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Common variable immune deficiency

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https://www.readbyqxmd.com/read/29678747/predictive-markers-for-humoral-influenza-vaccine-response-in-patients-with-common-variable-immunodeficiency-cvid
#1
Ann Gardulf, Hassan Abolhassani, Rolf Gustafson, Lars E Eriksson, Lennart Hammarström
BACKGROUND: A subgroup of patients with common variable immunodeficiencies (CVID) responds to vaccination. The aim of the study was to try to identify predictive markers for those who developed a humoral immune response after influenza vaccination. METHODS: 48 patients with CVID (29 females, 19 males, mean age 59.4 years) were vaccinated with the A(H1N1) influenza vaccine Pandemrix® and boosted after one month. Blood samples were collected prior to each vaccination and two months later...
April 17, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29675923/latent-therapeutic-demand-model-for-the-immunoglobulin-replacement-therapy-of-primary-immune-deficiency-disorders-in-the-usa
#2
J S Stonebraker, J Hajjar, J S Orange
BACKGROUND AND OBJECTIVES: Our research aim is to model latent therapeutic demand (LTD) for the immunoglobulin replacement therapy (IgGRT) of primary immune deficiency disorders (PIDDs) in the USA. Given the high level of variability of IgGRT use and major differences among American and European practices in the management of patients with PIDDs, we develop a USA-specific LTD model for common variable immune deficiency (CVID), hyper IGM syndrome, severe combined immune deficiency, Wiskott-Aldrich syndrome and X-linked agammaglobulinemia (XLA)...
April 20, 2018: Vox Sanguinis
https://www.readbyqxmd.com/read/29651973/the-clinical-and-immunological-features-of-patients-with-primary-antibody-deficiencies
#3
Gholamreza Azizi, Yasser Bagheri, Marzieh Tavakol, Forough Askarimoghaddam, Kiyoomars Poorrostami, Hosein Rafiemanesh, Reza Yazdani, Fatemeh Kiaee, Sima Habibi, Kosar Abouhamzeh, Hamed Mohammadi, Mostafa Qorbani, Hassan Abolhassani, Asghar Aghamohammadi
BACKGROUND: Primary antibody deficiency (PAD) comprises a range of diseases from early to late terminal B cells defects and is associated with the various clinical complications. METHODS: A total of 461 patients (311 males and 150 females) with PADs enrolled in the retrospective cohort study and for all patients' demographic information, clinical records and laboratory data were collected to investigate clinical complications. RESULTS: The most prevalent first presentations of immunodeficiency were respiratory tract infections in 63...
April 12, 2018: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/29542377/implanted-ports-in-adults-with-primary-immunodeficiency
#4
James C Barton, Jackson Clayborn Barton, Luigi F Bertoli
BACKGROUND: We sought to learn more about the utility and safety of implanted ports for monthly immunoglobulin G infusions in adults with primary immune deficiency. METHODS: We reviewed charts of adults who were referred to a single practice during the interval 2006-2016 for evaluation and management of frequent or severe upper and lower respiratory tract and other infections, subnormal total immunoglobulin G or immunoglobulin G subclasses, and suboptimal responses to polyvalent pneumococcal polysaccharide vaccinations; were diagnosed to have primary immune deficiency; and were advised to undergo immunoglobulin G therapy...
March 1, 2018: Journal of Vascular Access
https://www.readbyqxmd.com/read/29530546/body-mass-index-represents-a-good-predictor-of-vitamin-d-status-in-women-independently-from-age
#5
Simona Delle Monache, Patrizia Di Fulvio, Ester Iannetti, Luca Valerii, Ludovica Capone, Maria Giovanna Nespoli, Mauro Bologna, Adriano Angelucci
BACKGROUND & AIMS: Vitamin D is a pleiotropic hormone targeting several tissues and is involved in basic homeostatic processes, including bone mineralization, immune response and muscle strength. Although hypovitaminosis D is common in Europe and North America, representing a risk factor for several chronic diseases, the contribution of factors other than sun exposure is largely underestimated. METHODS: In our study, we retrospectively collected data from medical records of women with age between 19 and 80 screened in Central Italy (42°N) for increased risk of metabolic syndrome...
March 2, 2018: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29470661/granulomatous-lymphocytic-interstitial-lung-disease-in-22q11-2-deletion-syndrome-a-case-report-and-literature-review
#6
REVIEW
Amika K Sood, William Funkhouser, Brian Handly, Brent Weston, Eveline Y Wu
PURPOSE OF REVIEW: Granulomatous-lymphocytic interstitial lung disease (GLILD) has classically been associated with common variable immune deficiency (CVID), but is increasingly being reported in other immunodeficiencies. We describe the second reported case of GLILD in a patient with 22q11.2 deletion syndrome (22q11.2DS) and review the recent literature surrounding GLILD. RECENT FINDINGS: GLILD is characterized by granulomata and lymphoproliferation. Consensus statements and retrospective and case-control studies have better elucidated the clinicopathological and radiographic manifestations of GLILD, allowing for its differentiation from similar conditions like sarcoidosis...
February 22, 2018: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/29398461/presence-of-immune-deficiency-increases-the-risk-of-hospitalization-in-patients-with-norovirus-infection
#7
Keith A Sacco, Thanai Pongdee, Matthew J Binnicker, Mark Espy, Darrell Pardi, Sahil Khanna, Avni Y Joshi
Norovirus is an emerging pathogen causing gastroenteritis. We sought to identify factors associated with clinical outcomes in a cohort of patients with laboratory-confirmed norovirus infection. We performed a retrospective chart review of patients with positive norovirus polymerase chain reaction in stool between October 1, 2015, and May 31, 2016. 128 unique patients were identified during the study period, 64 of whom had immune deficiency, of which only 3 patients had a primary immune deficiency (common variable immune deficiency), while 61 patients had a secondary immune deficiency...
December 5, 2017: Diagnostic Microbiology and Infectious Disease
https://www.readbyqxmd.com/read/29378793/role-of-the-inducible-adhesin-cpals7-in-binding-of-candida-parapsilosis-to-extracellular-matrix-under-fluid-shear
#8
Matthew N Neale, Kyle A Glass, Sarah J Longley, Denny J Kim, Sonia S Laforce-Nesbitt, Jeremy D Wortzel, Sunil K Shaw, Joseph M Bliss
The yeast, Candida parapsilosis , is an increasingly common cause of systemic fungal infections among the immune compromised, including premature infants. Adhesion to host surfaces is an important step in pathogenesis, but this process has not been extensively studied in this organism. A microfluidics assay was developed to test the ability of C. parapsilosis to adhere to immobilized host extracellular matrix proteins under physiologic fluid shear conditions. Growth in mammalian tissue culture media at 37°C for 3-6 hours led to induction of an adhesive phenotype at shear forces of 1-5 dynes/cm2 in some isolates of C...
January 29, 2018: Infection and Immunity
https://www.readbyqxmd.com/read/29360733/maternal-vitamin-d-status-and-infant-infection
#9
Sara Moukarzel, Marlies Ozias, Elizabeth Kerling, Danielle Christifano, Jo Wick, John Colombo, Susan Carlson
Maternal vitamin D status during pregnancy may modulate fetal immune system development and infant susceptibility to infections. Vitamin D deficiency is common during pregnancy, particularly among African American (AA) women. Our objective was to compare maternal vitamin D status (plasma 25(OH)D concentration) during pregnancy and first-year infections in the offspring of African American (AA) and non-AA women. We used medical records to record frequency and type of infections during the first year of life of 220 term infants (69 AA, 151 non-AA) whose mothers participated in the Kansas University DHA Outcomes Study...
January 23, 2018: Nutrients
https://www.readbyqxmd.com/read/29341423/variable-immune-deficiency-related-to-deletion-size-in-chromosome-22q11-2-deletion-syndrome
#10
Blaine Crowley, Melanie Ruffner, Donna M McDonald McGinn, Kathleen E Sullivan
The clinical features of 22q11.2 deletion syndrome include virtually every organ of the body. This review will focus on the immune system and the differences related to deletion breakpoints. A hypoplastic thymus was one of the first features described in this syndrome and low T cell counts, as a consequence of thymic hypoplasia, are the most commonly described immunologic feature. These are most prominently seen in early childhood and can be associated with increased persistence of viruses. Later in life, evidence of T cell exhaustion may be seen and secondary deficiencies of antibody function have been described...
January 17, 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29184600/-common-variable-immune-deficiency-lately-revealed-by-gastrointestinal-problems-about-a-case
#11
Fatima Ezzaitouni, Youssef Thiyfa, Mohamed Tahiri, Fouad Haddad, Wafaa Hliwa, Ahmed Bellabah, Wafaa Badre
Common Variable Immune Deficiency (CVID) is rare. It is a constitutional deficit of humoral immunity characterized by recurrent bacterial infections and by increased frequency of tumors, autoimmune or granulomatous diseases. Gastrointestinal manifestations are very variable and sometimes reveal common variable immune deficiency. We report the case of a 31-year old patient with a history of childhood recurrent respiratory infections complicated by bronchiectasis and with a 3-year history of recurrent glairy diarrhea...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/29184394/highly-specific-reasons-for-nonadherence-to-antiretroviral-therapy-results-from-the-german-adherence-study
#12
Johanna Boretzki, Eva Wolf, Carmen Wiese, Sebastian Noe, Annamaria Balogh, Anja Meurer, Ivanka Krznaric, Alexander Zink, Christian Lersch, Christoph D Spinner
Background: Reasons for and frequency of nonadherence to antiretroviral therapy (ART) may have changed due to pharmacological improvements. In addition, the importance of known non-pharmacologic reasons for nonadherence is unclear. Methods: We performed a cross-sectional, noninterventional, multicenter study to identify current reasons for nonadherence. Patients were categorized by physicians into the following adherence groups: good, unstable, or poor adherence...
2017: Patient Preference and Adherence
https://www.readbyqxmd.com/read/29153154/a-100-year-review-from-ascorbic-acid-to-zinc-mineral-and-vitamin-nutrition-of-dairy-cows
#13
W P Weiss
Mineral and vitamin nutrition of dairy cows was studied before the first volume of the Journal of Dairy Science was published and is still actively researched today. The initial studies on mineral nutrition of dairy cows were simple balance experiments (although the methods available at the time for measuring minerals were anything but simple). Output of Ca and P in feces, urine, and milk was subtracted from intake of Ca and P, and if values were negative it was often assumed that cows were lacking in the particular mineral...
December 2017: Journal of Dairy Science
https://www.readbyqxmd.com/read/29126302/the-clinical-utility-of-measuring-igg-subclass-immunoglobulins-during-immunological-investigation-for-suspected-primary-antibody-deficiencies
#14
Antony R Parker, Markus Skold, David B Ramsden, J Gonzalo Ocejo-Vinyals, Marcos López-Hoyos, Stephen Harding
Measurement of IgG subclass concentrations is a standard laboratory test run as part of a panel to investigate the suspicion of antibody deficiency. The assessment is clinically important when total IgG is within the normal age-specific reference range. The measurement is useful for diagnosis of IgG subclass deficiency, to aid the diagnosis of specific antibody deficiency, as a supporting test for the diagnosis of common variable immunodeficiency, as well as for risk stratification of patients with low IgA...
November 8, 2017: Laboratory Medicine
https://www.readbyqxmd.com/read/29093626/faecal-microbiota-transplantation-in-patients-with-clostridium-difficile-and-significant-comorbidities-as-well-as-in-patients-with-new-indications-a-case-series
#15
Perttu Lahtinen, Eero Mattila, Veli-Jukka Anttila, Jyrki Tillonen, Matti Teittinen, Pasi Nevalainen, Seppo Salminen, Reetta Satokari, Perttu Arkkila
Fecal microbiota transplantation (FMT) is effective in recurrent Clostridium difficile infection (rCDI). Knowledge of the safety and efficacy of FMT treatment in immune deficient patients is scarce. FMT has been suggested as a potential method for an increasing number of new indications besides rCDI. Among our FMT-treated rCDI patients, we reviewed those with major comorbidities: two human immunodeficiency virus patients, six haemodialysis patients, two kidney transplant patients, two liver transplant patients and a patient with chronic lymphatic leukaemia...
October 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29080979/autoimmune-cytopenias-and-associated-conditions-in-cvid-a-report-from-the-usidnet-registry
#16
Elizabeth J Feuille, Niloofar Anooshiravani, Kathleen E Sullivan, Ramsay L Fuleihan, Charlotte Cunningham-Rundles
PURPOSE: Autoimmune cytopenia is frequently a presenting manifestation of common variable immune deficiency (CVID). Studies characterizing the CVID phenotype associated with autoimmune cytopenias have mostly been limited to large referral centers. Here, we report prevalence of autoimmune cytopenias in CVID from the USIDNET Registry and compare the demographics and clinical features of patients with and without this complication. METHODS: Investigators obtained demographic, laboratory, and clinical data on CVID patients within the USIDNET Registry...
January 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29019451/autoimmunity-in-a-cohort-of-471-patients-with-primary-antibody-deficiencies
#17
Gholamreza Azizi, Marzieh Tavakol, Hosein Rafiemanesh, Fatemeh Kiaee, Reza Yazdani, Amin Heydari, Kosar Abouhamzeh, Pardis Anvari, Sara Mohammadikhajehdehi, Laleh Sharifia, Yasser Bagheri, Hamed Mohammadi, Hassan Abolhassani, Asghar Aghamohammadi
OBJECTIVES: The aim of this study was to evaluate the frequency of autoimmunity in primary antibody deficiency (PAD). METHODS: A total of 471 patients with PADs enrolled in this retrospective cohort study. For all patients' demographic information, clinical records and laboratory data were collected to investigate autoimmune complications. RESULTS: Autoimmune disorders as the first presentation of immunodeficiency were recorded in 11 patients (2...
November 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28993767/genomic-programming-of-human-neonatal-dendritic-cells-in-congenital-systemic-and-in-vitro-cytomegalovirus-infection-reveal-plastic-and-robust-immune-pathway-biology-responses
#18
Widad Dantoft, Pablo Martínez-Vicente, James Jafali, Lara Pérez-Martínez, Kim Martin, Konstantinos Kotzamanis, Marie Craigon, Manfred Auer, Neil T Young, Paul Walsh, Arnaud Marchant, Ana Angulo, Thorsten Forster, Peter Ghazal
Neonates and especially premature infants are highly susceptible to infection but still can have a remarkable resilience that is poorly understood. The view that neonates have an incomplete or deficient immune system is changing. Human neonatal studies are challenging, and elucidating host protective responses and underlying cognate pathway biology, in the context of viral infection in early life, remains to be fully explored. In both resource rich and poor settings, human cytomegalovirus (HCMV) is the most common cause of congenital infection...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28983810/dysregulation-of-innate-lymphoid-cells-in-common-variable-immunodeficiency
#19
REVIEW
Paul J Maglione, Montserrat Cols, Charlotte Cunningham-Rundles
Common variable immunodeficiency (CVID) is the most prevalent symptomatic primary immune deficiency. With widespread use of immunoglobulin replacement therapy, non-infectious complications, such as autoimmunity, chronic intestinal inflammation, and lung disease, have replaced infections as the major cause of morbidity and mortality in this immune deficiency. The pathogenic mechanisms that underlie the development of these complications in CVID are not known; however, there have been numerous associated laboratory findings...
October 5, 2017: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/28983794/somatic-hypermutation-defects-in-common-variable-immune-deficiency
#20
REVIEW
María Belén Almejun, Mercedes Borge
Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by impaired antibody production and recurrent infections. In the last 20 years, several groups have reported that B cells from CVID patients have an impaired somatic hypermutation (SHM). The reported frequency of this defect among CVID patient cohorts is highly variable and so is the methodology used to evaluate this process. Interestingly, the low level of SHM on B cells from CVID patients has been correlated with the presence of infectious and non-infectious complications...
October 5, 2017: Current Allergy and Asthma Reports
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