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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

Int Immunol 2019 Feb 6. Epub 2019 Feb 6.

Department of Microbiology and Immunology, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan.

Ten-eleven translocation (TET) proteins regulate DNA methylation and gene expression by converting 5-methylcytosine (5mC) to 5-hydroxymethylcytosine (5hmC). Although Tet2/Tet3 deficiency has been reported to lead to myeloid cell, B cell, and invariant natural killer T (iNKT) cell malignancy, the effect of TET on regulatory T cells (Tregs) has not been elucidated. We found that Tet2/Tet3 deficiency in Tregs led to lethal hyperproliferation of CD4+Foxp3+ T cells in the spleen and mesenteric lymph nodes after 5 months of age. Read More

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

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

Immunity 2019 Jan 21. Epub 2019 Jan 21.

Immunobiology Laboratory, Fundación Centro Nacional de Investigaciones Cardiovasculares Carlos III (CNIC), Melchor Fernández Almagro 3, Madrid 28029, Spain. Electronic address:

Production of interleukin-17 (IL-17) and IL-22 by T helper 17 (Th17) cells and group 3 innate lymphoid cells (ILC3s) in response to the gut microbiota ensures maintenance of intestinal barrier function. Here, we examined the mechanisms whereby the immune system detects microbiota in the steady state. A Syk-kinase-coupled signaling pathway in dendritic cells (DCs) was critical for commensal-dependent production of IL-17 and IL-22 by CD4 T cells. Read More

J Allergy Clin Immunol 2019 Jan 29. Epub 2019 Jan 29.

Research Institute of Internal Medicine, Division of Surgery, Inflammatory Diseases and Transplantation, Oslo University Hospital, Rikshospitalet, Norway; Institute of Clinical Medicine, University of Oslo, Norway; Norwegian PSC Research Center, Division of Surgery, Inflammatory Diseases and Transplantation, Oslo University Hospital Rikshospitalet, Norway; Section of Gastroenterology, Department of Transplantation Medicine, Oslo University Hospital Rikshospitalet, Norway.

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

J Allergy Clin Immunol 2019 Jan 18. Epub 2019 Jan 18.

Department of Gastroenterology and Hepatology, University of Alabama at Birmingham, Birmingham, Ala. Electronic address:

Haematologica 2019 Jan 17. Epub 2019 Jan 17.

UHN

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

Clin Chem 2019 Jan 9. Epub 2019 Jan 9.

Research Centre for Metrological Traceability in Laboratory Medicine (CIRME), University of Milan, Milan, Italy.

Background: To identify an IgA deficiency, the availability of reliable IgA lower reference limits is essential, especially in pediatrics. In this study, we reported the results of an intercomparison study aimed to verify the status of standardization of IgA measurements using 11 commercially available measuring systems (MSs).

Methods: After confirming its commutability, the ERM- DA470k/IFCC reference material was used for the trueness evaluation of IgA MSs. Read More

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

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

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

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

Front Pediatr 2018 3;6:377. Epub 2018 Dec 3.

Division of Pediatric Rheumatology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Autoinflammatory diseases (AID) are diseases of the innate immune system, characterized by recurrent episodes of localized or systemic inflammation. Vasculitis may accompany AID. The causes of the association of vasculitis with monogenic AID are still debated. Read More

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

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

J Ayub Med Coll Abbottabad 2018 Jul-Sep;30(3):372-376

Nusrat Riaz College, Quetta-Pakistan.

Background: Iron deficiency anaemia (IDA) is common in Pakistani population and an important cause of morbidity. Celiac disease (CD) is an important contributor and its diagnosis is frequently missed especially if present in atypical/non-diarrhoeal form. Non-responders to iron replacement therapy typically raises suspicion of celiac disease as underlying cause. Read More

Gut Microbes 2018 Nov 18:1-5. Epub 2018 Nov 18.

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

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

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

Schizophr Res 2018 Nov 8. Epub 2018 Nov 8.

Department of Psychiatry, Columbia University Medical Center, New York, NY 10032, United States of America.

Background: Immunological, nutritional, and microbial factors have been implicated in the pathophysiology of schizophrenia, but the interrelationship among measures is understudied. In particular, an increase in the levels of the pro-inflammatory cytokine interleukin-6 (IL-6) is associated with all phases of the illness, and may be associated with other inflammatory markers. Vitamin D is a modulator of the immune system, and LPS antibodies are an indirect measure of gut barrier function. Read More

Clin Transl Allergy 2018 2;8:44. Epub 2018 Nov 2.

5Allergy Unit, Hospital Universitario de Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria, Spain.

Background And Objective: Severe alpha1 antitrypsin deficiency has been clearly associated with pulmonary emphysema, but its relationship with bronchial asthma remains controversial. Some deficient alpha 1 antitrypsin (AAT) genotypes seem to be associated with asthma development. The objective of this study was to analyze the distribution of AAT genotypes in asthmatic patients allergic to house dust mites (HDM), and to asses a possible association between these genotypes and severe asthma. Read More

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

Nutrients 2018 Nov 5;10(11). Epub 2018 Nov 5.

Faculty of Commodity Science, Poznań University of Economics and Business, 61-875 Poznań, Poland.

The importance of a gluten-free diet (GFD) in the treatment of celiac disease and other gluten-related disorders is undisputable. However, strict GFD often lead to nutritional imbalances and, therefore, to deficiencies. One of the most common deficiencies from a GFD are an insufficient amount of Ca, Fe, Mg, and Zn. Read More

Ann Allergy Asthma Immunol 2018 Nov 7;121(5):619-620. Epub 2018 Jul 7.

Department of Pulmonary and Critical Care, Allergy/Immunology Fellowship, University Hospitals, Cleveland Medical Center, Cleveland, Ohio; Allergy/Immunology Associates Inc., Mayfield Heights, Ohio.

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

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

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

Eur J Pediatr 2019 Jan 29;178(1):51-60. Epub 2018 Sep 29.

Pediatric Unit, Department of Woman, Child and Urologic Diseases, University of Bologna, S. Orsola-Malpighi Hospital, Bologna, Italy.

Selective IgA deficiency is defined as absolute or partial when serum IgA level is < 7 mg/dl or 2 SD below normal for age, respectively. Few data are available on partial selective IgA deficiency, as probably most children with low serum IgA are seldom referred to a specialist clinic in common pediatric practice. The aim of our study was to better define the profile of both symptomatic forms and their clinical outcome in a pediatric immunology setting. Read More

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

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

Nat Immunol 2018 Nov 24;19(11):1224-1235. Epub 2018 Sep 24.

Department of Immunology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Dendritic cells (DCs) play an integral role in regulating mucosal immunity and homeostasis, but the signaling network mediating this function of DCs is poorly defined. We identified the noncanonical NF-κB-inducing kinase (NIK) as a crucial mediator of mucosal DC function. DC-specific NIK deletion impaired intestinal immunoglobulin A (IgA) secretion and microbiota homeostasis, rendering mice sensitive to an intestinal pathogen, Citrobacter rodentium. Read More

Turk J Gastroenterol 2018 07;29(4):419-426

Dr Carol Davila Central Military Emergency University Hospital, Bucharest, Romania.

Background/aims: Despite its high prevalence, affecting about 1% of the general population, celiac disease (CD) remains heavily underdiagnosed. Among the reasons for underdiagnosis, awareness among medical professionals has been little studied. The aim of this survey was to determine physicians' knowledge in regard to CD in adult patients. Read More

Clin Immunol 2018 Dec 19;197:139-153. Epub 2018 Sep 19.

College of Pharmacy, Oregon State University, Corvallis, OR, United States. Electronic address:

Common variable immunodeficiency (CVID), the most common symptomatic primary antibody deficiency, is accompanied in some patients by a duodenal inflammation and malabsorption syndrome known as CVID enteropathy (E-CVID).The goal of this study was to investigate the immunological abnormalities in CVID patients that lead to enteropathy as well as the contribution of intestinal microbiota to this process.We found that, in contrast to noE-CVID patients (without enteropathy), E-CVID patients have exceedingly low levels of IgA in duodenal tissues. Read More

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

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

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

Adv Exp Med Biol 2018 ;1108:99-106

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

Respiratory tract infections in children are one of the most common causes for medical consultations. When the infections are of recurring nature, they are a major reason for the diagnostics for primary immunodeficiency that is in about 65% of cases underlain by disorders of humoral immunity. This study seeks to retrospectively evaluate the history of recurrent respiratory tract infections in children with humoral disorders and the associations among deficiencies in the immune system components. Read More

Cureus 2018 Jun 30;10(6):e2900. Epub 2018 Jun 30.

Internal Medicine, Icahn School of Medicine at Mount Sinai Queen Hospital Center, New York, USA.

When very low or undetectable high density lipoprotein (HDL)-cholesterol (HDL-C) is encountered in clinical practice, a paraproteinemia should be suspected in the absence of genetic or more obvious secondary causes. We reported a case of artifactually low HDL-C in a 68-year-old man with a past medical history of vitamin B12 deficiency. Lipid panel showed total cholesterol (TC) 144 mg/dl, triglycerides (TG) 79 mg/dl, HDL-C 5 mg/dl, and low density lipoprotein (LDL) 123 mg/dl. Read More

Transfusion 2018 Oct 4;58(10):2320-2325. Epub 2018 Sep 4.

Department of Medicine, Michael G. DeGroote School of Medicine, McMaster University, Hamilton, Ontario, Canada.

Background: Transfusion-associated anaphylaxis has been associated with anti-immunoglobulin A (anti-IgA) of IgG class in patients with IgA deficiency. In recent years, however, the frequency and clinical impact of this syndrome has been questioned. We present a case of recurrent red blood cell (RBC) transfusion-associated anaphylactoid reactions (rigors, hypertension, transient monocytopenia, and neutropenia) associated with anti-IgA. Read More

Pediatr Crit Care Med 2018 09;19(9S Suppl 1):S163-S169

Division of Pathology and Laboratory Medicine, Children's National Health System, Washington, DC.

Objectives: To present the recommendations and supporting literature for selection and processing of RBC products in critically ill children developed by the Pediatric Critical Care Transfusion and Anemia Expertise Initiative.

Design: Consensus conference series of international, multidisciplinary experts in RBC transfusion management of critically ill children METHODS:: The panel of 38 experts developed evidence-based, and when evidence was lacking, expert-based clinical recommendations as well as research priorities for RBC transfusions in critically ill children. The RBC processing subgroup included five experts. Read More

Clin Exp Rheumatol 2018 Sep-Oct;36(5):934. Epub 2018 Jul 19.

Institute of Infection, Immunity and Inflammation, University of Glasgow, UK; and Rheumatology Department, Army Shared Fund "NIMTS" Hospital, Athens, Greece.

Zh Nevrol Psikhiatr Im S S Korsakova 2018 ;118(5. Vyp. 2):64-68

Burdenko Voronezh State Medical University, Voronezh, Russia.

Aim: To study serological and genetic markers of gluten intolerance in children and teenagers with autism spectrum disorders (ASD) and Down's syndrome (DS).

Material And Methods: Thirty-three children with ASD (group 1) and 8 with DS (group 2), aged from 2.5 to 15 years, were examined. Read More

Vet Dermatol 2018 Dec 23;29(6):482-e162. Epub 2018 Aug 23.

Department of Clinical Sciences, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, 300 West Drake Road, Fort Collins, CO, 80525, USA.

Background: Juvenile onset generalized demodicosis (JOGD) is thought to occur due to immunological abnormalities and is over-represented in pit bull terrier-type dogs.

Animals: Twelve pit bull terrier-type dogs with JOGD and 12 age-matched healthy pit bull terrier-type dogs.

Objective: To investigate immunological differences between age-matched healthy and JOGD pit bull terrier-type dogs by flow cytometry, multiplex, molecular and serological assays. Read More

Intern Med 2019 Jan 10;58(1):145-146. Epub 2018 Aug 10.

Department of General Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan.

Int J Environ Res Public Health 2018 08 9;15(8). Epub 2018 Aug 9.

Pediatric Clinic, Department of Medical and Surgical Sciences, Università degli Studi di Perugia, 06132 Perugia, Italy.

: Imunoglobulin A (IgA) deficiency (IgAD) is the most common form of primary immunodeficiency in Western countries. There have been several reports on IgAD complicated by glomerulonephritis in adults, but only very few cases of IgAD with nephropathy have been reported in children. We present two cases of IgAD with relapsing nephrotic syndrome in pediatric age. Read More

Biotechniques 2018 Aug;65(2):71-77

University of Alabama at Birmingham, Department of Microbiology, Birmingham, AL, USA.

Aims: IgA nephropathy, the most common primary glomerulonephritis worldwide, is characterized by glomerular deposition of galactose-deficient IgA1 and elevated serum levels of this IgA1 glycoform. Current ELISA methods lack sensitivity to assess galactose deficiency using small amounts of IgA1, which limits studies in primary cells due to modest IgA1 production in isolated peripheral-blood lymphocytes.

Methods: Lectin from Helix pomatia was conjugated to biotin or acridinium ester and used in ELISA to detect galactose deficiency of IgA1 using small amounts of IgA1. Read More

Avicenna J Med Biotechnol 2018 Jul-Sep;10(3):192-195

Department of Pediatric Immunology, Faculty of Medicine, Ege University, Izmir, Turkey.

Background: The Transmembrane Activator and Calcium modulator ligand Interactor (TACI), encoded by gene, is mutated in some patients with Common Variable Immunodeficiency (CVID) and IgA Deficiency (IgAD). The purpose of the study was to investigate for the first time in Turkish patients the prevalence of alterations in CVID, selective and partial IgAD patients.

Methods: Forty two CVID, 36 selective IgAD, 34 partial IgAD and 25 healthy controls were included. Read More

Mucosal Immunol 2019 01 7;12(1):85-96. Epub 2018 Aug 7.

Mucosal Immunobiology Section, Laboratory of Molecular Immunology, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, MD, USA.

Despite studies indicating the effects of IL-21 signaling in intestinal inflammation, its roles in intestinal homeostasis and infection are not yet clear. Here, we report potent effects of commensal microbiota on the phenotypic manifestations of IL-21 receptor deficiency. IL-21 is produced highly in the small intestine and appears to be critical for mounting an IgA response against atypical commensals such as segmented filamentous bacteria and Helicobacter, but not to the majority of commensals. Read More

Zhonghua Er Ke Za Zhi 2018 Aug;56(8):628-632

Department of Rheumatology and Immunology, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Child Infection and Immunity, Chongqing 400014, China.

To investigate the clinical, immunological, and molecular manifestations of nuclear factor kappa-B subunit 2 (NFκB2) gene mutation associated common variable immunodeficiency (CVID) . A 14-month-old boy diagnosed with NFκB2-mutated CVID was admitted into Children's Hospital of Chongqing Medical University in December 2015. The clinical manifestations, biochemical tests, immunological function, molecular features, treatment, and follow-up of the patient were analyzed. Read More