have a significant impact on patients' morbidity and mortality. The most common autoimmune presentations are autoimmune cytopenias, but organ-related autoimmunity is also frequently observed. From a pulmonology perspective, granulomatous/lymphocytic interstitial lung disease (GLILD) is a severe immunological complication which significantly worsens the clinical outcome of these patients and for which there are currently few guidelines or protocols for treatment...
July 3, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28658731/-angioedema-as-initial-manifestation-of-hypogammaglobulinemia
#12
Eunice López-Rocha, Patricia O'Farril-Romanillos, Saraid Cerda-Reyes, Edgar A Medina-Torres, Sara E Espinosa-Padilla, José G Huerta-López, Lizbeth Blancas-Galicia
Common variable immunodeficiency is characterized by hypogammaglobulinemia and the inability to respond to vaccines. Patients mostly manifest infections, however only less than 5 % have pathological conditions as autoimmunity, granulomatous inflammation, and splenomegaly or lymphoproliferative disease among others, without showing infections. We report the case of a woman who debuted with localized cutaneous affection, facial angioedema, without other early symptoms. After diagnosis splenomegaly and bronchiectasis were documented...
April 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/28651547/autoantibodies-against-baff-april-or-il21-an-alternative-pathogenesis-for-antibody-deficiencies
#13
Marian-Christopher Pott, Natalie Frede, Jennifer Wanders, Lennart Hammarström, Erik-Oliver Glocker, Cristina Glocker, Fariba Tahami, Bodo Grimbacher
BACKGROUND: The ability of anti-cytokine antibodies to play a disease-causing role in the pathogenesis of immunodeficiencies is widely accepted. The aim of this study was to investigate whether autoantibodies against BAFF (important B cell survival signal), APRIL (important plasma cell survival signal), or Interleukin-21 (important cytokine for immunoglobulin class switch) present an alternative mechanism for the development of the following primary antibody deficiencies (PADs): common variable immune deficiency (CVID) or selective IgA deficiency (sIgAD)...
June 26, 2017: BMC Immunology
https://www.readbyqxmd.com/read/28641569/a-review-on-defects-of-dendritic-cells-in-common-variable-immunodeficiency
#14
Laleh Sharifi, Naeimeh Tavakolinia, Fatemeh Kiaee, Nima Rezaei, Monireh Mohsenzadegan, Mansoureh Shariat, Reza Yazdani, Abbas Mirshafiey, Asghar Aghamohammadi, Gholamreza Azizi
BACKGROUND AND OBJECTIVES: Common variable immunodeficiency (CVID) is the most important primary that is associated with clinical complications including recurrent infections, malignancy and autoimmune diseases. The genetic cause of CVID is mostly unknown and only a few genetic causes are identified. The various options are proposed for determining the etiology of CVID patients, such as T- and B-cell defects, Toll-like receptors (TLRs) impairments, altered cytokine production as well as blemished dendritic cells (DCs)...
June 13, 2017: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/28623282/a-nationwide-study-of-severe-and-protracted-diarrhoea-in-patients-with-primary-immunodeficiency-diseases
#15
Wen-I Lee, Chien-Chang Chen, Tang-Her Jaing, Liang-Shiou Ou, Chuen Hsueh, Jing-Long Huang
Diarrhoea lasting longer than 14 days and failing to respond to conventional management is defined as severe and protracted diarrhoea (SD). In this study, we investigated the prevalence, pathogens and prognosis of SD in primary immunodeficiency diseases (PIDs). Among 246 patients with predominantly paediatric-onset PIDs from 2003-2015, 21 [Btk (six), IL2RG (four), WASP, CD40L, gp91 (three each), gp47, RAG2 (one each)] and five [CVID (four), SCID (one)] without identified mutations had SD before prophylactic treatment...
June 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28611475/murine-lrba-deficiency-causes-ctla-4-deficiency-in-tregs-without-progression-to-immune-dysregulation
#16
Deborah Burnett, Ian Parish, Etienne Masle-Farquhar, Robert Brink, Christopher Goodnow
Inherited mutations in Lipopolysaccharide Responsive Beige-like Anchor (LRBA) cause a recessive human immune dysregulation syndrome with memory B cell and antibody deficiency (common variable immunodeficiency, CVID), inflammatory bowel disease, enlarged spleen and lymph nodes, accumulation of activated T cells, and multiple autoimmune diseases. To understand the pathogenesis of the syndrome, C57BL/6 mice carrying a homozygous truncating mutation in Lrba were produced using CRISPR/Cas9-mediated gene targeting...
June 14, 2017: Immunology and Cell Biology
https://www.readbyqxmd.com/read/28606051/comparison-of-bone-mineral-density-in-common-variable-immunodeficiency-and-x-linked-agammaglobulinaemia-patients
#17
Ali Mohebbi, Gholamreza Azizi, Naeimeh Tavakolinia, Mehdi Karimipour, Fatemeh Kiaee, Reza Yazdani, Sareh Sadat Ebrahimi, Hosein Rafiemanesh, Vahid Ziaee, Hassan Abolhassani, Asghar Aghamohammadi, Farzaneh Abbasi, Fatemeh Sayarifard, Mehran Ebrahimi, Javad Tafaroji
BACKGROUND: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders, resulting from different defects in development and function of B cell lineage. Common variable immunodeficiency (CVID) and X-linked agammaglobulinemia (XLA) are two of the major types of PADs. Optimal growth and subsequently bone health could potentially compromise due to the interference of several factors in PAD with childhood onset. In the present study, our aim was to evaluate bone mineral density (BMD) of patients with CVID and XLA...
June 11, 2017: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/28600865/immune-deficiency-and-autoimmunity-in-patients-with-ctla-4-mutations
#18
REVIEW
Nisha Verma, Siobhan O Burns, Lucy Sk Walker, David M Sansom
Immune deficiency disorders are a heterogeneous group of diseases of variable genetic aetiology. Whilst the hallmark of immunodeficiency is susceptibility to infection, it is increasingly clear that autoimmunity is prevalent suggestive of a more general immune dysregulation in some cases. With the increasing use of genetic technologies the underlying causes of immune dysregulation are beginning to emerge. Here we provide a review of the heterozygous mutations found in the immune checkpoint protein CTLA-4, which was originally identified in cases of Common Variable Immunodeficiency Disorders (CVID) with accompanying autoimmunity...
June 10, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28591035/a-case-report-of-lymphoid-intestitial-pneumonia-in-common-variable-immunodeficiency-oligoclonal-expansion-of-effector-lymphocytes-with-preferential-cytomegalovirus-specific-immune-response-and-lymphoproliferative-disease-promotion
#19
Przemyslaw Zdziarski, Andrzej Gamian, Grzegorz Dworacki
RATIONALE: Lymphoid interstitial pneumonia (LIP) is a rare disease with lymphocytic infiltration of the alveolar interstitial and air spaces, sometimes classified as a clonal lymphoproliferative disease (LPD) with high prevalence in patients with immunodysregulation. Although association of mucosa-associated lymphoid tissue (MALT) lymphoma development with infectious agents has been well described, it is not so in the case of LIP. Attempts to demonstrate an infective cause by direct microbe detection have failed, but association with atypical specific immune response to opportunistic infectious agent has not been studied...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28554560/th1-phenotype-of-t-follicular-helper-cells-indicates-an-ifn%C3%AE-associated-immune-dysregulation-in-cd21low-cvid-patients
#20
Susanne Unger, Maximilian Seidl, Pauline van Schouwenburg, Mirzokhid Rakhmanov, Alla Bulashevska, Natalie Frede, Bodo Grimbacher, Jens Pfeiffer, Klaudia Schrenk, Luis Munoz, Leif Hanitsch, Ina Stumpf, Fabian Kaiser, Oliver Hausmann, Florian Kollert, Sigune Goldacker, Mirjam van der Burg, Baerbel Keller, Klaus Warnatz
BACKGROUND: A subgroup of patients with Common Variable Immunodeficiency (CVID) suffers from immune dysregulation, manifesting as autoimmunity, lymphoproliferation and organ inflammation, and thereby increasing morbidity and mortality. Therefore, treatment of these complications demands a deeper comprehension of their etiology and pathophysiology. OBJECTIVES: Based on the identification of an Interferon (IFN) signature in CVID patients with secondary complications and a skewed T follicular helper cell (TFH) differentiation in defined monogenic immunodeficiencies, we sought to determine the profile of CD4 memory T cells in blood and secondary lymphatic tissues of these patients...
May 26, 2017: Journal of Allergy and Clinical Immunology
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