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https://www.readbyqxmd.com/read/28093361/neil1-is-a-candidate-gene-associated-with-common-variable-immunodeficiency-in-a-patient-with-a-chromosome-15q24-deletion
#1
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/28068853/chronic-lung-disease-in-common-variable-immune-deficiency-cvid-a-pathophysiological-role-for-microbial-and-non-b-cell-immune-factors
#2
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/28054583/common-variable-immunodeficiency-patients-with-a-phenotypic-profile-of-immunosenescence-present-with-thrombocytopenia
#3
Jan Stuchlý, Veronika Kanderová, Marcela Vlková, Ivana Heřmanová, Lucie Slámová, Ondřej Pelák, Eli Taraldsrud, Dalibor Jílek, Pavlína Králíc Ková, Børre Fevang, Marie Trková, Ondřej Hrušák, Eva Froňková, Anna Šedivá, Jiří Litzman, Tomáš Kalina
Common variable immunodeficiency (CVID) is a heterogeneous group of diseases. Our aim was to define sub-groups of CVID patients with similar phenotypes and clinical characteristics. Using eight-color flow cytometry, we analyzed both B- and T-cell phenotypes in a cohort of 88 CVID patients and 48 healthy donors. A hierarchical clustering of probability binning "bins" yielded a separate cluster of 22 CVID patients with an abnormal phenotype. We showed coordinated proportional changes in naïve CD4+ T-cells (decreased), intermediate CD27- CD28+ CD4+ T-cells (increased) and CD21low B-cells (increased) that were stable for over three years...
January 5, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28017520/microbial-dysbiosis-in-common-variable-immune-deficiencies-evidence-causes-and-consequences
#4
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/28011187/mucosal-associated-invariant-t-cells-are-depleted-and-functionally-altered-in-patients-with-common-variable-immunodeficiency
#5
Serena Arduini, Jean Dunne, Niall Conlon, Conleth Feighery, Derek G Doherty
Common variable immunodeficiency (CVID) is a primary immunoglobulin deficiency characterized by recurrent infections and complications, including autoimmunity, enteropathy, polyclonal lymphocytic infiltration or lymphoid malignancy. Innate T cells can support B cell maturation and antibody production. We investigated the numbers, phenotypes and functions of circulating B cell, γδ T cell, invariant natural killer T (iNKT) cell and mucosal-associated invariant T (MAIT) cell subsets in 23 CVID patients and 27 healthy controls...
December 21, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
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/28000208/when-to-initiate-immunoglobulin-replacement-therapy-igrt-in-antibody-deficiency-a-practical-approach
#7
REVIEW
Stephen Jolles, Helen Chapel, Jiri Litzman
Primary antibody deficiencies (PAD) constitute the majority of all primary immunodeficiency diseases (PID) and immunoglobulin replacement forms the mainstay of therapy for many patients in this category. Secondary antibody deficiencies (SAD) represents a larger and expanding number of patients resulting from the use of a wide range of immunosuppressive therapies, in particular those targeting B cells, and may also result from renal or gastrointestinal immunoglobulin losses. While there are clear similarities between primary and secondary antibody deficiencies, there are also significant differences...
December 21, 2016: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27982172/salivary-immunoglobulins-in-individuals-with-common-variable-immunodeficiency
#8
Karin Sá Fernandes, Michella Bezerra Lima, Cíntia de Paula Martins, Maria Cristina Dos-Santos, Fabio Daumas Nunes, Cristina Maria Kokron, Marina Gallottini
Oral manifestations of common variable immunodeficiency (CVID) are rare, have rarely been studied and have given controversial results. There are few data about IgA, IgG, and IgM antibody salivary levels in the literature, and there are few papers about the clinical impact of antibody deficiencies and CVID on the oral health of such patients. The aim of this study was to measure serum and salivary IgA, IgG, and IgM levels in CVID participants and controls, and to associate immunoglobulin levels with caries and periodontal disease...
October 2016: Brazilian Dental Journal
https://www.readbyqxmd.com/read/27923702/nfkb1-regulates-human-nk-cell-maturation-and-effector-functions
#9
Vassilios Lougaris, Ornella Patrizi, Manuela Baronio, Giovanna Tabellini, Giacomo Tampella, Eufemia Damiati, Natalie Frede, Jos W M van der Meer, Manfred Fliegauf, Bodo Grimbacher, Silvia Parolini, Alessandro Plebani
NFKB1, a component of the canonical NF-κB pathway, was recently reported to be mutated in a limited number of CVID patients. CVID-associated mutations in NFKB2 (non-canonical pathway) have previously been shown to impair NK cell cytotoxic activity. Although a biological function of NFKB1 in non-human NK cells has been reported, the role of NFKB1 mutations for human NK cell biology and disease has not been investigated yet. We decided therefore to evaluate the role of monoallelic NFKB1 mutations in human NK cell maturation and functions...
December 3, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/27919819/patterns-of-constitutively-phosphorylated-kinases-in-b-cells-are-associated-with-disease-severity-in-common-variable-immunodeficiency
#10
Eli Taraldsrud, Pål Aukrust, Silje Jørgensen, Ole Christian Lingjærde, Johanna Olweus, June H Myklebust, Børre Fevang
Patients with common variable immunodeficiency (CVID) constitute a clinically and immunologically heterogeneous group characterized by B-cell dysfunction with hypogammaglobulinemia and defective immunoglobulin class switch of unknown etiology. Current classification systems are insufficient to achieve precise disease management. Characterization of signaling pathways essential for B-cell differentiation and class switch could provide new means to stratify patients. We evaluated constitutive and induced signaling by phospho-specific flow cytometry in 26 CVID patients and 18 healthy blood donors...
December 3, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/27902982/limitation-of-simultaneous-analysis-of-t-cell-receptor-and-%C3%AE%C2%BA-deleting-recombination-excision-circles-based-on-multiplex-real-time-polymerase-chain-reaction-in-common-variable-immunodeficiency-patients
#11
Faranaz Atschekzei, Fareed Ahmad, Torsten Witte, Roland Jacobs, Reinhold E Schmidt
AIM OF STUDY: We used a triplex real-time polymerase chain reaction (PCR) to classify our common variable immunodeficiency (CVID) patients into distinct groups according to the amount of their T-cell receptor excision circles (TRECs) and κ-deleting recombination excision circles (KRECs). MATERIALS AND METHODS: TREC and KREC analysis was performed using a multiplex real-time PCR assay. The T- and B-lymphocyte subsets were measured by flow cytometry. RESULTS: The copy number of TRECs and KRECs was significantly reduced in CVID patients compared to healthy controls...
2016: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/27896807/fdg-pet-ct-imaging-of-therapeutic-response-in-granulomatous-lymphocytic-interstitial-lung-disease-glild-in-common-variable-immunodeficiency-cvid
#12
S Jolles, E Carne, M Brouns, T El-Shanawany, P Williams, C Marshall, P Fielding
Common variable immunodeficiency (CVID) is the most common severe adult primary immunodeficiency and is characterized by a failure to produce antibodies leading to recurrent predominantly sinopulmonary infections. Improvements in the prevention and treatment of infection with immunoglobulin replacement and antibiotics have resulted in malignancy, autoimmune, inflammatory and lymphoproliferative disorders emerging as major clinical challenges in the management of patients who have CVID. In a proportion of CVID patients, inflammation manifests as granulomas that frequently involve the lungs, lymph nodes, spleen and liver and may affect almost any organ...
January 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27888588/lps-responsive-beige-like-anchor-gene-mutation-associated-with-possible-bronchiolitis-obliterans-organizing-pneumonia-associated-with-hypogammaglobulinemia-and-normal-igm-phenotype-and-low-number-of-b-cells
#13
Sima Shokri, Mohammad Nabavi, Tatjana Hirschmugl, Asghar Aghamohammadi, Saba Arshi, Mohamad Hassan Bemanian, Morteza Fallahpour, Rasool Molatefi, Mahsa Rekabi, Narges Eslami, Javad Ahmadian, Kian Darabi, Gholam Reza Sedighi, Maryam Monajemzadeh, Mohammadreza Modaresi, Nima Parvaneh, Kaan Boztug, Nima Rezaei
LPS-Responsive Beige-like Anchor (LRBA) deficiency is a disease which has recently been described in a group of patients with common variable immunodeficiency (CVID) in association with autoimmunity and/or inflammatory bowel disease (IBD)-like phenotype. We here describe a 10-year-old boy who experienced recurrent infections, mainly in the respiratory system, associated with thrombocytopenia and anemia. Immunological workup showed low numbers of B cells and low IgG, but normal IgM levels. In spite of therapeutic doses of antibiotics, antivirals, and antifungal agents, in addition to immunoglobulin replacement therapy, he developed disseminated involvement of both lungs with peripheral nodules; transbronchial lung biopsy revealed possible bronchiolitis obliterans organizing pneumonia (BOOP)...
October 2016: Acta Medica Iranica
https://www.readbyqxmd.com/read/27856306/enhanced-formation-of-giant-cells-in-common-variable-immunodeficiency-relation-to-granulomatous-disease
#14
Timothy H Scott-Taylor, Karen Whiting, Ruth Pettengell, David A Webster
Peripheral monocytes from patients with common variable immunodeficiency (CVID) had on average a 2 fold greater tendency to form giant cells in medium without additional cytokines. Giant cell formation was faster and 3 to 5 fold higher in most CVID cells compared to normal. Addition of IL4, GMCSF, IFNγ, TNFa and both T cell and monocyte conditioned media promoted monocyte fusion of some CVID individuals over 5 fold the normal average level, with combinations of cytokines and monokines acting synergistically...
November 14, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/27851622/1988-pneumococcal-sepsis-in-itp-post-splenectomy-and-vaccination-due-to-coexisting-cvid
#15
Anusha Shanbhag, Oscar Llanos Ulloa, Nikhil Meena
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27836061/common-variable-immunodeficiency-disorders-cvid-diagnoses-of-exclusion-especially-combined-immune-defects
#16
EDITORIAL
Helen Chapel
No abstract text is available yet for this article.
November 2016: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/27795213/-clinic-of-humoral-primary-immunodeficiencies-in-adults-experience-in-a-tertiary-hospital
#17
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/27753065/chronic-norovirus-infection-and-common-variable-immunodeficiency
#18
Jeremy Woodward, Effrossyni Gkrania-Klotsas, Dinakantha Kumararatne
Chronic infection with Norovirus is emerging as a significant risk for patients with immunodeficiency - either primary or secondary to therapeutic immunosuppression. Patients with primary immunodeficiency present a range of pathological responses to Norovirus infection. Asymptomatic infections occur and differentiating viral carriage or prolonged viral shedding after self-limiting infection from infection causing protracted diarrhoea can be challenging due to relatively mild pathological changes that may mimic other causes of diarrhoea in such patients (for instance pathogenic bacteria or parasites or graft-versus-host disease)...
October 18, 2016: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27749582/nfkb2-mutation-in-common-variable-immunodeficiency-and-isolated-adrenocorticotropic-hormone-deficiency-a-case-report-and-review-of-literature
#19
Chuan Shi, Fen Wang, Anli Tong, Xiao-Qian Zhang, Hong-Mei Song, Zheng-Yin Liu, Wei Lyu, Yue-Hua Liu, Wei-Bo Xia
BACKGROUND: Common variable immunodeficiency (CVID) with central adrenal insufficiency is a recently defined clinical syndrome caused by mutations in the nuclear factor kappa-B subunit 2 (NFKB2) gene. We present the first case of NFKB2 mutation in Asian population. METHODS AND RESULTS: An 18-year-old Chinese female with adrenocorticotropic hormone (ACTH) deficiency was admitted due to adrenal crisis and pneumonia. She had a history of recurrent respiratory infections since childhood and ectodermal abnormalities were noted during physical examination...
October 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27717724/comparison-of-various-classifications-for-patients-with-common-variable-immunodeficiency-cvid-using-measurement-of-b-cell-subsets
#20
R Yazdani, R Seify, M Ganjalikhani-Hakemi, H Abolhassani, N Eskandari, F Golsaz-Shirazi, B Ansaripour, E Salehi, G Azizi, N Rezaei, A Aghamohammadi
BACKGROUND: Common variable immunodeficiency (CVID) is a heterogeneous disease, characterised by hypogammaglobulinaemia leading to recurrent infections and various complications. The aim of this study was to classify CVID patients based on four known classifications (Paris, Freiburg, EUROclass, and B-cell patterns) by measurement of B-cell subsets and to assess the relation of each classification with clinical manifestations. METHODS: We measured all B-cell subsets as both absolute count and percentage in 30 CVID patients and 30 healthy individuals using four-colour flow cytometry...
October 4, 2016: Allergologia et Immunopathologia
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