2,589 results match your criteria Immunoglobulin M Deficiency


New S19 Mutant to Improve Distinction Between Infected and Vaccinated Animals.

Iran J Biotechnol 2019 Sep 1;17(3):e2159. Epub 2019 Sep 1.

Department of Microbiology, Faculty of Veterinary Medicine, University of Tehran, Tehran, Iran.

Background: Using Strain 19 (S19) to control bovine brucellosis is restricted due to induce antibodies to the O-side chain of the smooth lipopolysaccharide (LPS) which may be difficult to differentiate vaccinated and infected animals. Furthermore, it is virulent for humans and can induce abortion to cattle.

Objectives: The aim of this study was to employ gene knockout S19 for the first time to eliminate diagnostic defects and obtain the attenuated mutant strain. Read More

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http://dx.doi.org/10.29252/ijb.2159DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7080968PMC
September 2019

The epidemiology and clinical features of selective immunoglobulin M deficiency: A single-center study in China.

J Clin Lab Anal 2020 Mar 10:e23289. Epub 2020 Mar 10.

Department of Clinical Laboratory, Zhejiang Provincial People's Hospital, Hangzhou Medical College, Hangzhou, China.

Background: Selective immunoglobulin M deficiency (SIgMD) is a rare primary immunodeficiency that is frequently reported in Western countries. However, large epidemiological and clinical studies of SIgMD in China are still lacking. Herein, we describe a cohort of SIgMD subjects in a large tertiary university hospital in China. Read More

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http://dx.doi.org/10.1002/jcla.23289DOI Listing
March 2020
1.144 Impact Factor

Lack of Gut Secretory Immunoglobulin A in Memory B-Cell Dysfunction-Associated Disorders: A Possible Gut-Spleen Axis.

Front Immunol 2019 8;10:2937. Epub 2020 Jan 8.

Department of Molecular Medicine, Sapienza University, Rome, Italy.

B-1a B cells and gut secretory IgA (SIgA) are absent in asplenic mice. Human immunoglobulin M (IgM) memory B cells, which are functionally equivalent to mouse B-1a B cells, are reduced after splenectomy. To demonstrate whether IgM memory B cells are necessary for generating IgA-secreting plasma cells in the human gut. Read More

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http://dx.doi.org/10.3389/fimmu.2019.02937DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6960143PMC
January 2020

Post-rituximab immunoglobulin M (IgM) hypogammaglobulinemia.

Autoimmun Rev 2020 Mar 7;19(3):102466. Epub 2020 Jan 7.

Department of Dermatology, Tufts University School of Medicine, The Center for Blistering Diseases, Boston, MA, USA. Electronic address:

Rituximab is a B cell depleting monoclonal antibody that targets the B cell-specific cell surface antigen CD20 and is currently used to treat several autoimmune diseases. The elimination of mature CD20-positive B lymphocytes committed to differentiate into autoantibody-producing plasma cells is considered to be the major effect of rituximab, that makes it a beneficial biological agent in treating autoimmune diseases. Hypogammaglobulinemia has been reported after rituximab therapy in patients with lymphoma and rheumatoid arthritis. Read More

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http://dx.doi.org/10.1016/j.autrev.2020.102466DOI Listing

Associations of Interleukin-5 With Plaque Development and Cardiovascular Events.

JACC Basic Transl Sci 2019 Dec 6;4(8):891-902. Epub 2019 Nov 6.

Department of Clinical Sciences Malmö, Lund University, Lund, Sweden.

Experimental studies have suggested an atheroprotective role of interleukin (IL)-5 through the stimulation of natural immunoglobulin M antibody expression. In the present study we show that there are no associations between baseline levels of IL-5 and risk for development of coronary events or stroke during a 15.7 ± 6. Read More

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http://dx.doi.org/10.1016/j.jacbts.2019.07.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6939009PMC
December 2019

Dietary biotin deficiency decreased growth performance and impaired the immune function of the head kidney, spleen and skin in on-growing grass carp (Ctenopharyngodon idella).

Fish Shellfish Immunol 2020 Feb 17;97:216-234. Epub 2019 Dec 17.

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

The aim of this study was to investigate the effects of dietary biotin deficiency on the growth performance and immune function of the head kidney, spleen and skin in on-growing grass carp (Ctenopharyngodon idella). A total of 540 on-growing grass carp (117.11 ± 0. Read More

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http://dx.doi.org/10.1016/j.fsi.2019.12.033DOI Listing
February 2020

Rare TACI Mutation in a 3-Year-Old Boy With CVID Phenotype.

Front Pediatr 2019 15;7:418. Epub 2019 Oct 15.

Division of Pediatric Immunology and Rheumatology, Department of Pediatrics, Sapienza University of Rome, Rome, Italy.

Common variable immunodeficiency (CVID) is the most common and clinically relevant primary immunodeficiency (PID). Genetic basis of CVID remains largely unknown. However, in a minority of CVID patients, a number of distinct genetic defects affecting the normal processes of B cell maturation and differentiation into memory B cells have now been identified, resulting in markedly reduced serum levels of immunoglobulin G (IgG) and low immunoglobulin A (IgA) or immunoglobulin M (IgM), with impaired antibody responses, despite the presence of normal levels of B cells. Read More

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http://dx.doi.org/10.3389/fped.2019.00418DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6803509PMC
October 2019

Impairment of PD-L2 positive B1a cells enhances susceptibility to sepsis in RasGRP1-deficient mice.

Cell Immunol 2019 12 16;346:103993. Epub 2019 Oct 16.

Center for Immunobiology, Western Michigan University Homer Stryker M.D. School of Medicine, Kalamazoo, MI, USA; Department of Biomedical Sciences, Western Michigan University Homer Stryker M.D. School of Medicine, Kalamazoo, MI, USA.

RasGRP1 is a key molecule that mediates antigen-initiated signaling for activation of the RAS-MAPK pathway in lymphocytes. Patients with aberrant RasGRP1 expression experience lymphocyte dysfunction and are afflicted with recurrent microbial infections. Yet, the underlying mechanism that accounts for microbial infection remains unknown. Read More

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http://dx.doi.org/10.1016/j.cellimm.2019.103993DOI Listing
December 2019

[Complicated viral to cholestatic hepatitis in a child with glucose-6-phosphate dehydrogenase deficiency from a high andean region].

Rev Peru Med Exp Salud Publica 2019 Apr-Jun;36(2):366-368. Epub 2019 Aug 22.

Servicio de Pediatría del Hospital II Huamanga de ESSALUD. Huamanga, Ayacucho.

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http://dx.doi.org/10.17843/rpmesp.2019.362.4075DOI Listing

Patient characteristics and analgesic efficacy of antiviral therapy in postherpetic neuralgia.

Med Hypotheses 2019 Oct 22;131:109323. Epub 2019 Jul 22.

Department of Anesthesiology, Chi Mei Medical Center, Tainan, Taiwan; Department of the Senior Citizen Service Management, Chia Nan University of Pharmacy and Science, Tainan, Taiwan. Electronic address:

Postherpetic neuralgia (PHN) is the most common complication of shingles caused by reactivation of varicella zoster virus (VZV). Management of PHN is often suboptimal while using current conventional treatments. Antiviral therapy was used to reduce PHN-associated pain in two small trials which showed conflicting results. Read More

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http://dx.doi.org/10.1016/j.mehy.2019.109323DOI Listing
October 2019
2 Reads

Dietary iron deficiency impaired intestinal immune function of on-growing grass carp under the infection of Aeromonas hydrophila: Regulation of NF-κB and TOR signaling.

Fish Shellfish Immunol 2019 Oct 10;93:669-682. Epub 2019 Aug 10.

Animal Nutrition Institute, Sichuan Agricultural University, Chengdu, 6111.0930, China; Fish Nutrition and Safety Production University Key Laboratory of Sichuan Province, Sichuan Agricultural University, Chengdu, 6111.0930, China; Key Laboratory of Animal Disease-resistant Nutrition, Sichuan Province, China. Electronic address:

Iron is an important mineral element for fish. In this study, we investigated the influences of dietary iron deficiency on intestinal immune function as well as underlying signaling of on-growing grass carp (Ctenopharyngodon idella). Fish were fed with six graded level of dietary iron for sixty days, and a fourteen days' challenge test under infection of Aeromonas hydrophila thereafter. Read More

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http://dx.doi.org/10.1016/j.fsi.2019.08.021DOI Listing
October 2019

Clinical, genetic and immunological characteristics of 40 Chinese patients with CD40 ligand deficiency.

Scand J Immunol 2019 Oct 21;90(4):e12798. Epub 2019 Jul 21.

Ministry of Education Key Laboratory of Child Development and Disorders, Children's Hospital of Chongqing Medical University, Chongqing, China.

CD40 ligand (CD40L) deficiency is a rare but life-threatening primary immunodeficiency caused by mutations in the CD40L gene. Here, we investigated a cohort of 40 genetically diagnosed CD40L-deficient patients from the Chinese mainland, analysed their clinical and genetic data, and examined CD40L expression, the proportion of T cell subsets, B cell subsets and T follicular helper (Tfh) cells. The aim was to provide a complete picture of CD40L deficiency. Read More

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http://dx.doi.org/10.1111/sji.12798DOI Listing
October 2019
7 Reads

Topical application of nebulized human IgG, IgA and IgAM in the lungs of rats and non-human primates.

Respir Res 2019 May 22;20(1):99. Epub 2019 May 22.

CSL Behring AG, Research, Bern, Switzerland.

Background: Recurrent and persistent infections are known to affect airways of patients with Primary Immunodeficiency despite appropriate replacement immunoglobulin serum levels. Interestingly, patients with Chronic Obstructive Pulmonary Disease or with non-CF bronchiectasis also show similar susceptibility to such infections. This may be due to the limited availability of immunoglobulins from the systemic circulation in the conductive airways, resulting in local immunodeficiency. Read More

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http://dx.doi.org/10.1186/s12931-019-1057-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6532128PMC
May 2019
11 Reads

Defining Primary Selective IgM Deficiency.

J Clin Immunol 2019 05 9;39(4):350-352. Epub 2019 May 9.

Hoag Medical Group, Hoag Hospital, Newport Beach, CA, USA.

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http://dx.doi.org/10.1007/s10875-019-00641-4DOI Listing
May 2019
1 Read

Clinical and immunological features in a cohort of patients with partial DiGeorge syndrome followed at a single center.

Blood 2019 06 23;133(24):2586-2596. Epub 2019 Apr 23.

Department of Paediatric Immunology.

DiGeorge syndrome (DGS) is a primary immunodeficiency characterized by various degrees of T-cell deficiency. In partial DGS (pDGS), other risk factors could predispose to recurrent infections, autoimmunity, and allergy. The aim of this study was to assess the effect of different factors in the development of infections, autoimmunity, and/or allergy in patients with pDGS. Read More

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http://dx.doi.org/10.1182/blood.2018885244DOI Listing
June 2019
12 Reads
10.452 Impact Factor

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

Intern Med 2019 Aug 17;58(15):2185-2190. Epub 2019 Apr 17.

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

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

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http://dx.doi.org/10.2169/internalmedicine.2607-18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6709322PMC
August 2019
6 Reads

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

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

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

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

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

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

Challenges in investigating patients with isolated decreased serum IgM: The SIMcal study.

Scand J Immunol 2019 Jun 25;89(6):e12763. Epub 2019 Apr 25.

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

The clinical consequences of isolated decreased serum immunoglobulin (Ig)M are not sufficiently known. Therefore, it is difficult to determine the clinical policy following such a finding. Only few reported IgM-deficient patients fulfil the European Society for Immunodeficiencies (ESID) diagnostic criteria for selective IgM deficiency (true sIgMdef), or their diagnosis is uncertain due to insufficient laboratory data (possible sIgMdef). Read More

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http://dx.doi.org/10.1111/sji.12763DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6850338PMC
June 2019
8 Reads
1.882 Impact Factor

BAFF-driven B cell hyperplasia underlies lung disease in common variable immunodeficiency.

JCI Insight 2019 03 7;4(5). Epub 2019 Mar 7.

Division of Clinical Immunology, Department of Medicine.

Background: Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency and is frequently complicated by interstitial lung disease (ILD) for which etiology is unknown and therapy inadequate.

Methods: Medical record review implicated B cell dysregulation in CVID ILD progression. This was further studied in blood and lung samples using culture, cytometry, ELISA, and histology. Read More

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https://insight.jci.org/articles/view/122728
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http://dx.doi.org/10.1172/jci.insight.122728DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6483510PMC
March 2019
12 Reads

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

Scand J Immunol 2019 Jun 18;89(6):e12759. Epub 2019 Mar 18.

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

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

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http://dx.doi.org/10.1111/sji.12759DOI Listing
June 2019
11 Reads

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

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

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

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

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

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

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http://dx.doi.org/10.1097/ICB.0000000000000854DOI Listing
January 2019
13 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
14 Reads

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
July 2019
14 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
November 2019
43 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
28 Reads

Report of a Chinese Cohort with Activated Phosphoinositide 3-Kinase δ Syndrome.

J Clin Immunol 2018 11 29;38(8):854-863. Epub 2018 Nov 29.

Department of Clinical Immunology, Children's Hospital of Fudan University, 399 Wanyuan Road, Shanghai, 201102, China.

Purpose: We aimed to report the clinical manifestations and immunological features of activated phosphatidylinositol 3-kinase δ syndrome 1 (APDS1) in a Chinese cohort. Moreover, we investigated the efficacy and safety of rapamycin therapy for Chinese patients with APDS1.

Methods: Fifteen Chinese patients with APDS1 from 14 unrelated families were enrolled in this study. Read More

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http://dx.doi.org/10.1007/s10875-018-0568-xDOI Listing
November 2018
8 Reads

Decreased nematode clearance and anti-phosphorylcholine-specific IgM responses in mannose-binding lectin-deficient mice.

Immunol Cell Biol 2019 03 6;97(3):305-316. Epub 2019 Jan 6.

Department of Comparative Biomedical Sciences, The Royal Veterinary College, Royal College Street, London, NW1 0TU, UK.

Brugia malayi is a nematode that causes human lymphatic filariasis. Previously, we showed that mannose-binding lectin (MBL)-A is necessary for clearance of B. malayi microfilariae in mice and presence of MBL-A is linked with maximal levels of parasite-specific IgM. Read More

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http://dx.doi.org/10.1111/imcb.12219DOI Listing
March 2019
4 Reads

Immune/microbial interface perturbation in human IgA deficiency.

Gut Microbes 2019 18;10(3):429-433. Epub 2018 Nov 18.

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

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

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https://www.tandfonline.com/doi/full/10.1080/19490976.2018.1
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http://dx.doi.org/10.1080/19490976.2018.1546520DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6546332PMC
December 2019
27 Reads

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

Pediatrics 2018 11;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
25 Reads

Mild Hypogammaglobulinemia Can Be a Serious Condition.

Front Immunol 2018 15;9:2384. Epub 2018 Oct 15.

Department of Tranzo, Tilburg University, Tilburg, Netherlands.

Most patients with primary antibody deficiency (PAD) suffer from less well-described and understood forms of hypogammaglobulinemia (unclassified primary antibody deficiency, unPAD). Because of the moderately decreased immunoglobulin levels compared to CVID, unPAD is generally considered to be clinically mild and not very relevant. To describe our cohort of-mainly-unPAD patients, and to analyze whether subgroups can be identified. Read More

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https://www.frontiersin.org/article/10.3389/fimmu.2018.02384
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http://dx.doi.org/10.3389/fimmu.2018.02384DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6196282PMC
September 2019
57 Reads

Enhanced Antibody Production in Clever-1/Stabilin-1-Deficient Mice.

Front Immunol 2018 8;9:2257. Epub 2018 Oct 8.

MediCity Research Laboratory, University of Turku, Turku, Finland.

Clever-1, encoded by the gene, is a scavenger and leukocyte trafficking receptor expressed by subsets of vascular and lymphatic endothelial cells and immunosuppressive macrophages. Monocyte Clever-1 also modulates T cell activation. However, nothing is known about the possible links between B cell function and Clever-1. Read More

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https://www.frontiersin.org/article/10.3389/fimmu.2018.02257
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http://dx.doi.org/10.3389/fimmu.2018.02257DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6187969PMC
September 2019
12 Reads

The circulating immunoglobulins negatively impact on the parasite clearance in the liver of Leishmania donovani-infected mice via dampening ROS activity.

Biochem Biophys Res Commun 2018 11 15;506(1):20-26. Epub 2018 Oct 15.

Department of Parasitology and Infectious Diseases, Gifu University Graduate School of Medicine, Gifu, Japan; Domain of Integrated Life Systems, Center for Highly Advanced Integration of Nano and Life Sciences (G-CHAIN), Gifu University, Gifu, Japan. Electronic address:

Visceral leishmaniasis, the most severe form of leishmaniasis, is caused by Leishmania donovani and L. infantum. Immunity to Leishmania infection has been shown to depend on the development of Th1 cells; however, the roles of B cells and antibodies during infection remain unclear. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S0006291X183220
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http://dx.doi.org/10.1016/j.bbrc.2018.10.055DOI Listing
November 2018
47 Reads

Growth Hormone (GH) Deficient Mice With GHRH Gene Ablation Are Severely Deficient in Vaccine and Immune Responses Against .

Front Immunol 2018 2;9:2175. Epub 2018 Oct 2.

GIGA-I3 Center of Immunoendocrinology, University of Liège, Liège, Belgium.

The precise impact of the somatotrope axis upon the immune system is still highly debated. We have previously shown that mice with generalized ablation of growth hormone (GH) releasing hormone (GHRH) gene () have normal thymus and T-cell development, but present a marked spleen atrophy and B-cell lymphopenia. Therefore, in this paper we have investigated vaccinal and anti-infectious responses of mice against , a pathogen carrying T-independent antigens. Read More

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http://dx.doi.org/10.3389/fimmu.2018.02175DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6176084PMC
September 2019
33 Reads

Endogenous IL-10 maintains immune tolerance but IL-10 gene transfer exacerbates autoimmune cholangitis.

J Autoimmun 2018 12 28;95:159-170. Epub 2018 Sep 28.

Department of Clinical Laboratory Sciences and Medical Biotechnology, College of Medicine, National Taiwan University, Taipei, Taiwan; Department of Laboratory Medicine, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan. Electronic address:

The immunomodulatory effect of IL-10 as an immunosuppressive and anti-inflammatory cytokine is well known. Taking advantage of our established mouse model of autoimmune cholangitis using 2-octynoic acid conjugated ovalbumin (2-OA-OVA) induction, we compared liver pathology, immune cell populations and antimitochondrial antibodies between IL-10 knockout and wild type mice immunized with 2-OA-OVA. At 10 weeks post immunization, portal inflammation and fibrosis were more severe in 2-OA-OVA immunized IL-10 knockout mice than in wild type mice. Read More

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http://dx.doi.org/10.1016/j.jaut.2018.09.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6289859PMC
December 2018
13 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
8 Reads

Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort.

Authors:
Reza Yazdani Hassan Abolhassani Fatemeh Kiaee Sima Habibi Gholamreza Azizi Marzieh Tavakol Zahra Chavoshzadeh Seyed Alireza Mahdaviani Tooba Momen Mohammad Gharagozlou Masoud Movahedi Amir Ali Hamidieh Nasrin Behniafard Mohammamd Nabavi Mohammad Hassan Bemanian Saba Arshi Rasol Molatefi Roya Sherkat Afshin Shirkani Reza Amin Soheila Aleyasin Reza Faridhosseini Farahzad Jabbari-Azad Iraj Mohammadzadeh Javad Ghaffari Alireza Shafiei Arash Kalantari Mahboubeh Mansouri Mehrnaz Mesdaghi Delara Babaie Hamid Ahanchian Maryam Khoshkhui Habib Soheili Mohammad Hossein Eslamian Taher Cheraghi Abbas Dabbaghzadeh Mahmoud Tavassoli Rasoul Nasiri Kalmarzi Seyed Hamidreza Mortazavi Sara Kashef Hossein Esmaeilzadeh Javad Tafaroji Abbas Khalili Fariborz Zandieh Mahnaz Sadeghi-Shabestari Sepideh Darougar Fatemeh Behmanesh Hedayat Akbari Mohammadreza Zandkarimi Farhad Abolnezhadian Abbas Fayezi Mojgan Moghtaderi Akefeh Ahmadiafshar Behzad Shakerian Vahid Sajedi Behrang Taghvaei Mojgan Safari Marzieh Heidarzadeh Babak Ghalebaghi Seyed Mohammad Fathi Behzad Darabi Saeed Bazregari Nasrin Bazargan Morteza Fallahpour Alireza Khayatzadeh Naser Javahertrash Bahram Bashardoust Mohammadali Zamani Azam Mohsenzadeh Sarehsadat Ebrahimi Samin Sharafian Ahmad Vosughimotlagh Mitra Tafakoridelbari Maziar Rahim Parisa Ashournia Anahita Razaghian Arezou Rezaei Ashraf Samavat Setareh Mamishi Hossein Ali Khazaei Javad Mohammadi Babak Negahdari Nima Parvaneh Nima Rezaei Vassilios Lougaris Silvia Giliani Alessandro Plebani Hans D Ochs Lennart Hammarström Asghar Aghamohammadi

J Allergy Clin Immunol Pract 2019 03 19;7(3):864-878.e9. Epub 2018 Sep 19.

Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran; Iranian Primary Immunodeficiencies Network (IPIN), Tehran University of Medical Sciences, Tehran, Iran. Electronic address:

Background: Predominantly antibody deficiencies (PADs) are the most common primary immunodeficiencies, characterized by hypogammaglobulinemia and inability to generate effective antibody responses.

Objective: We intended to report most common monogenic PADs and to investigate how patients with PAD who were primarily diagnosed as suffering from agammaglobulinemia, hyper-IgM (HIgM) syndrome, and common variable immunodeficiency (CVID) have different clinical and immunological findings.

Methods: Stepwise next-generation sequencing and Sanger sequencing were performed for confirmation of the mutations in the patients clinically diagnosed as suffering from agammaglobulinemia, HIgM syndrome, and CVID. Read More

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http://dx.doi.org/10.1016/j.jaip.2018.09.004DOI Listing
March 2019
69 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
45 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
30 Reads

Limited Innovations After More Than 65 Years of Immunoglobulin Replacement Therapy: Potential of IgA- and IgM-Enriched Formulations to Prevent Bacterial Respiratory Tract Infections.

Front Immunol 2018 23;9:1925. Epub 2018 Aug 23.

Section Pediatric Infectious Diseases, Laboratory of Medical Immunology, Radboud Institute for Molecular Life Sciences, Nijmegen, Netherlands.

Patients with primary immunoglobulin deficiency have lower immunoglobulin levels or decreased immunoglobulin function, which makes these patients more susceptible to bacterial infection. Most prevalent are the selective IgA deficiencies (~1:3,000), followed by common variable immune deficiency (~1:25,000). Agammaglobulinemia is less common (~1:400,000) and is characterized by very low or no immunoglobulin production resulting in a more severe disease phenotype. Read More

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http://dx.doi.org/10.3389/fimmu.2018.01925DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6115500PMC
September 2019
15 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
18 Reads

Absence of Persistent Hepatitis E Virus Infection in Antibody-Deficient Patients Is Associated With Transfer of Antigen-Neutralizing Antibodies From Immunoglobulin Products.

J Infect Dis 2019 01;219(2):245-253

Department of Clinical Immunology, Royal Free London National Health Service Foundation Trust.

Background: Persistent hepatitis E virus (HEV) infection is described in a number of immunosuppressive conditions. We aimed to determine the risk of persistent HEV infection in patients with primary or secondary antibody deficiency.

Methods: Two hundred forty-five antibody-deficient patients receiving regular immunoglobulin replacement therapy were tested for HEV RNA and anti-HEV immunoglobulin G (IgG). Read More

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http://dx.doi.org/10.1093/infdis/jiy504DOI Listing
January 2019
52 Reads

Antibodies aggravate the development of ischemic heart failure.

Am J Physiol Heart Circ Physiol 2018 11 10;315(5):H1358-H1367. Epub 2018 Aug 10.

University Hospital Halle, Department of Internal Medicine-III , Halle , Germany.

Heart-specific antibodies have been widely associated with myocardial infarction (MI). However, it remains unclear whether autoantibodies mediate disease progression or are a byproduct of cardiac injury. To disambiguate the role of immunoglobulins in MI, we characterized the development of ischemic heart failure in agammaglobulinemic mice (AIDμS). Read More

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http://dx.doi.org/10.1152/ajpheart.00144.2018DOI Listing
November 2018
17 Reads

Long-term follow-up of an activated PI3K-δ syndrome 2 in patient presenting with an agammaglobulinemia phenotype.

Ann Allergy Asthma Immunol 2018 12 4;121(6):739-740.e1. Epub 2018 Aug 4.

Department of Clinical Immunology, Saint-Louis Hospital, Assistance Publique Hôpitaux de Paris, Paris, France; EA3518, Université Paris Diderot Paris 7, Paris, France. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S10811206183062
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http://dx.doi.org/10.1016/j.anai.2018.07.043DOI Listing
December 2018
34 Reads

Low-dose vitamin A therapy on T lymphocyte function in neonatal pneumonia.

Eur Rev Med Pharmacol Sci 2018 07;22(13):4371-4374

Department of Neonatology, The Children & Women's Healthcare of Laiwu City, Laiwu 271199, P.R. China.

Objective: We aimed at studying the effect of adjuvant therapy with low-dose vitamin A on the function of T lymphocytes in neonatal pneumonia.

Patients And Methods: We recruited 60 cases of neonatal pneumonia which were randomly divided in two equal groups. The control group was treated with conventional anti-inflammatory therapy and aerosol inhalation. Read More

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http://dx.doi.org/10.26355/eurrev_201807_15437DOI Listing
July 2018
8 Reads

Expanding the Spectrum of EBV-positive Marginal Zone Lymphomas: A Lesion Associated With Diverse Immunodeficiency Settings.

Am J Surg Pathol 2018 10;42(10):1306-1316

Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, Bethesda.

Traditionally low-grade B-cell lymphomas have been excluded from the category of monomorphic posttransplant lymphoproliferative disorders. However, recent reports identified Epstein-Barr virus-positive (EBV) extranodal marginal zone lymphomas (MZL), almost exclusively seen in the posttransplant setting. Some reported cases responded to reduced immunosuppression, suggesting that they should be considered as a form of posttransplant lymphoproliferative disorders. Read More

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http://dx.doi.org/10.1097/PAS.0000000000001113DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6133753PMC
October 2018
26 Reads

Anti-annexin A5 antibodies and 25-hydroxy-cholecalciferol in female patients with primary antiphospholipid syndrome.

Clin Rheumatol 2018 Dec 12;37(12):3359-3364. Epub 2018 Jun 12.

University of Belgrade, Faculty of Pharmacy, Department for Medical Biochemistry, Belgrade, Serbia.

Vascular antiphospholipid syndrome (VAPS) and obstetric (OAPS) are different entities because some patients only develop thrombosis (without recurrent pregnancy losses) and vice versa. Only two articles have reported that low 25-hydroxy-cholecalciferol (vitamin D3, VD3) levels were not correlated with the presence of conventional antiphospholipid antibodies (aPL Abs: anticardiolipin (aCL), anti-beta2glycoprotein I (aβ2gpI), and lupus anticoagulant (LA)), but no article analyzed the association of VD3 and anti-annexin A5 (aanxA5) Abs. The aim of our study was to investigate the association between VD3, multiple positivity of conventional aPL and aanxA5 Abs levels only in female OAPS vs. Read More

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http://dx.doi.org/10.1007/s10067-018-4170-7DOI Listing
December 2018
11 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
8 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
48 Reads