5,800 results match your criteria Immunoglobulin G Deficiency


CLASS SWITCHING AND HIGH AFFINITY IgG PRODUCTION BY B CELLS IS DISPENSABLE FOR THE DEVELOPMENT OF HYPERTENSION IN MICE.

Cardiovasc Res 2020 Jul 1. Epub 2020 Jul 1.

Department of Molecular Physiology and Biophysics, Vanderbilt University, Nashville, TN, USA.

Aims: Elevated serum immunoglobulins have been associated with experimental and human hypertension for decades but whether immunoglobulins and B cells play a causal role in hypertension pathology is unclear. In this study, we sought to determine the role of B cells and high-affinity class-switched immunoglobulins on hypertension and hypertensive end-organ damage to determine if they might represent viable therapeutic targets for this disease.

Methods And Results: We purified serum IgG from mice exposed to vehicle or angiotensin (Ang) II to induce hypertension and adoptively transferred these to wild type (WT) recipient mice receiving a subpressor dose of Ang II. Read More

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http://dx.doi.org/10.1093/cvr/cvaa187DOI Listing

Effect of lead exposure and nutritional iron-deficiency on immune response: A vaccine challenge study in rats.

J Immunotoxicol 2020 Dec;17(1):144-152

Food and Drug Toxicology Research Center, ICMR-National Institute of Nutrition, Hyderabad, India.

The prevalence of iron (Fe) deficiency and subclinical lead (Pb) toxicity is high in developing countries like India, and information on their potential additive effects on immune responses is scant. The current study assessed immune parameters in dual Pb-exposed\Fe-deficient weanling SD rats. Rats were fed a control (CD) or Fe-deficient (ID) diet for 4 weeks and then evaluated for hemoglobin (Hb) and serum Fe status. Read More

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http://dx.doi.org/10.1080/1547691X.2020.1773973DOI Listing
December 2020

DOCK8 is essential for LFA-1 dependent positioning of T follicular helper cells in germinal centers.

JCI Insight 2020 Jun 23. Epub 2020 Jun 23.

Division of Immunology, Boston Children's Hospital and Harvard Medical School, Boston, United States of America.

T follicular helper (Tfh) cell migration into germinal centers (GC) is essential for the generation of GC B cells and antibody responses to T dependent (TD) antigens. This process requires interactions between LFA-1 on Tfh cells and ICAMs on B cells. The mechanisms underlying defective antibody responses to TD antigens in DOCK8 deficiency are incompletely understood. Read More

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http://dx.doi.org/10.1172/jci.insight.134508DOI Listing

A New Intravenous Immune Globulin: Novel or Not?

Ann Pharmacother 2020 Jun 21:1060028020934722. Epub 2020 Jun 21.

Raabe College of Pharmacy, Ohio Northern University, Ada, OH, USA.

To assess the clinical use and determine the place in therapy for immune globulin intravenous (IV), human-slra, a recently approved IV immune globulin for the treatment of primary immune deficiency diseases (PIDD). A PubMed and MEDLINE search (2010 to April 2020) was conducted for relevant articles. Data were also obtained from the package insert. Read More

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http://dx.doi.org/10.1177/1060028020934722DOI Listing

Antibodies From Children With PANDAS Bind Specifically to Striatal Cholinergic Interneurons and Alter Their Activity.

Am J Psychiatry 2020 Jun 16:appiajp202019070698. Epub 2020 Jun 16.

Department of Psychiatry (Xu, Liu, Fahey, Frick, Duman, Williams, Pittenger), Child Study Center (Leckman, Vaccarino, Pittenger), Department of Pediatrics (Leckman), and Department of Neuroscience (Vaccarino), Yale University School of Medicine, New Haven, Conn.; Hunter James Kelly Research Institute, University at Buffalo, State University of New York, Buffalo (Frick); Department of Psychiatry, Massachusetts General Hospital and Harvard Medical School (Williams), Boston; Pediatrics and Developmental Neuroscience Branch, NIMH, Bethesda, Md. (Swedo); PANDAS Physicians Network (Swedo); and Interdepartmental Neuroscience Program, Yale University, New Haven, Conn. (Pittenger).

Objective: Pediatric obsessive-compulsive disorder (OCD) sometimes appears rapidly, even overnight, often after an infection. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections, or PANDAS, describes such a situation after infection with . PANDAS may result from induced autoimmunity against brain antigens, although this remains unproven. Read More

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http://dx.doi.org/10.1176/appi.ajp.2020.19070698DOI Listing

Deficiency of CFHR plasma proteins and autoantibody positive hemolytic uremic syndrome: treatment rationale, outcomes, and monitoring.

Pediatr Nephrol 2020 Jun 12. Epub 2020 Jun 12.

Division of Nephrology, Phoenix Children's Hospital, Phoenix, AZ, USA.

Deficiency of Complement Factor H Related (CFHR) plasma proteins and Autoantibody Positive Hemolytic Uremic Syndrome (DEAP-HUS) is a subtype of atypical hemolytic uremic syndrome, known to be associated with significant morbidity. Its pathogenesis is linked to the production of IgG autoantibodies against complement factor H, a regulator of the alternative complement pathway. The binding of the autoantibodies to the C terminal of complement factor H interferes with its regulatory function, leading to increased activation of the alternative complement pathway and consequent endothelial cellular damage. Read More

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http://dx.doi.org/10.1007/s00467-020-04652-xDOI Listing

Biochemical Evaluation of Processed Ascites in Patients Undergoing Cell-Free and Concentrated Ascites Reinfusion Therapy.

Ther Apher Dial 2020 Jun 10. Epub 2020 Jun 10.

Department of Nephrology, Shinonoi General Hospital.

The biochemical composition of processed ascites is not well researched and may differ among institutions. This prospective study was conducted to evaluate the biochemical characteristics of processed ascites of 11 patients with liver cirrhosis and carcinoma who underwent cell-free and concentrated ascites reinfusion therapy. The ascites due to carcinoma were more acidic and had higher lactate dehydrogenase activity than those due to liver cirrhosis. Read More

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http://dx.doi.org/10.1111/1744-9987.13541DOI Listing

Immunoglobulin G Deficiency in Children with Recurrent Respiratory Infections with and Without History of Allergy.

Adv Exp Med Biol 2020 Jun 10. Epub 2020 Jun 10.

Institute of Agricultural Engineering, Wroclaw University of Environmental and Life Science, Wroclaw, Poland.

Recurrent respiratory tract infections (RTI) are one of the most common diseases in childhood. Frequent infections adversely affect the development of a child and may lead to suspicion of immunodeficiency. An additional allergy component is thought conducive to infection occurrence. Read More

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http://dx.doi.org/10.1007/5584_2020_541DOI Listing

Measuring the cellular memory B cell response after vaccination in patients after allogeneic stem cell transplantation.

Ann Hematol 2020 Jun 9. Epub 2020 Jun 9.

Department of Biology, Division of Genetics, Nikolaus-Fiebiger-Center for Molecular Medicine, Friedrich-Alexander-University Erlangen-Nuremberg, Erlangen, Germany.

After allogeneic hematopoietic stem cell transplantation (HSCT), patients are repetitively vaccinated to reduce the risk of infection caused by the immune deficiency following allogeneic HSCT. By the vaccination of transplanted patients, the humoral memory function can be restored in the majority of cases. It is unknown, however, to what extent memory B cells derived from the donor contribute to the mobilization of antibody-secreting cells and long-term humoral memory in patients after allogeneic HSCT. Read More

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http://dx.doi.org/10.1007/s00277-020-04072-9DOI Listing

Tonsillar granuloma associated with hypogammaglobulinemia.

Allergy Asthma Clin Immunol 2020 29;16:43. Epub 2020 May 29.

Research Unit of Biomedicine, University of Oulu, Oulu, Finland.

Background: Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Read More

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http://dx.doi.org/10.1186/s13223-020-00441-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7257222PMC

[Successful management of acquired factor V deficiency developing shortly after induction of hemodialysis].

Rinsho Ketsueki 2020 ;61(5):445-450

Department of Hematology, Gunma University Graduate School of Medicine.

Autoimmune factor V deficiency (AiF5D) is caused by autoantibodies to coagulation factor V (FV); its clinical manifestations range from asymptomatic to fatal hemorrhage. Herein, we report the case of a 68-year-old man who was diagnosed with end-stage renal disease at the time of a femoral fracture and developed AiF5D after initiating hemodialysis. A wound infection that occurred after joint replacement was treated with antibiotics; however, it was poorly controlled. Read More

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http://dx.doi.org/10.11406/rinketsu.61.445DOI Listing
January 2020

A randomized multicenter clinical trial to evaluate the efficacy of melatonin in the prophylaxis of SARS-CoV-2 infection in high-risk contacts (MeCOVID Trial): A structured summary of a study protocol for a randomised controlled trial.

Trials 2020 Jun 3;21(1):466. Epub 2020 Jun 3.

Clinical Pharmacology Department, Clinical Trial Unit, La Paz University Hospital - IdiPAZ, Paseo de la Castellana, 261, 28046, Madrid, Spain.

Objectives: Primary objective: to evaluate the efficacy of melatonin as a prophylactic treatment on prevention of symptomatic SARS-CoV-2 infection among healthcare workers at high risk of SARS-CoV-2 exposure. Secondary objectives: To evaluate the efficacy of melatonin as a prophylactic treatment on prevention of asymptomatic SARS-CoV-2 infection.To evaluate the efficacy of melatonin to prevent the development of severe COVID-19 in the participants enrolled in this study who develop SARS-CoV-2 infection along the trial. Read More

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http://dx.doi.org/10.1186/s13063-020-04436-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7267766PMC

High Flow 20% SQ IgG in Patients with Primary Immunodeficiency Disease.

Ann Allergy Asthma Immunol 2020 May 27. Epub 2020 May 27.

Division of Allergy and Immunology, Department of Pediatrics, University of South Florida. Electronic address:

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http://dx.doi.org/10.1016/j.anai.2020.05.022DOI Listing

Delivery of subcutaneous immunoglobulin by rapid "push" infusion for primary immunodeficiency patients in Manitoba: a retrospective review.

Allergy Asthma Clin Immunol 2020 13;16:34. Epub 2020 May 13.

3Section of Pediatric Allergy and Clinical Immunology, University of Manitoba, Winnipeg, Canada.

Background: Both intravenous and subcutaneous human immune globin G (IgG) replacement (IVIG and SCIG, respectively) reduce severe infection and increase serum IgG levels in primary immune deficiency disorder (PIDD) patients who require replacement. SCIG can be administered either with the aid of an infusion pump, or by patients or caregivers themselves, using butterfly needles and a syringe ("SCIG push"). SCIG offers advantages over IVIG, including higher steady state IgG levels, improved patient quality of life indicators, and decreased cost to the healthcare system, and for these reasons, SCIG has been increasingly used in Manitoba starting in 2007. Read More

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http://dx.doi.org/10.1186/s13223-020-00431-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7218483PMC

Brain control of humoral immune responses amenable to behavioural modulation.

Nature 2020 05 29;581(7807):204-208. Epub 2020 Apr 29.

Tsinghua-Peking Center for Life Sciences, Tsinghua University, Beijing, China.

It has been speculated that brain activities might directly control adaptive immune responses in lymphoid organs, although there is little evidence for this. Here we show that splenic denervation in mice specifically compromises the formation of plasma cells during a T cell-dependent but not T cell-independent immune response. Splenic nerve activity enhances plasma cell production in a manner that requires B-cell responsiveness to acetylcholine mediated by the α9 nicotinic receptor, and T cells that express choline acetyl transferase probably act as a relay between the noradrenergic nerve and acetylcholine-responding B cells. Read More

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http://dx.doi.org/10.1038/s41586-020-2235-7DOI Listing
May 2020
42.351 Impact Factor

Detection of circulating natural antibodies against CD25, MUC1, and VEGFR1 for early diagnosis of non-small cell lung cancer.

FEBS Open Bio 2020 May 11. Epub 2020 May 11.

First Hospital of Jilin University, Changchun, China.

We previously demonstrated that a deficiency of natural antibodies against CD25, Mucin 1 (MUC1), and vascular endothelial growth factor receptor 1 (VEGFR1) could contribute to high risk of non-small cell lung cancer (NSCLC). This study was designed to investigate whether natural IgG antibodies against POU domain class 5 transcription factor 1 (POU5F1), tumor necrosis factor-α (TNF-α), and the combination of CD25, VEGFR1, and MUC1 could play an anti-tumorigenic role against developing NSCLC. An ELISA was developed in-house to examine plasma IgG against peptide antigens derived from POU5F1, TNF-α, and a combination of peptide antigens derived from CD25, MUC1, and VEGFR1 in 211 patients with NSCLC and 200 healthy controls. Read More

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http://dx.doi.org/10.1002/2211-5463.12878DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7327917PMC

Hypogammaglobulinemia and Risk of Exacerbation and Mortality in Patients with COPD.

Int J Chron Obstruct Pulmon Dis 2020 16;15:799-807. Epub 2020 Apr 16.

Division of Emergencies and Critical Care, Department of Research and Development, Oslo University Hospital, Ullevål, Oslo, Norway.

Introduction: Chronic obstructive pulmonary disease (COPD) may, in some patients, be characterized by recurring acute exacerbations. Often these exacerbations are associated with airway infections. As immunoglobulins (Ig) are important parts of the immune defence against airway infections, the aim of this study was to relate the levels of circulating immunoglobulins to clinical features in unselected patients with COPD included in a Norwegian multicenter study. Read More

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http://dx.doi.org/10.2147/COPD.S236656DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7173948PMC

Plau/Plaur double-deficiency did not worsen lesion severity or vascular integrity after traumatic brain injury.

Neurosci Lett 2020 Jun 1;729:134935. Epub 2020 May 1.

A. I. Virtanen Institute for Molecular Sciences, University of Eastern Finland, PO Box 1627, FI-70211, Kuopio, Finland. Electronic address:

Binding of urokinase-type plasminogen activator receptor (uPAR) to its ligand uPA or to its plasma membrane partner, platelet-derived growth factor receptor β (PDGFRβ), promotes neuroprotection, cell proliferation, and angiogenesis. Following injury, single deficiency in uPA or uPAR leads in increased tissue loss and compromised vascular remodeling. We hypothesized that double-deficiency of uPAR (Plaur) and uPA (Plau) would result in increased lesion area and poor vascular integrity after traumatic brain injury (TBI). Read More

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http://dx.doi.org/10.1016/j.neulet.2020.134935DOI Listing

Defective formation of IgA memory B cells, Th1 and Th17 cells in symptomatic patients with selective IgA deficiency.

Clin Transl Immunology 2020 May 29;9(5):e1130. Epub 2020 Apr 29.

Department of Immunology Erasmus MC University Medical Center Rotterdam The Netherlands.

Objective: Selective IgA deficiency (sIgAD) is the most common primary immunodeficiency in Western countries. Patients can suffer from recurrent infections and autoimmune diseases because of a largely unknown aetiology. To increase insights into the pathophysiology of the disease, we studied memory B and T cells and cytokine concentrations in peripheral blood. Read More

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http://dx.doi.org/10.1002/cti2.1130DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7190975PMC

Genetic Variants of the MGAT5 Gene Are Functionally Implicated in the Modulation of T Cells Glycosylation and Plasma IgG Glycome Composition in Ulcerative Colitis.

Clin Transl Gastroenterol 2020 Apr;11(4):e00166

Instituto de Investigação e Inovação em Saúde (I3S)/Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), Porto, Portugal.

Objectives: The impact of genetic variants (single nucleotide polymorphisms [SNPs]) in the clinical heterogeneity of ulcerative colitis (UC) remains unclear. We showed that patients with UC exhibit a deficiency in MGAT5 glycogene transcription in intestinal T cells associated with a hyperimmune response. Herein, we evaluated whether MGAT5 SNPs might functionally impact on T cells glycosylation and plasma IgG glycome in patients with UC, as well as in UC clinical outcomes. Read More

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http://dx.doi.org/10.14309/ctg.0000000000000166DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7263653PMC

Activated PI3K-delta syndrome in an Egyptian pediatric cohort with primary immune deficiency.

Allergol Immunopathol (Madr) 2020 Apr 26. Epub 2020 Apr 26.

Department of Pediatrics, Faculty of Medicine, Zagazig University, Zagazig City, Al Sharqia Governorate, Egypt. Electronic address:

Background: Activated Phospho-Inositide 3 (PI3) Kinases Delta syndrome (APDS) can underlie primary immune deficiency. The prevalence and phenotypic characterization of these patients are not well described in Egypt.

Objectives: To describe patients with APDS in hospitalized children with recurrent respiratory tract infections with suspected primary immune deficiency. Read More

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http://dx.doi.org/10.1016/j.aller.2019.12.006DOI Listing

Blockade of BAFF Reshapes the Hepatic B Cell Receptor Repertoire and Attenuates Autoantibody Production in Cholestatic Liver Disease.

J Immunol 2020 Jun 24;204(12):3117-3128. Epub 2020 Apr 24.

Emory Vaccine Center, Division of Microbiology and Immunology, Yerkes National Primate Research Center, Emory University School of Medicine, Atlanta, GA 30329;

Defects in biliary transport proteins, MDR3 in humans and Mdr2 in mice, can lead to a spectrum of cholestatic liver disorders. Although B cell disorders and the aberrant Ab production are the leading extrahepatic manifestations of cholestatic liver diseases, the mechanism underlying this phenomenon is incompletely understood. Using mice with deficiency of Mdr2 that progressively develop cholestatic liver disease, we investigated the contributions of BAFF to aberrant IgG autoantibody production and hepatic fibrosis. Read More

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http://dx.doi.org/10.4049/jimmunol.1900391DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7306885PMC

Add-on or alone? Inhaled nebulized immunoglobulin reduces upper airway infections: 24 months of real-life experience.

Immunotherapy 2020 Apr 19;12(6):389-394. Epub 2020 Apr 19.

Children's Hospital, Skåne University Hospital & Lund University, Lund, Sweden.

Patients with antibody deficiencies might suffer from acute/chronic upper respiratory tract infections (URTI), despite apparently adequate levels of replacement IgG. This pilot study aimed to ascertain whether inhaled nebulized immunoglobulin (INHIG) could reduce the number of URTI episodes. Three young, male sibling patients with antibody deficiency who, despite ongoing treatment, were suffering from frequent URTI and recurrent otitis media. Read More

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http://dx.doi.org/10.2217/imt-2019-0136DOI Listing

HLA-A and -B Type and Haplotype Frequencies in IgG Subclass Deficiency Subgroups.

Arch Immunol Ther Exp (Warsz) 2020 Apr 20;68(3):14. Epub 2020 Apr 20.

Southern Iron Disorders Center, Birmingham, AL, USA.

We sought to determine whether HLA-A and -B type and haplotype frequencies differ between subgroups of adults with IgG subclass deficiency (IgGSD). We retrospectively compared type and haplotype frequencies of three subgroups of 269 unrelated adult IgGSD patients (70 subnormal IgG1; 121 subnormal IgG3; 78 subnormal IgG1/IgG3) and controls (1,321 for types; 751 for haplotypes). We selected types and haplotypes because their uncorrected frequencies differed significantly from controls in a previous adult IgGSD/common variable immunodeficiency cohort: A*24; B*14; B*35; B*40; B*49; B*50; B*58; B*62; A*01,B*08; A*02,B*44; A*02,B*60; A*03,B*07; A*03,B*14; A*03,B*44; A*31,B*40; and A*32,B*14. Read More

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http://dx.doi.org/10.1007/s00005-020-00572-8DOI Listing

Utilization of intravenous or subcutaneous immunoglobulins in secondary immune deficiency (ULTIMATE): A retrospective multicenter study.

Clin Immunol 2020 Jun 11;215:108419. Epub 2020 Apr 11.

Service de Médecine Interne, Centre de Référence Maladies Autoimmunes Systémiques Rares d'Ile de France, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Service de Médecine Interne, Hôpital Henri Mondor, AP-HP, Créteil, France. Electronic address:

Introduction: We conducted a retrospective multicenter cohort study of patients receiving Immunoglobulin replacement therapy (IgRT) for secondary immune deficiency (SID) during 2012.

Methods: Data were retrospectively collected from the first dose of Ig administered in 2012 to 1 year afterward in terms of the indication for IgRT, as well as efficacy and safety.

Results: In total, 16 hospitals participated in the study, and 368 patients were included. Read More

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http://dx.doi.org/10.1016/j.clim.2020.108419DOI Listing

Phenotypic variability in a child with Felty's syndrome: a case report.

BMC Pediatr 2020 Apr 7;20(1):153. Epub 2020 Apr 7.

Department of Rheumatology, Children's Hospital of Fudan University, 399 Wan-yuan Road, Shanghai, 201102, China.

Background: Felty's syndrome (FS) is characterized by the triad of rheumatoid arthritis (RA), splenomegaly and neutropenia. The arthritis is typically severe and virtually always associated with high-titer rheumatoid factor. The presence of persistent neutropenia is generally required to make the diagnosis. Read More

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http://dx.doi.org/10.1186/s12887-020-02054-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7137412PMC

AKT2 deficiency impairs formation of the BCR signalosome.

Cell Commun Signal 2020 Apr 6;18(1):56. Epub 2020 Apr 6.

Chongqing Key Laboratory of Child Infection and Immunity, Children's Hospital of Chongqing Medical University, Chongqing, China.

Background: AKT2 is one of the key molecules that involves in the insulin-induced signaling and the development of cancer. In B cells, the function of AKT2 is unclear.

Methods: In this study, we used AKT2 knockout mice model to study the role of AKT2 in BCR signaling and B cell differentiation. Read More

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http://dx.doi.org/10.1186/s12964-020-00534-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7133013PMC

Immunological and Clinical Phenotyping in Primary Antibody Deficiencies: a Growing Disease Spectrum.

J Clin Immunol 2020 May 2;40(4):592-601. Epub 2020 Apr 2.

Section of Rheumatology, Allergy and Immunology, Department of Internal Medicine, Yale University School of Medicine, 300 Cedar Street, New Haven, CT, 06520, USA.

Purpose: Although common variable immunodeficiency (CVID) is considered the most prevalent symptomatic primary antibody deficiency (PAD), there is a population with symptomatic PADs that do not meet criteria for CVID. We analyzed clinical and immunological profiles of patients with different PADs to better understand the differences and similarities between CVID and other PADs.

Methods: We extracted clinical and laboratory data of patients with PADs from electronic medical records. Read More

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http://dx.doi.org/10.1007/s10875-020-00773-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7260109PMC

[Effect of Lepidium meyenii (Maca) on neurotransmitter level and neuro-endocrine-immune network of deficiency-cold and deficiency-heat syndrome rats].

Zhongguo Zhong Yao Za Zhi 2020 Mar;45(5):1004-1010

School of Basic Medical Science, Beijing University of Chinese Medicine Beijing 100029, China.

The aim of this paper was to study the effect of Lepidium meyenii(Maca) on cyclic nucleotides, neurotransmitter levels and hypothalamic-pituitary-adrenal axis and immunization of deficiency-cold and deficiency-heat syndrome rats, in order to explore the cold and hot medicinal properties of Maca. SD rats were divided into blank group, deficiency-cold syndrome group, Cinnamomi Cortex of deficiency-cold syndrome(30 g·kg~(-1)) group, high and low-dose Maca groups(2.4, 1. Read More

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http://dx.doi.org/10.19540/j.cnki.cjcmm.20190916.401DOI Listing

Immunoglobulin G4 deficiency can be a new entity for primary recurrent miscarriage: Successful pregnancy in two cases after treatment with intravenous immunoglobulin.

Turk J Obstet Gynecol 2019 Dec 28;16(4):274-277. Epub 2020 Feb 28.

Fertility and Infertility Center, Isfahan, Iran.

Recurrent miscarriage is one of the complications of pregnancy in which the potential role of immunologic factors has already been mentioned. Here, two young women with recurrent miscarriage were consulted in the infertility center. The diagnosis of immunoglobulin G4 (IgG4) deficiency was made through the reduction of IgG4 Ig levels and normal total IgG titer. Read More

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http://dx.doi.org/10.4274/tjod.galenos.2019.02650DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7090269PMC
December 2019

No evidence for hypogammaglobulinemia in patients with paroxysmal nocturnal hemoglobinuria (PNH) chronically treated with ravulizumab.

PLoS One 2020 27;15(3):e0230869. Epub 2020 Mar 27.

Department of Hematology, West German Cancer Center, University Hospital Essen, Essen, Germany.

Introduction: Ravulizumab (ALXN1210) is a long-lasting recycling IgG monoclonal antibody with an increased affinity for the neonatal Fc receptor (FcRn). The FcRn is essential for regulating IgG homeostasis. Saturation of the FcRn pathway is seen under high IgG doses as they compete with endogenous IgG to bind the FcRn by their Fc regions, resulting in enhanced IgG clearance. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0230869PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7101163PMC

Impaired proteolysis by SPPL2a causes CD74 fragment accumulation that can be recognized by anti-CD74 autoantibodies in human ankylosing spondylitis.

Eur J Immunol 2020 Mar 21. Epub 2020 Mar 21.

Center for Translational Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.

Ankylosing spondylitis (AS) is associated with autoantibody production to class II MHC-associated invariant chain peptide, CD74/CLIP. In this study, we considered that anti-CD74/CLIP autoantibodies present in sera from AS might recognize CD74 degradation products that accumulate upon deficiency of the enzyme signal peptide peptidase-like 2A (SPPL2a). We analyzed monocytes from healthy controls (n = 42), psoriatic arthritis (n = 25), rheumatoid arthritis (n = 16), and AS patients (n = 15) for SPPL2a enzyme activity and complemented the experiments using SPPL2a-sufficient and -deficient THP-1 cells. Read More

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http://dx.doi.org/10.1002/eji.201948502DOI Listing

Recurrent Herpes Simplex Virus 2 Lymphocytic Meningitis in Patient with IgG Subclass 2 Deficiency.

Emerg Infect Dis 2020 Apr;26(4):748-750

We report a case of a patient with a lifetime history of 8 episodes of recurrent lymphocytic meningitis. Our findings suggest that susceptibility to recurrent lymphocytic meningitis might be caused by low serum IgG subclass 2 immunodeficiency. Read More

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http://dx.doi.org/10.3201/eid2604.190406DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7101106PMC

Hereditary Deficiency of the Second Component of Complement: Early Diagnosis and 21-Year Follow-Up of a Family.

Medicina (Kaunas) 2020 Mar 10;56(3). Epub 2020 Mar 10.

Internal Medicine, Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, 20122 Milan, Italy.

Complement deficiencies are rare and often underdiagnosed primary immunodeficiencies that may be associated with invasive bacterial diseases. Serious infections with encapsulated organisms (mainly Streptococcus pneumoniae, but also Neisseria meningitides and Haemophilus influenzae type B) are frequent in patients with a deficiency of the second component of complement (C2), but no data are available on long-term follow-up. This study aimed to evaluate the long-term clinical outcome and the importance of an early diagnosis and subsequent infection prophylaxis in C2 deficiency. Read More

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http://dx.doi.org/10.3390/medicina56030120DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7143546PMC

Effects of vitamin A supplementation in the diet of breeding geese on offspring intestinal tissue morphology and immune performance.

Asian-Australas J Anim Sci 2020 Mar 12. Epub 2020 Mar 12.

College of Animal Science and Technology, Yangzhou University, Yangzhou, Jiangsu Province, 225009, China.

Objective: The effects of maternal and offspring dietary vitamin A (VA) supplementation on early body weight, digestive tract function and immune function in goslings were studied.

Methods: Yangzhou geese (180 d old) were randomly divided into 5 experimental groups of 15 females and 3 males (the males were kept until for slaughter). Eggs were collected for hatching during the peak laying period. Read More

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http://dx.doi.org/10.5713/ajas.19.0890DOI Listing

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

[A case report of BCL11B mutation induced neurodevelopmental disorder and literature review].

Zhonghua Er Ke Za Zhi 2020 Mar;58(3):223-227

Department of Rheumatology and Immunology, Children's Hospital of Chongqing Medical University, Chongqing Key Laboratory of Child Infection and Immunity, Chongqing 400014, China.

To analyze the clinical , immunological and genetic features of a child with BCL11B mutation induced neurodevelopmental disorder. The clinical data and genetic test of a child with BCL11B mutation hospitalized in the Department of Rheumatology and Immunology in Children's Hospital of Chongqing Medical University in December 2018 were extracted and analyzed. The literature was searched with "BCL11B mutation" and "immunodeficiency 49" as key words in Chinese databases and Pubmed until January 2019 was reviewed. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2020.03.012DOI Listing

Association between neutropenia and IgG antineutrophil antibodies in a case of deficiency due to two novel mutations.

Clin Case Rep 2020 Feb 25;8(2):313-316. Epub 2019 Dec 25.

Hans Christian Andersen's Children Hospital Odense University Hospital Odense Denmark.

This case suggests a mechanistic rationale for the clinical efficacy of intravenous immunoglobulins (IVIG) in treating CD40 ligand (CD40L) deficiency associated neutropenia as it is the first reported instance of free and cell-bound antineutrophil antibodies in a case of CD40L deficiency, accompanied by a prolonged and clinically severe neutropenia. Read More

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http://dx.doi.org/10.1002/ccr3.2621DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7044354PMC
February 2020

Iron and prebiotic fortified flour improves the immune function of iron deficient women of childbearing age.

Pak J Pharm Sci 2020 Jan;33(1(Supplementary)):253-261

University Institute of Diet and Nutritional Sciences, The University of Lahore, Islamabad Campus, Islamabad, Pakistan.

Micronutrient deficiencies (MNDs) are common worldwide, in both developing as well as developed countries. MNDs such as Iron Deficiency not only compromise the nutritional status of individuals but can also put them at an increased risk of developing various other diseases by negatively affecting their immunity. The objective of the current research was to determine the effects of prebiotics and iron fortificants on various immunoglobulins among iron deficient women belonging to childbearing age. Read More

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January 2020

Membranoproliferative glomerulonephritis related to a streptococcal infection in a girl with IgA deficiency: a case report.

BMC Nephrol 2020 Feb 27;21(1):68. Epub 2020 Feb 27.

Department of Pediatrics, Kindai University Faculty of Medicine, 377-2 Ohno-higashi, Osaka-sayama, Osaka, 589-8511, Japan.

Background: IgA deficiency associated with glomerulonephritis is rare. In particular, there is no prior report regarding the association between IgA deficiency and membranoproliferative glomerulonephritis (MPGN) in children. Herein, we describe the case of a 5-year-old girl with selective IgA deficiency and MPGN. Read More

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http://dx.doi.org/10.1186/s12882-020-01735-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7045629PMC
February 2020

Clinical and laboratory characteristics of clozapine-treated patients with schizophrenia referred to a national immunodeficiency clinic reveals a B-cell signature resembling common variable immunodeficiency (CVID).

J Clin Pathol 2020 Feb 24. Epub 2020 Feb 24.

Immunodeficiency Centre for Wales, University Hospital of Wales, Cardiff, UK.

Aims: An association between antibody deficiency and clozapine use in individuals with schizophrenia has recently been reported. We hypothesised that if clozapine-associated hypogammaglobulinaemia was clinically relevant this would manifest in referral patterns.

Methods: Retrospective case note review of patients referred and assessed by Immunology Centre for Wales (ICW) between January 2005 and July 2018 with extraction of clinical and immunological features for individuals with diagnosis of schizophrenia-like illness. Read More

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http://dx.doi.org/10.1136/jclinpath-2019-206235DOI Listing
February 2020

Unusual phenotype in patients with a hypomorphic mutation in the DCLRE1C gene: IgG hypergammaglobulinemia with IgA and IgE deficiency.

Clin Immunol 2020 Apr 21;213:108366. Epub 2020 Feb 21.

Immunology Clinic, Soroka University Medical Center, and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel.

The nuclease Artemis is a enzyme for V(D)J recombination allowing for the creation of T and B lymphocytes as well as for the repair of radiation-induced DNA double strand breaks encoded by the DCLRE1C gene. Artemis-null mutations are a known cause of severe combined immunodeficiencies (SCIDs) with radiosensitivity. Hypomorphic mutations in Artemis have been reported to cause a "leaky SCID" phenotype, typically with hypogammaglobulinemia. Read More

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http://dx.doi.org/10.1016/j.clim.2020.108366DOI Listing

Effect of rituximab or tumour necrosis factor inhibitors on lung infection and survival in rheumatoid arthritis-associated bronchiectasis.

Rheumatology (Oxford) 2020 Feb 17. Epub 2020 Feb 17.

Department of Rheumatology, Queen Elizabeth Hospital, GatesheadUK.

Objective: To evaluate rituximab (RTX) in patients with RA-associated bronchiectasis (RA-BR) and compare 5-year respiratory survival between those treated with RTX and TNF inhibitors (TNFi).

Methods: A retrospective observational cohort study of RA-BR in RTX or TNFi-treated RA patients from two UK centres over 10 years. BR was assessed using number of infective exacerbation/year. Read More

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http://dx.doi.org/10.1093/rheumatology/kez676DOI Listing
February 2020

Infection in Humans With STAT3-Deficiency Is Associated With Defective Interferon-Gamma and Th17 Responses.

Front Immunol 2020 28;11:38. Epub 2020 Jan 28.

Unité des Aspergillus, Institut Pasteur, Paris, France.

In humans, loss-of-function mutation in the gene is frequently associated with susceptibility to bacterial as well as fungal infections including aspergillosis, although its pathogenesis remains largely unknown. In the present study, we investigated the immune responses obtained after stimulation with in STAT3-deficient patients. conidial killing efficiencies of both monocytes and neutrophils isolated from whole blood samples of STAT3-deficient patients were not different compared to those of healthy controls. Read More

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http://dx.doi.org/10.3389/fimmu.2020.00038DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6997434PMC
January 2020

Influence of the 2012 European Guidelines in Diagnosis and Follow-up of Coeliac Children With Selective IgA Deficiency.

J Pediatr Gastroenterol Nutr 2020 Jul;71(1):59-63

Section of Pediatric Gastroenterology, Hospital Universitario Puerta de Hierro, Madrid, Spain.

Objectives: The aim of the study was to describe diagnostic criteria used in children with coeliac disease (CD) and selective IgA deficiency; to determine if the publication of the 2012 ESPGHAN criteria prompted any changes; to evaluate the evolution of serological markers.

Methods: Multicenter, retrospective, descriptive study of a cohort of children under 15 years with selective IgA deficiency diagnosed with CD (January 2006 to December 2016). Demographic, clinical, genetic, histological and IgG-based antibodies were collected at diagnosis and follow-up. Read More

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http://dx.doi.org/10.1097/MPG.0000000000002634DOI Listing

Correlations Among Subcutaneous Immunoglobulin Dosage, Immunoglobulin G Serum Pre-infusional Levels and Body Mass Index in Primary Antibody Deficiency Patients: A Pooled Analysis from the SHIFT/IBIS Studies.

Clin Drug Investig 2020 Mar;40(3):279-286

Department of Translational Medical Sciences and Center for Basic and Clinical Immunology Research (CISI), University of Naples Federico II, Via S. Pansini 5, 80131, Naples, Italy.

BACKGROUND AND OBJECTIVE: In recent years, two Italian non-interventional studies evaluated subcutaneous immunoglobulin (SCIG) treatment in patients affected by primary antibody deficiency (PAD). The SHIFT study considered patients who were treated with intravenous immunoglobulin (IVIG) or SCIG 16% (Vivaglobin) and then replaced this therapy with weekly treatments of SCIG 20% (Hizentra). The IBIS study evaluated patients previously taking a weekly SCIG 20% regimen, who instead began therapy with biweekly SCIG 20% to assess the correlation between the dose of immunoglobulin G (IgG) administered and the body mass index (BMI) of patients, determine if there is a need for dosage adjustments on a BMI basis, and identify the predictors of serum IgG trough levels in our cohort. Read More

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http://dx.doi.org/10.1007/s40261-020-00885-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035227PMC

BAFF Produced by Neutrophils and Dendritic Cells Is Regulated Differently and Has Distinct Roles in Antibody Responses and Protective Immunity against West Nile Virus.

J Immunol 2020 Mar 7;204(6):1508-1520. Epub 2020 Feb 7.

Department of Immunology, University of Washington, Seattle, WA 98109; and.

B cell activating factor (BAFF) is essential for B cells to develop and respond to Ags. Dysregulation of BAFF contributes to the development of some autoimmune diseases and malignancies. Little is known about when, where, and how BAFF is produced in vivo and about which BAFF-producing cells contribute to B cell responses. Read More

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http://dx.doi.org/10.4049/jimmunol.1901120DOI Listing

CMTM7 plays key roles in TLR-induced plasma cell differentiation and p38 activation in murine B-1 B cells.

Eur J Immunol 2020 Jun 18;50(6):809-821. Epub 2020 Feb 18.

Department of Immunology, School of Basic Medical Sciences, Peking University Health Science Center, NHC Key Laboratory of Medical Immunology (Peking University), Beijing, China.

Terminal differentiation of B cells into antibody-secreting cells is the foundation of humoral immune response. B-1 cells, which are different from B-2 cells, preferentially differentiate into plasma cells. CMTM7 is a MARVEL-domain-containing membrane protein predominantly expressed in B cells that plays an important role in B-1a cell development. Read More

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http://dx.doi.org/10.1002/eji.201948363DOI Listing

Intravenous immunoglobulin for preventing infection in preterm and/or low birth weight infants.

Cochrane Database Syst Rev 2020 01 29;1:CD000361. Epub 2020 Jan 29.

Scarborough, ON, Canada, M1S 1W9.

Background: Nosocomial infections continue to be a significant cause of morbidity and mortality among preterm and/or low birth weight (LBW) infants. Preterm infants are deficient in immunoglobulin G (IgG); therefore, administration of intravenous immunoglobulin (IVIG) may have the potential of preventing or altering the course of nosocomial infections.

Objectives: To use systematic review/meta-analytical techniques to determine whether IVIG administration (compared with placebo or no intervention) to preterm (< 37 weeks' postmenstrual age (PMA) at birth) or LBW (< 2500 g birth weight) infants or both is effective/safe in preventing nosocomial infection. Read More

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http://dx.doi.org/10.1002/14651858.CD000361.pub4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6988992PMC
January 2020

Low Levels of Vitamin D Promote Memory B Cells in Lupus.

Nutrients 2020 Jan 22;12(2). Epub 2020 Jan 22.

Lerner Research Institute, Department of Inflammation and Immunity, Lerner Research Institute, Cleveland Clinic, Cleveland, OH 44195, USA.

: Vitamin D deficiency is a known risk factor for Systemic Lupus Erythematosus (SLE), yet clinical trials have not demonstrated efficacy and few studies have utilized lupus models to understand the mechanism underlying this relationship. The mouse is a spontaneous model of lupus and Sjögren's syndrome, characterized by increased Th17 cells and peripheral B cell expansion. Vitamin D3 has anti-inflammatory properties, reduces Th17 cells and impairs B cell differentiation/activation. Read More

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http://dx.doi.org/10.3390/nu12020291DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7070834PMC
January 2020
3.148 Impact Factor