2,537 results match your criteria Immunoglobulin M Deficiency


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
2 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
1 Read

Adenosine Deaminase Two and Immunoglobulin M Accurately Differentiate Adult Sneddon's Syndrome of Unknown Cause.

Cerebrovasc Dis 2018 15;46(5-6):257-264. Epub 2019 Jan 15.

Department of Neurology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

Background: The association that exists between livedo reticularis (LR) and stroke is known as Sneddon's syndrome (SnS). The disorder is classified as primary SnS (PSnS), if the cause remains unknown and secondary SnS. The condition is rare and it occurs mainly sporadically. Read More

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http://dx.doi.org/10.1159/000495794DOI Listing
January 2019
3 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
6 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
2 Reads

Renal Amyloidosis in Deficiency of Adenosine Deaminase 2: Successful Experience With Canakinumab.

Pediatrics 2018 Nov;142(5)

Departments of Pediatric Rheumatology.

Deficiency of adenosine deaminase 2 (DADA2) is a rare autoinflammatory disease that was firstly described in patients with early-onset strokes, livedo reticularis, and periodic fever resembling polyarteritis nodosa. In reported case series, researchers described highly variable manifestations, including autoimmunity, immunodeficiency, hepatosplenomegaly, pancytopenia, ichthyosiform rash, and arthritis, in patients with DADA2. A thirteen-year-old female patient who was born to consanguineous parents was admitted to our hospital with generalized edema and leg pain. Read More

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http://pediatrics.aappublications.org/lookup/doi/10.1542/ped
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http://dx.doi.org/10.1542/peds.2018-0948DOI Listing
November 2018
12 Reads

Cochaperone Mzb1 is a key effector of Blimp1 in plasma cell differentiation and β1-integrin function.

Proc Natl Acad Sci U S A 2018 10 26;115(41):E9630-E9639. Epub 2018 Sep 26.

Department of Cellular and Molecular Immunology, Max Planck Institute of Immunobiology and Epigenetics, 79108 Freiburg, Germany;

Plasma cell differentiation involves coordinated changes in gene expression and functional properties of B cells. Here, we study the role of Mzb1, a Grp94 cochaperone that is expressed in marginal zone (MZ) B cells and during the terminal differentiation of B cells to antibody-secreting cells. By analyzing mice, we find that Mzb1 is specifically required for the differentiation and function of antibody-secreting cells in a T cell-independent immune response. Read More

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http://dx.doi.org/10.1073/pnas.1809739115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6187189PMC
October 2018
2 Reads

Anti-ADAMTS13 Autoantibodies against Cryptic Epitopes in Immune-Mediated Thrombotic Thrombocytopenic Purpura.

Thromb Haemost 2018 Oct 20;118(10):1729-1742. Epub 2018 Sep 20.

Laboratory for Thrombosis Research, IRF Life Sciences, KU Leuven Campus Kulak Kortrijk, Kortrijk, Belgium.

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is characterized by severe ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13) deficiency, the presence of anti-ADAMTS13 autoantibodies and an open ADAMTS13 conformation with a cryptic epitope in the spacer domain exposed. A detailed knowledge of anti-ADAMTS13 autoantibodies will help identifying pathogenic antibodies and elucidating the cause of ADAMTS13 deficiency. We aimed at cloning anti-ADAMTS13 autoantibodies from iTTP patients to study their epitopes and inhibitory characteristics. Read More

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http://dx.doi.org/10.1055/s-0038-1669459DOI Listing
October 2018
14 Reads

IgM Myeloma: case report and literature review.

Ann Biol Clin (Paris) 2018 Oct;76(5):575-578

GCS de Saintonge, Centres hospitaliers de Saint-Jean-d'Angély, Jonzac, Saintes et Royan, Saint-Jean-d'Angély, France.

The presence of serum monoclonal IgM is often associated with the diagnosis of Waldenström macroglobulinemia (WM) or other chronic lymphoproliferative disorders. IgM myeloma is a rare entity (0.5%). Read More

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http://dx.doi.org/10.1684/abc.2018.1380DOI Listing
October 2018
3 Reads

CD28 deficiency leads to accumulation of germinal-center independent IgM+ experienced B cells and to production of protective IgM during experimental malaria.

PLoS One 2018 27;13(8):e0202522. Epub 2018 Aug 27.

Departamento de Imunologia, Instituto de Ciências Biomédicas (ICB), Universidade de São Paulo (USP), São Paulo, Brazil.

Protective immunity to blood-stage malaria is attributed to Plasmodium-specific IgG and effector-memory T helper 1 (Th1) cells. However, mice lacking the costimulatory receptor CD28 (CD28KO) maintain chronic parasitemia at low levels and do not succumb to infection, suggesting that other immune responses contribute to parasite control. We report here that CD28KO mice develop long-lasting non-sterile immunity and survive lethal parasite challenge. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0202522PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6110469PMC
February 2019
3 Reads

Chronic Pulmonary Histoplasmosis Identified in a Young Patient with Selective Immunoglobulin M Deficiency.

Case Rep Infect Dis 2018 20;2018:8740204. Epub 2018 May 20.

Lincoln Medical and Mental Health Center, Bronx, NY, USA.

Chronic histoplasmosis is typically diagnosed in patients who are immunocompromised or severely debilitated and who either live in or who have travelled to endemic areas. We report the case of a young, otherwise immunocompetent male patient who presented to a New York hospital with lobar consolidation and was found to have chronic pulmonary histoplasmosis. He described no history of travel to an endemic area. Read More

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http://dx.doi.org/10.1155/2018/8740204DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5985102PMC
May 2018
2 Reads

Immunization with lipopolysaccharide-free outer membrane complexes protects against Acinetobacter baumannii infection.

Vaccine 2018 07 7;36(29):4153-4156. Epub 2018 Jun 7.

Clinical Unit of Infectious Diseases, Clinical Microbiology and Preventive Medicine, Institute of Biomedicine of Seville (IBiS), University Hospital Virgen del Rocío/CSIC/University of Seville, 41013 Seville, Spain. Electronic address:

Outer membrane complex (OMC) vaccines, which contain antigens from the bacterial outer membrane, have been developed for multiple Gram-negative bacteria. However, OMC vaccines demonstrate high endotoxin activity due to the presence of lipopolysaccharide in the bacterial outer membrane, thus precluding their use in humans. We isolated OMCs from an LPS-deficient strain of A. Read More

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http://dx.doi.org/10.1016/j.vaccine.2018.05.113DOI Listing
July 2018
23 Reads

Oxidized phospholipids are proinflammatory and proatherogenic in hypercholesterolaemic mice.

Nature 2018 06 6;558(7709):301-306. Epub 2018 Jun 6.

Department of Medicine, University of California, San Diego, La Jolla, CA, USA.

Oxidized phospholipids (OxPL) are ubiquitous, are formed in many inflammatory tissues, including atherosclerotic lesions, and frequently mediate proinflammatory changes . Because OxPL are mostly the products of non-enzymatic lipid peroxidation, mechanisms to specifically neutralize them are unavailable and their roles in vivo are largely unknown. We previously cloned the IgM natural antibody E06, which binds to the phosphocholine headgroup of OxPL, and blocks the uptake of oxidized low-density lipoprotein (OxLDL) by macrophages and inhibits the proinflammatory properties of OxPL. Read More

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http://www.nature.com/articles/s41586-018-0198-8
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http://dx.doi.org/10.1038/s41586-018-0198-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6033669PMC
June 2018
34 Reads

Hypomorphic mutations alter the preimmune repertoire at early stages of lymphoid development.

Blood 2018 Jul 9;132(3):281-292. Epub 2018 May 9.

Laboratory of Clinical Immunology and Microbiology, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD.

Hypomorphic mutations allowing residual T- and B-cell development have been found in patients presenting with delayed-onset combined immune deficiency with granulomas and/or autoimmunity (CID-G/AI) and abnormalities of the peripheral T- and B-cell repertoire. To examine how hypomorphic mutations affect the earliest stages of lymphocyte development, we used CRISPR/Cas9 to generate mouse models with mutations equivalent to those found in patients with CID-G/AI. Immunological characterization showed partial development of T and B lymphocytes, with persistence of naïve cells and preserved serum immunoglobulin but impaired antibody responses and presence of autoantibodies, thereby recapitulating the phenotype seen in patients with CID-G/AI. Read More

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http://dx.doi.org/10.1182/blood-2017-12-820985DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6053949PMC
July 2018
4 Reads

Selective IgM deficiency associated with adult-onset Still disease.

Ann Allergy Asthma Immunol 2018 Apr;120(4):444-446

Allergy/Immunology Fellowship, University Hospitals, Cleveland Medical Center, Mayfield Heights, Ohio; Allergy/Immunology Associates, Inc, Mayfield Heights, Ohio.

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http://dx.doi.org/10.1016/j.anai.2018.01.001DOI Listing
April 2018
1 Read

B-cell tumor development in -deficient mice.

Blood Adv 2018 03;2(6):703-714

INSERM, Unité Mixte de Recherche (UMR)1170, Institut Gustave Roussy, Villejuif, France.

The gene encodes an α-ketoglutarate-dependent dioxygenase able to oxidize 5-methylcytosine into 5-hydroxymethylcytosine, which is a step toward active DNA demethylation. is frequently mutated in myeloid malignancies but also in B- and T-cell malignancies. somatic mutations are also identified in healthy elderly individuals with clonal hematopoiesis. Read More

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http://dx.doi.org/10.1182/bloodadvances.2017014118DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5873234PMC
March 2018
32 Reads

Interleukin-25 (IL-25) has a protective role in atherosclerosis development in the aortic arch in mice.

J Biol Chem 2018 05 23;293(18):6791-6801. Epub 2018 Mar 23.

From the Department of Clinical Sciences, Skåne University Hospital Malmö, Lund University, 20213 Malmö, Sweden

Atherosclerosis is a chronic inflammatory disease characterized by the entrapment of apolipoprotein B-containing lipoproteins in the arterial intima, leading to local inflammation. T helper (Th) cell 1-mediated immune responses have been associated with atherosclerosis, and the cytokine interleukin-25 (IL-25 or IL-17E) has been reported to potentially regulate Th1 cell- and Th17 cell-related immune responses. In this study, we evaluated the effects of complete IL-25 deficiency or of a temporal IL-25 blockade on atherosclerosis development in apolipoprotein E-deficient () mice. Read More

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http://dx.doi.org/10.1074/jbc.RA117.000292DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5936805PMC
May 2018
5 Reads

Diminished CD40L expression on T-cells in a case of disseminated cryptococcosis.

Indian J Pathol Microbiol 2018 Jan-Mar;61(1):137-140

Department of Transplant Immunology and Immunogenetics, All India Institute of Medical Sciences, New Delhi, India.

X-linked hyperimmunoglobulin M (HIGM) syndrome may increase the susceptibility of patients to disseminated cryptococcal infections primarily due to CD40L deficiency that causes defective cross talk between T- and B-cells, thus preventing class switching. In HIGM syndrome, serum IgM levels are elevated with severe reduction in serum immunoglobulin G (IgG) and IgA levels. In addition, the expression of CD40L (CD154) on in vitro-activated T-cells is severely reduced or absent. Read More

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http://dx.doi.org/10.4103/IJPM.IJPM_761_16DOI Listing
November 2018
2 Reads

Dietary myo-inositol deficiency decreased intestinal immune function related to NF-κB and TOR signaling in the intestine of young grass carp (Ctenopharyngodon idella).

Fish Shellfish Immunol 2018 May 12;76:333-346. Epub 2018 Mar 12.

Animal Nutrition Institute, Sichuan Agricultural University, Chengdu 611130, China; Fish Nutrition and Safety Production University Key Laboratory of Sichuan Province, Sichuan Agricultural University, Chengdu 611130, China; Key Laboratory for Animal Disease-Resistance Nutrition of China Ministry of Education, Sichuan Agricultural University, Chengdu 611130, China. Electronic address:

In this study, we investigated the effects of dietary myo-inositol on the intestinal immune barrier function and related signaling pathway in young grass carp (Ctenopharyngodon idella). A total of 540 young grass carp (221.33 ± 0. Read More

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http://dx.doi.org/10.1016/j.fsi.2018.03.017DOI Listing
May 2018
5 Reads

Secondary antibody deficiency: a complication of anti-CD20 therapy for neuroinflammation.

J Neurol 2018 May 6;265(5):1115-1122. Epub 2018 Mar 6.

The Walton Centre NHS Trust, Liverpool, L97LJ, UK.

B-cell depleting anti-CD20 monoclonal antibody therapies are being increasingly used as long-term maintenance therapy for neuroinflammatory disease compared to many non-neurological diseases where they are used as remission-inducing agents. While hypogammaglobulinaemia is known to occur in over half of patients treated with medium to long-term B-cell-depleting therapy (in our cohort IgG 38, IgM 56 and IgA 18%), the risk of infections it poses seems to be under-recognised. Here, we report five cases of serious infections associated with hypogammaglobulinaemia occurring in patients receiving rituximab for neuromyelitis optica spectrum disorders. Read More

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http://link.springer.com/10.1007/s00415-018-8812-0
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http://dx.doi.org/10.1007/s00415-018-8812-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5937879PMC
May 2018
5 Reads

Effects of dietary lysine restriction on inflammatory responses in piglets.

Sci Rep 2018 02 5;8(1):2451. Epub 2018 Feb 5.

Key Laboratory of Agro-ecological Processes in Subtropical Region, Institute of Subtropical Agriculture, Chinese Academy of Sciences; Scientific Observing and Experimental Station of Animal Nutrition and Feed Science in South-Central, Ministry of Agriculture; Hunan Provincial Engineering Research Center for Healthy Livestock and Poultry Production, Changsha, Hunan, 410125, P.R. China.

The aim of this study was to investigate the effects of lysine restriction on inflammatory responses in piglets. 38 male piglets with similar body weight of 9.62 kg were randomly divided into control group (basal diet) and lysine-restricted group (diet containing 70% lysine of the control diet). Read More

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http://www.nature.com/articles/s41598-018-20689-3
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http://dx.doi.org/10.1038/s41598-018-20689-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5799382PMC
February 2018
15 Reads

Complement factor H protects mice from ischemic acute kidney injury but is not critical for controlling complement activation by glomerular IgM.

Eur J Immunol 2018 05 22;48(5):791-802. Epub 2018 Feb 22.

Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USA.

Natural IgM binds to glomerular epitopes in several progressive kidney diseases. Previous work has shown that IgM also binds within the glomerulus after ischemia/reperfusion (I/R) but does not fully activate the complement system. Factor H is a circulating complement regulatory protein, and congenital or acquired deficiency of factor H is a strong risk factor for several types of kidney disease. Read More

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http://dx.doi.org/10.1002/eji.201747240DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5992014PMC
May 2018
10 Reads

AIM associated with the IgM pentamer: attackers on stand-by at aircraft carrier.

Cell Mol Immunol 2018 Jun 29;15(6):563-574. Epub 2018 Jan 29.

Laboratory of Molecular Biomedicine for Pathogenesis, Center for Disease Biology and Integrative Medicine, Faculty of Medicine, The University of Tokyo, Tokyo, 113-0033, Japan.

Circulating immunoglobulin M (IgM) exists in a pentameric form, possessing a polyreactive nature that responds not only to foreign antigens but also to autoantigens; thus, it is involved in both beneficial and detrimental immune responses, including protection from infection and the progression of autoimmunity. On the other hand, IgM also behaves as a carrier of the apoptosis inhibitor of macrophage (AIM) protein, storing a large amount of the inactivated form of AIM in the blood through this association. Under different disease conditions, AIM can dissociate from IgM locally or systemically to exert its function, inducing the removal of various biological debris such as excess fat, bacteria, cancer cells or dead cell debris. Read More

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http://dx.doi.org/10.1038/cmi.2017.141DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6078943PMC
June 2018
7 Reads

The impaired immune function and structural integrity by dietary iron deficiency or excess in gill of fish after infection with Flavobacterium columnare: Regulation of NF-κB, TOR, JNK, p38MAPK, Nrf2 and MLCK signalling.

Fish Shellfish Immunol 2018 Mar 2;74:593-608. Epub 2018 Feb 2.

Animal Nutrition Institute, Sichuan Agricultural University, Chengdu 611130, China; Fish Nutrition and Safety Production University Key Laboratory of Sichuan Province, Sichuan Agricultural University, Chengdu 611130, China; Key Laboratory for Animal Disease-Resistance Nutrition of China Ministry of Education, Sichuan Agricultural University, Chengdu 611130, China. Electronic address:

The aim of this study was to investigate the effects and potential mechanisms of dietary iron on immune function and structural integrity in gill of young grass carp (Ctenopharyngodon idella). A total of 630 grass carp (242.32 ± 0. Read More

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http://dx.doi.org/10.1016/j.fsi.2018.01.027DOI Listing
March 2018
14 Reads

Immunization with gingipain A hemagglutinin domain of Porphyromonas gingivalis induces IgM antibodies binding to malondialdehyde-acetaldehyde modified low-density lipoprotein.

PLoS One 2018 12;13(1):e0191216. Epub 2018 Jan 12.

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

Treatment of periodontitis has beneficial effects on systemic inflammation markers that relate to progression of atherosclerosis. We aimed to investigate whether immunization with A hemagglutinin domain (Rgp44) of Porphyromonas gingivalis (Pg), a major etiologic agent of periodontitis, would lead to an antibody response cross-reacting with oxidized low-density lipoprotein (OxLDL) and how it would affect the progression of atherosclerosis in low-density lipoprotein receptor-deficient (LDLR-/-) mice. The data revealed a prominent IgM but not IgG response to malondialdehyde-acetaldehyde modified LDL (MAA-LDL) after Rgp44 and Pg immunizations, implying that Rgp44/Pg and MAA adducts may share cross-reactive epitopes that prompt IgM antibody production and consequently confer atheroprotection. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0191216PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5766137PMC
February 2018
23 Reads

Germ Line IgM Is Sufficient, but Not Required, for Antibody-Mediated Alphavirus Clearance from the Central Nervous System.

J Virol 2018 04 14;92(7). Epub 2018 Mar 14.

W. Harry Feinstone Department of Molecular Microbiology and Immunology, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland, USA

Sindbis virus (SINV) infection of neurons in the brain and spinal cord in mice provides a model system for investigating recovery from encephalomyelitis and antibody-mediated clearance of virus from the central nervous system (CNS). To determine the roles of IgM and IgG in recovery, we compared the responses of immunoglobulin-deficient activation-induced adenosine deaminase-deficient (AID), secretory IgM-deficient (sIgM), and AID sIgM double-knockout (DKO) mice with those of wild-type (WT) C57BL/6 mice for disease, clearance of infectious virus and viral RNA from brain and spinal cord, antibody responses, and B cell infiltration into the CNS. Because AID is essential for immunoglobulin class switch recombination and somatic hypermutation, AID mice produce only germ line IgM, while sIgM mice secrete IgG but no IgM and DKO mice produce no secreted immunoglobulin. Read More

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http://dx.doi.org/10.1128/JVI.02081-17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5972861PMC
April 2018
11 Reads

Age-associated B cells expanded in autoimmune mice are memory cells sharing H-CDR3-selected repertoires.

Eur J Immunol 2018 03 15;48(3):509-521. Epub 2018 Jan 15.

Department of Rheumatology and Inflammation Research, University of Gothenburg, Gothenburg, Sweden.

Age-associated B cells (ABCs) represent a distinct cell population expressing low levels of CD21 (CD21 ). The Ig repertoire expressed by ABCs in aged mice is diverse and exhibits signs of somatic hypermutation (SHM). A CD21 B-cell population is expanded in autoimmune diseases, e. Read More

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http://dx.doi.org/10.1002/eji.201747127DOI Listing
March 2018
5 Reads
4.034 Impact Factor

[Clinical therapeutic effect on bronchial asthma in children of different body constitutions treated with plaster and plaster].

Zhongguo Zhen Jiu 2017 Apr;37(4):386-390

Administration Office, Hebei Baoding Children Hospital, Baoding 071000, China.

Objective: To observe the difference in the clinical therapeutic effects on bronchial asthma in children of different body constitutions treated with plaster and plaster.

Methods: One hundred and twenty-two children of bronchial asthma were divided into three groups according to TCM body constitutions, 42 cases in the deficiency constitution group, 40 cases in the deficiency constitution group and 40 cases in the phlegm damp constitution group. From 2011 to 2013, the acupoint plaster was applied to all of the children in the three groups during the dog days and the third nine-day period after the winter solstice each year. Read More

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http://dx.doi.org/10.13703/j.0255-2930.2017.04.009DOI Listing
April 2017
8 Reads

Schnitzler syndrome with IgG gammopathy and elevated IL-1β and IL-17 in skin biopsy.

Ann Allergy Asthma Immunol 2018 Jan 20;120(1):99-101. Epub 2017 Nov 20.

Northwestern University Feinberg School of Medicine, Chicago, Illinois. Electronic address:

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http://dx.doi.org/10.1016/j.anai.2017.10.006DOI Listing
January 2018
10 Reads

The Impact of Human Parvovirus B19 Infection on Heart Failure and Anemia with Reference to Iron Metabolism Markers in an Adult Woman.

Intern Med 2018 Feb 1;57(3):403-407. Epub 2017 Nov 1.

Department of Internal Medicine, Saiyu Soka Hospital, Japan.

A 35-year-old woman with fever, edema and rash was admitted. Pleural effusion and cardiomegaly were observed. A laboratory analysis revealed anemia with iron deficiency and elevated human parvovirus B19 (B19V) immunoglobulin M. Read More

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http://dx.doi.org/10.2169/internalmedicine.8809-17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5827324PMC
February 2018
17 Reads

Truly selective primary IgM deficiency is probably very rare.

Clin Exp Immunol 2018 02 27;191(2):203-211. Epub 2017 Oct 27.

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

Isolated decreased serum-immunoglobulin (Ig)M has been associated with severe and/or recurrent infections, atopy and autoimmunity. However, the reported high prevalence of clinical problems in IgM-deficient patients may reflect the skewed tertiary centre population studied so far. Also, many papers on IgM deficiency have included patients with more abnormalities than simply IgM-deficiency. Read More

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http://dx.doi.org/10.1111/cei.13065DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5758373PMC
February 2018
18 Reads

Effective "activated PI3Kδ syndrome"-targeted therapy with the PI3Kδ inhibitor leniolisib.

Blood 2017 11 29;130(21):2307-2316. Epub 2017 Sep 29.

Novartis Institutes for BioMedical Research, Novartis Pharma AG, Basel, Switzerland; and.

Pathogenic gain-of-function variants in the genes encoding phosphoinositide 3-kinase δ (PI3Kδ) lead to accumulation of transitional B cells and senescent T cells, lymphadenopathy, and immune deficiency (activated PI3Kδ syndrome [APDS]). Knowing the genetic etiology of APDS afforded us the opportunity to explore PI3Kδ inhibition as a precision-medicine therapy. Here, we report in vitro and in vivo effects of inhibiting PI3Kδ in APDS. Read More

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http://dx.doi.org/10.1182/blood-2017-08-801191DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5701526PMC
November 2017
44 Reads

Natural immunoglobulin M initiates an inflammatory response important for both hepatic ischemia reperfusion injury and regeneration in mice.

Hepatology 2017 Sep 7. Epub 2017 Sep 7.

Department of Microbiology and Immunology, Medical University of South Carolina, Charleston, SC.

Complement plays a role in both hepatic ischemia reperfusion (IR) injury (IRI) and liver regeneration, but it is not clear how complement is activated in either process. We investigated the role of self-reactive immunoglobulin M (IgM) antibodies in activating complement after hepatic IR and liver resection. Natural IgM antibodies that recognize danger-associated molecular patterns (neoepitopes) activate complement following both hepatic IR and liver resection. Read More

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http://dx.doi.org/10.1002/hep.29512DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5842100PMC
September 2017
11 Reads

Threonine deficiency decreased intestinal immunity and aggravated inflammation associated with NF-κB and target of rapamycin signalling pathways in juvenile grass carp (Ctenopharyngodon idella) after infection with Aeromonas hydrophila.

Br J Nutr 2017 Jul;118(2):92-108

1Animal Nutrition Institute,Sichuan Agricultural University,Chengdu 611130,People's Republic of China.

This study aimed to investigate the impacts of dietary threonine on intestinal immunity and inflammation in juvenile grass carp. Six iso-nitrogenous semi-purified diets containing graded levels of threonine (3·99-21·66 g threonine/kg) were formulated and fed to fishes for 8 weeks, and then challenged with Aeromonas hydrophila for 14 d. Results showed that, compared with optimum threonine supplementation, threonine deficiency (1) decreased the ability of fish against enteritis, intestinal lysozyme activities (except in the distal intestine), acid phosphatase activities, complement 3 (C3) and C4 contents and IgM contents (except in the proximal intestine (PI)), and it down-regulated the transcript abundances of liver-expressed antimicrobial peptide (LEAP)-2A, LEAP-2B, hepcidin, IgZ, IgM and β-defensin1 (except in the PI) (P<0·05); (2) could up-regulate intestinal pro-inflammatory cytokines TNF-α, IL-1β, IL-6, IL-8 and IL-17D mRNA levels partly related to NF-κB signalling; (3) could down-regulate intestinal anti-inflammatory cytokine transforming growth factor (TGF)-β1, TGF-β2, IL-4/13A (not IL-4/13B) and IL-10 mRNA levels partly by target of rapamycin signalling. Read More

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http://dx.doi.org/10.1017/S0007114517001830DOI Listing
July 2017
23 Reads

Altered marginal zone and innate-like B cells in aged senescence-accelerated SAMP8 mice with defective IgG1 responses.

Cell Death Dis 2017 08 17;8(8):e3000. Epub 2017 Aug 17.

Centro Nacional de Microbiología, Instituto de Salud Carlos III (ISCIII), Majadahonda, Madrid, Spain.

Aging has a strong impact on the activity of the immune system, enhancing susceptibility to pathogens and provoking a predominant pre-inflammatory status, whereas dampening responses to vaccines in humans and mice. Here, we demonstrate a loss of marginal zone B lymphocytes (MZ, CD19CD45RCD21CD23) and a decrease of naive B cells (CD19IgD), whereas there is an enhancement of a CD19CD45R innate-like B cell population (B1REL) and the so-called aged B cell compartment (ABC, CD45RCD21CD23CD5CD11b) in aged senescence-accelerated (SAMP8) mice but not in aged senescence-resistant (SAMR1) mice. These changes in aged SAMP8 mice were associated with lower IgG isotype levels, displaying low variable gene usage repertoires of the immunoglobulin heavy chain (V) diversity, with a diminution on IgG1-memory B cells (CD11bGr1CD138IgMIgDCD19CD38IgG1), an increase in T follicular helper (T, CD4CXCR5PD1) cell numbers, and an altered MOMA-1 (metallophilic macrophages) band in primary follicles. Read More

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http://dx.doi.org/10.1038/cddis.2017.351DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5596542PMC
August 2017
28 Reads

Serum carnitine and acyl-carnitine in patients with meningitis due to tick-borne encephalitis virus infection.

Adv Clin Exp Med 2017 Mar-Apr;26(2):277-280

Department of Infectious Diseases and Neuroinfections, Medical University of Bialystok, Poland.

Background: Hard ticks are the main vectors of tick-borne encephalitis virus (TBEV). Free carnitine (FC) and acylcarnitines (AC) have the basic role in β-oxidation as well as the modulation of immune and nervous system. Homeostasis of carnitines in the TBE patients was not studied so far. Read More

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http://dx.doi.org/10.17219/acem/63006DOI Listing
September 2017
20 Reads

Brucella Causing Liver Abscess in a Child with Selective IgA Deficiency.

Indian Pediatr 2017 Jul;54(7):595-596

Department of Pediatric Medicine, Institute of Child Health, Kolkata, West Bengal, India. Correspondence to: Dr Devdeep Mukherjee, Uttara Cooperative Housing Society, 13, Broad Street, Kolkata 700 019, West Bengal, India.

Background: Brucella has been known to cause pyrexia of unknown origin.

Case Characteristics: 9-year-old boy with fever and abdominal pain; multiple abscesses within the liver on ultrasonography.

Observations: IgM Antibodies against Brucella were raised in his serum sample, and Brucella serum agglutination test was positive. Read More

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July 2017
9 Reads

Selective IgM Deficiency: Clinical and Laboratory Features of 17 Patients and a Review of the Literature.

J Clin Immunol 2017 Aug 21;37(6):559-574. Epub 2017 Jul 21.

Department of Clinical Immunology and Allergy, St. Anne's University Hospital in Brno, Pekarska 53, 65691, Brno, Czech Republic.

Purpose: Primary selective IgM deficiency (sIgMD) is a primary immunodeficiency with unclear pathogenesis and a low number of published cases.

Methods: We reviewed clinical and laboratory manifestations of 17 sIgMD patients. Serum IgM, IgG, and its subclasses, IgA, IgE, antibodies against tetanus toxoid, pneumococcal polysaccharides and Haemophilus influenzae type b, isohemagglutinins, and T and B lymphocyte subsets, expressions of IgM on B cells and B lymphocyte production of IgM were compared with previously reported case reports and a small series of patients, which included 81 subjects in total. Read More

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http://dx.doi.org/10.1007/s10875-017-0420-8DOI Listing
August 2017
9 Reads

Anti-Neu5Gc and anti-non-Neu5Gc antibodies in healthy humans.

PLoS One 2017 17;12(7):e0180768. Epub 2017 Jul 17.

Thomas E. Starzl Transplantation Institute, University of Pittsburgh, Pittsburgh, PA, United States of America.

Our group previously investigated the levels of anti-Gal and anti-nonGal IgM and IgG in a cohort of 75 healthy humans of various backgrounds, and found some significant differences related to factors such as age, gender, ABO blood group, diet, vaccination history, and geographic location during childhood. We have now expanded our cohort (n = 84) to investigate the levels of anti-Neu5Gc and anti-nonGal/nonNeu5Gc antibodies in healthy humans. Anti-nonGal and anti-nonGal/nonNeu5Gc human IgM and IgG binding to pRBCs and pAECs from GTKO/CD46 and GTKO/CD46/Neu5GcKO pigs were measured by flow cytometry. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0180768PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5513429PMC
September 2017
10 Reads
4 Citations
3.234 Impact Factor

The role of human natural killer-1 (HNK-1) carbohydrate in neuronal plasticity and disease.

Biochim Biophys Acta Gen Subj 2017 Oct 12;1861(10):2455-2461. Epub 2017 Jul 12.

Department of Biological Chemistry, Human Health Sciences, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan. Electronic address:

Background: The human natural killer-1 (HNK-1) carbohydrate, a unique trisaccharide possessing sulfated glucuronic acid in a non-reducing terminus (HSO-3GlcAß1-3Galß1-4GlcNAc-), is highly expressed in the nervous system and its spatiotemporal expression is strictly regulated. Mice deficient in the gene encoding a key enzyme, GlcAT-P, of the HNK-1 biosynthetic pathway exhibit almost complete disappearance of the HNK-1 epitope in the brain, significant reduction of long-term potentiation, and aberration of spatial learning and memory formation. In addition to its physiological roles in higher brain function, the HNK-1 carbohydrate has attracted considerable attention as an autoantigen associated with peripheral demyelinative neuropathy, which relates to IgM paraproteinemia, because of high immunogenicity. Read More

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http://dx.doi.org/10.1016/j.bbagen.2017.06.025DOI Listing
October 2017
8 Reads

[Clinical and immunological profile of 15 Moroccan patients with Hyper IgM syndrome].

Pan Afr Med J 2017 19;26:212. Epub 2017 Apr 19.

Laboratoire d'Immunologie Clinique, Inflammation et Allergie, Faculté de Médecine et Pharmacie, Université Hassan II, Casablanca, Maroc.

Hyper IgM syndrome is a well known genetic (primary) immunodeficiency disorder which was first described in 1961. It is caused by B lymphocyte deficiency characterized by normal or elevated serum IgM levels and low or zero levels of IgG, IgA, IgE resulting from isotype-switching deficiency. Clinical manifestations are dominated by recurrent infections, especially involving the digestive tube of the ENT sphere and the lungs. Read More

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http://dx.doi.org/10.11604/pamj.2017.26.212.10081DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5491719PMC
July 2017
7 Reads

Report of an unsual case of anophthalmia and craniofacial cleft in a newborn with Toxoplasma gondii congenital infection.

BMC Infect Dis 2017 07 3;17(1):459. Epub 2017 Jul 3.

Laboratory Inmunología Experimental, Instituto Nacional de Pediatría (INP), Secretaría de Salud, Torre de Investigación, Av. Insurgentes Sur 3700-C, Col Insurgentes Cuicuilco, 04530, Ciudad de México, DF, Mexico.

Background: We present one unusual case of anophthalmia and craniofacial cleft, probably due to congenital toxoplasmosis only.

Case Presentation: A two-month-old male had a twin in utero who disappeared between the 7 and the 14 week of gestation. At birth, the baby presented anophthalmia and craniofacial cleft, and no sign compatible with genetic or exposition/deficiency problems, like the Wolf-Hirschhorn syndrome or maternal vitamin A deficiency. Read More

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http://bmcinfectdis.biomedcentral.com/articles/10.1186/s1287
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http://dx.doi.org/10.1186/s12879-017-2565-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5494800PMC
July 2017
12 Reads

CD36 and Platelet-Activating Factor Receptor Promote House Dust Mite Allergy Development.

J Immunol 2017 08 30;199(3):1184-1195. Epub 2017 Jun 30.

Department of Microbiology, The University of Alabama at Birmingham, Birmingham, AL 35294

Over 89% of asthmatic children in underdeveloped countries demonstrate sensitivity to house dust mites (HDMs). The allergic response to HDMs is partially mediated by epithelial cell-derived cytokines that activate group 2 innate lymphoid cells, induce migration and activation of dendritic cells, and promote effector differentiation of HDM-specific TH2 cells. However, the contribution of innate receptor engagement on epithelial or dendritic cells by HDMs that ultimately mediates said innate and adaptive allergic responses is poorly understood. Read More

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http://www.jimmunol.org/lookup/doi/10.4049/jimmunol.1700034
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http://dx.doi.org/10.4049/jimmunol.1700034DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5538133PMC
August 2017
7 Reads

Immunological phenotype of the murine Lrba knockout.

Immunol Cell Biol 2017 10 25;95(9):789-802. Epub 2017 Jul 25.

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

Biallelic mutations in the human lipopolysaccharide responsive beige-like anchor (LRBA) gene lead to a primary immunodeficiency known as LRBA deficiency, characterized by a broad range of clinical manifestations including autoimmunity, organomegaly, hypogammaglobulinemia and recurrent infections. Considering the phenotypic heterogeneity in patients and the severity of the disease, our aim was to assess the role of LRBA in immune cells and to understand the underlying pathomechanisms through the study of a Lrba knockout (Lrba) mouse model. LRBA-deficient mice did not show severe clinical or immunological signs of disease, either at steady state under specific-pathogen-free conditions, after vaccination with T-dependent and T-independent antigens, or in the context of acute infections with lymphocytic choriomeningitis virus (LCMV) or Salmonella Typhimurium. Read More

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http://dx.doi.org/10.1038/icb.2017.52DOI Listing
October 2017
41 Reads

Concentrations of Pneumococcal IgA and IgM are compromised in some individuals with antibody deficiencies.

J Immunoassay Immunochem 2017 15;38(5):505-513. Epub 2017 Jun 15.

e The Binding Site Group Limited , Birmingham , UK.

The response to pneumococcal vaccination is assessed by measurement of antigen specific IgG only and is compromised in a number of antibody deficiencies. We measured the concentrations of Pneumococcal IgA and IgM in individuals with both normal and abnormal pneumococcal capsular polysaccharide (PCP) IgG concentrations. A higher number of individuals had abnormal pre-vaccination IgA and IgM concentrations below the lower limit of the normal range compared to the control group. Read More

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http://dx.doi.org/10.1080/15321819.2017.1337641DOI Listing
January 2018
69 Reads

A case report of lymphoid intestitial pneumonia in common variable immunodeficiency: Oligoclonal expansion of effector lymphocytes with preferential cytomegalovirus-specific immune response and lymphoproliferative disease promotion.

Medicine (Baltimore) 2017 Jun;96(23):e7031

aDepartment of Clinical Immunology, Lower Silesian Center for Cellular Transplantation bL Hirszfeld Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Wroclaw cDepartment of Immunology, Poznan University of Medical Sciences, Poznań, Poland.

Rationale: Lymphoid interstitial pneumonia (LIP) is a rare disease with lymphocytic infiltration of the alveolar interstitial and air spaces, sometimes classified as a clonal lymphoproliferative disease (LPD) with high prevalence in patients with immunodysregulation. Although association of mucosa-associated lymphoid tissue (MALT) lymphoma development with infectious agents has been well described, it is not so in the case of LIP. Attempts to demonstrate an infective cause by direct microbe detection have failed, but association with atypical specific immune response to opportunistic infectious agent has not been studied. Read More

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http://dx.doi.org/10.1097/MD.0000000000007031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5466213PMC
June 2017
10 Reads

Humoral Immune Reconstitution Kinetics after Allogeneic Hematopoietic Stem Cell Transplantation in Children: A Maturation Block of IgM Memory B Cells May Lead to Impaired Antibody Immune Reconstitution.

Biol Blood Marrow Transplant 2017 Sep 8;23(9):1437-1446. Epub 2017 May 8.

Division of Hematology, Oncology, and Blood and Marrow Transplantation, Children's Hospital Los Angeles, Los Angeles, California; University of Southern California Keck School of Medicine, Los Angeles, California.

Although T cell immune reconstitution after allogeneic hematopoietic stem cell transplantation (allo-HSCT) has been well studied, long-term B cell immune reconstitution remains less characterized. We evaluated humoral immune reconstitution among 71 pediatric allo-HSCT recipients. Although tetanus toxoid antibody levels were normal at 1 year after allo-HSCT, antipolysaccharide carbohydrate antibodies remained persistently low for up to 5 years. Read More

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http://dx.doi.org/10.1016/j.bbmt.2017.05.005DOI Listing
September 2017
17 Reads

The decreased growth performance and impaired immune function and structural integrity by dietary iron deficiency or excess are associated with TOR, NF-κB, p38MAPK, Nrf2 and MLCK signaling in head kidney, spleen and skin of grass carp (Ctenopharyngodon idella).

Fish Shellfish Immunol 2017 Jun 18;65:145-168. Epub 2017 Apr 18.

Animal Nutrition Institute, Sichuan Agricultural University, Chengdu 611130, China; Fish Nutrition and Safety Production University Key Laboratory of Sichuan Province, Sichuan Agricultural University, Chengdu 611130, China; Key Laboratory for Animal Disease-Resistance Nutrition of China Ministry of Education, Sichuan Agricultural University, Chengdu 611130, China. Electronic address:

This study was conducted to investigate the effects of dietary iron on the growth, and immune function and structural integrity in head kidney, spleen and skin as well as the underlying signaling of young grass carp (Ctenopharyngodon idella). Total 630 grass carp (242.32 ± 0. Read More

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http://dx.doi.org/10.1016/j.fsi.2017.04.009DOI Listing
June 2017
19 Reads

First Case of X-Linked Moesin Deficiency Identified After Newborn Screening for SCID.

J Clin Immunol 2017 05 4;37(4):336-338. Epub 2017 Apr 4.

Immune Deficiency Genetics Section, Laboratory of Host Defenses, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Building 10 CRC, Room 5-3950, 10 Center Drive, MSC 1456, Bethesda, MD, 20892, USA.

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http://dx.doi.org/10.1007/s10875-017-0391-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6082367PMC
May 2017
4 Reads

Changes in the Level of Immunoglobulins and CD4/CD8 Ratio in Young and Aged Mice with Estradiol Deficiency.

Immunol Invest 2017 Apr 7;46(3):305-313. Epub 2017 Feb 7.

a Department of Obstetrics and Gynecology , Jinhua Municipal Central Hospital , Zhejiang Province , People's Republic of China.

Studies demonstrated that deficiency in 17β-estradiol (E) in postmenopausal women influences their immune system. However, few studies have reported alterations in immunologic presentation during nonnatural menopause in young females. Here we compared the differences in immune response between young C57BL/6N mice with surgical or medical variectomy and aged C57BL/6N mice with the common feature of E deficiency following Con A stimulation. Read More

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http://dx.doi.org/10.1080/08820139.2016.1267203DOI Listing
April 2017
15 Reads