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https://www.readbyqxmd.com/read/29881587/improvement-of-common-variable-immunodeficiency-using-embryonic-stem-cell-therapy-in-a-patient-with-lyme-disease-a-clinical-case-report
#1
Richard Horowitz, Phyllis R Freeman
Bone marrow transplantation and stem cell therapies have been used for the treatment of common variable immunodeficiency (CVID) and other life-threatening medical disorders. This is the first known case report in the medical literature describing improvement of both Lyme disease and CVID with human embryonic stem cell therapy.
June 2018: Clinical Case Reports
https://www.readbyqxmd.com/read/29867917/delayed-diagnosis-and-complications-of-predominantly-antibody-deficiencies-in-a-cohort-of-australian-adults
#2
Charlotte A Slade, Julian J Bosco, Tran Binh Giang, Elizabeth Kruse, Robert G Stirling, Paul U Cameron, Fiona Hore-Lacy, Michael F Sutherland, Sara L Barnes, Stephen Holdsworth, Samar Ojaimi, Gary A Unglik, Joseph De Luca, Mittal Patel, Jeremy McComish, Kymble Spriggs, Yang Tran, Priscilla Auyeung, Katherine Nicholls, Robyn E O'Hehir, Philip D Hodgkin, Jo A Douglass, Vanessa L Bryant, Menno C van Zelm
Background: Predominantly antibody deficiencies (PADs) are the most common type of primary immunodeficiency in adults. PADs frequently pass undetected leading to delayed diagnosis, delayed treatment, and the potential for end-organ damage including bronchiectasis. In addition, PADs are frequently accompanied by comorbid autoimmune disease, and an increased risk of malignancy. Objectives: To characterize the diagnostic and clinical features of adult PAD patients in Victoria, Australia...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29867916/evaluating-the-genetics-of-common-variable-immunodeficiency-monogenetic-model-and-beyond
#3
Guillem de Valles-Ibáñez, Ana Esteve-Solé, Mònica Piquer, E Azucena González-Navarro, Jessica Hernandez-Rodriguez, Hafid Laayouni, Eva González-Roca, Ana María Plaza-Martin, Ángela Deyà-Martínez, Andrea Martín-Nalda, Mónica Martínez-Gallo, Marina García-Prat, Lucía Del Pino-Molina, Ivón Cuscó, Marta Codina-Solà, Laura Batlle-Masó, Manuel Solís-Moruno, Tomàs Marquès-Bonet, Elena Bosch, Eduardo López-Granados, Juan Ignacio Aróstegui, Pere Soler-Palacín, Roger Colobran, Jordi Yagüe, Laia Alsina, Manel Juan, Ferran Casals
Common variable immunodeficiency (CVID) is the most frequent symptomatic primary immunodeficiency characterized by recurrent infections, hypogammaglobulinemia and poor response to vaccines. Its diagnosis is made based on clinical and immunological criteria, after exclusion of other diseases that can cause similar phenotypes. Currently, less than 20% of cases of CVID have a known underlying genetic cause. We have analyzed whole-exome sequencing and copy number variants data of 36 children and adolescents diagnosed with CVID and healthy relatives to estimate the proportion of monogenic cases...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29850647/acute-gastrointestinal-bleeding-due-to-cytomegalovirus-colitis-in-a-patient-with-common-variable-immunodeficiency
#4
Pradeep Kathi, Maher Tama, Vivek Reddy, Shankerdas Kundumadam, Omar Al-Subee, Murray N Ehrinpreis
Common variable immunodeficiency (CVID) is a heterogeneous disorder with decreased production of immunoglobulins that presents with recurrent and serious infections in ∼90% of cases. Review of the literature showed only 12 cases of gastrointestinal infection of cytomegalovirus in patients with CVID. We present an unusual case of a CVID patient presenting with new-onset hematochezia. With proper clinical suspicion and endoscopic evaluation, cytomegalovirus colitis was diagnosed and acute bleeding was successfully treated endoscopically...
2018: ACG Case Reports Journal
https://www.readbyqxmd.com/read/29849668/evaluation-of-clinical-and-immunological-characteristics-of-children-with-common-variable-immunodeficiency
#5
Gülsüm Alkan, Sevgi Keles, İsmail Reisli
Background: Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder (PID) that typically presents with hypogammaglobulinemia and impaired antibody production. Objectives: This study aimed to promote the awareness of CVID, whose clinical spectrum is quite broad. Methods: The demographic, clinical, and laboratory characteristics of 12 children (seven males and five females) with CVID were analyzed retrospectively. The patients were diagnosed using the diagnostic criteria of the European Society for Primary Immunodeficiencies...
2018: International Journal of Pediatrics
https://www.readbyqxmd.com/read/29806948/keeping-it-in-the-family-the-case-for-considering-late-onset-combined-immunodeficiency-a-subset-of-common-variable-immunodeficiency-disorders
#6
Rohan Ameratunga, Yeri Ahn, Anthony Jordan, Klaus Lehnert, Shannon Brothers, See-Tarn Woon
Common Variable Immunodeficiency Disorders (CVID) are the most frequent symptomatic primary immune defect in adults. Within the broad spectrum of CVID, a proportion of patients present with a predominant T cell phenotype associated with increased mortality. These patients are termed late onset combined immunodeficiency (LOCID) and are currently separated from patients suffering from CVID. Areas covered: We have recently co-discovered a new CVID-like disorder caused by mutations of the NFKB1 gene. Members of this non-consanguineous NZ kindred have a very diverse spectrum of phenotypes in spite of carrying the identical mutation...
May 28, 2018: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/29780906/hla-a29-negative-birdshot-like-chorioretinopathy-associated-with-common-variable-immunodeficiency
#7
Razek Georges Coussa, Fares Antaki, David E Lederer
Purpose: To report the longest ophthalmic follow-up and the associated ocular complications of HLA-A29 negative Birdshot-like chorioretinopathy (BLCR) associated with common variable immunodeficiency (CVID). Observations: A 22-year-old man known for CVID presented with a 3-month history of decreased visual acuity OS. Funduscopy revealed significant cystoid macular edema OS, as well as optic disk edema and chorioretinal infiltrates without signs of vitritis OU. No infectious, inflammatory or neoplastic etiologies were identified...
June 2018: American Journal of Ophthalmology Case Reports
https://www.readbyqxmd.com/read/29780795/a-novel-de-novo-mutation-in-the-cd40-ligand-gene-in-a-patient-with-a-mild-x-linked-hyper-igm-phenotype-initially-diagnosed-as-cvid-new-aspects-of-old-diseases
#8
Tábata T França, Luiz F B Leite, Tiago A Maximo, Christiane G Lambert, Nuria B Zurro, Wilma C N Forte, Antonio Condino-Neto
Mutations in the CD40 ligand (CD40L) gene ( CD40LG ) lead to X-linked hyper-IgM syndrome (X-HIGM), which is a primary immunodeficiency (PID) characterized by decreased serum levels of IgG and IgA and normal or elevated IgM levels. Although most X-HIGM patients become symptomatic during the first or second year of life, during which they exhibit recurrent infections, some patients exhibit mild phenotypes, which are usually associated with hypomorphic mutations that do not abrogate protein expression or function...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29757592/the-profile-of-toll-like-receptor-2-tlr2-tlr4-and-their-cytosolic-downstream-signaling-pathway-in-common-variable-immunodeficiency-cvid-patients
#9
Laleh Sharifi, Asghar Aghamohammadi, Nima Rezaei, Reza Yazdani, Mahdi Mahmoudi, Mohammad Mehdi Amiri, Farimah Masoumi, Saied Bokaie, Parsova Tavasolian, Rouzbeh Sanaei, Mona Moshiri, Naeimeh Tavakolinia, Tina Alinia, Gholamreza Azizi, Abbas Mirshafiey
Common variable immunodeficiency (CVID) is the most common clinical primary antibody deficiency, characterized by increased susceptibility to recurrent bacterial infections. Since Toll-like receptors (TLRs) play an important role in the maturation and differentiation of B-cells, TLRs' defect can be involved in the pathogenesis of CVID. Therefore, we evaluated the expression of TLR2 and TLR4 and their signaling pathway; also their association with autoimmunity, B-cell subtypes and response to pneumovax-23 were assessed in CVID patients...
April 2018: Iranian Journal of Allergy, Asthma, and Immunology
https://www.readbyqxmd.com/read/29736405/renal-evaluation-in-common-variable-immunodeficiency
#10
Giovany Gomes Capistrano, Gdayllon Cavalcante Meneses, Fernanda Macedo de Oliveira Neves, Renata de Almeida Leitão, Alice Maria Costa Martins, Alexandre Braga Libório
Introduction: Common variable immunodeficiency (CVID) comprises a heterogeneous group of disorders characterized by impaired antibody production. Kidney involvement in CVID is described in isolated and sporadic case reports. The objective of this study was to study the renal function pattern in CVID patients through glomerular and tubular function tests. Methods: Study of 12 patients with CVID diagnosis and 12 healthy control individuals. Glomerular filtration rate (GFR), fractional excretion of sodium (FENa+ ) and potassium (FEK+ ), urinary concentration, and acidification capacity were measured...
2018: Journal of Immunology Research
https://www.readbyqxmd.com/read/29709555/genetic-screening-of-male-patients-with-primary-hypogammaglobulinemia-can-guide-diagnosis-and-clinical-management
#11
Nicolas Vince, Gaël Mouillot, Marion Malphettes, Sophie Limou, David Boutboul, Angélique Guignet, Véronique Bertrand, Philippe Pellet, Pierre-Antoine Gourraud, Patrice Debré, Eric Oksenhendler, Ioannis Théodorou, Claire Fieschi
The precise diagnosis of an immunodeficiency is sometimes difficult to assess, especially due to the large spectrum of phenotypic variation reported among patients. Common variable immunodeficiency disorders (CVID) do not have, for a large part, an identified genetic cause. The identification of a causal genetic mutation is important to confirm, or in some cases correct, the diagnosis. We screened >150 male patients with hypogammaglobulinemia for mutations in three genes involved in pediatric X-linked primary immunoglobulin deficiency: CD40LG, SH2D1A and BTK...
July 2018: Human Immunology
https://www.readbyqxmd.com/read/29678747/predictive-markers-for-humoral-influenza-vaccine-response-in-patients-with-common-variable-immunodeficiency
#12
Ann Gardulf, Hassan Abolhassani, Rolf Gustafson, Lars E Eriksson, Lennart Hammarström
BACKGROUND: A subgroup of patients with common variable immunodeficiencies (CVIDs) responds to vaccination. The aim of this study was to try to identify predictive markers for those with a humoral immune response after influenza vaccination. METHODS: Forty-eight patients with CVID (29 female and 19 male patients; mean age, 57.7 years) were vaccinated with the A(H1N1) influenza vaccine Pandemrix (GlaxoSmithKline, Wavre, Belgium) and boosted after 1 month. Blood samples were collected before each vaccination and 2 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
#13
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
#14
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/29619656/complications-associated-with-underweight-primary-immunodeficiency-patients-prevalence-and-associations-within-the-usidnet-registry
#15
Melanie A Ruffner, Kathleen E Sullivan
PURPOSE: The point prevalence of underweight status and obesity in primary immunodeficiency disease (PID) is unknown, despite the described associations between PID and weight loss and failure to thrive. The goal of this study is to estimate the prevalence of underweight status and obesity in PID patients and to investigate the associations between abnormal body weight and complications of PID. METHODS: Using the US Immunodeficiency Network (USIDNET), we performed a retrospective analysis of 653 pediatric (age 2 to 20 years) and 514 adult (age > 20) patient records with information on patient body mass index (BMI)...
April 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29618830/evaluation-of-the-tlr-negative-regulatory-network-in-cvid-patients
#16
Roozbeh Sanaei, Nima Rezaei, Asghar Aghamohammadi, Ali-Akbar Delbandi, Shahram Teimourian, Reza Yazdani, Parsova Tavasolian, Fatemeh Kiaee, Nader Tajik
Common variable immunodeficiency (CVID), a clinically symptomatic primary immunodeficiency disease (PID), is characterized by hypogammaglobulinemia leading to recurrent infections and various complications. Recently, some defects in the signaling of TLRs have been identified in CVID patients which led us to investigate the expression of TLR4 and 9 negative regulatory molecules and their upregulation status following their activation. Using TaqMan real-time PCR, SOCS1, TNFAIP3, RFN216, and IRAK-M transcripts among peripheral blood mononuclear cells (PBMCs) were measured with/without TLR4 and 9 activations...
April 5, 2018: Genes and Immunity
https://www.readbyqxmd.com/read/29618687/-successful-cord-blood-transplantation-in-a-patient-with-adult-onset-common-variable-immunodeficiency
#17
Yuhei Harutani, Shinobu Tamura, Hiroshi Kobata, Takehiro Oiwa, Yusuke Yamashita, Toshiki Mushino, Hiroki Hosoi, Shogo Murata, Akinori Nishikawa, Kohsuke Imai, Tomohiro Morio, Takashi Sonoki
Common variable immunodeficiency (CVID) is the most frequently diagnosed congenital immunodeficiency and is characterized by dysfunctional antibody production. It often occurs at the age of ≥10 years. Here we reported a case of a 46-year-old man confirmed with adult-onset CVID. He was effectively treated with cord blood transplant (CBT). The patient was observed with repeated upper respiratory infection a few years back and was referred to our department owing to a marked decrease in neutrophil counts and progression of anemia...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29614902/intravenous-immunoglobulin-ivig-efficiency-in-women-with-common-variable-immunodeficiency-cvid-decreases-significantly-during-pregnancy
#18
Makiko Egawa, Hirokazu Kanegane, Kohsuke Imai, Tomohiro Morio, Naoyuki Miyasaka
Immunoglobulin replacement therapy, including intravenous immunoglobulin (IVIG), is essential for pregnant women with common variable immunodeficiency (CVID) since it prevents infection and improves the health of the newborn. There are no established IVIG treatment protocols for pregnant women with CVID, and the relationship between IVIG treatment and maternal serum IgG changes during pregnancy remains unclear. Therefore, we reviewed the medical charts of four CVID patients, including one receiving subcutaneous immunoglobulin (SCIG), for IVIG dose and frequency, maternal serum IgG changes, obstetrical findings, and perinatal outcomes...
April 3, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29599028/phenotypic-characterization-of-patients-with-rheumatologic-manifestations-of-common-variable-immunodeficiency
#19
Maria J Gutierrez, Kathleen E Sullivan, Ramsay Fuleihan, Clifton O Bingham
Patients with common variable immunodeficiency (CVID) have a higher incidence of rheumatologic disorders. To delineate this clinical association, we investigated the phenotypic features of patients with CVID affected by these conditions. METHODS: We conducted a retrospective analysis of 870 pediatric and adult patients with CVID included in the United States Immunodeficiency Network (USIDNET) registry. Outcomes included clinical characteristics (age, gender, ethnicity, rheumatologic diagnosis, and comorbidities), infectious history and basic immunophenotype (serum immunoglobulin levels, CD19+ B cells, and CD4/CD8 ratio) in patients with CVID and rheumatologic disorders compared to those with non-inflammatory CVID...
February 23, 2018: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29574865/rheumatologic-complications-in-a-cohort-of-227-patients-with-common-variable-immunodeficiency
#20
G Azizi, F Kiaee, E Hedayat, R Yazdani, E Dolatshahi, T Alinia, L Sharifi, H Mohammadi, H Kavosi, F Jadidi-Niaragh, V Ziaee, H Abolhassani, A Aghamohammadi
Common variable immunodeficiency (CVID) is the most prevalent symptomatic type of human primary immunodeficiency diseases (PID). Clinically, CVID is characterized by increased susceptibility to infections and a wide variety of autoimmune and rheumatologic disorders. All patients with CVID registered in Iranian PID Registry (IPIDR) were enrolled in this retrospective cohort study. We investigated the frequency of rheumatologic diseases and its association with immunological and clinical phenotypes in patients with CVID...
May 2018: Scandinavian Journal of Immunology
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