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Common variable immunodeficiency

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https://www.readbyqxmd.com/read/29142526/monomorphic-epitheliotropic-intestinal-t-cell-lymphoma-complicated-by-common-variable-immunodeficiency
#1
Yang Liu, Yue-Hui Li, Ji Li, Jia-Ming Qian, Wei-Xun Zhou, Ya-Ping Luo
No abstract text is available yet for this article.
October 2017: Intestinal Research
https://www.readbyqxmd.com/read/29138951/chronic-diarrhea-in-common-variable-immunodeficiency-a-case-series-and-review-of-the-literature
#2
Antonio Pecoraro, Liliana Nappi, Ludovica Crescenzi, Francesco P D'Armiento, Arturo Genovese, Giuseppe Spadaro
Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by reduced immunoglobulin serum levels and absent or impaired antibody production. Clinical manifestations, including infections, inflammatory and autoimmune diseases, and malignancies, also involve various segments of the gastrointestinal tract. Chronic diarrhea is one of the most common gastrointestinal symptoms and may cause a wide spectrum of potentially life-threatening conditions as malabsorption and protein-energy malnutrition...
November 14, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29126302/the-clinical-utility-of-measuring-igg-subclass-immunoglobulins-during-immunological-investigation-for-suspected-primary-antibody-deficiencies
#3
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/29116409/chronic-meningoencephalitis-caused-by-echo-virus%C3%A2-6-in-a%C3%A2-patient-with-common-variable-immunodeficiency-successful-treatment-with-pleconaril
#4
Igor Radanović, Deni Rkman, Paulo Zekan, Marko Kutleša, Bruno Baršić
Chronic enteroviral meningoencephalitis, most commonly caused by echoviruses, can particularly be seen in agammaglobulinemic patients. In spite of the fact that no specific treatment for enteroviral infections exists, pleconaril is an antiviral drug reported to be efficient against enteroviral infections in infants and adults. We present a case of a 42-year-old male, previously diagnosed with common variable immunodeficiency, who presented with severe chronic meningoencephalitis caused by Echo virus 6 and was successfully treated with pleconaril...
November 7, 2017: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/29114494/giardiasis-mimicking-celiac-disease-in-a-patient-of-common-variable-immunodeficiency
#5
Kumar Saurabh, Vijaya Lakshmi Nag, Daisy Khera, Poonam Elhence
An adolescent boy presented to pediatrics outpatient department with complaints of recurrent diarrhea, nausea, vomiting, and pedal edema since 3-4 months, with no relief even after taking treatment. His investigation revealed decreased serum IgA, IgG, and IgM levels with hypoproteinemia. Duodenal biopsy showed features of celiac disease (CD), but tissue transglutaminase IgA was negative. In stool examination, plenty of Giardia lamblia cysts and eggs of Hymenolepis nana were present. He improved on treatment and remained asymptomatic for 4 months...
July 2017: Tropical Parasitology
https://www.readbyqxmd.com/read/29114388/epistatic-interactions-between-mutations-of-taci-tnfrsf13b-and-tcf3-result-in-a-severe-primary-immunodeficiency-disorder-and-systemic-lupus-erythematosus
#6
Rohan Ameratunga, Wikke Koopmans, See-Tarn Woon, Euphemia Leung, Klaus Lehnert, Charlotte A Slade, Jessica C Tempany, Anselm Enders, Richard Steele, Peter Browett, Philip D Hodgkin, Vanessa L Bryant
Common variable immunodeficiency disorders (CVID) are a group of primary immunodeficiencies where monogenetic causes account for only a fraction of cases. On this evidence, CVID is potentially polygenic and epistatic although there are, as yet, no examples to support this hypothesis. We have identified a non-consanguineous family, who carry the C104R (c.310T>C) mutation of the Transmembrane Activator Calcium-modulator and cyclophilin ligand Interactor (TACI, TNFRSF13B) gene. Variants in TNFRSF13B/TACI are identified in up to 10% of CVID patients, and are associated with, but not solely causative of CVID...
October 2017: Clinical & Translational Immunology
https://www.readbyqxmd.com/read/29103535/practical-approach-to-the-flattened-duodenal-biopsy
#7
REVIEW
Thomas C Smyrk
Celiac disease features duodenal intraepithelial lymphocytosis with or without villous atrophy. Lymphocytosis without villous atrophy will be proven to represent celiac disease in 10% to 20% of cases. The differential diagnosis is broad: Helicobacter pylori gastritis, NSAID injury and bacterial overgrowth are considerations. Lymphocytosis with villous atrophy is very likely to be celiac disease, but there are mimics to consider, including collagenous sprue, tropical sprue, drug injury, and common variable immunodeficiency...
December 2017: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/29103189/modulation-of-the-interleukin-21-pathway-with-interleukin-4-distinguishes-common-variable-immunodeficiency-patients-with-more-non-infectious-clinical-complications
#8
Marylin Desjardins, Marianne Béland, Marieme Dembele, Duncan Lejtenyi, Jean-Phillipe Drolet, Martine Lemire, Christos Tsoukas, Moshe Ben-Shoshan, Francisco J D Noya, Reza Alizadehfar, Christine T McCusker, Bruce D Mazer
PURPOSE: Common variable immunodeficiency (CVID) is characterized by hypogammaglobulinemia and clinical manifestations such as infections, autoimmunity, and malignancy. We sought to determine if responsiveness to interleukin-21 (IL-21), a key cytokine for B cell differentiation, correlates with distinct clinical phenotypes in CVID. METHODS: CVID subjects were recruited through the Canadian Primary Immunodeficiency Evaluative Survey registry. Peripheral blood mononuclear cells were cultured with anti-CD40 ± interferon-gamma, interleukin-4 (IL-4), IL-21, and/or IL-4+IL-21...
November 4, 2017: Journal of Clinical Immunology
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
#9
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/29083052/skewed-invariant-natural-killer-t-inkt-cells-impaired-inkt-b-cell-help-and-decreased-sap-expression-in-blood-lymphocytes-from-patients-with-common-variable-immunodeficiency
#10
L V Erazo-Borrás, J A Álvarez-Álvarez, C A Perez-Romero, J C Orrego-Arango, J L Franco-Restrepo, C M Trujillo-Vargas
Common variable immunodeficiency (CVID) is a syndrome with predominantly defective B cell function. However, abnormalities in the number and function of other lymphocyte subpopulations in peripheral blood (PB) have been described in most patients. We have analysed the distribution of iNKT cell subpopulations in the PB of CVID patients and the ability of these cells to provide in vitro cognate B cell help. The total of iNKT cells was reduced in the PB of CVID patients, especially CD4+, CD4-/CD8- and CCR5+/CXCR3+...
September 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/29080613/phylogeography-genetic-variability-and-structure-of-acanthamoeba-metapopulations-in-iran-inferred-by-18s-ribosomal-rna-sequences-a-systematic-review-and-meta-analysis
#11
REVIEW
Adel Spotin, Hamid Reza Moslemzadeh, Mahmoud Mahami-Oskouei, Ehsan Ahmadpour, Maryam Niyyati, Seyed Hossein Hejazi, Fatemeh Memari, Jafar Noori
OBJECTIVE: To verify phylogeography and genetic structure of Acanthamoeba populations among the Iranian clinical isolates and natural/artificial environments distributed in various regions of the country. METHODS: We searched electronic databases including Medline, PubMed, Science Direct, Scopus and Google Scholar from 2005 to 2016. To explore the genetic variability of Acanthamoeba sp, 205 sequences were retrieved from keratitis patients, immunosuppressed cases and environmental sources as of various geographies of Iran...
September 2017: Asian Pacific Journal of Tropical Medicine
https://www.readbyqxmd.com/read/29058572/lifetime-marijuana-and-alcohol-use-and-cognitive-dysfunction-in-people-with-human-immunodeficiency-virus-infection
#12
Sara A Lorkiewicz, Alicia S Ventura, Timothy C Heeren, Michael R Winter, Alexander Y Walley, Meg Sullivan, Jeffrey H Samet, Richard Saitz
BACKGROUND: Substance use is common among people with HIV infection. Alcohol, marijuana, and HIV can have negative effects on cognition. We examined associations between current and lifetime marijuana and alcohol use, and cognitive dysfunction in people with HIV infection. METHODS: We studied a cohort of 215 HIV-infected adults with Diagnostic and Statistical Manual of Mental Disorders Fourth Edition (DSM-IV) substance dependence or ever injection drug use. In adjusted cross-sectional regression analyses we tested the associations between current marijuana use, current heavy alcohol use, lifetime marijuana use, lifetime alcohol use, duration of heavy alcohol use (the independent variables), and three measures of cognitive dysfunction (dependent variables): both the i) memory and ii) attention domains from the Montreal Cognitive Assessment (MoCA), and iii) the 4-item cognitive function scale (CF4) from the Medical Outcomes Study HIV Health Survey (MOS-HIV)...
October 23, 2017: Substance Abuse
https://www.readbyqxmd.com/read/29050515/cytologic-and-ultrastructural-findings-of-bronchoalveolar-lavage-in-patients-with-chronic-granulomatous-disease
#13
Mikako Warren, Hiroyuki Shimada
Background Chronic granulomatous disease (CGD) is a hereditary immunodeficiency caused by mutations in genes encoding nicotinamide adenine dinucleotide phosphate oxidase enzyme complex, which lead to the inability to kill intracellular pathogens. Patients with CGD are susceptible to recurrent bacterial and fungal infections in their early lives. Although the recent survival rate has been significantly improved, early diagnosis is critical to prevent multiple organ impairment. In 1950s, CGD was first described as a disease with recurrent infections and visceral infiltration of granulomas and pigmented histiocytes...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29038665/c-reactive-protein-as-an-independent-cardiovascular-risk-predictor-in-hiv-patients-a-focused-review-of-published-studies
#14
REVIEW
Tarvinder S Gilotra, Stephen A Geraci
Patients infected with the human immunodeficiency virus (HIV+) are living longer and at heightened risk for developing cardiovascular events (CVEs). Commonly used prediction tools appear to misrepresent their CVE risk to varying degrees and in varying directions. Inclusion of markers of cellular infection, chronic immune activation and/or systemic inflammation into risk models might provide better predictive accuracy. Observational studies assessing the relationship of high-sensitivity C-reactive protein (hs-CRP) to CVE in HIV+ patients have reported inconsistent findings...
November 2017: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/29033928/unexpectedly-high-prevalence-of-common-variable-immunodeficiency-in-finland
#15
Jannica S Selenius, Timi Martelius, Sampsa Pikkarainen, Sanna Siitonen, Eero Mattila, Risto Pietikäinen, Pekka Suomalainen, Arja H Aalto, Janna Saarela, Elisabet Einarsdottir, Asko Järvinen, Martti Färkkilä, Juha Kere, Mikko Seppänen
BACKGROUND: Common variable immunodeficiency (CVID) is the most common primary immunodeficiency. Prevalence varies greatly between countries and studies. Most diagnostic criteria include hypogammaglobulinemia and impaired vaccine response. AIM: To evaluate the minimum prevalence as well as the clinical and immunological phenotypes of CVID in Southern Finland. METHODS: We performed a cross-sectional study to assess all adult CVID patients followed up in three hospital districts in Southern and South-Eastern Finland between April 2007 and August 2015...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29030829/review-diagnosing-common-variable-immunodeficiency-disorder-in-the-era-of-genome-sequencing
#16
REVIEW
Rohan Ameratunga, Klaus Lehnert, See-Tarn Woon, David Gillis, Vanessa L Bryant, Charlotte A Slade, Richard Steele
Common variable immunodeficiency disorders (CVID) are an enigmatic group of often heritable conditions, which may manifest for the first time in early childhood or as late as the eighth decade of life. In the last 5 years, next generation sequencing (NGS) has revolutionised identification of genetic disorders. However, despite the best efforts of researchers around the globe, CVID conditions have been slow to yield their molecular secrets. We have previously described the many clinical advantages of identifying the genetic basis of primary immunodeficiency disorders (PIDs)...
October 14, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/29030367/patients-with-common-variable-immunodeficiency-paradoxically-have-increased-rates-of-autoimmune-disorders
#17
Ammu Thampi Susheela, Andrew Hale
Common variable immunodeficiency (CVID), characterised by disordered B cell function, is one of the most common primary immunodeficiency disorders. Patients with CVID are at lifelong risk of recurrent infections, particularly of the respiratory and gastrointestinal tracts. Paradoxically, given their immunocompromised state, patients with CVID are also at significantly increased risk of autoimmune disorders, which are seen in almost 25% of cases. The authors report a 24-year-old female patient with CVID, manifested as severe hypogammaglobulinaemia with recurrent sinopulmonary infections and enterocolitis, who presented with transaminitis, chronic diarrhoea and haematemesis...
October 13, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29019451/autoimmunity-in-a-cohort-of-471-patients-with-primary-antibody-deficiencies
#18
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/28993765/prolonged-excretion-of-poliovirus-among-individuals-with-primary-immunodeficiency-disorder-an-analysis-of-the-world-health-organization-registry
#19
Grace Macklin, Yi Liao, Marina Takane, Kathleen Dooling, Stuart Gilmour, Ondrej Mach, Olen M Kew, Roland W Sutter
Individuals with primary immunodeficiency disorder may excrete poliovirus for extended periods and will constitute the only remaining reservoir of virus after eradication and withdrawal of oral poliovirus vaccine. Here, we analyzed the epidemiology of prolonged and chronic immunodeficiency-related vaccine-derived poliovirus cases in a registry maintained by the World Health Organization, to identify risk factors and determine the length of excretion. Between 1962 and 2016, there were 101 cases, with 94/101 (93%) prolonged excretors and 7/101 (7%) chronic excretors...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28983810/dysregulation-of-innate-lymphoid-cells-in-common-variable-immunodeficiency
#20
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
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