4,358 results match your criteria Immunoglobulin A Deficiency


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

IgA and FcαRI: Pathological Roles and Therapeutic Opportunities.

Front Immunol 2019 22;10:553. Epub 2019 Mar 22.

Department of Molecular Cell Biology and Immunology, Amsterdam UMC, Amsterdam, Netherlands.

Immunoglobulin A (IgA) is the most abundant antibody class present at mucosal surfaces. The production of IgA exceeds the production of all other antibodies combined, supporting its prominent role in host-pathogen defense. IgA closely interacts with the intestinal microbiota to enhance its diversity, and IgA has a passive protective role via immune exclusion. Read More

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https://www.frontiersin.org/article/10.3389/fimmu.2019.00553
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http://dx.doi.org/10.3389/fimmu.2019.00553DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6448004PMC
March 2019
2 Reads

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

Mapping of Signaling Pathways Linked to sIgAD Reveals Impaired IL-21 Driven STAT3 B-Cell Activation.

Front Immunol 2019 18;10:403. Epub 2019 Mar 18.

Department of Immunology, Landspítali-The National University Hospital of Iceland, Reykjavík, Iceland.

It has recently been shown that individuals with selective IgA deficiency (sIgAD) have defective B cell responses both to T cell dependent and independent mimicking stimulations. The complex intracellular signaling pathways from different stimuli leading to IgA isotype switching have not been fully elucidated. Thus, the main objective of this study was to delineate these pathways and their potential role in the immunopathology linked to sIgAD. Read More

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http://dx.doi.org/10.3389/fimmu.2019.00403DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6431630PMC
March 2019
2 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

Loss of the Golgi Matrix Protein 130 Cause Aberrant IgA1 Glycosylation in IgA Nephropathy.

Am J Nephrol 2019 27;49(4):307-316. Epub 2019 Mar 27.

Department of Nephrology, The Second Xiangya Hospital, Central South University, Changsha, China,

Background: Aberrant O-glycosylation IgA1 production is a major factor in the pathogenesis of IgA nephropathy, but the underlying mechanism is still unclear. IgA1 glycosylation modification is in Golgi, and downregulation of the Golgi peripheral membrane protein Golgi matrix protein 130 (GM130) could lead to glycosylation deficiency. In this study, we aimed to explore the role of GM130 in glycosylate deficiency IgA1 (Gd-IgA1) production. Read More

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http://dx.doi.org/10.1159/000499110DOI Listing
March 2019
6 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

Immunoglobulin A Dysgammaglobulinemia Is Associated with Pediatric-Onset Obsessive-Compulsive Disorder.

J Child Adolesc Psychopharmacol 2019 Mar 20. Epub 2019 Mar 20.

4 Allergy, Immunology, and Infectious Disease Program, University of South Florida, St. Petersburg, Florida.

Background: Inflammation and immune dysregulation have been implicated in the pathogenesis of pediatric-onset obsessive-compulsive disorder (OCD) and tic disorders such as Tourette syndrome (TS). Though few replicated studies have identified markers of immune dysfunction in this population, preliminary studies suggest that serum immunoglobulin A (IgA) concentrations may be abnormal in these children with these disorders.

Methods: This observational retrospective cohort study, conducted using electronic health records (EHRs), identified 206 children with pediatric-onset OCD and 1024 adults diagnosed with OCD who also had testing for serum levels of IgA. Read More

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http://dx.doi.org/10.1089/cap.2018.0043DOI Listing
March 2019
1 Read

PJP Infection in a Patient with High Output Ileostomy and Selective IgA Deficiency.

QJM 2019 Mar 19. Epub 2019 Mar 19.

Respiratory Department, Portiuncula University Hospital, Ballinasloe, Co. Galway, Ireland.

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http://dx.doi.org/10.1093/qjmed/hcz067DOI Listing
March 2019
3 Reads

The First Purine Nucleoside Phosphorylase Deficiency Patient Resembling IgA Deficiency and a Review of the Literature.

Immunol Invest 2019 Mar 19:1-21. Epub 2019 Mar 19.

a Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran, and the University of Medical Science , Tehran , Iran.

Purine nucleoside phosphorylase (PNP) deficiency is a rare autosomal recessive primary immunodeficiency disorder characterized by decreased numbers of T-cells, variable B-cell abnormalities, decreased amount of serum uric acid and PNP enzyme activity. The affected patients usually present with recurrent infections, neurological dysfunction and autoimmune phenomena. In this study, whole-exome sequencing was used to detect mutation in the case suspected of having primary immunodeficiency. Read More

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https://www.tandfonline.com/doi/full/10.1080/08820139.2019.1
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http://dx.doi.org/10.1080/08820139.2019.1570249DOI Listing
March 2019
3 Reads
1.903 Impact Factor

Transcriptional factor ATF3 protects against colitis by regulating follicular helper T cells in Peyer's patches.

Proc Natl Acad Sci U S A 2019 Mar 12;116(13):6286-6291. Epub 2019 Mar 12.

Joint Program in Immunology, Department of Internal Medicine, Affiliated Guangzhou Women and Children's Medical Center, Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou 510623, China;

Disruption of mucosal immunity plays a critical role in the pathogenesis of inflammatory bowel disease, yet its mechanism remains not fully elucidated. Here, we found that activating transcription factor 3 (ATF3) protects against colitis by regulating follicular helper T (T) cells in the gut. The expression of ATF3 in CD4 T cells was negatively correlated with the severity of ulcerative colitis in clinical patients. Read More

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http://dx.doi.org/10.1073/pnas.1818164116DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6442638PMC
March 2019
4 Reads

Celiac disease and severe vitamin D deficiency: the case for anti-tissue transglutaminase antibody screening.

Authors:
Riad A Sulimani

Arch Osteoporos 2019 Mar 4;14(1):30. Epub 2019 Mar 4.

Department of Internal Medicine, College of Medicine, King Saud University, P.O Box 7805, Riyadh, 11472, Saudi Arabia.

Vitamin D-deficient Saudi adolescent girls were screened for anti-tissue transglutaminase (IgA-tTG) antibodies to determine whether the presence of severe vitamin D deficiency was associated with celiac disease. All 9 participants who were positive for IgA-tTG antibodies had severe vitamin D deficiency (25(OH)D < 12.5 nmol/l), suggesting that this population should be screened for celiac disease. Read More

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http://dx.doi.org/10.1007/s11657-018-0554-1DOI Listing
March 2019
1 Read

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

Prevalence of autoimmune diseases and clinical significance of autoantibody profile: Data from National Institute of Hygiene in Rabat, Morocco.

Hum Immunol 2019 Feb 23. Epub 2019 Feb 23.

Laboratory of Human Pathologies Biology, Department of Biology, Faculty of Sciences, and Genomic Center of Human Pathologies, Faculty of Medicine and Pharmacy, Mohammed V University in Rabat, Morocco.

Aim: The objective of this study was to explore the prevalence of various autoimmune diseases (AIDs) in a large cohort of patients and to characterize the autoantibody profile in the patients with and without AIDs to confirm the diagnosis and to refine the Moroccan databases.

Patients And Method: Retrospective study was conducted in the Laboratory of autoimmunity National Institute of Hygiene (NIH) of Rabat in Morocco. A total of 3182 consecutive Moroccan patients (2183 females and 999 males) whose sera were tested for 14 autoantibody profile between 2010 and 2016. Read More

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http://dx.doi.org/10.1016/j.humimm.2019.02.012DOI Listing
February 2019
5 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

c-Maf-dependent T cell control of intestinal T17 cells and IgA establishes host-microbiota homeostasis.

Nat Immunol 2019 04 18;20(4):471-481. Epub 2019 Feb 18.

Institute of Immunology, Christian-Albrechts-Universität zu Kiel & Universitätsklinik Schleswig Holstein, Kiel, Germany.

Foxp3 regulatory T cells (T cells) are crucial for the maintenance of immune homeostasis both in lymphoid tissues and in non-lymphoid tissues. Here we demonstrate that the ability of intestinal T cells to constrain microbiota-dependent interleukin (IL)-17-producing helper T cell (T17 cell) and immunoglobulin A responses critically required expression of the transcription factor c-Maf. The terminal differentiation and function of several intestinal T cell populations, including RORγt T cells and follicular regulatory T cells, were c-Maf dependent. Read More

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http://dx.doi.org/10.1038/s41590-019-0316-2DOI Listing
April 2019
4 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

Loss of TET proteins in regulatory T cells promotes abnormal proliferation, Foxp3 destabilization, and IL-17 expression.

Int Immunol 2019 Feb 6. Epub 2019 Feb 6.

Department of Microbiology and Immunology, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan.

Ten-eleven translocation (TET) proteins regulate DNA methylation and gene expression by converting 5-methylcytosine (5mC) to 5-hydroxymethylcytosine (5hmC). Although Tet2/Tet3 deficiency has been reported to lead to myeloid cell, B cell, and invariant natural killer T (iNKT) cell malignancy, the effect of TET on regulatory T cells (Tregs) has not been elucidated. We found that Tet2/Tet3 deficiency in Tregs led to lethal hyperproliferation of CD4+Foxp3+ T cells in the spleen and mesenteric lymph nodes after 5 months of age. Read More

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http://dx.doi.org/10.1093/intimm/dxz008DOI Listing
February 2019
15 Reads

[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

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

Microbiota Sensing by Mincle-Syk Axis in Dendritic Cells Regulates Interleukin-17 and -22 Production and Promotes Intestinal Barrier Integrity.

Immunity 2019 Feb 29;50(2):446-461.e9. Epub 2019 Jan 29.

Immunobiology Laboratory, Fundación Centro Nacional de Investigaciones Cardiovasculares Carlos III (CNIC), Melchor Fernández Almagro 3, Madrid 28029, Spain. Electronic address:

Production of interleukin-17 (IL-17) and IL-22 by T helper 17 (Th17) cells and group 3 innate lymphoid cells (ILC3s) in response to the gut microbiota ensures maintenance of intestinal barrier function. Here, we examined the mechanisms whereby the immune system detects microbiota in the steady state. A Syk-kinase-coupled signaling pathway in dendritic cells (DCs) was critical for commensal-dependent production of IL-17 and IL-22 by CD4 T cells. Read More

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http://dx.doi.org/10.1016/j.immuni.2018.12.020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6382412PMC
February 2019
7 Reads

Selective IgA deficiency in humans is associated with reduced gut microbial diversity.

J Allergy Clin Immunol 2019 Jan 29. Epub 2019 Jan 29.

Research Institute of Internal Medicine, Division of Surgery, Inflammatory Diseases and Transplantation, Oslo University Hospital, Rikshospitalet, Norway; Institute of Clinical Medicine, University of Oslo, Oslo, Norway; Norwegian PSC Research Center, Division of Surgery, Inflammatory Diseases and Transplantation, Oslo University Hospital, Rikshospitalet, Norway; Section of Gastroenterology, Department of Transplantation Medicine, Oslo University Hospital Rikshospitalet, Rikshospitalet, Norway.

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http://dx.doi.org/10.1016/j.jaci.2019.01.019DOI Listing
January 2019
4 Reads

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

Systemic antibody responses to gut commensal bacteria: How and why do I know you?

Authors:
Peter J Mannon

J Allergy Clin Immunol 2019 Apr 18;143(4):1353-1354. Epub 2019 Jan 18.

Department of Gastroenterology and Hepatology, University of Alabama at Birmingham, Birmingham, Ala. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S00916749193008
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http://dx.doi.org/10.1016/j.jaci.2018.12.1019DOI Listing
April 2019
12 Reads

Resolution of celiac disease, IgA deficiency and platelet refractoriness after allogeneic bone marrow transplantation for acute leukemia.

Haematologica 2019 Mar 17;104(3):e121-e123. Epub 2019 Jan 17.

University Health Network (UHN), Laboratory Medicine Program (LMP), Department of Laboratory Hematology (Blood Transfusion Laboratory [BTL])

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http://dx.doi.org/10.3324/haematol.2018.203943DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6395320PMC
March 2019
5 Reads

Pediatric immunoglobulin A complex secretory component deficiency.

Pediatr Int 2018 Jul;60(7):662-663

Department of Pediatrics, Nippon Medical School, Tokyo, Japan.

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http://dx.doi.org/10.1111/ped.13575DOI Listing
July 2018
2 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

Trueness Evaluation and Verification of Interassay Agreement of 11 Serum IgA Measuring Systems: Implications for Medical Decisions.

Clin Chem 2019 Mar 9;65(3):473-483. Epub 2019 Jan 9.

Research Centre for Metrological Traceability in Laboratory Medicine (CIRME), University of Milan, Milan, Italy.

Background: To identify an IgA deficiency, the availability of reliable IgA lower reference limits is essential, especially in pediatrics. In this study, we reported the results of an intercomparison study aimed to verify the status of standardization of IgA measurements using 11 commercially available measuring systems (MSs).

Methods: After confirming its commutability, the ERM-DA470k/IFCC reference material was used for the trueness evaluation of IgA MSs. Read More

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http://dx.doi.org/10.1373/clinchem.2018.297655DOI Listing
March 2019
3 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

Vasculitis in Systemic Autoinflammatory Diseases.

Front Pediatr 2018 3;6:377. Epub 2018 Dec 3.

Division of Pediatric Rheumatology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Autoinflammatory diseases (AID) are diseases of the innate immune system, characterized by recurrent episodes of localized or systemic inflammation. Vasculitis may accompany AID. The causes of the association of vasculitis with monogenic AID are still debated. Read More

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http://dx.doi.org/10.3389/fped.2018.00377DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6287042PMC
December 2018
19 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

Iron Deficiency Anaemia In Pakistan: Celiac Disease An Underlying Cause.

J Ayub Med Coll Abbottabad 2018 Jul-Sep;30(3):372-376

Nusrat Riaz College, Quetta-Pakistan.

Background: Iron deficiency anaemia (IDA) is common in Pakistani population and an important cause of morbidity. Celiac disease (CD) is an important contributor and its diagnosis is frequently missed especially if present in atypical/non-diarrhoeal form. Non-responders to iron replacement therapy typically raises suspicion of celiac disease as underlying cause. Read More

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January 2019
19 Reads

Immune/microbial interface perturbation in human IgA deficiency.

Gut Microbes 2018 Nov 18:1-5. Epub 2018 Nov 18.

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

In a recently published article we report the metagenomic analysis of human gut microbiomes evolved in the absence of immunoglobulin A (IgA). We show that human IgA deficiency is not associated with massive quantitative perturbations of gut microbial ecology. While our study underlines a rather expected pathobiont expansion, we at the same time highlight a less expected depletion in some typically beneficial symbionts. Read More

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https://www.tandfonline.com/doi/full/10.1080/19490976.2018.1
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http://dx.doi.org/10.1080/19490976.2018.1546520DOI Listing
November 2018
14 Reads

The effect of omalizumab treatment on IgE and other immunoglobulin levels in patients with chronic spontaneous urticaria and its association with treatment response.

Postepy Dermatol Alergol 2018 Oct 19;35(5):516-519. Epub 2018 Jul 19.

Department of Allergy and Clinical Immunology, Adnan Menderes University, Aydın, Turkey.

Introduction: Suppression of free immunoglobulin E (IgE) levels and an increase in total IgE levels are observed during omalizumab treatment. However, whether omalizumab has any effect on other immunoglobulins is unknown.

Aim: To investigate the effect of omalizumab treatment on serum IgE and other immunoglobulins, and demonstrate any association with response to treatment in patients with chronic spontaneous urticaria (CSU). Read More

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https://www.termedia.pl/doi/10.5114/ada.2017.71422
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http://dx.doi.org/10.5114/ada.2017.71422DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6232555PMC
October 2018
9 Reads

Inflammatory biomarkers in psychosis and clinical high risk populations.

Schizophr Res 2018 Nov 8. Epub 2018 Nov 8.

Department of Psychiatry, Columbia University Medical Center, New York, NY 10032, United States of America.

Background: Immunological, nutritional, and microbial factors have been implicated in the pathophysiology of schizophrenia, but the interrelationship among measures is understudied. In particular, an increase in the levels of the pro-inflammatory cytokine interleukin-6 (IL-6) is associated with all phases of the illness, and may be associated with other inflammatory markers. Vitamin D is a modulator of the immune system, and LPS antibodies are an indirect measure of gut barrier function. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09209964183061
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http://dx.doi.org/10.1016/j.schres.2018.10.017DOI Listing
November 2018
19 Reads

Alpha 1 antitrypsin distribution in an allergic asthmatic population sensitized to house dust mites.

Clin Transl Allergy 2018 2;8:44. Epub 2018 Nov 2.

5Allergy Unit, Hospital Universitario de Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria, Spain.

Background And Objective: Severe alpha1 antitrypsin deficiency has been clearly associated with pulmonary emphysema, but its relationship with bronchial asthma remains controversial. Some deficient alpha 1 antitrypsin (AAT) genotypes seem to be associated with asthma development. The objective of this study was to analyze the distribution of AAT genotypes in asthmatic patients allergic to house dust mites (HDM), and to asses a possible association between these genotypes and severe asthma. Read More

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http://dx.doi.org/10.1186/s13601-018-0231-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6214172PMC
November 2018
2 Reads

An X-Linked Hyper-IgM Patient Followed Successfully for 23 Years without Hematopoietic Stem Cell Transplantation.

Case Reports Immunol 2018 14;2018:6897935. Epub 2018 Oct 14.

Ege University Faculty of Medicine, Department of Medical Genetics, Izmir, Turkey.

When caring for patients with life-limiting diseases, improving survival and optimizing quality of life are the primary goals. For patients with X-linked hyper-IgM syndrome (XHIGM), the treatment modality has to be decided for a particular patient regarding hematopoietic stem cell transplantation or intravenous immunoglobulin replacement therapy with prophylaxis. A seven-year-old male patient was admitted with recurrent upper and lower respiratory tract infections and recurrent otitis media. Read More

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https://www.hindawi.com/journals/crii/2018/6897935/
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http://dx.doi.org/10.1155/2018/6897935DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6204170PMC
October 2018
5 Reads

The Handbook of Minerals on a Gluten-Free Diet.

Authors:
Iga Rybicka

Nutrients 2018 Nov 5;10(11). Epub 2018 Nov 5.

Faculty of Commodity Science, Poznań University of Economics and Business, 61-875 Poznań, Poland.

The importance of a gluten-free diet (GFD) in the treatment of celiac disease and other gluten-related disorders is undisputable. However, strict GFD often lead to nutritional imbalances and, therefore, to deficiencies. One of the most common deficiencies from a GFD are an insufficient amount of Ca, Fe, Mg, and Zn. Read More

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http://www.mdpi.com/2072-6643/10/11/1683
Publisher Site
http://dx.doi.org/10.3390/nu10111683DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6266778PMC
November 2018
9 Reads

Salivary IgA deficiency in a patient with cystic fibrosis (genotype M470V/V520F).

Ann Allergy Asthma Immunol 2018 Nov 7;121(5):619-620. Epub 2018 Jul 7.

Department of Pulmonary and Critical Care, Allergy/Immunology Fellowship, University Hospitals, Cleveland Medical Center, Cleveland, Ohio; Allergy/Immunology Associates Inc., Mayfield Heights, Ohio.

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http://dx.doi.org/10.1016/j.anai.2018.07.003DOI Listing
November 2018
3 Reads

Clinical significance of serum and mesangial galactose-deficient IgA1 in patients with IgA nephropathy.

PLoS One 2018 2;13(11):e0206865. Epub 2018 Nov 2.

Division of Nephrology, Department of Medicine, Showa University School of Medicine, Tokyo, Japan.

Introduction: Galactose-deficient IgA1 (Gd-IgA1) is a critical pathogenic factor for IgA nephropathy (IgAN), but its value as a disease-specific biomarker remains controversial. We aimed to clarify the clinical significance of Gd-IgA1 in patients with IgAN.

Methods: We retrospectively reviewed 111 patients who were diagnosed with IgAN based on the findings of renal biopsies (RB) at Showa University Hospital since 2007. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0206865PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6214568PMC
April 2019
10 Reads

Effect of methionine deficiency on duodenal and jejunal IgA B cell count and immunoglobulin level of broilers.

Authors:
B Wu L Li T Ruan X Peng

Iran J Vet Res 2018 ;19(3):165-171

Department of Animal Pathology, College of Life Science, China West Normal University, Sichuan, China.

Dietary methionine (met) is reported to enhance antibody production and boost cell-mediated immunity in chickens. Methionine deficiency has been shown to affect the development of the lymphoid organs and the generation of antibodies in chickens. This study is designed to investigate the effects of met deficiency on IgA B cells and immunoglobulins (sIgA, IgA, IgG and IgM) for a 6 week period in the duodenum and jejunum of Cobb broiler chicken using immunohistochemistry and enzyme-linked immunosorbent assay (ELISA) techniques. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6184025PMC
January 2018
2 Reads

BAFF and BAFF-Receptor in B Cell Selection and Survival.

Front Immunol 2018 8;9:2285. Epub 2018 Oct 8.

Faculty of Medicine, Center for Chronic Immunodeficiency, Medical Center - University of Freiburg, Freiburg, Germany.

The BAFF-receptor (BAFFR) is encoded by the TNFRSF13C gene and is one of the main pro-survival receptors in B cells. Its function is impressively documented in humans by a homozygous deletion within exon 2, which leads to an almost complete block of B cell development at the stage of immature/transitional B cells. The resulting immunodeficiency is characterized by B-lymphopenia, agammaglobulinemia, and impaired humoral immune responses. Read More

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https://www.frontiersin.org/article/10.3389/fimmu.2018.02285
Publisher Site
http://dx.doi.org/10.3389/fimmu.2018.02285DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6186824PMC
October 2018
21 Reads

Prevalence and Clinical Features of Celiac Disease in Healthy School-Aged Children.

Dig Dis Sci 2019 01 12;64(1):173-181. Epub 2018 Oct 12.

Department of Medical Biochemistry, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey.

Background/aims: The aim of this study was to determine the prevalence of celiac disease (CD) in healthy school-aged children in the northern region of Cyprus and to investigate the existence of potential markers that may accompany CD. This is the first study to measure the prevalence of CD in the northern region of Cyprus.

Methods: This study included 3792 school-aged children who were between the ages of 6 and 10 years between January 2015 and October 2016. Read More

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http://link.springer.com/10.1007/s10620-018-5320-0
Publisher Site
http://dx.doi.org/10.1007/s10620-018-5320-0DOI Listing
January 2019
31 Reads