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Common variable immunodeficiency

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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/28791687/skin-signs-of-primary-immunodeficiencies-how-to-find-the-genes-to-check
#2
REVIEW
M Ettinger, J Schreml, K Wirsching, M Berneburg, S Schreml
Primary immunodeficiencies (PIDs) are a heterogeneous group of rare diseases that result from defects in immune system development and/or function. The clinical manifestations of PIDs are highly variable, but most disorders involve at least an increased susceptibility to infection. Furthermore, cutaneous manifestations are very common in PIDs. As an easy accessible organ, skin canbecrucial for early diagnosis and treatment. This is relevant for preventing significant disease-associated morbidity and mortality...
August 9, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28781847/management-of-common-variable-immunodeficiency-by-subcutaneous-igg-self-administration-during-pregnancy-a-case-report
#3
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/28761856/psoriasis-an-unusual-autoimmune-manifestation-in-a-boy-with-common-variable-immunodeficiency
#4
Ankur Kumar Jindal, Amit Rawat, Avinash Sharma, Sunil Dogra, Deepti Suri, Surjit Singh
No abstract text is available yet for this article.
July 2017: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/28755066/two-sides-of-the-same-coin-pediatric-onset-and-adult-onset-common-variable-immune-deficiency
#5
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/28736065/common-variable-immunodeficiency-associated-granulomatous-and-lymphocytic-interstitial-lung-disease-successfully-treated-with-a-combination-regimen-of-rituximab-and-azathioprine
#6
Sananda I Moctezuma, Carlos M Panizo, Manuel F Landecho
No abstract text is available yet for this article.
July 20, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28735808/autoimmunity-and-its-association-with-regulatory-t-cells-and-b-cell-subsets-in-patients-with-common-variable-immunodeficiency
#7
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
#8
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/28730326/infectious-diseases-causing-autonomic-dysfunction
#9
REVIEW
Francisco Javier Carod Artal
OBJECTIVES: To review infectious diseases that may cause autonomic dysfunction. METHODS: Review of published papers indexed in medline/embase. RESULTS: Autonomic dysfunction has been reported in retrovirus (human immunodeficiency virus (HIV), human T-lymphotropic virus), herpes viruses, flavivirus, enterovirus 71 and lyssavirus infections. Autonomic dysfunction is relatively common in HIV-infected patients and heart rate variability is reduced even in early stages of infection...
July 20, 2017: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/28722362/-transient-hypogammaglobulinemia-of-infancy
#10
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
#11
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
#12
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
#13
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
#14
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/28712180/causes-of-chronic-non-infectious-diarrhoea-in-infants-less-than-6-months-of-age-rarely-recognized-entities
#15
Iqra Mushtaq, Huma Ashraf Cheema, Hassan Suleman Malik, Nadia Waheed, Muhammad Almas Hashmi, Hassan Suleman Malik
BACKGROUND: Non-infectious causes of chronic diarrhoea are important and easily missed. The study was done with the objectives to identify different causes of chronic non-infectious diarrhoea in infants less than 6 months of age. METHODS: All patients less than 6 months of age presenting for the first time to a Paediatric Gastroenterology tertiary care centre with a history of chronic diarrhoea and negative stool cultures were enrolled over a period of 8 months...
January 2017: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/28711959/a-multicentre-study-on-the-efficacy-safety-and-pharmacokinetics-of-iqymune%C3%A2-a-highly-purified-10-liquid-intravenous-immunoglobulin-in-patients-with-primary-immune-deficiency
#16
Gergely Krivan, Ludmila Chernyshova, Larysa Kostyuchenko, Andrzej Lange, Zoltan Nyul, Beata Derfalvi, Jacek Musial, Anne Bellon, Martin Kappler, Alain Sadoun, Ewa Bernatowska
This multicentre, open-label, prospective, single-arm study was designed to evaluate the efficacy, pharmacokinetics, and safety of IqYmune®, a highly purified 10% polyvalent immunoglobulin preparation for intravenous administration in patients with primary immunodeficiency. IqYmune® was administered to 62 patients (aged 2-61 years) with X-linked agammaglobulinemia or common variable immune deficiency at a dose from 0.22 to 0.97 g/kg every 3 to 4 weeks for 12 months with an infusion rate up to 8 mL/kg/h...
July 15, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28708268/screening-protocols-to-monitor-respiratory-status-in-primary-immunodeficiency-disease-findings-from-a-european-survey-and-subclinical-infection-working-group
#17
Stephen Jolles, Silvia Sánchez-Ramón, Isabella Quinti, Pere Soler-Palacín, Carlo Agostini, Benoit Florkin, Louis-Jean Couderc, Nicholas Brodszki, Alison Jones, Hilary Longhurst, Klaus Warnatz, Filomeen Haerynck, Andrea Matucci, Esther de Vries
Many patients with primary immunodeficiency (PID) who have antibody deficiency develop progressive lung disease due to underlying subclinical infection and inflammation. To understand how these patients are monitored we conducted a retrospective survey based on patient records of 13 PID centres across Europe, regarding the care of 1,061 adult and 178 paediatric patients with PID on IgG replacement. The most common diagnosis was common variable immunodeficiency in adults (75%), and hypogammaglobulinaemia in children (39%)...
July 14, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28674534/human-cd56-dim-cd16-dim-cells-as-an-individualized-natural-killer-cell-subset
#18
Mathieu Amand, Gilles Iserentant, Aurélie Poli, Marwan Sleiman, Virginie Fievez, Isaura Pilar Sanchez, Nicolas Sauvageot, Tatiana Michel, Nasséra Aouali, Bassam Janji, Claudia Milena Trujillo-Vargas, Carole Seguin-Devaux, Jacques Zimmer
Human natural killer (NK) cells can be subdivided in several subpopulations on the basis of the relative expression of the adhesion molecule CD56 and the activating receptor CD16. Whereas blood CD56(bright)CD16(dim/-) NK cells are classically viewed as immature precursors and cytokine producers, the larger CD56(dim)CD16(bright) subset is considered as the most cytotoxic one. In peripheral blood of healthy donors, we noticed the existence of a population of CD56(dim)CD16(dim) NK cells that was frequently higher in number than the CD56(bright) subsets and even expanded in occasional control donors but also in transporter associated with antigen processing-deficient patients, two familial hemophagocytic lymphohistiocytosis type II patients, and several common variable immunodeficiency patients...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28672090/efficacy-of-mycophenolate-on-lung-disease-and-autoimmunity-in-children-with-immunodeficiency
#19
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
#20
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
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