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Common variable immunodeficiency

V Balugo-López, E Hernández García de la Barrera, J Sastre
No abstract text is available yet for this article.
October 2016: Journal of Investigational Allergology & Clinical Immunology
Jeremy Woodward, Effrossyni Gkrania-Klotsas, Dinakantha Kumararatne
Chronic infection with Norovirus is emerging as a significant risk for patients with immunodeficiency - either primary or secondary to therapeutic immunosuppression. Patients with primary immunodeficiency present a range of pathological responses to Norovirus infection. Asymptomatic infections occur and differentiating viral carriage or prolonged viral shedding after self-limiting infection from infection causing protracted diarrhoea can be challenging due to relatively mild pathological changes that may mimic other causes of diarrhoea in such patients (for instance pathogenic bacteria or parasites or graft-versus-host disease)...
October 18, 2016: Clinical and Experimental Immunology
Chuan Shi, Fen Wang, Anli Tong, Xiao-Qian Zhang, Hong-Mei Song, Zheng-Yin Liu, Wei Lyu, Yue-Hua Liu, Wei-Bo Xia
BACKGROUND: Common variable immunodeficiency (CVID) with central adrenal insufficiency is a recently defined clinical syndrome caused by mutations in the nuclear factor kappa-B subunit 2 (NFKB2) gene. We present the first case of NFKB2 mutation in Asian population. METHODS AND RESULTS: An 18-year-old Chinese female with adrenocorticotropic hormone (ACTH) deficiency was admitted due to adrenal crisis and pneumonia. She had a history of recurrent respiratory infections since childhood and ectodermal abnormalities were noted during physical examination...
October 2016: Medicine (Baltimore)
R Yazdani, R Seify, M Ganjalikhani-Hakemi, H Abolhassani, N Eskandari, F Golsaz-Shirazi, B Ansaripour, E Salehi, G Azizi, N Rezaei, A Aghamohammadi
BACKGROUND: Common variable immunodeficiency (CVID) is a heterogeneous disease, characterised by hypogammaglobulinaemia leading to recurrent infections and various complications. The aim of this study was to classify CVID patients based on four known classifications (Paris, Freiburg, EUROclass, and B-cell patterns) by measurement of B-cell subsets and to assess the relation of each classification with clinical manifestations. METHODS: We measured all B-cell subsets as both absolute count and percentage in 30 CVID patients and 30 healthy individuals using four-colour flow cytometry...
October 4, 2016: Allergologia et Immunopathologia
María Belén Almejún, Bárbara Carolina Campos, Virginia Patiño, Miguel Galicchio, Marta Zelazko, Matías Oleastro, Pablo Oppezzo, Silvia Danielian
BACKGROUND: Common variable immunodeficiency is a heterogeneous syndrome characterized by impaired immunoglobulin production and usually presents with a normal quantity of peripheral B cells. Most attempts aiming to classify these patients have mainly been focused on T cell phenotypes or B cell phenotypes and their ability to produce protective antibodies, but it is still a major challenge to find a suitable classification that includes the clinical and immunological heterogeneity of these patients...
October 3, 2016: Journal of Allergy and Clinical Immunology
Elif Azarsız, Neslihan Karaca, Erturk Levent, Necil Kutukculer, Eser Sozmen
BACKGROUND: Common variable immunodeficiency (CVID) is a rare clinically symptomatic primary immunodeficiency disorder which manifests a wide variability of symptoms, complications. Atherosclerosis in CVID patients have not been investigated yet contrary to other severe clinical complications. We aimed to investigate the chitotriosidase enzyme's role as an inflammation and atherosclerosis marker in pediatric CVID patients. METHODS: CVID patients (n =24) and healthy controls (n = 23) evaluated for chitotriosidase activity with other inflammation markers (hsCRP, myeloperoxidase, serum amyloid A, ferritin), lipid profile and echocardiographic findings (carotid artery intima media thickness - cIMT, brachial artery flow-mediated vazodilatation - FMD%)...
October 4, 2016: Annals of Clinical Biochemistry
Jenna Thuc-Uyen Nguyen, Ari Green, Michael R Wilson, Joseph L DeRisi, Katherine Gundling
Common variable immunodeficiency is a rare disorder of immunity associated with a myriad of clinical manifestations including recurrent infections, autoimmunity, and malignancy. Though rare, neurologic complications have been described in a small number of case reports and case series of CVID patients. In this article, we present a patient with CVID who suffered significant neurologic morbidity and categorize the reported range of neurologic complications associated with CVID. Our case highlights the complex nature of neurologic manifestations in CVID patients, and our review of the current database suggests that infection and inflammatory neurologic disorders are the cause of most neurologic presentations...
October 4, 2016: Journal of Clinical Immunology
Esmaeil Mortaz, Payam Tabarsi, Davod Mansouri, Adnan Khosravi, Johan Garssen, Aliakbar Velayati, Ian M Adcock
The life span of patients with primary and secondary immunodeficiency is increasing due to recent improvements in therapeutic strategies. While the incidence of primary immunodeficiencies (PIDs) is 1:10,000 births, that of secondary immunodeficiencies are more common and are associated with posttransplantation immune dysfunction, with immunosuppressive medication for human immunodeficiency virus or with human T-cell lymphotropic virus infection. After infection, malignancy is the most prevalent cause of death in both children and adults with (PIDs)...
2016: Frontiers in Immunology
Daniel Peckham, Thomas Scambler, Sinisa Savic, Michael F McDermott
Immune-mediated autoinflammatory diseases are occupying an increasingly prominent position among the pantheon of debilitating conditions that afflict mankind. This review focuses on some of the key developments which have occurred since the original description of autoinflammatory disease in 1999, and focuses on underlying mechanisms that trigger autoinflammation. The monogenic autoinflammatory disease range has expanded considerably during that time, and now includes a broad spectrum of disorders, including relatively common conditions such as cystic fibrosis and subsets of systemic lupus erythematosus...
September 29, 2016: Journal of Pathology
Reza Yazdani, Mazdak Ganjalikhani-Hakemi, Mohammad Esmaeili, Hassan Abolhassani, Shahram Vaeli, Abbas Rezaei, Zohre Sharifi, Gholamreza Azizi, Nima Rezaei, Asghar Aghamohammadi
Common variable immunodeficiency (CVID) is a heterogeneous group of primary immunodeficiency characterized by recurrent infectious. We evaluated whether defective PI3K/Akt/FoxO pathway could influence B-cell fate. Determination of B-cell subsets in CVD patients and healthy donors (HDs) were performed using flow cytometry. We evaluated mRNA level and protein expression of PI3K, Akt and FoxO molecules using real-time PCR and flow cytometry, respectively. Moreover, phosphorylated Akt (p-Akt) expression in B-cells has also been measured by flowcytometry...
September 21, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
Wondemagegn Mulu, Yohannes Zenebe, Bayeh Abera, Mulat Yimer, Tadesse Hailu
BACKGROUND: Young women aged 15-24 years are members of key populations at higher risk for Human Immunodeficiency Virus (HIV) acquisition through sexual intercourse. In areas where unprotected sex is a common practice, Hepatitis B virus (HBV) commonly transmitted via sexual and parenteral routes. The study aimed at determining HIV and HBV infections prevalence in young women attending health institutions for abortion care in Bahir Dar city, Ethiopia. METHODS: A cross - sectional study was conducted from January 2015 to June 2015...
2016: BMC Public Health
T V Latysheva, E A Latysheva, I A Martynova, G E Aminova
Primary immunodeficiencies (PIDs) are a group of congenital diseases of the immune system, which numbers more than 230 nosological entities associated with lost, decreased, or wrong function of its one or several components. Due to the common misconception that these are extremely rare diseases that occur only in children and lead to their death at an early age, PIDs are frequently ruled out by physicians of related specialties from the range of differential diagnosis. The most common forms of PIDs, such as humoral immunity defects, common variable immune deficiency, X-linked agammaglobulinemia, selective IgA deficiency, etc...
2016: Terapevticheskiĭ Arkhiv
Gholamreza Azizi, Hassan Abolhassani, Mohammad Hosein Asgardoon, Tina Alinia, Reza Yazdani, Javad Mohammadi, Nima Rezaei, Hans D Ochs, Asghar Aghamohammadi
INTRODUCTION: Common variable immunodeficiency (CVID) comprises a large heterogeneous group of patients with primary antibody deficiency. AREAS COVERED: The affected patients are characterized by increased susceptibility to infections and low levels of serum immunoglobulin. However, enteropathy, granulomatous organ infiltrates, malignancy, inflammatory and autoimmune conditions are also prevalent. The concomitance of immunodeficiency and autoimmunity appears to be paradoxical and creates difficulties in the management of autoimmune complications affecting these patients...
September 16, 2016: Expert Review of Clinical Immunology
Jing Zhao, Rui Tian, Tao An, Jing Xu, Tingting Cui, Xuefeng Sun, Yong Chen, Mengzhao Wang, Baiqiang Cai, Jinming Gao, Rong Huang, Zuojun Xu
No abstract text is available yet for this article.
September 16, 2016: American Journal of Respiratory and Critical Care Medicine
Delfien J A Bogaert, Marieke De Bruyne, Veronique Debacker, Pauline Depuydt, Katleen De Preter, Carolien Bonroy, Jan Philippe', Victoria Bordon, Bart N Lambrecht, Tessa Kerre, Andrea Cerutti, Karim Y Vermaelen, Filomeen Haerynck, Melissa Dullaers
The etiology of primary antibody deficiencies is largely unknown. Beside rare monogenic forms, the majority of cases seem to have a more complex genetic basis. Whereas common variable immunodeficiency has been investigated in-depth, only few reports exist on milder primary antibody deficiencies like idiopathic primary hypogammaglobulinemia and IgG subclass deficiency. We performed flow cytometric immunophenotyping in 33 common variable immunodeficiency, 23 idiopathic primary hypogammaglobulinemia and 21 IgG subclass deficiency patients, and in 47 asymptomatic first-degree family members of patients and 101 unrelated healthy controls...
September 15, 2016: Haematologica
Supriya M Paranjpe, Avani Koticha, Preeti R Mehta
Common Variable Immunodeficiency (CVID) is a primary immunodeficiency characterized by low antibody levels and recurrent infections. This makes an individual more prone to recurrent respiratory and gastrointestinal tract infections. In cases where there is persistent positive finding of intestinal parasites in stool, a high index of suspicion should be raised to rule out immunodeficiency state. Early diagnosis of such cases will help in reducing the morbidity and better management of the patient. A case of CVID in 18-year-old male with recurrent lower respiratory tract infection and chronic diarrhoea due to Giardia lamblia is reported herewith...
July 2016: Journal of Clinical and Diagnostic Research: JCDR
Sarah A Williams, Laura E Moench, Fatima Khan, Gregory Vercellotti, Michael A Linden
Common variable immunodeficiency (CVID) comprises a heterogeneous group of disorders of humoral immunity, characterized by marked deficiencies in serum immunoglobulins. Immune dysregulation causes susceptibility to recurrent bacterial infections, as well as autoimmune and lymphoproliferative disorders. Although the lymphoid cells comprising the atypical proliferations are often clonally related, their malignant potential and clinical significance differ from similar lesions in individuals with immunocompetence...
November 2016: Laboratory Medicine
H Marefat, H Abolhassani, M Ghareje Daghi, G Azizi, A Aghamohammadi
Purpose. To report early onset steroid induced posterior subcapsular cataract in a case of common variable immunodeficiency. Methods. Case report. Results. Here we report a 14-yearold male of steroid induced bilateral posterior subcapsular cataract in a common variable immunodeficiency patient with damaging mutations in Glutathione reductase gene, leading to hypersensitivity of patient to glucocorticoid (GC) products. Conclusions. In order to reduce the ocular side effects of the GCs there are some advisements, including a complete history, regular examination, GC should be prescribed in minimal dosage and minimal course, and as possible GC-sparing drugs should always be considered...
September 2016: European Annals of Allergy and Clinical Immunology
C Poli, R Hoyos-Bachiloglu, A Borzutzky
BACKGROUND: The epidemiology and hospitalisation trends of primary immunodeficiency (PID) in Chile are unknown. We aimed to evaluate hospitalisation trends and demographic characteristics of PID admissions in Chile. METHODS: PID admissions between 2001 and 2010 (ICD-10 codes D70.0, D70.4, D71, 72.0, D76.1, D80-D84, E70.3, G11.3) were reviewed using national hospital discharge databases. RESULTS: During the study period, 5486 admissions due to PID were registered (0...
August 28, 2016: Allergologia et Immunopathologia
Julio César Cambray-Gutiérrez, Ulises Noel García-Ramírez, Leonel Gerardo Del Rivero-Hernández, Patricia López-Pérez, Aurora Chávez-García
BACKGROUND: Common variable immunodeficiency is the most commonly-diagnosed primary immunodeficiency in adults; it is characterized by recurrent sinopulmonary and gastrointestinal infections, and increased incidence of malignancy and autoimmune processes. Many patients begin to have clinical manifestations during reproductive age. CASE REPORT: A 34-year-old woman with 12 weeks of gestation who was diagnosed with common variable immunodeficiency after recurrent episodes of rhinosinusitis, pharyngoamygdalitis, and pneumonia...
July 2016: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
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