4,623 results match your criteria Immunoglobulin A Deficiency

O- and N-Glycosylation of Serum Immunoglobulin A is Associated with IgA Nephropathy and Glomerular Function.

J Am Soc Nephrol 2021 Jun 14. Epub 2021 Jun 14.

M Falchi, Department of Twin Research & Genetic Epidemiology, King's College London, London, United Kingdom of Great Britain and Northern Ireland.

Immunoglobulin A (IgA) nephropathy (IgAN) is the most common primary glomerular disease worldwide, and is a leading cause of renal failure. The disease mechanisms are not completely understood, but a higher abundance of galactose-deficient IgA is recognized to play a crucial role in IgAN pathogenesis. While both types of human IgA (IgA1 and IgA2) have several -glycans as posttranslational modification, only IgA1 features extensive hinge-region -glycosylation. Read More

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Collagenous Gastritis in Primary Selective IgM Deficiency: Transition to EBV+ Gastric Adenocarcinoma.

Case Reports Immunol 2021 25;2021:5574944. Epub 2021 May 25.

Division of Basic and Clinical Immunology, University of California, Irvine, California, USA.

Selective IgM deficiency (SIgMD) and isolated collagenous gastritis are two independent rare disorders. Our purpose is to report the 1 case of SIgMD and isolated collagenous gastritis and collagenous gastritis that has transitioned to EBV + gastric adenocarcinoma. Gastric biopsy tissue was analyzed by EBV-related encoded RNA in situ hybridization assay. Read More

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Coexistence of immune-mediated diseases in sarcoidosis Frequency and clinical significance in 1737 patients.

Joint Bone Spine 2021 Jun 8:105236. Epub 2021 Jun 8.

Department of Medicine, Universitat de Barcelona, Barcelona, Spain; Department of Autoimmune Diseases, ICMiD, Hospital Clinic, Barcelona, Spain. Electronic address:

Objective: To analyze whether immune-mediated diseases (IMDs) occurs in sarcoidosis more commonly than expected in the general population, and how concomitant IMDs influence the clinical presentation of the disease.

Methods: We searched for coexisting IMDs in patients included in the SARCOGEAS-cohort, a multicenter nationwide database of consecutive patients diagnosed according to the ATS/ESC/WASOG criteria. Comparisons were made considering the presence or absence of IMD clustering, and odds ratios (OR) and their 95% confidence intervals (CI) were calculated as the ratio of observed cases of every IMD in the sarcoidosis cohort to the observed cases in the general population. Read More

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IgA Deficiency and Membranoproliferative Glomerulonephritis: A Case Report.

Int Med Case Rep J 2021 3;14:377-380. Epub 2021 Jun 3.

Department of Medicine, Nephrology and Dialysis Unit, SS Annunziata Hospital, "G. d'Annunzio" University, Chieti, Italy.

Background: Immunoglobulin A deficiency (IgAD) is the most common form of primary immunodeficiency in western countries. It can be associated with the development of autoimmune diseases both in adults and in children even though the exact pathophysiology is not fully defined.

Case Presentation: We report here a case of a young patient who developed nephrotic syndrome secondary to membranoproliferative glomerulonephritis associated with the incidental finding of IgAD. Read More

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TNFRSF13B polymorphisms counteract microbial adaptation to natural IgA.

JCI Insight 2021 Jun 10. Epub 2021 Jun 10.

Department of Microbiology and Immunology, University of Michigan, Ann Arbor, United States of America.

TNFRSF13B encodes the "transmembrane-activator and CAML-interactor" (TACI) receptor, which drives plasma cell differentiation. Although TNFRSF13B supports host defense, dominant-negative TNFRSF13B alleles are common in humans and other species and only rarely associate with disease. We reasoned the high frequency of disruptive TNFRSF13B alleles reflects balancing selection, the loss of function conferring advantage in some settings. Read More

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SARS-CoV-2 infectious virus, viral RNA in nasopharyngeal swabs, and serostatus of symptomatic COVID-19 outpatients in the United States.

medRxiv 2021 Jun 1. Epub 2021 Jun 1.

Background: SARS-CoV-2 infectious virus isolation in the upper airway of COVID-19 patients is associated with higher levels of viral RNA. However, comprehensive evaluation of the relationships between host and disease factors and infectious, replication competent virus is needed.

Methods: Symptomatic COVID-19 outpatients were enrolled from the United States. Read More

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The Prevalence of Atopic Manifestations in 313 Iranian Patients with Inborn Errors of Immunity.

Int Arch Allergy Immunol 2021 May 31:1-5. Epub 2021 May 31.

Immunology and Allergy Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Introduction: Inborn errors of immunity (IEIs) are rare inherited disorders with a broad spectrum of manifestations. Here, we aimed to delineate the atopy burden in a cohort of patients with IEIs.

Methods: 313 patients with IEIs were enrolled in the study within a 9-years period, and data were collected via a questionnaire. Read More

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Protein-Activated Kinase 3 (PAK3)-Related Intellectual Disability Associated with Combined Immunodeficiency: A Case Report.

Am J Case Rep 2021 May 20;22:e930966. Epub 2021 May 20.

Department of Pediatrics, Faculty of Medicine, Health Sciences Center, Kuwait University, Jabriya, Kuwait.

BACKGROUND X-linked intellectual disabilities constitute a group of clinically and genetically heterogeneous disorders that are divided into syndromic and nonsyndromic forms. PAK3 mutations are associated with X-linked nonsyndromic forms of intellectual disability, with the most common clinical features being cognitive deficit, large ears, oral motor hypotonia, and neurobehavioral abnormalities. These mutations have been reported to be associated with either loss of the PAK3 protein or loss of its kinase activity. Read More

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Clinical and Demographic Characteristics of Cutaneous Mastocytosis in Childhood: Single-center Experience.

J Pediatr Hematol Oncol 2021 May 18. Epub 2021 May 18.

Departments of Pediatric Hematology and Oncology Pediatric Allergy and Immunology, Ankara City Hospital, Children's Hospital Department of Pediatric Hematology and Oncology, Ankara Atilim University Department of Genetics, Ankara City Hospital, Ankara, Turkey.

Introduction: Mastocytosis is a rare and heterogenous disease, and in children it is generally limited to the skin and tends to regress spontaneously in adolescence.

Aim: In this study, demographic, clinical, and laboratory characteristics of pediatric patients with mastocytosis, and also coexisting diseases were investigated.

Results: A total of 61 pediatric patients were included in the study. Read More

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Reconvalescent plasma/camostat mesylate in early SARS-CoV-2 Q-PCR positive high-risk individuals (RES-Q-HR): a structured summary of a study protocol for a randomized controlled trial.

Trials 2021 May 17;22(1):343. Epub 2021 May 17.

Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital Duesseldorf, Medical Faculty Heinrich-Heine-University Duesseldorf, Moorenstr. 5, D-40225, Duesseldorf, Germany.

Objectives: Currently, there are no approved treatments for early disease stages of COVID-19 and few strategies to prevent disease progression after infection with SARS-CoV-2. The objective of this study is to evaluate the safety and efficacy of convalescent plasma (CP) or camostat mesylate administered within 72 h of diagnosis of SARS-CoV-2 infection in adult individuals with pre-existing risk factors at higher risk of getting seriously ill with COVID-19. Camostat mesylate acts as an inhibitor of the host cell serine protease TMPRSS2 and prevents the virus from entering the cell. Read More

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Innate Mechanisms in Selective IgA Deficiency.

Front Immunol 2021 26;12:649112. Epub 2021 Apr 26.

Cell Biology and Immunology Group, Wageningen University & Research, Wageningen, Netherlands.

Selective IgA deficiency (SIgAD), characterized by a serum IgA level below 0.07 mg/ml, while displaying normal serum levels of IgM and IgG antibodies, is the most frequently occurring primary immunodeficiency that reveals itself after the first four years after birth. These individuals with SIgAD are for the majority healthy and even when they are identified they are usually not investigated further or followed up. Read More

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Isolated IgG2 Deficiency is an Independent Risk Factor for Exacerbations in Bronchiectasis.

QJM 2021 May 10. Epub 2021 May 10.

Centre for Inflammation Research at the University of Edinburgh, Queen's Medical Research Institute, Edinburgh BioQuarter, Edinburgh, EH16 4TJ, Scotland.

Background: Immunoglobulin G (IgG) subclass 2 deficiency is the most frequent IgG subclass deficiency identified in patients with bronchiectasis, but its clinical significance is not known.

Aim: To analyse if bronchiectasis patients with isolated IgG2 deficiency at risk of recurrent exacerbations and/or hospitalisation? Do patients with IgG2 deficiency have worse disease progression?

Design And Methods: This is a retrospective study (2015-2020) exploring independent risk factors for recurrent exacerbations (three or more per year) and/or hospitalisation with bronchiectasis exacerbations using multivariable models using binary logistic regression. There was no patient with IgG deficiency, IgG 1, 3 or 4 deficiency, or IgA or IgM deficiency included. Read More

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β -Glucan Improves Protective Qi Status in Adults with Protective Qi Deficiency-A Randomized, Placebo-Controlled, and Double-Blinded Trial.

Chin J Integr Med 2021 May 8. Epub 2021 May 8.

Research and Development Department, USANA Health Science, Inc., Salt Lake City, UT, 84121, USA.

Objective: To test the hypothesis that β -glucan enhances protective qi (PQi), an important Chinese medicine (CM) concept which stipulates that a protective force circulates throughout the body surface and works as the first line of defense against "external pernicious influences".

Methods: A total of 138 participants with PQi deficiency (PQD) were randomized to receive β -glucan (200 mg daily) or placebo for 12 weeks. Participants' PQi status was assessed every 2 weeks via conventional diagnosis and a standardized protocol from which a PQD severity and risk score was derived. Read More

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Immunoglobulin A-specific deficiency induces spontaneous inflammation specifically in the ileum.

Gut 2021 May 7. Epub 2021 May 7.

Department of Immunology, Medical Research Institute, TMDU, Bunkyo-ku, Tokyo, Japan

Objective: Although immunoglobulin A (IgA) is abundantly expressed in the gut and known to be an important component of mucosal barriers against luminal pathogens, its precise function remains unclear. Therefore, we tried to elucidate the effect of IgA on gut homeostasis maintenance and its mechanism.

Design: We generated various IgA mutant mouse lines using the CRISPR/Cas9 genome editing system. Read More

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Coexistence of Acquired Hemophilia and Antiphospholipid Serology in Monoclonal Gammopathy Patient.

Int Med Case Rep J 2021 28;14:261-264. Epub 2021 Apr 28.

Department of Internal Medicine, Groupe Hospitalier Sud-Ile de France, Melun, 77000, France.

Acquired hemophilia is a rare coagulopathy with hemorrhage into the skin, muscle, or soft tissues and mucous membranes and caused by inhibitor antibodies, mainly against FVIII. We report a case of acquired hemophilia presenting with diffuse cutaneous hemorrhage and hemothorax. The patient was found to have acquired an FVIII inhibitor and a high titer of anti β2 glycoprotein 1 IgG and IgM, and anticardiolipin IgM in the context of IgA kappa-type monoclonal gammopathy. Read More

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Ovariectomy-Induced Hepatic Lipid and Cytochrome P450 Dysmetabolism Precedes Serum Dyslipidemia.

Int J Mol Sci 2021 Apr 26;22(9). Epub 2021 Apr 26.

Centre for Experimental Medicine, Institute for Clinical and Experimental Medicine, 14021 Prague, Czech Republic.

Ovarian hormone deficiency leads to increased body weight, visceral adiposity, fatty liver and disorders associated with menopausal metabolic syndrome. To better understand the underlying mechanisms of these disorders in their early phases of development, we investigated the effect of ovariectomy on lipid and glucose metabolism. Compared to sham-operated controls, ovariectomized Wistar female rats markedly increased whole body and visceral adipose tissue weight ( ˂ 0. Read More

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Immunoglobulin deficiencies in treated patients suffering from rheumatoid arthritis.

Rheumatol Int 2021 Jul 29;41(7):1273-1280. Epub 2021 Apr 29.

Ambulantes Rheumazentrum Erfurt, Tschaikowskistraße 22, 99096, Erfurt, Germany.

Immunoglobulins and antibodies to immunoglobulins (autoimmunoglobulins) have been identified to be implicated in the pathogenesis of rheumatoid arthritis (RA). Immunoglobulin deficiencies have been suggested to account for the increased risk of infections in RA patients. This study was carried out to determine the prevalence of immunoglobulin deficiencies in patients with RA and the identification of putative contributing factors. Read More

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Low-Risk Human Leukocyte Antigen Genes and Mild Villous Atrophy Typify Celiac Disease With Immunoglobulin A Deficiency.

J Pediatr Gastroenterol Nutr 2021 Jun;72(6):889-893

Gastroenterologia Pediatrica, Clinica Pediatrica e Malattie Rare, Ospedale Pediatrico Microcitemico A. Cao, ARNAS Brotzu, Cagliari.

Objectives: We aimed to establish if in celiac disease (CD) with immunoglobulin A deficiency (IgAD) duodenal histopathology is influenced by human leukocyte antigen (HLA)-DQB1∗02 alleles dosage. Clinical differences between patients with CD and patients with CD and IgAD (CD-IgAD) were also evaluated.

Methods: Five hundred and sixteen CD and 16 patients with CD-IgAD, enrolled over the time of 8 years, took part in this study. Read More

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Immunodeficiency, Centromeric Region Instability, and Facial Anomalies Syndrome (ICF) in a Boy with Variable Clinical and Immunological Presentations.

Iran J Allergy Asthma Immunol 2021 Apr 17;20(2):249-254. Epub 2021 Apr 17.

Department of Allergy and Clinical Immunology, Iran University of Medical Sciences, Tehran, Iran.

Immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome is a rare primary immunodeficiency disorder characterized by recurrent infections and low immunoglobulin levels due to variable combined immunodeficiency, and centromeric region instability, and facial dysmorphism. We describe a 12-year-old boy with recurrent respiratory tract infections, facial anomalies, scoliosis, and psychomotor retardation. He had recurrent pneumonia with low serum IgG and IgM levels during infancy and preschool age. Read More

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Pericardial effusion, cardiomegaly, oedema, and IgA deficiency in a child: coeliac disease.

Lancet 2021 Apr;397(10284):1576

Department of Paediatrics and Neonatology, University Children's Hospital of Saarland, Homburg, Germany.

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Compensatory IgM to the Rescue: Patients with Selective IgA Deficiency Have Increased Natural IgM Antibodies to MAA-LDL and No Changes in Oral Microbiota.

Immunohorizons 2021 Apr 23;5(4):170-181. Epub 2021 Apr 23.

Medical Microbiology and Immunology, Research Unit of Biomedicine, University of Oulu, Oulu, Finland.

IgA is the most abundant Ab in the human body. However, most patients with selective IgA deficiency (SIgAD) are asymptomatic. IgM, and to lesser extent IgG Abs, are generally presumed to compensate for the lack of IgA in SIgAD by multiplying and adopting functions of IgA. Read More

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IgA Antibodies and IgA Deficiency in SARS-CoV-2 Infection.

Front Cell Infect Microbiol 2021 6;11:655896. Epub 2021 Apr 6.

Department of Laboratory Medicine, Research Area Multimodal Medicine, Diagnostic Immunology and Research Unit, Bambino Gesù Children's Hospital IRCCS, Rome, Italy.

A large repertoire of IgA is produced by B lymphocytes with T-independent and T-dependent mechanisms useful in defense against pathogenic microorganisms and to reduce immune activation. IgA is active against several pathogens, including rotavirus, poliovirus, influenza virus, and SARS-CoV-2. It protects the epithelial barriers from pathogens and modulates excessive immune responses in inflammatory diseases. Read More

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Celiac disease: Understandings in diagnostic, nutritional, and medicinal aspects.

Int J Immunopathol Pharmacol 2021 Jan-Dec;35:20587384211008709

Laboratory of Immunology, Center of Clinical Research, University Hospital Mohammed VI, Marrakech, Morocco.

Celiac disease (CD) is characterized by clinical polymorphism, with classic, asymptomatic or oligosymptomatic, and extra-intestinal forms, which may lead to diagnostic delay and exposure to serious complications. CD is a multidisciplinary health concern involving general medicine, pediatric, and adult gastroenterology, among other disciplines. Immunology and pathology laboratories have a fundamental role in diagnosing and monitoring CD. Read More

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The clinical relevance of IgM and IgA anti-pneumococcal polysaccharide ELISA assays in patients with suspected antibody deficiency.

Clin Exp Immunol 2021 Apr 20. Epub 2021 Apr 20.

Department of Tranzo, Tilburg University, Tilburg, the Netherlands.

Unlike immunoglobulin (Ig)G pneumococcal polysaccharide (PnPS)-antibodies, PnPS IgA and IgM-antibodies are not routinely determined for the assessment of immunocompetence. It is not yet known whether an isolated inability to mount a normal IgM or IgA-PnPS response should be considered a relevant primary antibody deficiency (PAD). We studied the clinical relevance of anti-PnPS IgM and IgA-assays in patients with suspected primary immunodeficiency in a large teaching hospital in 's-Hertogenbosch, the Netherlands. Read More

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A randomised, double-blind, placebo-controlled, pilot trial of intravenous plasma purified alpha-1 antitrypsin for SARS-CoV-2-induced Acute Respiratory Distress Syndrome: a structured summary of a study protocol for a randomised, controlled trial.

Trials 2021 Apr 19;22(1):288. Epub 2021 Apr 19.

Royal College of Surgeons in Ireland, Dublin, Ireland.

Objectives: The primary objective is to demonstrate that, in patients with PCR-confirmed SARS-CoV-2 resulting in Acute Respiratory Distress Syndrome (ARDS), administration of 120mg/kg of body weight of intravenous Prolastin®(plasma-purified alpha-1 antitrypsin) reduces circulating plasma levels of interleukin-6 (IL-6). Secondary objectives are to determine the effects of intravenous Prolastin® on important clinical outcomes including the incidence of adverse events (AEs) and serious adverse events (SAEs).

Trial Design: Phase 2, randomised, double-blind, placebo-controlled, pilot trial. Read More

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Clinical heterogeneity among pediatric patients with autoimmune type 1 diabetes stratified by immunoglobulin deficiency.

Pediatr Diabetes 2021 Apr 10. Epub 2021 Apr 10.

Department of Pediatrics, Oncology, and Hematology, Medical University of Łódź, Łódź, Poland.

Background: Type 1 diabetes (T1D) may coexist with primary immunodeficiencies, indicating a shared genetic background.

Objective: To evaluate the prevalence and clinical characteristics of immunoglobulin deficiency (IgD) among children with T1D.

Methods: Serum samples and medical history questionnaires were obtained during routine visits from T1D patients aged 4-18 years. Read More

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Antibody deficiencies are more common in adult versus pediatric recurrent acute rhinosinusitis.

Am J Otolaryngol 2021 Mar 29;42(5):103004. Epub 2021 Mar 29.

Departments of Otolaryngology, West Virginia University School of Medicine, Morgantown, WV, USA.

Purpose: To evaluate the frequency and types of humoral immunodeficiencies (HID) in pediatric and adult patients with recurrent (RARS). Patients with HID commonly present with upper respiratory tract infections. Their pathophysiology in children is different than adult counterparts. Read More

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[Acupuncture combined with powder for children with cerebral palsy and its effect on serum immune indexes and nerve growth related protein].

Zhongguo Zhen Jiu 2021 Mar;41(3):288-92

Rehabilitation Center, Children's Hospital of Zhengzhou University/Henan Children's Hospital/Zhengzhou Children's Hospital, Zhengzhou 450002, China.

Objective: To observe the efficacy of acupuncture combined with powder for children with cerebral palsy (liver- stagnation, spleen-kidney deficiency syndrome) and its effect on serum immune indexes and nerve growth related protein.

Methods: A total of 180 children with cerebral palsy were randomly divided into a combined group (60 cases, 2 cases dropped off), an acupuncture group (60 cases, 4 cases dropped off) and a Chinese medication group (60 cases, 5 cases dropped off). On the basis of conventional treatment, the children in the combined group were treated with acupuncture [Baihui (GV 20), Sishencong (EX-HN 1), Shenting (GV 24), Benshen (GB 13), 30 min each time, twice a day] and powder; the children in the acupuncture group were treated with acupuncture, and the children in the Chinese medication group were treated with powder, the treatment was same with the combined group. Read More

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Comprehensive comparison between 222 CTLA-4 haploinsufficiency and 212 LRBA deficiency patients: a systematic review.

Clin Exp Immunol 2021 07 3;205(1):28-43. Epub 2021 May 3.

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

Cytotoxic T lymphocyte antigen 4 (CTLA-4) haploinsufficiency (CHAI) and lipopolysaccharide-responsive beige-like anchor (LRBA) deficiency (LATAIE) are newly identified inborn errors of immunity with shared molecular pathomechanisms and clinical manifestations. In this review, we aimed to provide differential comparisons regarding demographic, clinical, immunological and molecular characteristics between these two similar conditions. A literature search was conducted in PubMed, Web of Science and Scopus databases and included studies were systematically evaluated. Read More

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IgA Deficiency Is Not Systematically Ruled Out in Patients Undergoing Celiac Disease Testing.

Dig Dis Sci 2021 Mar 26. Epub 2021 Mar 26.

Gastroenterology Department, Hospital Británico de Buenos Aires, Perdriel 74 (1012), Buenos Aires, Argentina.

Background: Guidelines for celiac disease (CD) testing recommend total serum IgA determination alongside anti-transglutaminase IgA antibodies. It is not well known if lack of serum IgA determination is a common finding in clinical practice.

Aim: To determine the prevalence of lack of serum IgA determination among patients screened for celiac disease. Read More

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