keyword
MENU ▼
Read by QxMD icon Read
search

CVID

keyword
https://www.readbyqxmd.com/read/27923702/nfkb1-regulates-human-nk-cell-maturation-and-effector-functions
#1
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
#2
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 2, 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
#3
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...
December 1, 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
#4
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...
November 28, 2016: 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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
https://www.readbyqxmd.com/read/27713077/noninfectious-complications-in-patients-with-pediatric-onset-common-variable-immunodeficiency-correlated-with-defects-in-somatic-hypermutation-but-not-in-class-switch-recombination
#13
María Belén Almejún, Bárbara Carolina Campos, Virginia Patiño, Miguel Galicchio, Marta Zelazko, Matías Oleastro, Pablo Oppezzo, Silvia Danielian
BACKGROUND: Common variable immunodeficiency (CVID) is a heterogeneous syndrome characterized by impaired immunoglobulin production and usually presents with a normal quantity of peripheral B cells. Most attempts aiming to classify these patients have mainly been focused on T- or B-cell phenotypes and their ability to produce protective antibodies, but it is still a major challenge to find a suitable classification that includes the clinical and immunologic heterogeneity of these patients...
October 3, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27705887/annals-express-chitotriosidase-enzyme-activity-is-this-a-possible-chronic-inflammation-marker-in-children-with-common-variable-immunodeficiency-and-early-atherosclerosis
#14
Elif Azarsız, Neslihan Karaca, Erturk Levent, Necil Kutukculer, Eser Sozmen
BACKGROUND: Common variable immunodeficiency (CVID) is a rare clinically symptomatic primary immunodeficiency disorder which manifests a wide variability of symptoms, complications. Atherosclerosis in CVID patients have not been investigated yet contrary to other severe clinical complications. We aimed to investigate the chitotriosidase enzyme's role as an inflammation and atherosclerosis marker in pediatric CVID patients. METHODS: CVID patients (n =24) and healthy controls (n = 23) evaluated for chitotriosidase activity with other inflammation markers (hsCRP, myeloperoxidase, serum amyloid A, ferritin), lipid profile and echocardiographic findings (carotid artery intima media thickness - cIMT, brachial artery flow-mediated vazodilatation - FMD%)...
October 4, 2016: Annals of Clinical Biochemistry
https://www.readbyqxmd.com/read/27704236/neurologic-complications-of-common-variable-immunodeficiency
#15
Jenna Thuc-Uyen Nguyen, Ari Green, Michael R Wilson, Joseph L DeRisi, Katherine Gundling
Common variable immunodeficiency is a rare disorder of immunity associated with a myriad of clinical manifestations including recurrent infections, autoimmunity, and malignancy. Though rare, neurologic complications have been described in a small number of case reports and case series of CVID patients. In this article, we present a patient with CVID who suffered significant neurologic morbidity and categorize the reported range of neurologic complications associated with CVID. Our case highlights the complex nature of neurologic manifestations in CVID patients, and our review of the current database suggests that infection and inflammatory neurologic disorders are the cause of most neurologic presentations...
October 4, 2016: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/27703456/cancers-related-to-immunodeficiencies-update-and-perspectives
#16
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
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
#17
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/27664934/impaired-akt-phosphorylation-in-b-cells-of-patients-with-common-variable-immunodeficiency
#18
Reza Yazdani, Mazdak Ganjalikhani-Hakemi, Mohammad Esmaeili, Hassan Abolhassani, Shahram Vaeli, Abbas Rezaei, Zohre Sharifi, Gholamreza Azizi, Nima Rezaei, Asghar Aghamohammadi
Common variable immunodeficiency (CVID) is a heterogeneous group of primary immunodeficiency characterized by recurrent infectious. We evaluated whether defective PI3K/Akt/FoxO pathway could influence B-cell fate. Determination of B-cell subsets in CVD patients and healthy donors (HDs) were performed using flow cytometry. We evaluated mRNA level and protein expression of PI3K, Akt and FoxO molecules using real-time PCR and flow cytometry, respectively. Moreover, phosphorylated Akt (p-Akt) expression in B-cells has also been measured by flowcytometry...
September 21, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/27636680/autoimmunity-in-common-variable-immunodeficiency-epidemiology-pathophysiology-and-management
#19
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
https://www.readbyqxmd.com/read/27630845/chronic-giardiasis-in-a-case-of-common-variable-immunodeficiency-cvid-a-case-report
#20
Supriya M Paranjpe, Avani Koticha, Preeti R Mehta
Common Variable Immunodeficiency (CVID) is a primary immunodeficiency characterized by low antibody levels and recurrent infections. This makes an individual more prone to recurrent respiratory and gastrointestinal tract infections. In cases where there is persistent positive finding of intestinal parasites in stool, a high index of suspicion should be raised to rule out immunodeficiency state. Early diagnosis of such cases will help in reducing the morbidity and better management of the patient. A case of CVID in 18-year-old male with recurrent lower respiratory tract infection and chronic diarrhoea due to Giardia lamblia is reported herewith...
July 2016: Journal of Clinical and Diagnostic Research: JCDR
keyword
keyword
10061
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"