5,568 results match your criteria Immunoglobulin G Deficiency


Disseminated cryptococcosis with immunoglobulin G subclass deficiency.

Pediatr Int 2019 Feb 11. Epub 2019 Feb 11.

Division of Pediatric Infectious Disease, Department of Pediatrics, St Joseph's Children's Hospital, Paterson, New Jersey, USA.

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http://dx.doi.org/10.1111/ped.13741DOI Listing
February 2019
1 Read

The total IgM, IgA and IgG antibody responses to pneumococcal polysaccharide vaccination (Pneumovax®23) in a healthy adult population and patients diagnosed with primary immunodeficiencies.

Vaccine 2019 Feb 5;37(10):1350-1355. Epub 2019 Feb 5.

Department of Laboratory Medicine and Pathology, Mayo Clinic , Rochester, MN, USA.

Background: Interpretation of the responses to the pneumococcal polysaccharide vaccine (Pneumovax®23, PPV) has proven challenging. In addition, there are few studies documenting the longevity of these responses.

Methods: The age-specific PPV IgM, IgA, IgG and IgG2 concentrations were determined pre, 4-6 weeks and 6 years post-vaccination in the serum of Prevnar®-naïve adults using VaccZyme™ pneumococcal capsular polysaccharide ELISAs. Read More

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http://dx.doi.org/10.1016/j.vaccine.2019.01.035DOI Listing
February 2019
1 Read

[Characterization of celiac disease in chilean public hospitals].

Rev Chil Pediatr 2018 Dec;89(6):709-717

Instituto de Nutrición y Tecnología de los Alimentos, Universidad de Chile, Chile.

Introduction: The worldwide prevalence of celiac disease (CD) is ~1% of the population. In Chile, the National Health Survey 2009-2010 showed a serological prevalence in individuals older than 15 years of 0.76% (IgA-tTG2), which corresponded in Concepción to 0. Read More

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http://dx.doi.org/10.4067/S0370-41062018005001001DOI Listing
December 2018
1 Read

The role of Mannose Binding Lectin in the immune response against Borrelia burgdorferi sensu lato.

Sci Rep 2019 Feb 5;9(1):1431. Epub 2019 Feb 5.

Amsterdam UMC, University of Amsterdam, Center for Experimental and Molecular Medicine, Meibergdreef 9, Amsterdam, Netherlands.

The causative agents of Lyme borreliosis, spirochetes belonging to the Borrelia burgdorferi sensu lato group, have developed several ways to protect themselves against killing by the host complement system. In addition, it has been shown that serum sensitive isolates are (partially) protected by the Ixodes Tick Salivary Lectin Pathway Inhibitor (TSLPI) protein; a salivary gland protein that inhibits the function of Mannose Binding Lectin (MBL). MBL is a C-type lectin that recognizes oligosaccharides on pathogens and activates the complement system via the lectin pathway. Read More

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http://dx.doi.org/10.1038/s41598-018-37922-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6363739PMC
February 2019
1 Read

TREC and KREC Levels as a Predictors of Lymphocyte Subpopulations Measured by Flow Cytometry.

Front Physiol 2018 21;9:1877. Epub 2019 Jan 21.

Department of Paediatrics, Sechenov University, Moscow, Russia.

Primary immunodeficiency diseases (PID) is a heterogeneous group of disorders caused by genetic defects of the immune system, which manifests clinically as recurrent infections, autoimmune diseases, or malignancies. Early detection of other PID remains a challenge, particularly in older children due to milder and less specific symptoms, a low level of clinician PID awareness and poor provision of hospital laboratories with appropriate devices. T-cell recombination excision circles (TREC) and kappa-deleting element recombination circle (KREC) in a dried blood spot and in peripheral blood using real-time polymerase chain reaction (PCR) are used as a tool for severe combined immune deficiency but not in PID. Read More

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http://dx.doi.org/10.3389/fphys.2018.01877DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6348265PMC
January 2019
2 Reads

Adiponectin deficiency has no effect in murine autoimmune myocarditis.

Cytokine 2019 Apr 1;116:139-149. Epub 2019 Feb 1.

Department of Cardiology, University of Heidelberg, Heidelberg, Germany. Electronic address:

Background: Adiponectin is a hormone that together with its receptors modulates a number of metabolic processes including gluconeogenesis and lipid catabolism. It belongs to the C1QTNF (complement C1q tumor necrosis factor-related protein) family, which has a variety of members with high amino acid sequence homology and overlapping functions. Concentration of adiponectin in blood is inversely correlated with body fat percentage and cardiac risk factors like blood pressure and CRP (C-reactive protein) level. Read More

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http://dx.doi.org/10.1016/j.cyto.2018.12.022DOI Listing
April 2019
1 Read

Laboratory Diagnostics in Thrombophilia.

Hamostaseologie 2019 Feb 31;39(1):49-61. Epub 2019 Jan 31.

Department of Hematology and Oncology, Internal Medicine III, University Hospital Regensburg, Regensburg, Germany.

A thrombophilic disorder is a hereditary or acquired condition that increases the risk of thrombosis. The most common hereditary thrombophilias that predispose to venous thrombosis in the Caucasian population are the heterozygous forms of the factor V Leiden and prothrombin G20210A mutation that are generally detected by direct DNA genotyping. Immunologic antigen assays and chromogenic or clot-based activity assays are used to identify deficiencies in the natural coagulation inhibitors antithrombin, protein C and protein S. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-1677840
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http://dx.doi.org/10.1055/s-0039-1677840DOI Listing
February 2019
8 Reads

Dysregulated TRAF3 and BCL2 Expression Promotes Multiple Classes of Mature Non-hodgkin B Cell Lymphoma in Mice.

Front Immunol 2018 11;9:3114. Epub 2019 Jan 11.

Instituto de Investigaciones Biomédicas "Alberto Sols", CSIC-UAM, Madrid, Spain.

TNF-Receptor Associated Factor (TRAF)-3 is a master regulator of B cell homeostasis and function. TRAF3 has been shown to bind and regulate various proteins involved in the control of innate and adaptive immune responses. Previous studies showed that TRAF3 overexpression renders B cells hyper-reactive to antigens and Toll-like receptor (TLR) agonists, while TRAF3 deficiency has been implicated in the development of a variety of B cell neoplasms. Read More

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http://dx.doi.org/10.3389/fimmu.2018.03114DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6338067PMC
January 2019
2 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
2 Reads

LRRK2 is involved in the pathogenesis of system lupus erythematosus through promoting pathogenic antibody production.

J Transl Med 2019 Jan 22;17(1):37. Epub 2019 Jan 22.

Shanghai Institute of Immunology, Department of Immunology and Microbiology, Shanghai Jiao Tong University School of Medicine, Shanghai, 200025, China.

Background: Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease characterized by the presence of pathogenic autoantibodies associated with polyclonal B cell hyperreactivity. Previous study reported that autophagy-related gene Leucine-rich repeat kinase 2 (LRRK2) was likely a susceptible gene for SLE. However, the pathogenic function of LRRK2 in SLE is undefined. Read More

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http://dx.doi.org/10.1186/s12967-019-1786-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6343316PMC
January 2019
3 Reads
3.930 Impact Factor

Isolated IgG4-related gastric disease presenting as diffuse gastric wall thickening with ulcer.

J Radiol Case Rep 2018 Sep 30;12(9):9-20. Epub 2018 Sep 30.

Department of Anatomical Pathology, Singapore General Hospital, Singapore.

An 81-year-old male presented with loss of appetite, early satiety and iron deficiency anaemia. A computed tomography (CT) scan of the abdomen and pelvis during initial work-up revealed diffuse gastric mural thickening associated with a large ulcer and adjacent gastro-hepatic lymphadenopathy. The CT appearances, together with the clinical features, were highly suspicious for an infiltrative type of gastric malignancy. Read More

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http://radiologycases.com/index.php/radiologycases/article/v
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http://dx.doi.org/10.3941/jrcr.v12i9.3493DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6312045PMC
September 2018
6 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

Fn14 Deficiency Ameliorates Anti-dsDNA IgG-Induced Glomerular Damage in SCID Mice.

J Immunol Res 2018 16;2018:1256379. Epub 2018 Dec 16.

Department of Dermatology, The Second Affiliated Hospital, School of Medicine, Xi'an Jiaotong University, Xi'an 710004, China.

Many studies have demonstrated that anti-dsDNA IgG is closely associated with lupus nephritis. Recently, it was found that activation of the fibroblast growth factor-inducible 14 (Fn14) signaling pathway damages glomerular filtration barrier in MRL/lpr lupus-prone mice. However, MRL/lpr mice have high titers of serum autoantibodies other than anti-dsDNA IgG. Read More

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https://www.hindawi.com/journals/jir/2018/1256379/
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http://dx.doi.org/10.1155/2018/1256379DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6311848PMC
December 2018
4 Reads

A 9.5-year-old boy with recurrent neurological manifestations and severe hypertension, treated initially for polyarteritis nodosa, was subsequently diagnosed with adenosine deaminase type 2 deficiency (DADA2) which responded to anti-TNF-α.

Paediatr Int Child Health 2019 Jan 15:1-4. Epub 2019 Jan 15.

a Department of Paediatric Rheumatology, Cerrahpasa Medical School , Istanbul University , Istanbul , Turkey.

A 9.5-year-old boy was referred with a 2-year history of recurrent fever, myalgia, abdominal pain and various neurological manifestations associated with increased acute phase reactants and IgG level. During the recent episode, severe hypertension and right-sided hemiparesis developed and angiography demonstrated irregularities and stenosis in renal and mesenteric artery branches. Read More

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http://dx.doi.org/10.1080/20469047.2018.1559495DOI Listing
January 2019
2 Reads

CMV-Specific Immune Response-New Patients, New Insight: Central Role of Specific IgG during Infancy and Long-Lasting Immune Deficiency after Allogenic Stem Cell Transplantation.

Int J Mol Sci 2019 Jan 11;20(2). Epub 2019 Jan 11.

Hirszfeld Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, 53-114 Wrocław, Poland.

Although the existing paradigm states that cytomegalovirus (CMV) reactivation is under the control of the cellular immune response, the role of humoral and innate counterparts are underestimated. The study analyzed the host⁻virus interaction i.e. Read More

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http://www.mdpi.com/1422-0067/20/2/271
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http://dx.doi.org/10.3390/ijms20020271DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6358762PMC
January 2019
6 Reads

[Clinical and genetic manifestations of immunodeficiency, centromeric instability, and facial anomalies syndrome: a case report and literature review].

Zhonghua Er Ke Za Zhi 2019 Jan;57(1):55-59

Department of Nephrology and Immunology, Qingdao Women and Children's Hospital, Qingdao 266000, China.

To analyze the clinical and genetic features of immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome with a case report and literature review. The clinical data and genetic test of a girl diagnosed with ICF syndrome in the Department of Nephrology and Immunology in Qingdao Women and Children's Hospital in December 2016 were extracted and analyzed. "ICF syndrome" "immunodeficiency, centromeric instability and facial anomalies syndrome" "ICF syndrome and DNMT3B" were used as key words to search Chinese databases and Pubmed for literature until March 2018, and the literature was reviewed. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2019.01.013DOI Listing
January 2019
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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
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AGA Clinical Practice Update on Diagnosis and Monitoring of Celiac Disease: Changing Utility of Serology and Histologic Measures: Expert Review.

Gastroenterology 2018 Dec 19. Epub 2018 Dec 19.

Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota.

Purpose: The purpose of this clinical practice update is to define key modalities in the diagnosis and monitoring of celiac disease (CD) in adults as well as in children and adolescents.

Methods: The recommendations outlined in this expert review are based on available published evidence, including cohort and case-control studies of the diagnostic process as well as controlled and descriptive studies of disease management. Best Practice Advice 1: Serology is a crucial component of the detection and diagnosis of CD, particularly tissue transglutaminase-immunoglobulin A (TG2-IgA), IgA testing, and less frequently, endomysial IgA testing. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00165085183540
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http://dx.doi.org/10.1053/j.gastro.2018.12.010DOI Listing
December 2018
1 Read

High-resolution computed tomography findings in humoral primary immunodeficiencies and correlation with pulmonary function tests.

World J Radiol 2018 Nov;10(11):172-183

Institute of Radiology, Department of Medicine, University of Udine, Azienda Sanitaria Universitaria Integrata di Udine, Udine 33100, Italy.

Aim: To compare high-resolution computed tomography (HRCT) findings between humoral primary immunodeficiencies (hPIDs) subtypes; to correlate these findings to pulmonary function tests (PFTs).

Methods: We retrospectively identified 52 consecutive adult patients with hPIDs who underwent 64-row HRCT and PFTs at the time of diagnosis. On a per-patient basis, an experienced radiologist recorded airway abnormalities (bronchiectasis, airway wall thickening, mucus plugging, tree-in-bud, and air-trapping) and parenchymal-interstitial abnormalities (consolidations, ground-glass opacities, linear and/or irregular opacities, nodules, and bullae/cysts) found on HRCT. Read More

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http://www.wjgnet.com/1949-8470/full/v10/i11/172.htm
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http://dx.doi.org/10.4329/wjr.v10.i11.172DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6288673PMC
November 2018
12 Reads

Common variable immune deficiency: Dissection of the variable.

Immunol Rev 2019 Jan;287(1):145-161

Division of Clinical Immunology, Icahn School of Medicine at Mount Sinai, New York, New York.

Starting about 60 years ago, a number of reports appeared that outlined the severe clinical course of a few adult subjects with profound hypogammaglobinemia. Puzzled by the lack of family history and adult onset of symptoms in most, the name "acquired" hypogammaglobinemia was given, but later altered to the current name common variable immune deficiency. Pathology reports remarked on the loss of lymph node architecture and paucity of plasma cells in lymphoid tissues in these subjects. Read More

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http://doi.wiley.com/10.1111/imr.12728
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http://dx.doi.org/10.1111/imr.12728DOI Listing
January 2019
7 Reads

Differentiation of Common Variable Immunodeficiency From IgG Deficiency.

J Allergy Clin Immunol Pract 2018 Dec 14. Epub 2018 Dec 14.

The Division of Clinical Immunology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY. Electronic address:

Background: Common variable immunodeficiency (CVID) and IgG deficiency are 2 of the more prevalent primary humoral immune defects. The former is defined by consensus with criteria for quantitative and qualitative antibody defects, whereas the latter is used to describe patients with reduced IgG, who commonly have recurrent sinopulmonary infections but do not fulfill CVID criteria. However, these patients are often given this diagnosis. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22132198183082
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http://dx.doi.org/10.1016/j.jaip.2018.12.004DOI Listing
December 2018
9 Reads

Synergistic convergence of microbiota-specific systemic IgG and secretory IgA.

J Allergy Clin Immunol 2018 Dec 7. Epub 2018 Dec 7.

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

Background: Commensals induce local IgA responses essential to the induction of tolerance to gut microbiota, but it remains unclear whether antimicrobiota responses remain confined to the gut.

Objective: The aim of this study was to investigate systemic and intestinal responses against the whole microbiota under homeostatic conditions and in the absence of IgA.

Methods: We analyzed blood and feces from healthy donors, patients with selective IgA deficiency (SIgAd), and patients with common variable immunodeficiency (CVID). Read More

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http://dx.doi.org/10.1016/j.jaci.2018.09.036DOI Listing
December 2018
16 Reads

Screening for Humoral Immunodeficiency in Patients with Community-Acquired Pneumonia.

J Hosp Med 2019 01 28;14(1):33037. Epub 2018 Nov 28.

Rochester Regional Health, Rochester, New York, USA.

Background: Immunodeficiency is an underrecognized risk factor for infections, such as community-acquired pneumonia (CAP).

Objective: We evaluated patients admitted with CAP for humoral immunodeficiency.

Design: Prospective Cohort Study. Read More

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http://dx.doi.org/10.12788/jhm.3106DOI Listing
January 2019
9 Reads

Vitamin D deficiency increases blood-brain barrier dysfunction after ischemic stroke in male rats.

Exp Neurol 2019 Feb 28;312:63-71. Epub 2018 Nov 28.

Department of Emergency Medicine, Brain Research Laboratory, Emory University, Atlanta, GA 30322, USA. Electronic address:

Because vitamin D hormone deficiency (VDH) can worsen severity and outcome for ischemic stroke, we examined the role of VDH in maintaining blood-brain-barrier (BBB integrity) in a rat model of stroke. In most types of stroke, the BBB is markedly compromised, potentially leading to a cascade of injury processes and functional deficits, so we examined a number of biomarkers associated with BBB disruption to determine whether VDH deficiency would further compromise the BBB following a stroke. Male Wistar rats were randomly assigned to one of two diet cohorts, VDH-sufficient (VDH) and VDH. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00144886183027
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http://dx.doi.org/10.1016/j.expneurol.2018.11.005DOI Listing
February 2019
9 Reads

TACI Contributes to Host Resistance by Controlling T Follicular Helper Cell Response and Germinal Center Formation.

Front Immunol 2018 9;9:2612. Epub 2018 Nov 9.

US Food and Drug Administration, Division of Bacterial Allergenic and Parasitic Diseases, Center for Biologics Evaluation and Research, Silver Spring, MD, United States.

The delay in parasite-specific B cell development leaves people in malaria endemic areas vulnerable to repeated infections. Here, we investigated the role of transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI), a molecule involved in the generation of antigen-specific antibody secreting cells, in host response to non-lethal infection. We found that TACI deficiency not only resulted in higher peak parasitemia levels in challenged mice, but also led to a delay in parasite clearance and anti- Merozoite Surface Protein 1(C-terminal 19-kDa fragment [rMSP-1]) protein and anti-rMSP-1 and anti- IgG antibody development. Read More

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http://dx.doi.org/10.3389/fimmu.2018.02612DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6237915PMC
November 2018
2 Reads

Personalized Therapy: Immunoglobulin Replacement for Antibody Deficiency.

Immunol Allergy Clin North Am 2019 Feb;39(1):95-111

Allergy Partners of North Texas, 7777 Forest Lane, Suite B-332, Dallas, TX 75230, USA. Electronic address:

Immunoglobulin replacement therapy is the cornerstone of management for most primary immunodeficiency disease patients. The selection of a particular product, dose, and route of administration requires an understanding of the features of therapeutic immunoglobulin as well as patient-specific risk factors in order to maximize efficacy and tolerability and minimize risk. Individualizing therapy, taking into consideration the burdens of care, is necessary in order to optimize patient outcomes. Read More

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http://dx.doi.org/10.1016/j.iac.2018.08.001DOI Listing
February 2019
13 Reads

Increased susceptibility against Cryptococcus neoformans of lupus mouse models (pristane-induction and FcGRIIb deficiency) is associated with activated macrophage, regardless of genetic background.

J Microbiol 2019 Jan 19;57(1):45-53. Epub 2018 Nov 19.

Department of Microbiology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.

The severity of cryptococcosis in lupus from varying genetic-backgrounds might be different due to the heterogeneity of lupus-pathogenesis. This study explored cryptococcosis in lupus mouse models of pristane-induction (normal genetic-background) and FcGRIIb deficiency (genetic defect). Because the severity of lupus nephritis, as determined by proteinuria and serum creatinine, between pristane and FcGRIIb-/- mice were similar at 6-month-old, Cryptococcus neoformans was intravenously administered in 6-month-old mice and were age-matched with wild-type. Read More

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http://dx.doi.org/10.1007/s12275-019-8311-8DOI Listing
January 2019
1 Read

Allogeneic Stem Cell Transplantation after Fanconi Anemia Conditioning in Children with Ataxia-Telangiectasia Results in Stable T Cell Engraftment and Lack of Infections despite Mixed Chimerism.

Biol Blood Marrow Transplant 2018 Nov 7;24(11):2245-2249. Epub 2018 Jul 7.

Department of Pediatric Bone Marrow Transplantation, Oncology, and Hematology, Wroclaw Medical University, Wroclaw, Poland.

Ataxia-telangiectasia (A-T) syndrome is an autosomal recessive chromosomal breakage syndrome caused by mutation of the ataxia-telangiectasia mutated gene manifested by progressive neurodegeneration, telangiectasias of sclera and skin, immune deficiency with sinopulmonary infections, and increased incidence of lymphoid malignancies and solid tumors. Three children with A-T underwent allogeneic stem cell transplantation (SCT) using protocols for Fanconi anemia. All 3 patients were engrafted with a mixed donor-recipient chimerism, but the full donor engraftment was observed in the T lymphocyte compartment. Read More

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http://dx.doi.org/10.1016/j.bbmt.2018.07.001DOI Listing
November 2018
12 Reads

Privigen® has similar pharmacokinetic properties in primary and secondary immune deficiency.

Int Immunopharmacol 2019 Jan 15;66:119-126. Epub 2018 Nov 15.

CSL Behring LLC, 1020 First Avenue, King of Prussia, PA 19406, USA. Electronic address:

Purpose: Primary (PID) and secondary immune deficiencies (SID) represent diverse groups of diagnoses, yet both can be effectively treated with intravenous immunoglobulin (IVIG) replacement therapy. Guidelines for the use of IVIG in SID vary due to the paucity of data. The objective was to analyze available IVIG Privigen® (IgPro10, CSL Behring, Bern, Switzerland) data on Efficiency Index (EI) and pharmacokinetic (PK) parameters in patients with PID and SID. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15675769183074
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http://dx.doi.org/10.1016/j.intimp.2018.11.008DOI Listing
January 2019
9 Reads

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

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

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

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

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

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

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

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

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

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

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

Impaired innate immune signaling due to combined Toll-like receptor 2 and 4 deficiency affects both periodontitis and atherosclerosis in response to polybacterial infection.

Pathog Dis 2018 Nov 1;76(8). Epub 2018 Nov 1.

Department of Periodontology, College of Dentistry, University of Florida, Gainesville, FL 32610, USA.

Plasma membrane-associated Toll-like receptor (TLR2 and TLR4) signaling contributes to oral microbe infection-induced periodontitis and atherosclerosis. We recently reported that either TLR2 or TLR4 receptor deficiency alters recognition of a consortium of oral pathogens, modifying host responses in periodontitis and atherosclerosis. We evaluated the effects of combined TLR2-/-TLR4-/- double knockout mice on innate immune signaling and induction of periodontitis and atherosclerosis after polybacterial infection with Porphyromonas gingivalis, Treponema denticola, Tannerella forsythia and Fusobacterium nucleatum in a mouse model. Read More

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https://academic.oup.com/femspd/advance-article/doi/10.1093/
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http://dx.doi.org/10.1093/femspd/fty076DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6251349PMC
November 2018
13 Reads

Emicizumab, a humanized bispecific antibody to coagulation factors IXa and X with a factor VIIIa-cofactor activity.

Int J Hematol 2018 Oct 22. Epub 2018 Oct 22.

Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.

Hemophilia A is a congenital disorder caused by deficiency or malfunction of coagulation factor (F) VIII. While exogenously provided FVIII effectively reduces bleeding complications in many hemophilia A patients, multiple efforts are underway to develop new drugs to meet the needs that conventional FVIII agents do not. We have been long engaged in creating and clinically developing a humanized anti-FIXa/FX asymmetric bispecific IgG antibody with a FVIIIa-cofactor activity. Read More

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http://link.springer.com/10.1007/s12185-018-2545-9
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http://dx.doi.org/10.1007/s12185-018-2545-9DOI Listing
October 2018
11 Reads

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

Authors:
B Wu L Li T Ruan X Peng

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

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

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

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

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

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

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

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

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

Complement-dependent bystander injury to neurons in AQP4-IgG seropositive neuromyelitis optica.

J Neuroinflammation 2018 Oct 22;15(1):294. Epub 2018 Oct 22.

Departments of Medicine and Physiology, University of California, 1246 Health Sciences East Tower, 513 Parnassus Ave, San Francisco, CA, 94143-0521, USA.

Background: Aquaporin-4-immunoglobulin G (AQP4-IgG) seropositive neuromyelitis optica spectrum disorder (herein called NMO) is an autoimmune disease of the central nervous system in which AQP4-IgG binding to AQP4 on astrocytes results in complement-dependent astrocyte injury and secondary inflammation, demyelination, and neuron loss. We previously reported evidence for a complement bystander mechanism for early oligodendrocyte injury in NMO. Herein, we tested the hypothesis that complement bystander injury, which involves diffusion to nearby cells of activated soluble complement components from complement-injured astrocytes, is a general phenomenon that may contribute to neuronal injury in NMO. Read More

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http://dx.doi.org/10.1186/s12974-018-1333-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6198534PMC
October 2018
1 Read

LGI1 antibodies alter Kv1.1 and AMPA receptors changing synaptic excitability, plasticity and memory.

Brain 2018 Nov;141(11):3144-3159

Hans-Berger Department of Neurology, Jena University Hospital, Jena, Germany.

Leucine-rich glioma-inactivated 1 (LGI1) is a secreted neuronal protein that forms a trans-synaptic complex that includes the presynaptic disintegrin and metalloproteinase domain-containing protein 23 (ADAM23), which interacts with voltage-gated potassium channels Kv1.1, and the postsynaptic ADAM22, which interacts with AMPA receptors. Human autoantibodies against LGI1 associate with a form of autoimmune limbic encephalitis characterized by severe but treatable memory impairment and frequent faciobrachial dystonic seizures. Read More

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https://academic.oup.com/brain/advance-article/doi/10.1093/b
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http://dx.doi.org/10.1093/brain/awy253DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6202570PMC
November 2018
7 Reads

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

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

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

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

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

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

Quantitative B-lymphocyte deficiency and increased TCRγδ T-lymphocytes in acute infectious spondylodiscitis.

Sci Rep 2018 Oct 11;8(1):15174. Epub 2018 Oct 11.

Viro-Imunology Research Unit, Department of Infectious Diseases, Rigshospitalet, University Hospital of Copenhagen, Copenhagen, Denmark.

Acute infectious spondylodiscitis (AIS) is a serious infection of the spine with rising incidence and a mortality of 3-6%. The role of the immune system in AIS is largely unknown. We performed extensive B and T-lymphocyte phenotyping in patients with AIS at diagnosis and after treatment cessation. Read More

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http://dx.doi.org/10.1038/s41598-018-33318-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6181974PMC
October 2018
3 Reads

pathogenicity of IgG from patients with anti-SRP or anti-HMGCR autoantibodies in immune-mediated necrotising myopathy.

Ann Rheum Dis 2019 Jan 11;78(1):131-139. Epub 2018 Oct 11.

Normandie Univ, UNIROUEN, IRIB, Inserm, U1234, Departement of Immunology, Rouen University Hospital, Rouen, France

Objectives: In autoimmunity, autoantibodies (aAb) may be simple biomarkers of disease or true pathogenic effectors. A form of idiopathic inflammatory myopathy associated with anti-signal recognition particle (SRP) or anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) aAb has been individualised and is referred to as immune-mediated necrotising myopathy (IMNM). The level of aAb correlates with IMNM activity and disease may respond to immunosuppression, suggesting that they are pathogenic. Read More

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http://ard.bmj.com/lookup/doi/10.1136/annrheumdis-2018-21351
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http://dx.doi.org/10.1136/annrheumdis-2018-213518DOI Listing
January 2019
10 Reads

Resolution of inflammation in neuromyelitis optica spectrum disorders.

Mult Scler Relat Disord 2019 Jan 2;27:34-41. Epub 2018 Oct 2.

Department of Neurology, The First Hospital of Jilin University, Xinmin Street 71#, Changchun 130021, China. Electronic address:

Background: Neuromyelitis optica spectrum disorders (NMOSD) are a spectrum of neuroinflammatory disorders associated with autoimmune antibodies against aquaporin-4 (AQP4). Accumulating evidence suggests that inflammation is involved in NMOSD pathogenesis. Resolution of inflammation, which is a highly regulated process mediated by specialized pro-resolving lipid mediators (SPMs) is important to prevent over-responsive inflammation. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22110348183036
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http://dx.doi.org/10.1016/j.msard.2018.09.040DOI Listing
January 2019
16 Reads

Expansion of Regulatory T Cells with IL-2/IL-2 Antibody Complex Protects against Transient Ischemic Stroke.

J Neurosci 2018 Nov 5;38(47):10168-10179. Epub 2018 Oct 5.

Pittsburgh Institute of Brain Disorders and Recovery, and Department of Neurology,

Regulatory T cells (Tregs) are known to protect against ischemic stroke. However, the low frequency of Tregs restricts their clinical utility. This study investigated whether expanding the number of Tregs with the IL-2/IL-2 antibody complex (IL-2/IL-2Ab) could improve stroke outcomes and further elaborated the mechanisms of protection in male mice. Read More

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http://dx.doi.org/10.1523/JNEUROSCI.3411-17.2018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6246882PMC
November 2018
2 Reads

Evaluation of screening for coeliac disease in children with juvenile idiopathic arthritis.

Acta Paediatr 2018 Sep 28. Epub 2018 Sep 28.

Department of Women's and Children's Health, Uppsala University, Uppsala, Sweden.

Aim: To study the prevalence of coeliac disease (CD) in children with Juvenile idiopathic arthritis (JIA), by screening a population-based cohort of children with JIA using autoantibodies against tissue transglutaminase (anti-TG2).

Methods: All children diagnosed with JIA in three Swedish counties, with disease onset between 2007 and 2014, were included prospectively. Serum levels of IgA anti-TG2 antibodies, IgG anti-TG2 antibodies, and total IgA were analysed. Read More

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http://dx.doi.org/10.1111/apa.14598DOI Listing
September 2018
4 Reads

Performance evaluation of serum IgG subclass quantification using a SPAPLUS turbidimetric analyzer and comparison with the BNII nephelometer.

Scand J Clin Lab Invest 2018 10 27;78(6):496-500. Epub 2018 Sep 27.

a Department of Laboratory Medicine & Genetics , Samsung Medical Center, Sungkyunkwan University School of Medicine , Seoul , Korea.

IgG consists of four subclasses: IgG1, IgG2, IgG3, and IgG4. Changes in the serum concentration of each subclass reflect different clinical situations, and quantification of each subclass is important to assess patients' clinical states. Herein, we evaluated the analytical performance of the SPAPLUS turbidimetric analyzer (The Binding Site, Birmingham, UK) for IgG subclass. Read More

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https://www.tandfonline.com/doi/full/10.1080/00365513.2018.1
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http://dx.doi.org/10.1080/00365513.2018.1502464DOI Listing
October 2018
13 Reads

Fc Gamma Receptor IIb Expressed in Hepatocytes Promotes Lipid Accumulation and Gluconeogenesis.

Int J Mol Sci 2018 Sep 26;19(10). Epub 2018 Sep 26.

State Key Laboratory of Medical Molecular Biology, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences, Department of Pathophysiology, Peking Union Medical College, Beijing 100005, China.

Non-alcoholic fatty liver disease (NAFLD) is characterized by ectopic lipid accumulation in the liver, usually combined with hepatic insulin resistance. Fc-gamma receptor-IIb (FcγRIIb) and its ligand are reported to be associated with obesity and type 2 diabetes mellitus (T2DM). As knowledge about FcγRIIb in the literature is mostly generated from studies on skeletal muscle tissue, the expression and function of FcγRIIb in the liver and hepatocytes are largely unknown. Read More

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http://dx.doi.org/10.3390/ijms19102932DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6213401PMC
September 2018
7 Reads

Clozapine is associated with secondary antibody deficiency.

Br J Psychiatry 2018 Sep 27:1-7. Epub 2018 Sep 27.

Professor of Clinical Immunology,Immunodeficiency Centre for Wales,University Hospital of Wales,UK.

Background: Schizophrenia affects 1% of the population. Clozapine is the only medication licensed for treatment-resistant schizophrenia and is intensively monitored to prevent harm from neutropenia. Clozapine is also associated with increased risk of pneumonia although the mechanism is poorly understood. Read More

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http://dx.doi.org/10.1192/bjp.2018.152DOI Listing
September 2018
7 Reads

Effect of lycopene supplementation on cardiovascular parameters and markers of inflammation and oxidation in patients with coronary vascular disease.

Food Sci Nutr 2018 Sep 13;6(6):1770-1777. Epub 2018 Aug 13.

Lycotec Ltd Cambridge UK.

Oxidative stress and antioxidant deficiency play a pivotal role in initiation, development, and outcomes of cardiovascular disease. Pharmacokinetic parameters as well as the impact of highly bioavailable lycopene on cardiovascular variables, markers of inflammation and oxidation were investigated during a 30-day clinical trial in patients with coronary vascular disease. The patients were randomized into two major groups and were supplemented with a single 7 mg daily dose of lycopene ingested either in the form of lactolycopene (68 patients) or in the form of lycosome-formulated GA lycopene (74 patients). Read More

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http://doi.wiley.com/10.1002/fsn3.734
Publisher Site
http://dx.doi.org/10.1002/fsn3.734DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6145244PMC
September 2018
9 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