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https://www.readbyqxmd.com/read/28345437/clinical-profile-dosing-and-quality-of-life-outcomes-in-primary-immune-deficiency-patients-treated-at-home-with-immunoglobulin-g-data-from-the-ideal-patient-registry
#1
Sean Kearns, Loretta Kristofek, William Bolgar, Luqman Seidu, Samantha Kile
BACKGROUND: Patients with primary immune deficiency (PID) often require immunoglobulin G (IgG, commonly referred to as Ig) replacement therapy to prevent infections and associated comorbidities. Ig therapy can be given either through intravenous or subcutaneous routes, and both can be done in the home setting. There is limited information available on the real-world diagnosis, management, and outcomes of this patient population, given the variable disease presentation and treatment options...
April 2017: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/28337685/type-iii-hypersensitivity-reaction-to-subcutaneous-insulin-preparations-in-a-type-1-diabetic
#2
Benjamin R Murray, Jolene R Jewell, Kyle J Jackson, Olabunmi Agboola, Brianna R Alexander, Poonam Sharma
Management of type 1 diabetes in patients who have insulin hypersensitivity is a clinical challenge and places patients at risk for recurrent diabetic ketoacidosis (DKA). Hypersensitivity reactions can be due to the patient's response to the insulin molecule itself or one of the injection's non-insulin components. It is therefore crucial for clinicians to quickly recognize the type of hypersensitivity reaction that is occurring and identify potentially immunogenic additives for the purpose of directing therapy as various insulin preparations have differing ingredients...
March 23, 2017: Journal of General Internal Medicine
https://www.readbyqxmd.com/read/28324805/limited-role-of-interferon-kappa-ifnk-truncating-mutations-in-common-variable-immunodeficiency
#3
Faranaz Atschekzei, Thilo Dörk, Peter Schürmann, Robert Geffers, Torsten Witte, Reinhold E Schmidt
We used whole exome sequencing to determine the genetic background of CVID in two non-consanguineous German families. We identified IFNK (interferon-kappa) as the only candidate gene that harbored truncating mutations in affected members from both families. One family segregated c.30_31insTGTT, a known frameshift variant, while the other family segregated the novel IFNK mutation p.K199X that creates a premature stop codon. We sequenced the whole coding region of IFNK in a further series of 167 CVID patients and 192 healthy controls...
March 18, 2017: Cytokine
https://www.readbyqxmd.com/read/28323147/increase-of-circulating-%C3%AE-4%C3%AE-7-conventional-memory-cd4-and-regulatory-t-cells-in-patients-with-common-variable-immunodeficiency-cvid
#4
Karina Mescouto de Melo, Susanne Unger, Baerbel Keller, Sylvia Gutenberger, Ina Stumpf, Sigune Goldacker, Klaus Warnatz
This study investigated whether circulating α4β7(+) expressing T cells could serve as a potential marker for gastrointestinal (GI) disease activity in patients with CVID. The analysis of α4β7(+) T cells in the peripheral blood of 36 patients and 22 healthy donors (HD) revealed increased percentages of α4β7(+) conventional memory CD4 T cells and Tregs, but not among CD8 T-cell populations in patients with CVID compared to HD. No differences between patients with and without chronic or acute GI symptoms were observed...
March 17, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28289412/follicular-t-cells-from-smb-common-variable-immunodeficiency-patients-are-skewed-toward-a-th1-phenotype
#5
Vanesa Cunill, Antonio Clemente, Nallibe Lanio, Carla Barceló, Valero Andreu, Jaume Pons, Joana M Ferrer
Germinal center follicular T helper (GCTfh) cells are essential players in the differentiation of B cells. Circulating follicular T helper (cTfh) cells share phenotypic and functional properties with GCTfh cells. Distinct subpopulations of cTfh with different helper capabilities toward B cells can be identified: cTfh1 (CXCR3(+)CCR6(-)), cTfh2 (CXCR3(-)CCR6(-)), and cTfh17 (CXCR3(-)CCR6(+)). Alterations in cTfh function and/or distribution have been associated with autoimmunity, infectious diseases, and more recently, with several monogenic immunodeficiencies...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28284485/abnormality-of-regulatory-t-cells-in-common-variable-immunodeficiency
#6
REVIEW
Gholamreza Azizi, Nasim Hafezi, Hamed Mohammadi, Reza Yazdai, Tina Alinia, Marzieh Tavakol, Asghar Aghamohammadi, Abbas Mirshafiey
Common variable immunodeficiency (CVID) is a heterogeneous group of primary antibody deficiencies (PAD) which is defined by recurrent infections, hypogammaglobulinemia and defects in B-cell differentiation into plasma cells and memory B cells. T cell abnormalities have also been described in CVID patients. Several studies reported that Treg frequencies and their functional characteristics are disturbed and might account for the aberrant immune responses observed in CVID patients. The aim of this review is to describe phenotypic and functional characteristics of Treg cells, and to review the literature with respect to the reported Treg defects and its association with the clinical manifestation in CVID...
December 29, 2016: Cellular Immunology
https://www.readbyqxmd.com/read/28254202/predictors-of-granulomatous-lymphocytic-interstitial-lung-disease-in-common-variable-immunodeficiency
#7
Stella Hartono, Megan S Motosue, Shakila Khan, Vilmarie Rodriguez, Vivek N Iyer, Rohit Divekar, Avni Y Joshi
BACKGROUND: A subset of patients with common variable immunodeficiency (CVID) develop granulomatous lymphocytic interstitial lung disease (GLILD), which is associated with early mortality. OBJECTIVE: To determine a set of clinical and/or laboratory parameters that correlate with GLILD. METHODS: A retrospective, nested case-control (patients with CVID diagnosed with GLILD compared with patients with CVID without a diagnosis of GLILD) medical record review was undertaken at Mayo Clinic, Rochester, MN...
February 18, 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/28240614/diagnostic-and-therapeutic-considerations-in-patients-with-hypogammaglobulinemia-after-rituximab-therapy
#8
Ruba Kado, Georgiana Sanders, W Joseph McCune
PURPOSE OF REVIEW: There are no established guidelines for evaluating and treating hypogammaglobulinemia in patients with rheumatic disease who receive B-cell depleting agents. The purpose of this article is to review findings in the work-up and treatment of common variable immunodeficiency (CVID) that can guide our evaluation of patients with autoimmune disease who develop hypogammaglobulinemia after rituximab/B-cell depleting therapy. RECENT FINDINGS: Infection rates are higher in rheumatic disease patients who develop hypogammaglobulinemia than those who do not...
May 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28236292/the-role-of-genomics-in-common-variable-immunodeficiency-disorders
#9
REVIEW
Anne-Kathrin Kienzler, Chantal E Hargreaves, Smita Y Patel
The advent of next generation sequencing (NGS) and 'omic' technologies has revolutionised the field of genetics and its implementation in healthcare has the potential to realise precision medicine. Primary immunodeficiencies (PID) are a group of rare diseases which have benefitted from NGS, with a massive increase in causative genes identified in the past few years. Common Variable Immunodeficiency Disorders (CVID) are a heterogeneous form of PID and the most common form of antibody failure in children and adults...
February 25, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28188716/-immunological-alterations-in-common-variable-immunodeficiency
#10
Laura Berrón-Ruiz
Common variable immunodeficiency (CVID) is the largest group of symptomatic primary immune deficiencies; it is characterized by hypogammaglobulinemia, poor response to vaccines and increased susceptibility to infections. Cellular phenotypes and abnormalities have been described both in adaptive and innate immune response. Several classifications of common variable immunodeficiency are based on defects found on T and B cells, which have been correlated with clinical manifestations. In recent years, significant progress has been made in elucidating the genetic mechanisms that result in a IDCV phenotype...
January 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/28159733/identifying-functional-defects-in-patients-with-immune-dysregulation-due-to-lrba-and-ctla-4-mutations
#11
Tie Zheng Hou, Nisha Verma, Jennifer Wanders, Alan Kennedy, Blagoje Soskic, Daniel Janman, Neil Halliday, Behzad Rowshanravan, Austen Worth, Waseem Qasim, Helen Baxendale, Hans Stauss, Suranjith Seneviratne, Olaf Neth, Peter Olbrich, Sophie Hambleton, Peter D Arkwright, Siobhan O Burns, Lucy S K Walker, David M Sansom
Heterozygous CTLA-4 deficiency has been reported as a monogenic cause of common variable immune deficiency (CVID) with features of immune dysregulation. Direct mutation in CTLA-4 leads to defective regulatory T cell function associated with impaired ability to control levels of the CTLA-4 ligands, CD80 and CD86. However, additional mutations affecting the CTLA-4 pathway, such as those recently reported for LRBA, indirectly affect CTLA-4 expression resulting in clinically similar disorders. Robust phenotyping approaches sensitive to defects in the CTLA-4 pathway are therefore required to inform understanding of such immune dysregulation syndromes...
February 3, 2017: Blood
https://www.readbyqxmd.com/read/28154406/cvid-and-celiac-disease
#12
Silje F Jørgensen, Henrik M Reims, Pål Aukrust, Knut E A Lundin, Børre Fevang
No abstract text is available yet for this article.
February 2017: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/28154271/diffuse-nodular-lymphoid-hyperplasia-of-the-intestine-caused-by-common-variable-immunodeficiency-and-refractory-giardiasis
#13
Jung Hye Choi, Dong Soo Han, Jieun Kim, Kijong Yi, Young-Ha Oh, Yongsoo Kim
Diffuse nodular lymphoid hyperplasia of the gastrointestinal tract is a rare disease characterized by numerous small polypoid nodules in the small intestine, large intestine, or both. It is associated with immunodeficiency and infection, such as Giardia lamblia and Helicobacter pylori. Although diffuse nodular lymphoid hyperplasia associated with common variable immunodeficiency (CVID) and giardiasis is already known, a few studies have reported a regression of the lymphoid nodules after the eradication of infection...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28134088/evolving-spectrum-of-lrba-deficiency-associated-chronic-arthritis-is-there-a-causative-role-in-juvenile-idiopathic-arthritis
#14
Sulaiman M Al-Mayouf, Hamzah Naji, Khalid Alismail, Anas M Alazami, Farrukh Sheikh, Walter Conca, Hamoud Al-Mousa
Lipopolysaccharide-responsive, beige-like anchor protein (LRBA) deficiency causes common variable immunodeficiency (CVID) disorders and autoimmunity. LRBA deficiency has become a clinically variable syndrome with a wide spectrum of clinical manifestations. We report a patient with LRBA deficiency associated chronic non-erosive arthritis. This report highlights the spectrum of arthritis in such patients and the potential causative role of LRBA gene in juvenile arthritis.
January 27, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28126376/igg-trough-levels-and-progression-of-pulmonary-disease-in-pediatric-and-adult-common-variable-immunodeficiency-disorder-patients
#15
Willemijn J M Janssen, Firdaus Mohamed Hoesein, Annick A J M Van de Ven, Jacobien Maarschalk, Florien van Royen, Pim A de Jong, Elisabeth A M Sanders, Joris M van Montfrans, Pauline M Ellerbroek
No abstract text is available yet for this article.
January 23, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28124237/increased-incidence-of-fatigue-in-patients-with-primary-immunodeficiency-disorders-prevalence-and-associations-within-the-us-immunodeficiency-network-registry
#16
Joud Hajjar, Danielle Guffey, Charles G Minard, Jordan S Orange
INTRODUCTION: Patients with primary immunodeficiency (PID) often report fatigue, yet this symptom has not been studied in PID. Fatigue affects 6-7.5% of healthy adults. The goal of this study is to estimate the prevalence of fatigue in patients with PID and investigate its associated factors. METHODS: We analyzed 2537 PID patients registered in USIDNET to determine responses to the field "fatigue" in the core registry form. Demographics, immune phenotypes, and comorbid conditions were compared between fatigued and non-fatigued patients to identify relevant associations and potential drivers...
February 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28093361/neil1-is-a-candidate-gene-associated-with-common-variable-immunodeficiency-in-a-patient-with-a-chromosome-15q24-deletion
#17
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 14, 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
#18
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
#19
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
#20
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...
March 2017: Trends in Immunology
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