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Selective IGA Deficiency

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https://www.readbyqxmd.com/read/27920422/population-based-screening-for-selective-immunoglobulin-a-iga-deficiency-in-lithuanian-children-using-a-rapid-antibody-based-fingertip-test
#1
Vaidotas Urbonas, Jolita Sadauskaite, Rimante Cerkauskiene, Arvydas Kaminskas, Markku Mäki, Kalle Kurppa
BACKGROUND Selective immunoglobulin A (IgA) deficiency is the most common inherited immunodeficiency disorder worldwide. An early diagnosis is advocated because of the increased risk of infections, autoimmune diseases, and allergic reactions. We investigated the usefulness of a rapid point-of-care test in detecting for IgA deficiency in a population with a previously unknown prevalence. MATERIAL AND METHODS Altogether, 1000 children aged 11-13 years from randomly selected Lithuanian schools were enrolled. A point-of-care test with a fingertip sample was used to screen for the presence of IgA deficiency in children whose parents gave consent...
December 6, 2016: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/27884168/ataxia-telangiectasia-a-review
#2
REVIEW
Cynthia Rothblum-Oviatt, Jennifer Wright, Maureen A Lefton-Greif, Sharon A McGrath-Morrow, Thomas O Crawford, Howard M Lederman
DEFINITION OF THE DISEASE: Ataxia telangiectasia (A-T) is an autosomal recessive disorder primarily characterized by cerebellar degeneration, telangiectasia, immunodeficiency, cancer susceptibility and radiation sensitivity. A-T is often referred to as a genome instability or DNA damage response syndrome. EPIDEMIOLOGY: The world-wide prevalence of A-T is estimated to be between 1 in 40,000 and 1 in 100,000 live births. CLINICAL DESCRIPTION: A-T is a complex disorder with substantial variability in the severity of features between affected individuals, and at different ages...
November 25, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27800605/b-cell-activation-with-cd40l-or-cpg-measures-the-function-of-b-cell-subsets-and-identifies-specific-defects-in-immunodeficient-patients
#3
Emiliano Marasco, Chiara Farroni, Simona Cascioli, Valentina Marcellini, Marco Scarsella, Ezio Giorda, Eva Piano Mortari, Lucia Leonardi, Alessia Scarselli, Diletta Valentini, Caterina Cancrini, Marzia Duse, Ola Grimsholm, Rita Carsetti
Around 65% of primary immunodeficiencies are antibody deficiencies. Functional tests are useful tools to study B-cell functions in vitro. However, no accepted guidelines for performing and evaluating functional tests have been issued yet. Here, we report our experience on the study of B-cell functions in infancy and throughout childhood. We show that T-independent stimulation with CpG measures proliferation and differentiation potential of memory B cells. Switched memory B cells respond better than IgM memory B cells...
November 1, 2016: European Journal of Immunology
https://www.readbyqxmd.com/read/27795213/-clinic-of-humoral-primary-immunodeficiencies-in-adults-experience-in-a-tertiary-hospital
#4
Julio César Cambray-Gutiérrez, Diana Andrea Herrera-Sánchez, Lizbeth Blancas-Galicia, Patricia María O'Farrill-Romanillos
BACKGROUND: Primary immunodeficiencies (PID) are characterized by alteration of the components of the immune system. Humoral deficiencies represent 50%. The most common are selective IgA deficiency, Bruton agammaglobulinemia, and common variable immunodeficiency (CVID). OBJECTIVE: To describe the epidemiological and clinical characteristics of adults with humoral PID, cared for in a Primary Humoral Immunodeficiencies Clinic. METHODS: A descriptive cross-sectional study that included a year of analysis, including 35 patients with humoral PID, 31 with CVID, and 4 with Bruton agammaglobulinemia...
October 2016: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/27790582/salivary-iga-versus-hiv-and-dental-caries
#5
Pradip Kumar Mandal, Malay Mitra, Sonu Acharya, Chiranjit Ghosh, Susant Mohanty, Subrata Saha
INTRODUCTION: The inter-relationship of Human Immunodeficiency Virus (HIV) infection and dental caries as well as Salivary Immunoglobulin-A (S-IgA) level appear to remain under explored while a manual and electronic search of the literature was made. Hence, the present study was undertaken to assess the relationship of S-IgA and dental caries status in HIV positive children. AIM: The aim of this study was to find out the relationship of S-IgA antibody with dental caries by measuring the concentration of IgA in saliva of HIV positive and negative children and determine the dental caries status in HIV positive and HIV negative children, which may help in treatment planning and prevention of the same...
September 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27763681/selective-iga-deficiency-epidemiology-pathogenesis-clinical-phenotype-diagnosis-prognosis-and-management
#6
Reza Yazdani, Gholamreza Azizi, Hassan Abolhassani, Asghar Aghamohammadi
Selective immunoglobulin A deficiency (SIgAD) is the most common primary antibody deficiency. Although more patients with SIgAD are asymptomatic, selected patients suffer from different clinical complications such as pulmonary infections, allergies, autoimmune diseases, gastrointestinal disorders and malignancy. Pathogenesis of SIgAD is still unknown, however, a defective terminal differentiation of B-cells and defect in switching to IgA-producing plasma cells are presumed to be responsible. Furthermore, some cytogenic defects and monogenic mutations are associated with SIgAD...
October 20, 2016: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/27734646/cavitary-lung-lesions-in-a-difficult-to-treat-asthma-patient
#7
R Ragesh, Animesh Ray, Agrima Mian, Surabhi Vyas, S K Sharma
We describe an interesting case of severe asthma who was not showing satisfactory response to standard treatment. Investigations revealed him to be suffering from allergic bronchopulmonary aspergillosis (ABPA). After starting systemic steroids he showed marked improvement initially only to have recurrent symptoms within a year. He was investigated further and found to have chronic pulmonary aspergillosis in the form of chronic cavitary pulmonary aspergillosis (CCPA) and aspergilloma as also the presence of selective IgA deficiency...
April 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27723758/common-variants-at-pvt1-atg13-ambra1-ahi1-and-clec16a-are-associated-with-selective-iga-deficiency
#8
Paola G Bronson, Diana Chang, Tushar Bhangale, Michael F Seldin, Ward Ortmann, Ricardo C Ferreira, Elena Urcelay, Luis Fernández Pereira, Javier Martin, Alessandro Plebani, Vassilios Lougaris, Vanda Friman, Tomáš Freiberger, Jiri Litzman, Vojtech Thon, Qiang Pan-Hammarström, Lennart Hammarström, Robert R Graham, Timothy W Behrens
Selective immunoglobulin A deficiency (IgAD) is the most common primary immunodeficiency in Europeans. Our genome-wide association study (GWAS) meta-analysis of 1,635 patients with IgAD and 4,852 controls identified four new significant (P < 5 × 10(-8)) loci and association with a rare IFIH1 variant (p.Ile923Val). Peak new variants (PVT1, P = 4.3 × 10(-11); ATG13-AMBRA1, P = 6.7 × 10(-10); AHI1, P = 8.4 × 10(-10); CLEC16A, P = 1.4 × 10(-9)) overlapped with autoimmune markers (3/4) and correlated with 21 putative regulatory variants, including expression quantitative trait loci (eQTLs) for AHI1 and DEXI and DNase hypersensitivity sites in FOXP3(+) regulatory T cells...
November 2016: Nature Genetics
https://www.readbyqxmd.com/read/27681397/salivary-iga-and-dental-caries-in-hiv-patients-a-pilot-study
#9
Sonu Acharya, Pradip Kumar Mandal
INTRODUCTION: The interrelationship of human immunodeficiency virus (HIV) infection and dental caries, as well as Salivary IgA (S-IgA) level, appear to remain underexplored while a manual and electronic search of the literature was made. Hence, this study was undertaken to assess the relationship of S-IgA and dental caries status in HIV +ve children. AIM: The aim of this study was to find out the relationship of S-IgA antibody with dental caries by measuring the concentration of IgA in saliva of HIV +ve and HIV -ve children and to determine the dental caries status in HIV +ve and HIV -ve children, which may help in treatment planning and prevention of the same...
October 2016: Journal of the Indian Society of Pedodontics and Preventive Dentistry
https://www.readbyqxmd.com/read/27647947/galactose-deficient-iga1-as-a-candidate-urinary-polypeptide-marker-of-iga-nephropathy
#10
Hitoshi Suzuki, Landino Allegri, Yusuke Suzuki, Stacy Hall, Zina Moldoveanu, Robert J Wyatt, Jan Novak, Bruce A Julian
In patients with IgA nephropathy (IgAN), circulatory IgA1 and IgA1 in mesangial deposits contain elevated amounts of galactose-deficient IgA1 (Gd-IgA1). We hypothesized that a fraction of Gd-IgA1 from the glomerular deposits and/or circulation may be excreted into the urine and thus represent a disease-specific biomarker. Levels of urinary IgA and Gd-IgA1 were determined in 207 patients with IgAN, 205 patients with other renal diseases, and 57 healthy controls, recruited in USA, Japan, and Italy. Urinary IgA was similarly elevated in patients with IgAN and renal-disease controls compared with healthy controls...
2016: Disease Markers
https://www.readbyqxmd.com/read/27636936/-pulmonary-manifestations-in-adult-patients-with-a-defect-in-humoral-immunity
#11
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
https://www.readbyqxmd.com/read/27540116/clathrin-light-chains-role-in-selective-endocytosis-influences-antibody-isotype-switching
#12
Shuang Wu, Sophia R Majeed, Timothy M Evans, Marine D Camus, Nicole M L Wong, Yvette Schollmeier, Minjong Park, Jagan R Muppidi, Andrea Reboldi, Peter Parham, Jason G Cyster, Frances M Brodsky
Clathrin, a cytosolic protein composed of heavy and light chain subunits, assembles into a vesicle coat, controlling receptor-mediated endocytosis. To establish clathrin light chain (CLC) function in vivo, we engineered mice lacking CLCa, the major CLC isoform in B lymphocytes, generating animals with CLC-deficient B cells. In CLCa-null mice, the germinal centers have fewer B cells, and they are enriched for IgA-producing cells. This enhanced switch to IgA production in the absence of CLCa was attributable to increased transforming growth factor β receptor 2 (TGFβR2) signaling resulting from defective endocytosis...
August 30, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27536208/a-rare-case-of-addison-s-disease-hepatitis-thyreoiditis-positive-igg-anti-tissue-transglutaminase-antibodies-and-partial-iga-deficiency
#13
Marta P Baleva, Snejina Mihaylova, Petja Yankova, Iliana Atanasova, Milena Nikolova-Vlahova, Elissaveta Naumova
INTRODUCTION: Selective IgA deficiency (IgAD) is the most prevalent type of primary immune deficiencies, but partial IgA deficiency is even more common. Addison's disease is a rare condition associated with primary adrenal insufficiency due to infection or autoimmune destruction of the adrenals. The association between IgA deficiency and Addison's disease is very rare. CASE AND LABORATORY DATA: We observed a 22-year-old male patient with marked darkening of the skin, especially on the palms and areolae, jaundice on the skin and sclera, astheno-adynamia, hypotension (80/50 mm Hg), and pain in the right hypochondrium...
2016: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/27492260/lymphoma-secondary-to-congenital-and-acquired-immunodeficiency-syndromes-at-a-turkish-pediatric-oncology-center
#14
Hikmet G Tanyildiz, Handan Dincaslan, Gulsan Yavuz, Emel Unal, Aydan Ikinciogulları, Figen Dogu, Nurdan Tacyildiz
The prevalence of lymphoma in primary immunodeficiency cases and autoimmune diseases, as well as on a background of immunodeficiency following organ transplants, is increasing. The lymphoma treatment success rate is known to be a low prognosis. Our study aimed to emphasize the low survival rates in immunodeficient vs. immunocompetent lymphoma patients and also to investigate the effect of rituximab in patients with ataxia telangiectasia and other immunodeficiencies. We summarized the clinical characteristics and treatment results of 17 cases with primary immunodeficiency that developed non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL) retrospectively...
October 2016: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/27324471/biomarkers-and-targeted-new-therapies-for-iga-nephropathy
#15
Rosanna Coppo
IgA nephropathy (IgAN) has variable clinical presentation and outcome. There is a need to identify children who have the potential to progress to end stage renal disease (ESRD). Biomarkers related to the pathogenetic process of IgAN can detect risk factors and identify targets for new therapies. Galactose-deficient IgA1 (Gd-IgA1) is a specific biomarker of IgAN and could be the first treatment target. In experimental mice, reduction of IgA1 deposits and hematuria was observed after treatment with a bacterial protease that selectively cleaves human IgA1...
June 20, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27257449/disseminated-cat-scratch-disease-in-an-adult-with-selective-iga-deficiency
#16
Aaron Rohr, Ryan Ash, John Vadaparampil, Jacqueline Hill, Louis Wetzel
A 51-year-old man with history of undiagnosed pulmonary nodules 4 years prior, presented with right-sided chest pain. Acute cardiac workup was negative, and a chest computed tomography examination demonstrated marked improvement in bilateral pulmonary nodules. A concordant abdominal computed tomography examination showed new subcentimeter hypodense lesions throughout the liver and spleen, mild progressive abdominopelvic lymphadenopathy, and new small lytic lesions of T11 and L4 vertebrae. A positron emission tomography examination demonstrated hypermetabolic activity of these abdominopelvic lesions suggesting metastatic disease...
June 2016: Radiology case reports
https://www.readbyqxmd.com/read/27158541/clinical-utility-of-quantitative-multi-antibody-polycheck-immunoassays-in-the-diagnosis-of-coeliac-disease
#17
Ewa Konopka, Maciej Grzywnowicz, Beata Oralewska, Joanna Cielecka-Kuszyk, Ilona Trojanowska, Bożena Cukrowska
AIM: To evaluate the clinical utility of multi-antibody strategies in the diagnosis of coeliac disease (CD), the new quantitative Polycheck immunoassays were analysed. METHODS: Polycheck Celiac Panels (PCPs) are immunoenzyme screening assays for the quantitative measurement of coeliac-specific immunoglobulin class G (IgG) or class A (IgA) in serum. Lines of relevant antigens are coated together with five IgG or IgA standard lines used for the standard curve as positive control...
May 6, 2016: World Journal of Gastrointestinal Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/27126754/the-role-of-nurses-and-dietitians-in-managing-paediatric-coeliac-disease
#18
REVIEW
Chi-Yee Fok, Kate Sara Holland, Elena Gil-Zaragozano, Siba Prosad Paul
Coeliac disease (CD) is an immune-mediated genetic condition elicited by the ingestion of gluten, leading to proximal small bowel enteropathy. It affects around 1% of the population, although only a small proportion of cases are actually diagnosed. It is a multisystem disorder presenting with both gastrointestinal and extra-intestinal manifestations such as diarrhoea, abdominal pain, constipation, vomiting, iron deficiency anaemia, faltering growth, dental enamel defects, short stature, liver disease, arthropathy and recurrent aphthous ulcers...
April 28, 2016: British Journal of Nursing: BJN
https://www.readbyqxmd.com/read/27120139/unusual-presentation-concurrent-iga-deficiency-and-idiopathic-pulmonary-hemosiderosis
#19
Mustafa Erkoçoğlu, Ersoy Civelek, Can Naci Kocabaş
Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder that should be considered in the differential diagnosis of patients with hemoptysis, recurrent alveolar hemorrhage, dyspnea and iron deficiency anemia (IDA). The frequent association of autoimmune disorders with IPH and a favorable response to steroids suggest the presence of an underlying immune disorder. Here we present a case of a patient with cough, fever, and cyanosis who was also diagnosed with IPH and concurrent selective immunoglobulin A deficiency...
April 27, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27083494/a-prospective-ten-year-follow-up-of-patients-with-chronic-urticaria
#20
P C L Dionigi, M C S Menezes, W C N Forte
BACKGROUND: Chronic urticaria can be the initial clinical presentation of a number of different diseases. The objective of the present study was to report the associated diseases during a ten-year clinical-laboratory follow-up in patients with an initial diagnosis of chronic spontaneous urticaria (CSU) of unknown cause. METHODS: A prospective, longitudinal cohort study with a ten-year clinical-laboratory follow-up was conducted. Patients with a history of urticarial plaques of over six weeks presenting as the only clinical symptom were selected...
July 2016: Allergologia et Immunopathologia
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