5,598 results match your criteria Immunoglobulin G Deficiency


Measurement of Typhim Vi IgG as a Diagnostic Tool to Determine Anti-polysaccharide Antibody Production Deficiency in Children.

Front Immunol 2019 2;10:654. Epub 2019 Apr 2.

Department of Immunology, IML and IdSSC, Hospital Clínico San Carlos, Madrid, Spain.

The assessment of specific polysaccharide antibody production plays a pivotal role in the diagnosis of humoral primary immunodeficiencies (PID). The response to 23-valent pneumococcal vaccine (PPV) remains the gold standard for the diagnosis of polysaccharide antibodies. However, in Spain, the interpretation of pure polysaccharide 23-valent immunization is hampered by the high endemicity of pneumococcal disease and the generalization of the 13-valent adjuvant pneumococcal vaccination. Read More

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https://www.frontiersin.org/article/10.3389/fimmu.2019.00654
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http://dx.doi.org/10.3389/fimmu.2019.00654DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6455213PMC
April 2019
2 Reads

Coordinated signals from the DNA repair enzymes PARP-1 and PARP-2 promotes B-cell development and function.

Cell Death Differ 2019 Apr 17. Epub 2019 Apr 17.

Cancer Research Program, Hospital del Mar Medical Research Institute (IMIM), Barcelona, Spain.

Poly (ADP-ribose) polymerase (PARP)-1 and PARP-2 regulate the function of various DNA-interacting proteins by transferring ADP-ribose emerging from catalytic cleavage of cellular β-NAD. Hence, mice lacking PARP-1 or PARP-2 show DNA perturbations ranging from altered DNA integrity to impaired DNA repair. These effects stem from the central role that PARP-1 and PARP-2 have on the cellular response to DNA damage. Read More

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http://www.nature.com/articles/s41418-019-0326-5
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http://dx.doi.org/10.1038/s41418-019-0326-5DOI Listing
April 2019
1 Read

Acute-onset Autoimmune Hepatitis in a Patient with Selective Immunoglobulin M Deficiency.

Intern Med 2019 Apr 17. Epub 2019 Apr 17.

Division of Gastroenterology, Tohoku University Graduate School of Medicine, Japan.

Selective immunoglobulin M deficiency (SIGMD) is an uncommon primary immunodeficiency disorder. We herein report an SIGMD patient with autoimmune hepatitis. A 21-year-old Japanese man was transferred to our hospital because of acute liver dysfunction. Read More

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http://dx.doi.org/10.2169/internalmedicine.2607-18DOI Listing

Clinical, immunologic, and molecular spectrum of patients with LPS-responsive beige-like anchor protein (LRBA) deficiency: a systematic review.

J Allergy Clin Immunol Pract 2019 Apr 14. Epub 2019 Apr 14.

Non-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran. Electronic address:

Background: LPS-responsive beige-like anchor protein (LRBA) deficiency is a primary immunodeficiency and immune dysregulation syndrome caused by biallelic mutations in the LRBA gene. These mutations usually abrogate the protein expression of LRBA, leading to a broad spectrum of clinical phenotypes including autoimmunity, chronic diarrhea, hypogammaglobulinemia and recurrent infections.

Objective: Our aim was to systematically collect all studies reporting on the clinical manifestations, molecular and laboratory findings, and management of patients with LRBA deficiency. Read More

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http://dx.doi.org/10.1016/j.jaip.2019.04.011DOI Listing

The Clinical Features of Bronchiectasis Associated with Alpha-1 Antitrypsin Deficiency, Common Variable Immunodeficiency and Primary Ciliary Dyskinesia--Results from the U.S. Bronchiectasis Research Registry.

Chronic Obstr Pulm Dis 2019 Apr 9;6(2). Epub 2019 Apr 9.

Oregon Health Sciences University Hospital, Portland.

Objective: This study compares and contrasts the clinical features of non-cystic fibrosis bronchiectasis with 3 uncommon disorders known to be associated with bronchiectasis but with distinctly different underlying defined pathophysiologic derangements, namely severe alpha-1 antitrypsin deficiency (AATD), common variable immunodeficiency (CVI) and primary ciliary dyskinesia (PCD).

Methods: The Bronchiectasis Research Registry provides a central database for studying patients with non-cystic fibrosis bronchiectasis. This report consists of information from 13 U. Read More

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http://dx.doi.org/10.15326/jcopdf.6.2.2018.0156DOI Listing
April 2019
6 Reads

Lifelong immunoglobulin replacement is not always necessary: A case description of a patient with recurrent infections and hypogammaglobulinemia.

Int J Immunopathol Pharmacol 2019 Jan-Dec;33:2058738419843364

5 Jan Biziel University Hospital No. 2, Ludwik Rydygier Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University in Toruń, Bydgoszcz, Poland.

Humoral immunodeficiency with accompanying infections is an indication for human immunoglobulin replacement therapy. Whether treatment will be lifelong or necessary only temporarily depends on the nature of deficiency: primary (persistent) or secondary (persistent or transient). It is not always easy to distinguish between primary and secondary immunodeficiency, especially in adults. Read More

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http://dx.doi.org/10.1177/2058738419843364DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6458662PMC
April 2019
1 Read

Pathogenic NFKB2 variant in the ankyrin repeat domain (R635X) causes a variable antibody deficiency.

Clin Immunol 2019 Apr 3;203:23-27. Epub 2019 Apr 3.

Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Department of Pediatric Immunology, Rheumatology and Infectious diseases, Meibergdreef 9, Amsterdam, The Netherlands; Amsterdam UMC, University of Amsterdam, Department of Experimental Immunology, Amsterdam Infection & Immunity Institute, Meibergdreef 9, Amsterdam, The Netherlands. Electronic address:

Genetic studies are identifying an increasing number of monogenic causes of Common Variable Immunodeficiency (CVID). Pathogenic variants in the C-terminus of NFKB2 have been identified in the subset of CVID patients whose immunodeficiency is associated with ectodermal dysplasia and central adrenal insufficiency. We describe 2 unrelated CVID pedigrees with 4 cases of pathogenic stop gain variants (c. Read More

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http://dx.doi.org/10.1016/j.clim.2019.03.010DOI Listing
April 2019
2 Reads

[Clinical research of auricular gold-needle therapy in treatment of chronic fatigue syndrome of deficiency constitution].

Zhongguo Zhen Jiu 2019 Feb;39(2):128-32

Foshan Chinese Medicine Hospital Affiliated to Guangzhou University of TCM, Foshan 528000, Guangdong Province.

Objective: To observe the clinical therapeutic effects of auricular gold-needle therapy on chronic fatigue syndrome of deficiency constitution and explore its potential mechanism.

Methods: A total of 120 patients were randomized into an auricular gold-needle therapy group, an auricular point pressure therapy group and a Chinese herb group, 40 cases in each one. Additionally, a health control group (40 cases) was set up, without any intervention. Read More

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http://dx.doi.org/10.13703/j.0255-2930.2019.02.004DOI Listing
February 2019
2 Reads

Retrospective Analysis of Serology Results from Adnan Menderes University Faculty of Medicine Parasitology Laboratory from 2007 to 2017

Turkiye Parazitol Derg 2019 Mar;43(1):1-4

Adnan Menderes Üniversitesi Tıp Fakültesi, Parazitoloji Anabilim Dalı, Aydın, Türkiye

Objective: is a common apicomplexan parasite of humans and can cause significant morbidity and mortality due to congenital transmission and in patients with immune deficiency. The aim of this study was to evaluate serology results of 11 years and to determine compatibility of serologic diagnosis methods.

Methods: The study was conducted between 2007 and 2017, and anti- IgG antibodies were investigated by an in-house Enzyme Linked Immunosorbent Assay (ELISA) and Indirect Fluorescence Antibody (IFA) methods. Read More

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http://cms.galenos.com.tr/Uploads/Article_26286/TPD-43-1-En.
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http://dx.doi.org/10.4274/tpd.galenos.2018.6098DOI Listing
March 2019
4 Reads

Common variable immunodeficiency syndrome with chronic diarrhoea.

BMJ Case Rep 2019 Mar 31;12(3). Epub 2019 Mar 31.

Department of Gastroenterology, Medanta-The Medicity, Gurgaon, India.

Common variable immunodeficiency syndrome (CVID) is a heterogeneous disorder characterised by diminished levels of IgG, IgA and/or IgM, and recurrent bacterial infections. Sinopulmonary infections are most commonly reported followed by gastrointestinal (GI) infections. GI tract represents the largest immune organ with abundance of lymphoid cells, its involvement can manifest variably ranging from asymptomatic involvement to florid symptoms and signs. Read More

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http://dx.doi.org/10.1136/bcr-2018-228240DOI Listing
March 2019
1 Read

Inherited Thrombotic Thrombocytopenic Purpura Revealed by Recurrent Strokes in a Male Adult: Case Report and Literature Review.

J Stroke Cerebrovasc Dis 2019 Mar 28. Epub 2019 Mar 28.

Department of Neurology, Bicêtre Hospital, AP-HP, University Paris Sud Saclay, Le Kremlin-Bicêtre, France.

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy related to a severe deficiency of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). In this article, we describe the first case of a young male adult suffering from a hereditary TTP revealed by recurrent strokes, relapsing despite antiplatelet and anticoagulant therapy. Because of the persistent moderate thrombocytopenia, plasmatic ADAMTS13 activity was investigated and was found lower than 5% in the absence of anti-ADAMTS13 IgG. Read More

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http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2019.03.011DOI Listing
March 2019
2 Reads

Impact of immunoglobulin G2 subclass level on late-onset bacterial infection after allogeneic hematopoietic stem cell transplantation.

Transpl Infect Dis 2019 Mar 31:e13086. Epub 2019 Mar 31.

Division of Hematology, Department of Medicine, Keio University School of Medicine, Tokyo, Japan.

Background: Immunoglobulin (Ig) G2 subclass deficiency is known to be associated with recurrent bacterial respiratory infections caused by capsulated bacteria and is found mostly in pediatric patients. However, its impact after allogeneic hematopoietic stem cell transplantation (HSCT) has not been fully assessed.

Methods: We retrospectively evaluated the relationship between IgG2 subclass levels and bacterial pneumonia in 74 adult patients who survived longer than 2 years after allogeneic HSCT. Read More

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http://dx.doi.org/10.1111/tid.13086DOI Listing
March 2019
2 Reads

B-lymphocyte-intrinsic and -extrinsic defects in secretory immunoglobulin A production in the neural crest-conditional deletion of endothelin receptor B model of Hirschsprung-associated enterocolitis.

FASEB J 2019 Mar 25:fj201801913R. Epub 2019 Mar 25.

Division of Pediatric Surgery, Department of Surgery, University of Tennessee Health Sciences Center, Memphis, Tennessee, USA.

Hirschsprung disease (HSCR) is a common cause of intestinal obstruction in the newborn. Hirschsprung-associated enterocolitis (HAEC) is a significant and life-threatening complication of HSCR, affecting up to 60% of patients. Animal models of endothelin receptor B ( EdnrB) mutation reliably model human HSCR and HAEC. Read More

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https://www.fasebj.org/doi/10.1096/fj.201801913R
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http://dx.doi.org/10.1096/fj.201801913RDOI Listing
March 2019
10 Reads

Germinal Center-Derived Antibodies Promote Atherosclerosis Plaque Size and Stability.

Circulation 2019 Mar 21. Epub 2019 Mar 21.

Department of Medicine and Center for Molecular Medicine, Karolinska Institutet, Sweden.

Background: Atherosclerosis progression is modulated by interactions with the adaptive immune system. Humoral immunity can help protect against atherosclerosis formation, however the existence, origin and function of putative atherogenic-antibodies is controversial. How such atherosclerosis-promoting antibodies could affect the specific composition and stability of plaques, as well as the vasculature generally, remains unknown. Read More

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http://dx.doi.org/10.1161/CIRCULATIONAHA.118.038534DOI Listing
March 2019
1 Read
14.430 Impact Factor

Expansion of regulatory T cells using low-dose interleukin-2 attenuates hypertension in an experimental model of systemic lupus erythematos.

Am J Physiol Renal Physiol 2019 Mar 20. Epub 2019 Mar 20.

Physiology & Biophysics, University of Mississippi Medical Center, United States.

Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disorder that is characterized by prevalent hypertension, renal injury, and cardiovascular disease. Numerous studies report a low prevalence and/or impaired function of regulatory T cells (T) in both patients with SLE and murine models of the disease. Evidence suggests that T dysfunction in SLE results from a deficiency in interleukin (IL)-2. Read More

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http://dx.doi.org/10.1152/ajprenal.00616.2018DOI Listing
March 2019
1 Read

Immunoglobulin replacement for secondary immunodeficiency after B-cell targeted therapies in autoimmune rheumatic disease: Systematic literature review.

Autoimmun Rev 2019 May 4;18(5):535-541. Epub 2019 Mar 4.

Frimley Health NHS Foundation Trust, Portsmouth Rd, Frimley, UK. Electronic address:

Background: Consensus guidelines are not available for the use of immunoglobulin replacement therapy (IGRT) in patients developing iatrogenic secondary antibody deficiency following B-cell targeted therapy (BCTT) in autoimmune rheumatic disease.

Objectives: To evaluate the role of IGRT to manage hypogammaglobulinemia following BCTT in autoimmune rheumatic disease (AIRD).

Methods: Using an agreed search string we performed a systematic literature search on Medline with Pubmed as vendor. Read More

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http://dx.doi.org/10.1016/j.autrev.2019.03.010DOI Listing
May 2019
1 Read
7.933 Impact Factor

Defects in memory B-cell and plasma cell subsets expressing different immunoglobulin-subclasses in patients with CVID and immunoglobulin subclass deficiencies.

J Allergy Clin Immunol 2019 Feb 28. Epub 2019 Feb 28.

Department of Medicine, Cancer Research Centre (IBMCC, USAL-CSIC), Cytometry Service (NUCLEUS), University of Salamanca (USAL), Institute of Biomedical Research of Salamanca (IBSAL), Salamanca, Spain; Biomedical Research Networking Centre Consortium of Oncology (CIBERONC), number CB16/12/00400, Instituto de Salud Carlos III, Madrid, Spain. Electronic address:

Background: Predominantly antibody deficiencies (PADs) are the most prevalent primary immunodeficiencies, but their B-cell defects and underlying genetic alterations remain largely unknown.

Objective: We investigated patients with PADs for the distribution of 41 blood B-cell and plasma cell (PC) subsets, including subsets defined by expression of distinct immunoglobulin heavy chain subclasses.

Methods: Blood samples from 139 patients with PADs, 61 patients with common variable immunodeficiency (CVID), 68 patients with selective IgA deficiency (IgAdef), 10 patients with IgG subclass deficiency with IgA deficiency, and 223 age-matched control subjects were studied by using flow cytometry with EuroFlow immunoglobulin isotype staining. Read More

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http://dx.doi.org/10.1016/j.jaci.2019.02.017DOI Listing
February 2019
5 Reads

Safety and effectiveness of enzyme replacement therapy with agalsidase alfa in patients with Fabry disease: Post-marketing surveillance in Japan.

Mol Genet Metab 2019 Feb 20. Epub 2019 Feb 20.

Sumitomo Dainippon Pharma Co., Ltd., Osaka, Japan.

Fabry disease is a rare X-linked inherited multisystem disorder resulting from deficiency of the lysosomal enzyme alpha-galactosidase A. Currently, specific therapies, including enzyme replacement therapies, are available for Fabry disease, but clinical trials provide limited information on long-term safety and effectiveness. Agalsidase alfa was approved in Japan in 2006. Read More

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http://dx.doi.org/10.1016/j.ymgme.2019.02.005DOI Listing
February 2019
3 Reads

Galactose-deficient IgA1 and the corresponding IgG autoantibodies predict IgA nephropathy progression.

PLoS One 2019 22;14(2):e0212254. Epub 2019 Feb 22.

General Teaching Hospital, 1st Faculty of Medicine, Charles University, Department of Nephrology, Prague, Czech Republic.

Background: IgA nephropathy (IgAN), the most common primary glomerulonephritis worldwide, has serious outcomes with end-stage renal disease developing in 30-50% of patients. The diagnosis requires renal biopsy. Due to its inherent risks, non-invasive approaches are needed. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0212254PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6386256PMC
February 2019
6 Reads

Skewed B cell receptor repertoire and reduced antibody avidity in patients with DOCK8 deficiency.

Scand J Immunol 2019 Feb 21:e12759. Epub 2019 Feb 21.

Division of Immunology, Children's Hospital of Chongqing Medical University, Chongqing, China.

DOCK8 immunodeficiency syndrome (DIDS) is a combined immunodeficiency characterized by recurrent viral infections, severe atopy and early onset malignancy. Immunological abnormalities include lymphopenia, CD8 T-cell cytoskeleton dysfunction, defective B cell memory and variable serum immunoglobulin levels. Here, we analyse the B cell receptor repertoire (BCR) characteristics and antibody avidity of four DIDS patients, attempt to understand the dysregulated humoral immunity in DIDS patients with a normal antibody titre and suggest a scientific basis for intravenous immunoglobulin (IVIG) replacement therapy for these patients. Read More

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http://dx.doi.org/10.1111/sji.12759DOI Listing
February 2019

Fra1 Controls Rheumatoid Factor Autoantibody Production by Bone Marrow Plasma Cells and the Development of Autoimmune Bone Loss.

J Bone Miner Res 2019 Feb 19:e3705. Epub 2019 Feb 19.

Department of Medicine 3, Rheumatology and Immunology, Friedrich Alexander University Erlangen-Nuremberg and Universitätsklinikum Erlangen, Erlangen, Germany.

Next to proinflammatory cytokines, autoimmunity has been identified as a key trigger for osteoclast activation and bone loss. IgG-rheumatoid factor (IgG-RF) immune complexes, which are present in patients with rheumatoid arthritis, were shown to boost osteoclast differentiation. To date, the regulation of IgG-RF production in the absence of inflammatory triggers is unknown. Read More

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http://dx.doi.org/10.1002/jbmr.3705DOI Listing
February 2019
5 Reads

Disseminated cryptococcosis with immunoglobulin G subclass deficiency.

Pediatr Int 2019 Feb 11;61(2):198-199. Epub 2019 Feb 11.

Division of Pediatric Infectious Disease, Department of Pediatrics, St Joseph's Children's Hospital, Paterson, New Jersey, USA.

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http://dx.doi.org/10.1111/ped.13741DOI Listing
February 2019
7 Reads

The total IgM, IgA and IgG antibody responses to pneumococcal polysaccharide vaccination (Pneumovax®23) in a healthy adult population and patients diagnosed with primary immunodeficiencies.

Vaccine 2019 Feb 5;37(10):1350-1355. Epub 2019 Feb 5.

Department of Laboratory Medicine and Pathology, Mayo Clinic , Rochester, MN, USA.

Background: Interpretation of the responses to the pneumococcal polysaccharide vaccine (Pneumovax®23, PPV) has proven challenging. In addition, there are few studies documenting the longevity of these responses.

Methods: The age-specific PPV IgM, IgA, IgG and IgG2 concentrations were determined pre, 4-6 weeks and 6 years post-vaccination in the serum of Prevnar®-naïve adults using VaccZyme™ pneumococcal capsular polysaccharide ELISAs. Read More

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http://dx.doi.org/10.1016/j.vaccine.2019.01.035DOI Listing
February 2019
1 Read

[Characterization of celiac disease in chilean public hospitals].

Rev Chil Pediatr 2018 Dec;89(6):709-717

Instituto de Nutrición y Tecnología de los Alimentos, Universidad de Chile, Chile.

Introduction: The worldwide prevalence of celiac disease (CD) is ~1% of the population. In Chile, the National Health Survey 2009-2010 showed a serological prevalence in individuals older than 15 years of 0.76% (IgA-tTG2), which corresponded in Concepción to 0. Read More

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http://dx.doi.org/10.4067/S0370-41062018005001001DOI Listing
December 2018
3 Reads

The role of Mannose Binding Lectin in the immune response against Borrelia burgdorferi sensu lato.

Sci Rep 2019 Feb 5;9(1):1431. Epub 2019 Feb 5.

Amsterdam UMC, University of Amsterdam, Center for Experimental and Molecular Medicine, Meibergdreef 9, Amsterdam, Netherlands.

The causative agents of Lyme borreliosis, spirochetes belonging to the Borrelia burgdorferi sensu lato group, have developed several ways to protect themselves against killing by the host complement system. In addition, it has been shown that serum sensitive isolates are (partially) protected by the Ixodes Tick Salivary Lectin Pathway Inhibitor (TSLPI) protein; a salivary gland protein that inhibits the function of Mannose Binding Lectin (MBL). MBL is a C-type lectin that recognizes oligosaccharides on pathogens and activates the complement system via the lectin pathway. Read More

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http://dx.doi.org/10.1038/s41598-018-37922-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6363739PMC
February 2019
2 Reads

TREC and KREC Levels as a Predictors of Lymphocyte Subpopulations Measured by Flow Cytometry.

Front Physiol 2018 21;9:1877. Epub 2019 Jan 21.

Department of Paediatrics, Sechenov University, Moscow, Russia.

Primary immunodeficiency diseases (PID) is a heterogeneous group of disorders caused by genetic defects of the immune system, which manifests clinically as recurrent infections, autoimmune diseases, or malignancies. Early detection of other PID remains a challenge, particularly in older children due to milder and less specific symptoms, a low level of clinician PID awareness and poor provision of hospital laboratories with appropriate devices. T-cell recombination excision circles (TREC) and kappa-deleting element recombination circle (KREC) in a dried blood spot and in peripheral blood using real-time polymerase chain reaction (PCR) are used as a tool for severe combined immune deficiency but not in PID. Read More

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http://dx.doi.org/10.3389/fphys.2018.01877DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6348265PMC
January 2019
11 Reads

Adiponectin deficiency has no effect in murine autoimmune myocarditis.

Cytokine 2019 Apr 1;116:139-149. Epub 2019 Feb 1.

Department of Cardiology, University of Heidelberg, Heidelberg, Germany. Electronic address:

Background: Adiponectin is a hormone that together with its receptors modulates a number of metabolic processes including gluconeogenesis and lipid catabolism. It belongs to the C1QTNF (complement C1q tumor necrosis factor-related protein) family, which has a variety of members with high amino acid sequence homology and overlapping functions. Concentration of adiponectin in blood is inversely correlated with body fat percentage and cardiac risk factors like blood pressure and CRP (C-reactive protein) level. Read More

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http://dx.doi.org/10.1016/j.cyto.2018.12.022DOI Listing
April 2019
2 Reads

Laboratory Diagnostics in Thrombophilia.

Hamostaseologie 2019 Feb 31;39(1):49-61. Epub 2019 Jan 31.

Department of Hematology and Oncology, Internal Medicine III, University Hospital Regensburg, Regensburg, Germany.

A thrombophilic disorder is a hereditary or acquired condition that increases the risk of thrombosis. The most common hereditary thrombophilias that predispose to venous thrombosis in the Caucasian population are the heterozygous forms of the factor V Leiden and prothrombin G20210A mutation that are generally detected by direct DNA genotyping. Immunologic antigen assays and chromogenic or clot-based activity assays are used to identify deficiencies in the natural coagulation inhibitors antithrombin, protein C and protein S. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-1677840
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http://dx.doi.org/10.1055/s-0039-1677840DOI Listing
February 2019
14 Reads

Low levels of vitamin D and the relationship between vitamin D and Th2 axis-related cytokines in neuromyelitis optica spectrum disorders.

J Clin Neurosci 2019 Mar 25;61:22-27. Epub 2019 Jan 25.

Department of Neurology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China. Electronic address:

Objective: The impact of vitamin D have been studied in neuroinflammation disorders, and as the newly discovered Th2-related cytokines, IL-25, IL-31 and IL-33 may also play important roles in the lesions of neuromyelitis optica spectrum disorders (NMOSD). This study sought to investigate the clinical profiles of vitamin D and Th2 axis-related cytokines and their relationships in patients with NMOSD.

Methods: Eighty-four NMOSD patients and 84 healthy controls (HC) were evaluated for serum levels of the total vitamin D [25(OH)D], 25-hydroxyvitamin D2 [25(OH)D2] and 25-hydroxyvitamin D3 [25(OH)D3] by means of high performance liquid chromatographytandem mass spectrometry (HPLC-MS/MS). Read More

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http://dx.doi.org/10.1016/j.jocn.2018.11.024DOI Listing
March 2019
1 Read

Dysregulated TRAF3 and BCL2 Expression Promotes Multiple Classes of Mature Non-hodgkin B Cell Lymphoma in Mice.

Front Immunol 2018 11;9:3114. Epub 2019 Jan 11.

Instituto de Investigaciones Biomédicas "Alberto Sols", CSIC-UAM, Madrid, Spain.

TNF-Receptor Associated Factor (TRAF)-3 is a master regulator of B cell homeostasis and function. TRAF3 has been shown to bind and regulate various proteins involved in the control of innate and adaptive immune responses. Previous studies showed that TRAF3 overexpression renders B cells hyper-reactive to antigens and Toll-like receptor (TLR) agonists, while TRAF3 deficiency has been implicated in the development of a variety of B cell neoplasms. Read More

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http://dx.doi.org/10.3389/fimmu.2018.03114DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6338067PMC
January 2019
3 Reads

A CASE OF CAT SCRATCH DISEASE DIAGNOSED BY INDIRECT FLUORESCENT ANTIBODY ASSAY OF IGM SPECIFIC FOR A JAPANESE STRAIN OF Bartonella henselae.

Retin Cases Brief Rep 2019 Jan 23. Epub 2019 Jan 23.

Department of Ophthalmology, Kyushu University Graduate School of Medical Sciences, Fukuoka, Japan.

Purpose: To report a case of cat scratch disease-associated retinitis diagnosed with an indirect fluorescent antibody (IFA) assay for immunoglobulin M (IgM) specific for a strain (YH-01) of Bartonella henselae recently identified in Japan.

Methods: Case report of a 24-year-old pregnant woman presented with general fever, fatigue, as well as blurred vision, and a central visual field deficiency in her right eye and was suspected as cat scratch disease because she had started to feed a feral dog a month ago.

Results: The patient's serum tested negative, however, with an IFA assay for IgG or IgM specific for the Houston-1, common strain of B. Read More

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http://dx.doi.org/10.1097/ICB.0000000000000854DOI Listing
January 2019
4 Reads

LRRK2 is involved in the pathogenesis of system lupus erythematosus through promoting pathogenic antibody production.

J Transl Med 2019 Jan 22;17(1):37. Epub 2019 Jan 22.

Shanghai Institute of Immunology, Department of Immunology and Microbiology, Shanghai Jiao Tong University School of Medicine, Shanghai, 200025, China.

Background: Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease characterized by the presence of pathogenic autoantibodies associated with polyclonal B cell hyperreactivity. Previous study reported that autophagy-related gene Leucine-rich repeat kinase 2 (LRRK2) was likely a susceptible gene for SLE. However, the pathogenic function of LRRK2 in SLE is undefined. Read More

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http://dx.doi.org/10.1186/s12967-019-1786-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6343316PMC
January 2019
11 Reads
3.930 Impact Factor

Isolated IgG4-related gastric disease presenting as diffuse gastric wall thickening with ulcer.

J Radiol Case Rep 2018 Sep 30;12(9):9-20. Epub 2018 Sep 30.

Department of Anatomical Pathology, Singapore General Hospital, Singapore.

An 81-year-old male presented with loss of appetite, early satiety and iron deficiency anaemia. A computed tomography (CT) scan of the abdomen and pelvis during initial work-up revealed diffuse gastric mural thickening associated with a large ulcer and adjacent gastro-hepatic lymphadenopathy. The CT appearances, together with the clinical features, were highly suspicious for an infiltrative type of gastric malignancy. Read More

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http://radiologycases.com/index.php/radiologycases/article/v
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http://dx.doi.org/10.3941/jrcr.v12i9.3493DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6312045PMC
September 2018
12 Reads

Antibody Responses in HIV-Infected Patients With Advanced Immunosuppression and Asymptomatic Cryptococcal Antigenemia.

Open Forum Infect Dis 2019 Jan 11;6(1):ofy333. Epub 2018 Dec 11.

Division of Infectious Diseases, Department of Medicine, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, New York.

Background: There are no host biomarkers of risk for HIV-associated cryptococcal meningitis (CM) except CD4+ T-cell deficiency. At present, serum cryptococcal antigen (CrAg) screening of those with CD4 <100 cells/µL is used to identify persons at risk for HIV-associated CM. We determined if plasma antibody profiles could discriminate CrAg+ from CrAg- patients. Read More

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http://dx.doi.org/10.1093/ofid/ofy333DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6329905PMC
January 2019
8 Reads

Fn14 Deficiency Ameliorates Anti-dsDNA IgG-Induced Glomerular Damage in SCID Mice.

J Immunol Res 2018 16;2018:1256379. Epub 2018 Dec 16.

Department of Dermatology, The Second Affiliated Hospital, School of Medicine, Xi'an Jiaotong University, Xi'an 710004, China.

Many studies have demonstrated that anti-dsDNA IgG is closely associated with lupus nephritis. Recently, it was found that activation of the fibroblast growth factor-inducible 14 (Fn14) signaling pathway damages glomerular filtration barrier in MRL/lpr lupus-prone mice. However, MRL/lpr mice have high titers of serum autoantibodies other than anti-dsDNA IgG. Read More

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https://www.hindawi.com/journals/jir/2018/1256379/
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http://dx.doi.org/10.1155/2018/1256379DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6311848PMC
April 2019
9 Reads

A 9.5-year-old boy with recurrent neurological manifestations and severe hypertension, treated initially for polyarteritis nodosa, was subsequently diagnosed with adenosine deaminase type 2 deficiency (DADA2) which responded to anti-TNF-α.

Paediatr Int Child Health 2019 Jan 15:1-4. Epub 2019 Jan 15.

a Department of Paediatric Rheumatology, Cerrahpasa Medical School , Istanbul University , Istanbul , Turkey.

A 9.5-year-old boy was referred with a 2-year history of recurrent fever, myalgia, abdominal pain and various neurological manifestations associated with increased acute phase reactants and IgG level. During the recent episode, severe hypertension and right-sided hemiparesis developed and angiography demonstrated irregularities and stenosis in renal and mesenteric artery branches. Read More

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http://dx.doi.org/10.1080/20469047.2018.1559495DOI Listing
January 2019
3 Reads

CMV-Specific Immune Response-New Patients, New Insight: Central Role of Specific IgG during Infancy and Long-Lasting Immune Deficiency after Allogenic Stem Cell Transplantation.

Int J Mol Sci 2019 Jan 11;20(2). Epub 2019 Jan 11.

Hirszfeld Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, 53-114 Wrocław, Poland.

Although the existing paradigm states that cytomegalovirus (CMV) reactivation is under the control of the cellular immune response, the role of humoral and innate counterparts are underestimated. The study analyzed the host⁻virus interaction i.e. Read More

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http://www.mdpi.com/1422-0067/20/2/271
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http://dx.doi.org/10.3390/ijms20020271DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6358762PMC
January 2019
11 Reads

[Clinical and genetic manifestations of immunodeficiency, centromeric instability, and facial anomalies syndrome: a case report and literature review].

Zhonghua Er Ke Za Zhi 2019 Jan;57(1):55-59

Department of Nephrology and Immunology, Qingdao Women and Children's Hospital, Qingdao 266000, China.

To analyze the clinical and genetic features of immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome with a case report and literature review. The clinical data and genetic test of a girl diagnosed with ICF syndrome in the Department of Nephrology and Immunology in Qingdao Women and Children's Hospital in December 2016 were extracted and analyzed. "ICF syndrome" "immunodeficiency, centromeric instability and facial anomalies syndrome" "ICF syndrome and DNMT3B" were used as key words to search Chinese databases and Pubmed for literature until March 2018, and the literature was reviewed. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2019.01.013DOI Listing
January 2019
2 Reads

Hypomorphic Mutations in the BCR Signalosome Lead to Selective Immunoglobulin M Deficiency and Impaired B-cell Homeostasis.

Front Immunol 2018 18;9:2984. Epub 2018 Dec 18.

Immunology Outpatient Clinic, Vienna, Austria.

B cell activation via the B cell receptor (BCR) signalosome involves participation of signaling molecules such as BTK and BLNK. Genetic defects in these molecules are known to impair B cell differentiation and subsequently lead to agammaglobulinemia. Here we identified novel mutations in BTK and BLNK in two unrelated patients that perturb the intrinsic B-cell receptor signaling pathway and lead to selective IgM deficiency, whereas production of other immunoglobulin isotypes and IgG antibody response remain intact. Read More

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https://www.frontiersin.org/article/10.3389/fimmu.2018.02984
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http://dx.doi.org/10.3389/fimmu.2018.02984DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6305442PMC
December 2018
12 Reads

Autoimmunity and immunodeficiency at the crossroad: autoimmune disorders as the presenting feature of selective IgM deficiency.

BMJ Case Rep 2019 Jan 3;12(1). Epub 2019 Jan 3.

Department of Experimental Rheumatology, Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK.

Selective immunoglobulin M deficiency (sIgMD) is an immunodeficiency with undefined pathogenesis and commonly presenting with recurrent infections. The European Society for Immunodeficiencies Registry defines sIgMD as a serum IgM level repeatedly below 2 SD of normal with normal levels of serum IgA, IgG and IgG subclasses, normal vaccination responses, absence of T-cell defects and absence of causative external factors. Rarely it can also be associated with autoimmune diseases. Read More

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http://dx.doi.org/10.1136/bcr-2017-223180DOI Listing
January 2019
6 Reads

AGA Clinical Practice Update on Diagnosis and Monitoring of Celiac Disease-Changing Utility of Serology and Histologic Measures: Expert Review.

Gastroenterology 2019 03 19;156(4):885-889. Epub 2018 Dec 19.

Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota.

Purpose: The purpose of this clinical practice update is to define key modalities in the diagnosis and monitoring of celiac disease (CD) in adults as well as in children and adolescents.

Methods: The recommendations outlined in this expert review are based on available published evidence, including cohort and case-control studies of the diagnostic process as well as controlled and descriptive studies of disease management. Best Practice Advice 1: Serology is a crucial component of the detection and diagnosis of CD, particularly tissue transglutaminase-immunoglobulin A (TG2-IgA), IgA testing, and less frequently, endomysial IgA testing. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00165085183540
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http://dx.doi.org/10.1053/j.gastro.2018.12.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6409202PMC
March 2019
6 Reads

High-resolution computed tomography findings in humoral primary immunodeficiencies and correlation with pulmonary function tests.

World J Radiol 2018 Nov;10(11):172-183

Institute of Radiology, Department of Medicine, University of Udine, Azienda Sanitaria Universitaria Integrata di Udine, Udine 33100, Italy.

Aim: To compare high-resolution computed tomography (HRCT) findings between humoral primary immunodeficiencies (hPIDs) subtypes; to correlate these findings to pulmonary function tests (PFTs).

Methods: We retrospectively identified 52 consecutive adult patients with hPIDs who underwent 64-row HRCT and PFTs at the time of diagnosis. On a per-patient basis, an experienced radiologist recorded airway abnormalities (bronchiectasis, airway wall thickening, mucus plugging, tree-in-bud, and air-trapping) and parenchymal-interstitial abnormalities (consolidations, ground-glass opacities, linear and/or irregular opacities, nodules, and bullae/cysts) found on HRCT. Read More

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http://www.wjgnet.com/1949-8470/full/v10/i11/172.htm
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http://dx.doi.org/10.4329/wjr.v10.i11.172DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6288673PMC
November 2018
19 Reads

Common variable immune deficiency: Dissection of the variable.

Immunol Rev 2019 Jan;287(1):145-161

Division of Clinical Immunology, Icahn School of Medicine at Mount Sinai, New York, New York.

Starting about 60 years ago, a number of reports appeared that outlined the severe clinical course of a few adult subjects with profound hypogammaglobinemia. Puzzled by the lack of family history and adult onset of symptoms in most, the name "acquired" hypogammaglobinemia was given, but later altered to the current name common variable immune deficiency. Pathology reports remarked on the loss of lymph node architecture and paucity of plasma cells in lymphoid tissues in these subjects. Read More

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http://doi.wiley.com/10.1111/imr.12728
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http://dx.doi.org/10.1111/imr.12728DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6435035PMC
January 2019
16 Reads

Differentiation of Common Variable Immunodeficiency From IgG Deficiency.

J Allergy Clin Immunol Pract 2019 Apr 14;7(4):1277-1284. Epub 2018 Dec 14.

The Division of Clinical Immunology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY. Electronic address:

Background: Common variable immunodeficiency (CVID) and IgG deficiency are 2 of the more prevalent primary humoral immune defects. The former is defined by consensus with criteria for quantitative and qualitative antibody defects, whereas the latter is used to describe patients with reduced IgG, who commonly have recurrent sinopulmonary infections but do not fulfill CVID criteria. However, these patients are often given this diagnosis. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22132198183082
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http://dx.doi.org/10.1016/j.jaip.2018.12.004DOI Listing
April 2019
15 Reads

Synergistic convergence of microbiota-specific systemic IgG and secretory IgA.

J Allergy Clin Immunol 2019 Apr 13;143(4):1575-1585.e4. Epub 2018 Dec 13.

Sorbonne Université, INSERM, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Assistance Publique-Hôpitaux de Paris (AP-HP), Groupement Hospitalier Pitié-Salpêtrière, Département d'Immunologie, Paris, France. Electronic address:

Background: Commensals induce local IgA responses essential to the induction of tolerance to gut microbiota, but it remains unclear whether antimicrobiota responses remain confined to the gut.

Objective: The aim of this study was to investigate systemic and intestinal responses against the whole microbiota under homeostatic conditions and in the absence of IgA.

Methods: We analyzed blood and feces from healthy donors, patients with selective IgA deficiency (SIgAd), and patients with common variable immunodeficiency (CVID). Read More

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http://dx.doi.org/10.1016/j.jaci.2018.09.036DOI Listing
April 2019
25 Reads

Screening for Humoral Immunodeficiency in Patients with Community-Acquired Pneumonia.

J Hosp Med 2019 01 28;14(1):33037. Epub 2018 Nov 28.

Rochester Regional Health, Rochester, New York, USA.

Background: Immunodeficiency is an underrecognized risk factor for infections, such as community-acquired pneumonia (CAP).

Objective: We evaluated patients admitted with CAP for humoral immunodeficiency.

Design: Prospective Cohort Study. Read More

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http://dx.doi.org/10.12788/jhm.3106DOI Listing
January 2019
14 Reads

Vitamin D deficiency increases blood-brain barrier dysfunction after ischemic stroke in male rats.

Exp Neurol 2019 Feb 28;312:63-71. Epub 2018 Nov 28.

Department of Emergency Medicine, Brain Research Laboratory, Emory University, Atlanta, GA 30322, USA. Electronic address:

Because vitamin D hormone deficiency (VDH) can worsen severity and outcome for ischemic stroke, we examined the role of VDH in maintaining blood-brain-barrier (BBB integrity) in a rat model of stroke. In most types of stroke, the BBB is markedly compromised, potentially leading to a cascade of injury processes and functional deficits, so we examined a number of biomarkers associated with BBB disruption to determine whether VDH deficiency would further compromise the BBB following a stroke. Male Wistar rats were randomly assigned to one of two diet cohorts, VDH-sufficient (VDH) and VDH. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00144886183027
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http://dx.doi.org/10.1016/j.expneurol.2018.11.005DOI Listing
February 2019
15 Reads

TACI Contributes to Host Resistance by Controlling T Follicular Helper Cell Response and Germinal Center Formation.

Front Immunol 2018 9;9:2612. Epub 2018 Nov 9.

US Food and Drug Administration, Division of Bacterial Allergenic and Parasitic Diseases, Center for Biologics Evaluation and Research, Silver Spring, MD, United States.

The delay in parasite-specific B cell development leaves people in malaria endemic areas vulnerable to repeated infections. Here, we investigated the role of transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI), a molecule involved in the generation of antigen-specific antibody secreting cells, in host response to non-lethal infection. We found that TACI deficiency not only resulted in higher peak parasitemia levels in challenged mice, but also led to a delay in parasite clearance and anti- Merozoite Surface Protein 1(C-terminal 19-kDa fragment [rMSP-1]) protein and anti-rMSP-1 and anti- IgG antibody development. Read More

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http://dx.doi.org/10.3389/fimmu.2018.02612DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6237915PMC
November 2018
5 Reads