2,653 results match your criteria Immunoglobulin M Deficiency

Paraprotein associated heparin resistance during cardiopulmonary bypass.

Perfusion 2022 Jun 14:2676591221109143. Epub 2022 Jun 14.

Department of Cardiothoracic Surgery, 60086Royal North Shore Hospital, Sydney, NSW, Australia.

Introduction: Heparin resistance during cardiopulmonary bypass poses a significant intraoperative dilemma. Antithrombin deficiency related heparin resistance is well described, but less common causes are still poorly understood and inadequately managed.

Case Report: We present a case of heparin resistance during cardiopulmonary bypass in a gentleman with no previous haematological history or thrombotic risk factors. Read More

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Variants Disrupting CD40L Transmembrane Domain and Atypical X-Linked Hyper-IgM Syndrome: A Case Report With Leishmaniasis and Review of the Literature.

Front Immunol 2022 28;13:840767. Epub 2022 Apr 28.

Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.

X-linked hyper-IgM (XHIGM) syndrome is caused by mutations of the CD40LG gene, encoding the CD40L protein. The clinical presentation is characterized by early-onset infections, with profound hypogammaglobulinemia and often elevated IgM, susceptibility to opportunistic infections, such as pneumonia, biliary tract disease due to , and malignancy. We report a 41-year-old male presenting with recurrent leishmaniasis, hypogammaglobulinemia, and myopathy. Read More

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Naturally acquired antibodies against 7 Streptococcus pneumoniae serotypes in Indigenous and non-Indigenous adults.

PLoS One 2022 14;17(4):e0267051. Epub 2022 Apr 14.

NOSM University, Thunder Bay, ON, Canada.

Despite the use of pneumococcal conjugate vaccines for pediatric immunization, North American Indigenous populations continue to experience high burden of pneumococcal infections. Naturally acquired antibodies, which can protect unvaccinated adults against pneumococcal infections, have not previously been studied in Canadian Indigenous people. We analysed concentrations of natural serum IgG, IgM and IgA antibodies specific to 7 serotype-specific capsular polysaccharides (3, 6B, 9V, 14, 19A, 19F and 23F) in 141 healthy individuals (age between 18 and 80 years), including Indigenous adults living in 2 geographical different areas of Ontario, Canada, and non-Indigenous residing in northwestern Ontario. Read More

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Screening for Immunodeficiencies in Children With Invasive Pneumococcal Disease: Six-year Experience From a UK Children's Hospital.

Pediatr Infect Dis J 2022 07 7;41(7):575-578. Epub 2022 Jun 7.

From the Oxford Vaccine Group, Department of Paediatrics, University of Oxford, Oxford University Hospitals NHS Foundation Trust, and National Institute for Health Research Oxford Biomedical Research Centre, Oxford, United Kingdom.

Background: A previous study showed that investigation of children with invasive pneumococcal disease (IPD) revealed an immunodeficiency in up to 10% of cases. Following this report, we implemented a protocol to investigate children with IPD, to assess the proportion with an immunodeficiency in our setting.

Methods: We retrospectively identified patients who presented with IPD from January 2015 to November 2020 and collected data from medical records. Read More

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Hydroxychloroquine Therapy and Serum Immunoglobulin Levels in Women with IgG Subclass Deficiency and Systemic Lupus Erythematosus, Sjögren Syndrome, and Rheumatoid Arthritis: A Retrospective Study.

Arch Immunol Ther Exp (Warsz) 2022 Apr 11;70(1):14. Epub 2022 Apr 11.

Department of Medicine, Brookwood Medical Center, Birmingham, AL, USA.

Hydroxychloroquine (HCQ) therapy decreased immunoglobulin (Ig) levels in patients with Sjögren syndrome (SS) and rheumatoid arthritis (RA) in previous studies. We found no report of Ig levels of women with IgG subclass deficiency (IgGSD) and systemic lupus erythematosus (SLE), SS, or RA treated with HCQ. We retrospectively evaluated IgG, IgG subclass, IgA, and IgM levels and other characteristics of women at IgGSD diagnosis who did and did not take HCQ for SLE, SS, or RA. Read More

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Novel Insight Into Nutritional Regulation in Enhancement of Immune Status and Mediation of Inflammation Dynamics Integrated Study and of Teleost Grass Carp (): Administration of Threonine.

Front Immunol 2022 14;13:770969. Epub 2022 Mar 14.

Animal Nutrition Institute, Sichuan Agricultural University, Chengdu, China.

This study aims to investigate the effects of threonine (Thr) on immunoregulation and of teleost grass carp (). Juveniles (9.53 ± 0. Read More

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Protocol for the unclassified primary antibody deficiency (unPAD) study: Characterization and classification of patients using the ESID online Registry.

PLoS One 2022 25;17(3):e0266083. Epub 2022 Mar 25.

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

Background: Primary antibody deficiencies (PADs) without an identified monogenetic origin form the largest and most heterogeneous group of primary immunodeficiencies. These patients often remain undiagnosed for years and many present to medical attention in adulthood after several infections risking structural complications. Not much is known about their treatment, comorbidities, or prognosis, nor whether the various immunological forms (decreased total IgG, IgG subclass(es), IgM, IgA, specific antibody responses, alone or in combination(s)) should be considered as separate, clearly definable subgroups. Read More

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Atypical Autosomal Recessive AID Deficiency-Yet Another Piece of the Hyper-IgM Puzzle.

J Clin Immunol 2022 May 25;42(4):713-715. Epub 2022 Mar 25.

Immunology and Immunodeficiency Laboratory, Garvan Institute of Medical Research, 384 Victoria Street, Darlinghurst, New South Wales, 2010, Australia.

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Evaluation of Specific Antibody Responses in Patients with Selective IgA Deficiency and Ataxia Telangiectasia.

Endocr Metab Immune Disord Drug Targets 2022 Feb 8. Epub 2022 Feb 8.

Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran | Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran.

Background: Specific Antibody Deficiency (SAD) is a primary immunodeficiency disease (PID) characterized by the occurrence of recurrent infections and inadequate antibody response to polysaccharide new antigens.

Objective: This study aims to determine the titer of specific antibodies against unconjugated 23-valent pneumococcal polysaccharide vaccine (PPSV-23), the presence of SAD, and its association with clinical and laboratory findings in Ataxia-telangiectasia (A-T) and selective immunoglobulin A deficiency (SIgAD) patients.

Methods: 32 A-T patients and 43 SIgAD patients were included in the study. Read More

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February 2022

Serum Impedance in Children with Recurrent Respiratory Infections.

Adv Exp Med Biol 2022 ;1375:47-54

Third Department and Clinic of Pediatrics, Immunology, and Rheumatology of Developmental Age, Wroclaw Medical University, Wroclaw, Poland.

The role of disturbed immunoglobulin content during recurrent respiratory tract infections (RTI) might escape recognition in practical children's diagnostics. This study aims to investigate the potential changes in serum impedance caused by a constellation of decreases in the immunoglobulin IgA, IgG, and IgM content in RTI. The control group consisted of children suffering from RTI without any evident decreases in immunoglobulins. Read More

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Unique B-1 cells specific for both N-pyrrolated proteins and DNA evolve with apolipoprotein E deficiency.

J Biol Chem 2022 02 11;298(2):101582. Epub 2022 Jan 11.

Graduate School of Agricultural and Life Sciences, The University of Tokyo, Tokyo, Japan; Japan Agency for Medical Research and Development, CREST, Tokyo, Japan. Electronic address:

Lysine N-pyrrolation, a posttranslational modification, which converts lysine residues to N-pyrrole-L-lysine, imparts electronegative properties to proteins, causing them to mimic DNA. Apolipoprotein E (apoE) has been identified as a soluble receptor for pyrrolated proteins (pyrP), and accelerated lysine N-pyrrolation has been observed in apoE-deficient (apoE) hyperlipidemic mice. However, the impact of pyrP accumulation consequent to apoE deficiency on the innate immune response remains unclear. Read More

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February 2022

IgM to IgG Class Switching Is a Necessary Step for Pemphigus Phenotype Induction in Desmoglein 3-Specific B Cell Receptor Knock-in Mouse.

J Immunol 2022 02 7;208(3):582-593. Epub 2022 Jan 7.

Department of Dermatology, Keio University School of Medicine, Tokyo, Japan;

Pemphigus vulgaris is an autoimmune blistering disease caused by IgG targeting desmoglein 3 (Dsg3), an adhesion molecule of keratinocytes. Anti-Dsg3 IgG production is prevented in healthy individuals, but it is unclear how Dsg3-specific B cells are regulated. To clarify the immunological condition regulating Dsg3-specific B cells, a pathogenic anti-Dsg3 Ig (AK23) knock-in mouse was generated. Read More

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February 2022

Immunological evaluation of patients with orthopedic infections: taking the Cierny-Mader classification to the next level.

J Bone Jt Infect 2021 1;6(9):433-441. Epub 2021 Dec 1.

International Center for Limb Lengthening, Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore, Baltimore, Maryland, USA.

: Cierny-Mader osteomyelitis classification is used to label A, B, or C hosts based on comorbidities. This study's purpose was to define the "true" host status of patients with orthopedic infection using serologic markers to quantify the competence of their immune system while actively infected. : Retrospective chart review identified patients at a single-surgeon practice who were diagnosed with orthopedic infection between September 2013 and March 2020 and had immunological laboratory results. Read More

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December 2021

Loss of Id3 (Inhibitor of Differentiation 3) Increases the Number of IgM-Producing B-1b Cells in Ischemic Skeletal Muscle Impairing Blood Flow Recovery During Hindlimb Ischemia.

Arterioscler Thromb Vasc Biol 2022 01 23;42(1):6-18. Epub 2021 Nov 23.

Robert M. Berne Cardiovascular Research Center (V.O., C.A.M.), University of Virginia.

Objective: Neovascularization can maintain and even improve tissue perfusion in the setting of limb ischemia during peripheral artery disease. The molecular and cellular mechanisms mediating this process are incompletely understood. We investigate the potential role(s) for Id3 (inhibitor of differentiation 3) in regulating blood flow in a murine model of hindlimb ischemia (HLI). Read More

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January 2022

Tafazzin deficiency impairs mitochondrial metabolism and function of lipopolysaccharide activated B lymphocytes in mice.

FASEB J 2021 12;35(12):e22023

Diabetes Research Envisioned and Accomplished in Manitoba (DREAM) Theme, Children's Hospital Research Institute of Manitoba, Winnipeg, Manitoba, Canada.

B lymphocytes are responsible for humoral immunity and play a key role in the immune response. Optimal mitochondrial function is required to support B cell activity during activation. We examined how deficiency of tafazzin, a cardiolipin remodeling enzyme required for mitochondrial function, alters the metabolic activity of B cells and their response to activation by lipopolysaccharide in mice. Read More

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December 2021

Resolution of Laryngeal Oedema in a Patient with Acquired C1-Inhibitor Deficiency. a Case Report.

J Crit Care Med (Targu Mures) 2021 Apr 12;7(2):136-140. Epub 2021 May 12.

George Emil Palade University of Medicine, Pharmacy, Science, and Technology of Targu Mures, Mures Romania.

Introduction: Laryngeal oedema caused by acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a life-threatening condition. The swelling is bradykinin mediated and will not respond to the usual treatment with antihistamines, corticosteroids, or epinephrine. Instead, kallikrein-bradykinin-targeted therapies should be used promptly to prevent asphyxiation. Read More

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Glia maturation factor-γ is involved in S1P-induced marginal zone B-cell chemotaxis and optimal IgM production to type II T-independent antigen.

Int Immunol 2022 01;34(1):35-43

Department of Immunology, School of Basic Medical Sciences, Fudan University, Shanghai, China.

Marginal zone B cells (MZBs) represent a unique B-cell sub-population that rapidly differentiate into IgM-secreting plasma cells in response to T-independent (T-I) antigen. Sphingosine 1-phosphate (S1P) promotes MZB localization to the marginal zone. However, intracellular molecules involved in MZB localization and migration remain largely unknown. Read More

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January 2022

IgM Deficiency in Autoimmune Blistering Mucocutaneous Diseases Following Various Treatments: Long Term Follow-Up and Relevant Observations.

Front Immunol 2021 27;12:727520. Epub 2021 Sep 27.

Department of Dermatology, The Center for Blistering Diseases, Tufts University School of Medicine, Boston, MA, United States.

IgM deficiency has been reported in patients with many autoimmune diseases treated with Rituximab (RTX). It has not been studied, in detail, in autoimmune mucocutaneous blistering diseases (AIMBD). Our objectives were: (i) Examine the dynamics of IgM levels in patients with and without RTX. Read More

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November 2021

Effects of Se-Enriched Malt on the Immune and Antioxidant Function in the Se-Deprived Reclamation Merino Sheep in Southern Xinjiang.

Biol Trace Elem Res 2022 Aug 11;200(8):3621-3629. Epub 2021 Oct 11.

School of Life Science and Engineering, Southwest University of Science and Technology, Mianyang, 621010, China.

We have found that the Reclamation merino sheep in Southern Xinjiang, China, showed emaciation, stiff limbs, instability, and sudden death, which is related to the impairment of immune function and antioxidant capacity caused by selenium (Se) deficiency. The experiments were to study the effects of Se-enriched malt on the immune and antioxidant function in Se-deprived Reclamation merino sheep in Southern Xinjiang, China. The samples of soil and forage had been collected from tested pastures, and animal tissues were also collected in tested animals. Read More

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A 70-Year-Old Man With Cough and Recurrent Respiratory Infections.

Chest 2021 10;160(4):e347-e350

Division of Pulmonary and Critical Care Medicine, Department of Medicine, Mayo Clinic College of Medicine and Science, Rochester, MN. Electronic address:

Case Presentation: A 70-year-old man was referred for evaluation of recurrent respiratory infections requiring antibiotics and chronic cough over 3 years. Two months prior to presentation, he started to develop blood-tinged sputum but not frank hemoptysis. He otherwise denied any fever, chills, night sweats, or weight loss. Read More

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October 2021

RAG1 splicing mutation causes enhanced B cell differentiation and autoantibody production.

JCI Insight 2021 10 8;6(19). Epub 2021 Oct 8.

Department of Immunology, School of Basic Medical Sciences, Fudan University, Shanghai, China.

Hypomorphic RAG1 or RAG2 mutations cause primary immunodeficiencies and can lead to autoimmunity, but the underlying mechanisms are elusive. We report here a patient carrying a c.116+2T>G homozygous splice site mutation in the first intron of RAG1, which led to aberrant splicing and greatly reduced RAG1 protein expression. Read More

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October 2021

Tetraspanin CD53 modulates lymphocyte trafficking but not systemic autoimmunity in Lyn-deficient mice.

Immunol Cell Biol 2021 11 10;99(10):1053-1066. Epub 2021 Oct 10.

Centre for Inflammatory Diseases, Department of Medicine, Monash Medical Centre, Monash University, Clayton, VIC, Australia.

The leukocyte-restricted tetraspanin CD53 has been shown to promote lymphocyte homing to lymph nodes (LNs) and myeloid cell recruitment to acutely inflamed peripheral organs, and accelerate the onset of immune-mediated disease. However, its contribution in the setting of chronic systemic autoimmunity has not been investigated. We made use of the Lyn autoimmune model, generating Cd53 Lyn mice, and compared trafficking of immune cells into secondary lymphoid organs and systemic autoimmune disease development with mice lacking either gene alone. Read More

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November 2021

Serum Protein Electrophoresis May Be Used as a Screening Tool for Antibody Deficiency in Children and Adolescents.

Front Immunol 2021 23;12:712637. Epub 2021 Aug 23.

Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, São Paulo, Brazil.

Background: Patients with antibody deficiency may experience exceptionally long diagnostic delays, increasing the risk of life-threatening infections, end-organ damage, mortality, and health costs.

Objective: This study aimed to analyze serum protein electrophoresis and verify the correlation between calculated globulin (CG, total protein minus albumin levels) or electrophoretically determined serum gamma globulin fraction (Gamma) with IgG levels in children and adolescents under 18 years old (yo).

Methods: We analyzed serum protein electrophoresis (GC or Gamma) and IgG levels from 1215 children and adolescents under 18 yo, classified into 5 age groups. Read More

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December 2021

Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia.

J Clin Immunol 2021 11 3;41(8):1878-1892. Epub 2021 Sep 3.

The Royal Hospitals & Queen's University, Belfast, UK.

Patients with ataxia-telangiectasia (A-T) suffer from progressive cerebellar ataxia, immunodeficiency, respiratory failure, and cancer susceptibility. From a clinical point of view, A-T patients with IgA deficiency show more symptoms and may have a poorer prognosis. In this study, we analyzed mortality and immunity data of 659 A-T patients with regard to IgA deficiency collected from the European Society for Immunodeficiencies (ESID) registry and from 66 patients with classical A-T who attended at the Frankfurt Goethe-University between 2012 and 2018. Read More

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November 2021

Minimal-moderate variation of human oral virome and microbiome in IgA deficiency.

Sci Rep 2021 07 21;11(1):14913. Epub 2021 Jul 21.

Department of Physiology, Genetics, and Microbiology, University of Alicante, Alicante, Spain.

Immunoglobulin A (IgA) is the dominant antibody found in our mucosal secretions and has long been recognized to play an important role in protecting our epithelium from pathogens. Recently, IgA has been shown to be involved in gut homeostatic regulation by 'recognizing' and shaping our commensal microbes. Paradoxically, yet selective IgA-deficiency is often described as asymptomatic and there is a paucity of studies only focused on the mice and human gut microbiome context fully ignoring other niches of our body and our commensal viruses. Read More

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Histoplasmosis in Children; HIV/AIDS Not a Major Driver.

J Fungi (Basel) 2021 Jun 30;7(7). Epub 2021 Jun 30.

Faculty of Basic Medical Sciences, Department of Medical Microbiology and Parasitology, College of Medicine, University of Lagos, Lagos 100254, Nigeria.

The classification of histoplasmosis as an AIDS-defining illness has largely attributed its occurrence in people to the presence of HIV/AIDS especially in Africa. Prior to the advent of the HIV/AIDS epidemic, several cases of histoplasmosis were documented both in the pediatric and adult populations. Our review revealed 1461 reported cases of pediatric histoplasmosis globally in the last eight decades (1939-2021). Read More

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The lysophospholipid-binding molecule CD1D is not required for the alloimmunization response to fresh or stored RBCs in mice despite RBC storage driving alterations in lysophospholipids.

Transfusion 2021 07 28;61(7):2169-2178. Epub 2021 Jun 28.

Department of Pathology, University of Virginia, Charlottesville, Virginia, USA.

Background: Despite the significant adverse clinical consequences of RBC alloimmunization, our understanding of the signals that induce immune responses to transfused RBCs remains incomplete. Though RBC storage has been shown to enhance alloimmunization in the hen egg lysozyme, ovalbumin, and human Duffy (HOD) RBC alloantigen mouse model, the molecular signals leading to immune activation in this system remain unclear. Given that the nonclassical major histocompatibility complex (MHC) Class I molecule CD1D can bind to multiple different lysophospholipids and direct immune activation, we hypothesized that storage of RBCs increases lysophospholipids known to bind CD1D, and further that recipient CD1D recognition of these altered lipids mediates storage-induced alloimmunization responses. Read More

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Diagnostic and Management Errors in an Immunodeficient Patient with Pneumococcal Pneumonia with Bacteraemia due to Incorrect Assessment of the Patient's Immune Status.

Eur J Case Rep Intern Med 2021 26;8(4):002557. Epub 2021 Apr 26.

Department of Diagnostic and Generalist Medicine, Dokkyo Medical University Hospital, Mibu, Tochigi, Japan.

A 79-year-old woman presented with fever and pleural chest pain. Based on the assessment of mild community pneumonia in an immunocompetent patient, outpatient follow-up was planned. However, the patient was admitted several hours later with a diagnosis of pneumococcal pneumonia with bacteraemia. Read More

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C-C chemokine hepatocellular carcinoma motif ligand 5-deficiency promotes hepatocellular carcinoma progression by affecting B cell recruitment.

J Dig Dis 2021 Jul 20;22(7):433-441. Epub 2021 Jun 20.

Department of Hepatic Surgery, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.

Objective: To evaluate the expression of C-C motif chemokine ligand 5 (CCL5) in hepatocellular carcinoma (HCC) and to explore its role in regulating the immune microenvironment and the related mechanism in tumor immunity.

Methods: The mRNA expression level of CCL5 in HCC and adjacent non-cancerous tissues was measured by quantitative polymerase chain reaction and the protein expression was examined by immunohistochemistry. Serum CCL5 expression was measured by an enzyme-linked immunosorbent assay (ELISA). Read More

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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 04 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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