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    4149 results match your criteria Immunoglobulin A Deficiency

    1 OF 83

    MST1 deficiency promotes B cell responses by CD4(+) T cell-derived IL-4, resulting in hypergammaglobulinemia.
    Biochem Biophys Res Commun 2017 May 17. Epub 2017 May 17.
    Division of Life Sciences, College of Life Sciences and Biotechnology, Korea University, Seoul 02841, Republic of Korea. Electronic address:
    MST1 deficiency causes T and B cell lymphopenia, resulting in combined immunodeficiency. However, MST1-deficient patients also exhibit autoimmune-like symptoms such as hypergammaglobulinemia and autoantibody production. Recent studies have shown that the autoimmune responses observed in MST1-deficient patients were most likely attributable to defective regulatory T (Treg) cells instead of intrinsic signals in MST1-lacking B cells. Read More

    TNF receptor-associated periodic syndrome (TRAPS) mimicking chronic spontaneous urticaria.
    J Eur Acad Dermatol Venereol 2017 May 13. Epub 2017 May 13.
    Klinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Leipzig.
    Tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS) is a rare (about one per million) autosomal dominant autoinflammatory disease being typically characterized by recurrent fever episodes accompanied by variable gastrointestinal, musculoskeletal, neurological, lymphoid, urogenital and skin symptoms (table 1) [1-3]. We report on a 21 year old non atopic male with prediagnosed IgA deficiency, Marfan syndrome and hypothyroidism who first presented in our university allergy centre with three year history of antihistamine resistant, only slightly itching urticarial rash being accompanied by fatigue. Histology showed superficial perivascular lymphocytic dermatitis with eosinophils and intravascular neutrophil granulocytes. Read More

    Collagenous Gastritis in a Young Female With IgA Deficiency.
    Gastroenterology Res 2017 Apr 19;10(2):126-127. Epub 2017 Apr 19.
    UHS Wilson Medical Center, Binghamton, NY, USA.
    Collagenous gastritis, without colonic involvement, is exceptionally rare. It is not known to be associated with IgA deficiency and scleroderma. This is the first report of this type of association. Read More

    Recurrent respiratory tract infections (RRTI) in the elderly: A late onset mild immunodeficiency?
    Clin Immunol 2017 May 6;180:111-119. Epub 2017 May 6.
    Department of Infectious Diseases, Leiden University Medical Center, Leiden, The Netherlands.
    Elderly with late-onset recurrent respiratory tract infections (RRTI) often have specific anti-polysaccharide antibody deficiency (SPAD). We hypothesized that late-onset RRTI is caused by mild immunodeficiencies, such as SPAD, that remain hidden through adult life. We analyzed seventeen elderly RRTI patients and matched controls. Read More

    Acne conglobata in a long-term survivor with trisomy 13, accompanied by selective IgM deficiency.
    Am J Med Genet A 2017 May 7. Epub 2017 May 7.
    Department of Dermatology, Red Cross Sendai Hospital, Sendai, Japan.
    Trisomy 13 (T13) is a congenital chromosomal disorder that is usually fatal within 2 years of birth, and only a few patients have been reported to reach adolescence. Here, we report a male long-term survivor of T13, currently 15 years of age, with a several-year history of extensive acne conglobata (AC) with abscesses on the face and neck. Methicillin-resistant Staphylococcus aureus was consistently isolated from the pustular lesions. Read More

    A Case Report of Hypoglycemia and Hypogammaglobulinemia: DAVID syndrome in a patient with a novel NFKB2 mutation.
    J Clin Endocrinol Metab 2017 May 3. Epub 2017 May 3.
    Division of Allergy, Immunology & Rheumatology, Departments of Pediatrics, Stanford University School of Medicine, Stanford, California.
    Context: DAVID syndrome (Deficient Anterior pituitary with Variable Immune Deficiency) is a rare disorder in which children present with symptomatic ACTH deficiency preceded by hypogammaglobulinemia from B-cell dysfunction with recurrent infections, termed common variable immunodeficiency (CVID). Subsequent whole exome sequencing studies have revealed germline heterozygous C-terminal mutations of NFKB2 as either a cause of DAVID syndrome or of CVID without clinical hypopituitarism. However, to the best of our knowledge there have been no cases in which the endocrinopathy has presented in the absence of a prior clinical history of CVID. Read More

    Implementation of National Institute for Health and Care Excellence (NICE) guidance to measure immunoglobulin A with all coeliac screens: can an affordable solution be devised?
    Clin Exp Immunol 2017 May 2. Epub 2017 May 2.
    Department of Immunology, Eastbourne Hospital, Eastbourne, East Sussex, UK.
    There has been a dramatic increase in requests for coeliac disease (CD) serological screening using immunoglobulin (Ig)A tissue transglutaminase antibodies (IgA-tTG). Recently, the UK National Institute for Health and Care Excellence has revised its guidance, recommending that total IgA should also be measured in all samples. This is justified, as false-negative results may occur with IgA deficiency. Read More

    Syndrome Differentiation of IgA Nephropathy Based on Clinicopathological Parameters: A Decision Tree Model.
    Evid Based Complement Alternat Med 2017 26;2017:2697560. Epub 2017 Mar 26.
    Renal Division, Guangdong Provincial Hospital of Chinese Medicine, Guangzhou 510120, China.
    Background. IgA nephropathy is the most common cause of primary glomerulonephritis in China, and Traditional Chinese Medicine (TCM) is a vital treatment strategy. However, not all doctors prescribing TCM medicine have adequate knowledge to classify the syndrome accurately. Read More

    Lack of Utility of Anti-tTG IgG to Diagnose Celiac Disease When Anti-tTG IgA Is Negative.
    J Pediatr Gastroenterol Nutr 2017 May;64(5):726-729
    *Division of Pediatric Gastroenterology and Hepatology, College of Medicine †Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota ‡Division of Biomedical Statistics and Informatics §Division of Clinical Biochemistry and Immunology, Department of Laboratory Medicine and Pathology.
    Objectives: Guidelines for diagnosing celiac disease (CD) recommend initial testing with a highly sensitive serologic test for anti-tissue transglutaminase immunoglobulin A antibodies (tTG IgA). When the probability of CD is high, IgA deficiency should be considered. The 2 approaches to address this include measuring "both tTG IgA and tTG IgG" or measuring "total IgA. Read More

    Effect of threonine on secretory immune system using a chicken intestinal ex vivo model with lipopolysaccharide challenge.
    Poult Sci 2017 Apr 18. Epub 2017 Apr 18.
    Department of Animal Sciences, Purdue University, West Lafayette, IN 47906.
    Muc2: Secretory IgA (sIgA) and its transcytosis receptor, polymeric immunoglobulin receptor (pIgR), along with mucus, form the first lines of intestinal defense. Threonine (Thr) is a major component of intestinal mucins and IgA, which are highly secreted under lipopolysaccharide (LPS) induced inflammation. In the current study, the effect of Thr on the secretory immune system was determined in an ex vivo chicken ileal explant model. Read More

    Allergic and autoimmune disorders in families with selective IgA deficiency.
    Turk J Med Sci 2017 Apr 18;47(2):592-598. Epub 2017 Apr 18.
    Department of Pediatric Allergy and Immunology, Faculty of Medicine, Abant İzzet Baysal University, Bolu, Turkey.
    Background/aim: IgA deficiency is the most common human primary immunodeficiency. The prevalence of allergic disorders and autoimmunity is thought to be increased in selective IgA deficiency (sIgAD). However, it is currently unclear if these disorders coincide within these families. Read More

    Heat stress decreases expression of the cytokines, avian β-defensins 4 and 6 and Toll-like receptor 2 in broiler chickens infected with Salmonella Enteritidis.
    Vet Immunol Immunopathol 2017 Apr 27;186:19-28. Epub 2017 Feb 27.
    Neuroimmunomodulation Research Group, Department of Pathology, School of Veterinary Medicine, University of São Paulo, São Paulo, SP, Brazil.
    A high ambient temperature is a highly relevant stressor in poultry production. Heat stress (HS) has been reported to reduce animal welfare, performance indices and increase Salmonella susceptibility. Salmonella spp. Read More

    The protection role of Atg16l1 in CD11c(+)dendritic cells in murine colitis.
    Immunobiology 2017 Mar 16. Epub 2017 Mar 16.
    Department of Gastroenterology, The Second Hospital of Hebei Medical University, Hebei, China. Electronic address:
    The autophagy-related 16-like 1 gene (Atg16l1) is associated with inflammatory bowel disease (IBD) and has been shown to play an essential role in paneth cell function and intestinal homeostasis. However, the functional consequences of Atg16l1 deficiency in myeloid cells, particularly in dendritic cells (DCs), are not fully characterized. The aim of this study is to investigate the functional consequence of Atg16l1 in CD11c(+)DCs in murine colitis. Read More

    Short article: Mortality and differential diagnoses of villous atrophy without coeliac antibodies.
    Eur J Gastroenterol Hepatol 2017 May;29(5):572-576
    aFirst Department of Internal Medicine, Coeliac Centre Departments of bClinical Biochemistry cBiometry and Clinical Epidemiology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
    Objective: Villous atrophy (VA) of the small bowel is mainly related to coeliac disease (CD), whose diagnosis is made on the basis of positive endomysial/tissue transglutaminase antibodies while on a gluten-containing diet in the vast majority of patients. However, VA can also occur in other conditions whose epidemiology is little known. Our aim was to study the epidemiology and clinical features of these rare enteropathies. Read More

    Delay in diagnosis affects the clinical outcome in a cohort of cvid patients with marked reduction of iga serum levels.
    Clin Immunol 2017 Mar 25;180:1-4. Epub 2017 Mar 25.
    Department of Translational Medical Sciences, Allergy and Clinical Immunology, University of Naples Federico II, Naples, Italy.
    Common variable immunodeficiency disorders (CVID) represent a collection of diseases leading to an absent or strongly impaired antibody production. CVID presents a wide range of immunological abnormalities and clinical manifestations, including infections, inflammatory and autoimmune diseases, and malignancies. The aim of this observational study was to analyze the epidemiological and clinical features of a cohort of 75 Italian CVID patients, and evaluate the correlation with comorbidity and mortality. Read More

    Severe Aspergillus Pneumonia and Pulmonary Artery Hypertension in a Child with Autosomal Recessive Chronic Granulomatous Disease and Selective IgA Deficiency.
    J Clin Immunol 2017 May 24;37(4):333-335. Epub 2017 Mar 24.
    Allergy Immunology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

    Biochemical characterization and stability of immune globulin intravenous 10% liquid (Panzyga(®)).
    Biologicals 2017 Jan 27;45:33-38. Epub 2016 Oct 27.
    Octapharma Pharmazeutika Produktionsgesellschaft m.b.H., Vienna, Austria.
    Panzyga(®) is a new glycine-formulated immune globulin intravenous 10% liquid for the treatment of patients suffering from immunodeficiencies and autoimmune diseases. Panzyga(®) is a high purity, native and functional IgG product with an IgG subclass distribution equivalent to normal plasma. The levels of hemagglutinins and accompanying plasma proteins (including IgA and IgM) are low. Read More

    Changes in the Level of Immunoglobulins and CD4/CD8 Ratio in Young and Aged Mice with Estradiol Deficiency.
    Immunol Invest 2017 Apr 7;46(3):305-313. Epub 2017 Feb 7.
    a Department of Obstetrics and Gynecology , Jinhua Municipal Central Hospital , Zhejiang Province , People's Republic of China.
    Studies demonstrated that deficiency in 17β-estradiol (E2) in postmenopausal women influences their immune system. However, few studies have reported alterations in immunologic presentation during nonnatural menopause in young females. Here we compared the differences in immune response between young C57BL/6N mice with surgical or medical variectomy and aged C57BL/6N mice with the common feature of E2 deficiency following Con A stimulation. Read More

    Lithophagia as a clue for celiac disease: a case report and literature review.
    Gastroenterol Hepatol Bed Bench 2017 ;10(1):70-72
    Gastroenterohepatology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
    Lithophagia is a type of pica that might be resulted from Iron Deficiency Anemia (IDA) which is the frequent presenting signs of Celiac Disease (CD). A 5-year-old child with a two year history of the lithophagia with a, refractory IDA, abdominal distention and constipation. The child did not grow well and had failure to thrive. Read More

    Targeting C3a/C5a receptors inhibits human mesangial cell proliferation and alleviates immunoglobulin A nephropathy in mice.
    Clin Exp Immunol 2017 Mar 15. Epub 2017 Mar 15.
    Department of Nephrology, First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
    Complement activation has a deep pathogenic influence in immunoglobulin (Ig)A nephropathy (IgAN). C3a and C5a, small cleavage fragments generated by complement activation, are key mediators of inflammation. The fragments exert broad proinflammatory effects by binding to specific receptors (C3aR and C5aR, respectively). Read More

    The Specific Roles of Vitamins in the Regulation of Immunosurveillance and Maintenance of Immunologic Homeostasis in the Gut.
    Immune Netw 2017 Feb 23;17(1):13-19. Epub 2017 Feb 23.
    Laboratory of Vaccine Materials, National Institutes of Biomedical Innovation, Health and Nutrition (NIBIOHN), Osaka 567-0085, Japan.; Graduate School of Medicine, Graduate School of Pharmaceutical Sciences, Graduate School of Dentistry, Osaka University, Osaka 565-0871, Japan.; Division of Mucosal Immunology, Department of Microbiology and Immunology and International Research and Development Center for Mucosal Vaccines, The Institute of Medical Science, The University of Tokyo, Tokyo 108-8639, Japan.; Department of Microbiology and Immunology, Kobe University Graduate School of Medicine, Hyogo 650-0017, Japan.
    Vitamins are micronutrients which are essential for the maintenance of biological responses including immune system. Hence, vitamin deficiency increases a risk of infectious, allergic, and inflammatory diseases. Accumulating evidence has recently revealed the molecular and cellular mechanisms of vitamin-mediated regulation in the active and quiescent immune responses. Read More

    Levothyroxine therapy and impaired clearance are the strongest contributors to small intestinal bacterial overgrowth: Results of a retrospective cohort study.
    World J Gastroenterol 2017 Feb;23(5):842-852
    Thorsten Brechmann, Andre Sperlbaum, Wolff Schmiegel, Department of Gastroenterology and Hepatology, Berufsgenossenschaftliches Universitätsklinikum Bergmannsheil GmbH, Ruhr-University Bochum, 44789 Bochum, Germany.
    Aim: To identify a set of contributors, and weight and rank them on a pathophysiological basis.

    Methods: Patients who have undergone a lactulose or glucose hydrogen breath test to rule out small intestinal bacterial overgrowth (SIBO) for various clinical symptoms, including diarrhoea, weight loss, abdominal pain, cramping or bloating, were seen as eligible for inclusion in a retrospective single-centre study. Clinical data such as co-morbidities, medication, laboratory parameters and other possible risk factors have been identified from the electronic data system. Read More

    Antibody deficiency in patients with frequent exacerbations of Chronic Obstructive Pulmonary Disease (COPD).
    PLoS One 2017 17;12(2):e0172437. Epub 2017 Feb 17.
    Division of Allergy and Clinical Immunology, Department of Internal Medicine, the Johns Hopkins University School of Medicine, Baltimore, Maryland, United States of America.
    Chronic Obstructive Pulmonary Disease is the third leading cause of death in the US, and is associated with periodic exacerbations, which account for the largest proportion of health care utilization, and lead to significant morbidity, mortality, and worsening lung function. A subset of patients with COPD have frequent exacerbations, occurring 2 or more times per year. Despite many interventions to reduce COPD exacerbations, there is a significant lack of knowledge in regards to their mechanisms and predisposing factors. Read More

    [Clinical symptoms in IgA deficiency].
    Rev Alerg Mex 2017 Jan-Mar;64(1):34-39
    Santa Casa de São Paulo, Facultad de Ciencias Médicas. São Paulo, Brasil.
    Background: IgA deficiency is the most common primary immunodeficiency. Early diagnosis and clinical follow-up may improve the quality of life of patients with IgA deficiency. To this end, IgA deficiency should be further studied and better understood on its clinical manifestations. Read More

    Determination of Anti-Anisakis Simplex Antibodies and Relationship with αβ and γδ Lymphocyte Subpopulations in Patients with Crohn's Disease.
    Dig Dis Sci 2017 Apr 6;62(4):934-943. Epub 2017 Feb 6.
    Research Department, Arnau de Vilanova Hospital, c/San Clemente 12, 46015, Valencia, Spain.
    Background: The etiology of Crohn's disease (CD) is still unknown although new theories are based on defects in innate immunity. We have previously shown a decrease in γδ T cells in CD patients. Previous studies have shown a high prevalence of anti-A. Read More

    The clinical significance of complete class switching defect in Ataxia telangiectasia patients.
    Expert Rev Clin Immunol 2017 May 15;13(5):499-505. Epub 2017 Feb 15.
    a Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.
    Background: Ataxia telangiectasia (AT) is a primary immunodeficiency associated with recurrent infections. We aimed to investigate clinical and immunological classification in AT patients who suffer from a different spectrum of humoral immune defects.

    Methods: AT patients were categorized according to the ability of class switching and patients with hyper IgM (HIgM) profile were defined as class switching defect (CSD). Read More

    Myeloid ATG16L1 Facilitates Host-Bacteria Interactions in Maintaining Intestinal Homeostasis.
    J Immunol 2017 Mar 27;198(5):2133-2146. Epub 2017 Jan 27.
    F. Widjaja Foundation, Inflammatory Bowel and Immunobiology Research Institute, Cedars-Sinai Medical Center, Los Angeles, CA 90048;
    Intact ATG16L1 plays an essential role in Paneth cell function and intestinal homeostasis. However, the functional consequences of ATG16L1 deficiency in myeloid cells, particularly macrophages, are not fully characterized. We generated mice with Atg16l1 deficiency in myeloid and dendritic cells and showed that mice with myeloid Atg16l1 deficiency had exacerbated colitis in two acute and one chronic model of colitis with increased proinflammatory to anti-inflammatory macrophage ratios, production of proinflammatory cytokines, and numbers of IgA-coated intestinal microbes. Read More

    Recurrent Intestinal Obstruction in a Patient with Selective IgA Deficiency.
    Malays J Med Sci 2016 Nov 7;23(6):123-127. Epub 2016 Dec 7.
    Centre for Alimentary Studies, Endoscopy Centre 1, Lower Albert Road, Central Hong Kong, Hong Kong SAR, China; Quality Healthcare Medical Services, 6/F HK Pacific Centre, 28 Hankow Road, Tsim Sha Tsui, Hong Kong, China.
    A 32 year old woman presented with acute onset of abdominal pain and fever. An urgent computerised tomography (CT) of the whole abdomen showed dilated loop at the terminal ileum in the right lower abdomen with thickening of the wall and oedema. The CT was suggestive of distal small bowel obstruction at the ileum with surrounding wall oedema. Read More

    Mortality and differential diagnoses of villous atrophy without coeliac antibodies.
    Eur J Gastroenterol Hepatol 2017 Jan 11. Epub 2017 Jan 11.
    aFirst Department of Internal Medicine, Coeliac CentreDepartments of bClinical BiochemistrycBiometry and Clinical Epidemiology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
    Objective: Villous atrophy (VA) of the small bowel is mainly related to coeliac disease (CD), whose diagnosis is made on the basis of positive endomysial/tissue transglutaminase antibodies while on a gluten-containing diet in the vast majority of patients. However, VA can also occur in other conditions whose epidemiology is little known. Our aim was to study the epidemiology and clinical features of these rare enteropathies. Read More

    Low immunoglobulin E flags two distinct types of immune dysregulation.
    Clin Exp Immunol 2017 Mar 11;187(3):345-352. Epub 2017 Jan 11.
    Immunodeficiency Clinic, Medical Outpatient Unit and Immunodeficiency Laboratory, Department of Biomedicine, University Hospital Basel, Basel, Switzerland.
    During the last two decades, hyper-immunoglobulin (Ig)E syndromes have been characterized clinically and molecularly in patients with genetically determined primary immunodeficiencies. However, the detection of low IgE levels, defined here as below detection limit in the routine clinical immunology laboratory, has received little attention. We analysed the association of serum IgA, IgM and IgG levels (including IgG subclasses) with low, normal or high serum IgE levels in patients evaluated in a single-centre out-patient immunodeficiency and allergy clinic. Read More

    Prevalence of dermatologic diseases among patients with selective immunoglobulin A deficiency.
    Allergy Asthma Proc 2017 Jan;38(1):70-77
    Background: There are no published large-scale epidemiologic studies regarding the prevalence of skin diseases in patients with selective immunoglobulin A (IgA) deficiency (sIgAD). The purpose of this study was to investigate the prevalence of dermatological diseases in patients with sIgAD.

    Methods: This retrospective matched case-control study was based on data from the Leumit Healthcare Services data base (approximately 725,000 residents of Israel), which was searched for all subjects aged ≥12 years who had undergone serum total IgA measurements during 2004-14 for any reason. Read More

    Nodular lymphoid hyperplasia complicated with ileal Burkitt's lymphoma in an adult patient with selective IgA deficiency.
    Int J Surg Case Rep 2017 22;30:69-72. Epub 2016 Nov 22.
    University, Josip Juraj Strossmayer, Osijek University Hospital Centre, Clinic for Internal Medicine, Josip Huttler 4, Osijek, 31 000, Croatia.
    Introduction: Primary lymphomas of the small intestine are rare. Burkitt's lymphoma (BL) occurs sporadically in adults. Nodular lymphoid hyperplasia (NLH) is a rare disorder characterized by diffuse nodular lesions, which represent hyperplastic lymphoid follicles, and it is often associated with immunodeficiency syndromes. Read More

    Abnormal hematopoiesis and autoimmunity in human subjects with germline IKZF1 mutations.
    J Allergy Clin Immunol 2016 Dec 1. Epub 2016 Dec 1.
    Department of Pediatrics and Developmental Biology, Perinatal and Maternal Medicine, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan. Electronic address:
    Background: Ikaros, which is encoded by IKZF1, is a transcriptional factor that play a critical role in hematopoiesis. Somatic IKZF1 alterations are known to be involved in the pathogenesis of leukemia in human subjects. Recently, immunodeficiency caused by germline IKZF1 mutation has been described. Read More

    Population-Based Screening for Selective Immunoglobulin A (IgA) Deficiency in Lithuanian Children Using a Rapid Antibody-Based Fingertip Test.
    Med Sci Monit 2016 Dec 6;22:4773-4778. Epub 2016 Dec 6.
    Tampere Centre for Child Health Research, University of Tampere and Tampere University Hospital, Tampere, Finland.
    BACKGROUND Selective immunoglobulin A (IgA) deficiency is the most common inherited immunodeficiency disorder worldwide. An early diagnosis is advocated because of the increased risk of infections, autoimmune diseases, and allergic reactions. We investigated the usefulness of a rapid point-of-care test in detecting for IgA deficiency in a population with a previously unknown prevalence. Read More

    Secretory IgA Deficiency in Individual Small Airways Is Associated with Persistent Inflammation and Remodeling.
    Am J Respir Crit Care Med 2017 Apr;195(8):1010-1021
    1 Division of Allergy, Pulmonary and Critical Care Medicine, Department of Medicine, and.
    Rationale: Maintenance of a surface immune barrier is important for homeostasis in organs with mucosal surfaces that interface with the external environment; however, the role of the mucosal immune system in chronic lung diseases is incompletely understood.

    Objectives: We examined the relationship between secretory IgA (SIgA) on the mucosal surface of small airways and parameters of inflammation and airway wall remodeling in chronic obstructive pulmonary disease (COPD).

    Methods: We studied 1,104 small airways (<2 mm in diameter) from 50 former smokers with COPD and 39 control subjects. Read More

    Relationships between Mucosal Antibodies, Non-Typeable Haemophilus influenzae (NTHi) Infection and Airway Inflammation in COPD.
    PLoS One 2016 29;11(11):e0167250. Epub 2016 Nov 29.
    Clinical & Experimental Sciences, University of Southampton Faculty of Medicine, Southampton General Hospital, Tremona Road, Southampton, United Kingdom.
    Non-typeable Haemophilus influenzae (NTHi) is a key pathogen in COPD, being associated with airway inflammation and risk of exacerbation. Why some patients are susceptible to colonisation is not understood. We hypothesised that this susceptibility may be due to a deficiency in mucosal humoral immunity. Read More

    Impact of iron deficiency anemia on the function of the immune system in children.
    Medicine (Baltimore) 2016 Nov;95(47):e5395
    aDepartment of Pediatrics bDepartment of Clinical Pathology, Zagazig University, Zagazig, Egypt.
    The importance of iron deficiency as a public health problem is based ultimately on the seriousness of its consequences on health. The most extensively investigated consequences of iron deficiency involve work performance and immune function. The significance of the effects on work performance is generally accepted. Read More

    Ataxia telangiectasia: a review.
    Orphanet J Rare Dis 2016 Nov 25;11(1):159. Epub 2016 Nov 25.
    The Ataxia Telangiectasia Clinical Center, Departments of Pediatrics, Medicine and Pathology, Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.
    Definition Of The Disease: Ataxia telangiectasia (A-T) is an autosomal recessive disorder primarily characterized by cerebellar degeneration, telangiectasia, immunodeficiency, cancer susceptibility and radiation sensitivity. A-T is often referred to as a genome instability or DNA damage response syndrome.

    Epidemiology: The world-wide prevalence of A-T is estimated to be between 1 in 40,000 and 1 in 100,000 live births. Read More

    [Topical corticosteroids as a therapeutic alternative in linear immunoglobulin A bullous dermatosis in childhood: case report].
    Arch Argent Pediatr 2016 Dec;114(6):e440-e443
    Servicio de Dermatología, Hospital García Orcoyen, Estella, España.
    Linear immunoglobulin A dermatosis of childhood is a rare autoimmune disorder. Its etiology remains unknown, although it has been linked to drugs, infections, immunological diseases and lymphoproliferative processes. We report the case of a 6 year old girl who consulted for perioral bullous lesions without other symptoms. Read More

    Vitamin D deficiency may predict a poorer outcome of IgA nephropathy.
    BMC Nephrol 2016 Nov 2;17(1):164. Epub 2016 Nov 2.
    Renal Division, Department of Medicine, First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, Guangxi Zhuang Autonomous Region, China.
    Background: Experimental studies showed that 25-hydroxy-vitamin D [25(OH)D] deficiency (defined as 25-hydroxy-vitamin D < 15 ng/ml) has been associated with CKD progression. Patients with IgA nephropathy have an exceptionally high rate of severe 25(OH)D deficiency; however, it is not known whether this deficiency is a risk factor for progression of IgA nephropathy. We conducted this study to investigate the relationship between the plasma level of 25(OH)D and certain clinical parameters and renal histologic lesions in the patients with IgA nephropathy, and to evaluate whether the 25(OH)D level could be a good prognostic marker for IgA nephropathy progression. Read More

    Prevalence of selective immunoglobulin A deficiency in Greek children and adolescents with type 1 diabetes.
    World J Pediatr 2016 Nov 10;12(4):470-476. Epub 2016 Jun 10.
    4th Department of Pediatrics, Faculty of Medicine, Aristotle University of Thessaloniki, Papageorgiou General Hospital, Ring Road Nea Efkarpia, 56403, Thessaloniki, Greece.
    Background: The association of selective immunoglobulin A (IgA) deficiency with type 1 diabetes (T1D) remains unclear. This study was to evaluate serum IgA concentrations in Greek children and adolescents with T1D.

    Methods: In two hundred individuals with T1D, serum IgA concentrations were quantitatively determined using nephelometry. Read More

    RAGs and BUGS: An alliance for autoimmunity.
    Gut Microbes 2016 Nov 30;7(6):503-511. Epub 2016 Aug 30.
    a Milan Unit , Istituto di Ricerca Genetica e Biomedica, Consiglio Nazionale delle Ricerche , Milan , Italy.
    Hypomorphic Rag mutations in humans cause Omenn Syndrome (OS) a severe immunodeficiency associated with autoimmune-like manifestations mediated by oligoclonal activated T and B cells. The clinical and immunological spectrum of OS presentation is extremely broad. However, the role played by environmental triggers in the disease pathogenesis remains largely unknown. Read More

    B-cell activation with CD40L or CpG measures the function of B-cell subsets and identifies specific defects in immunodeficient patients.
    Eur J Immunol 2017 Jan 25;47(1):131-143. Epub 2016 Nov 25.
    B Cell Physiopathology Unit, Immunology Research Area, Ospedale Pediatrico Bambino Gesù IRCSS, Roma, Italy.
    Around 65% of primary immunodeficiencies are antibody deficiencies. Functional tests are useful tools to study B-cell functions in vitro. However, no accepted guidelines for performing and evaluating functional tests have been issued yet. Read More

    [Clinic of humoral primary immunodeficiencies in adults. Experience in a tertiary hospital].
    Rev Alerg Mex 2016 Oct-Dec;63(4):334-341
    Instituto Mexicano del Seguro Social, Centro Médico Nacional Siglo XXI, Hospital de Especialidades, Servicio de Alergia e Inmunología Clínica, Ciudad de México, México.
    Background: Primary immunodeficiencies (PID) are characterized by alteration of the components of the immune system. Humoral deficiencies represent 50%. The most common are selective IgA deficiency, Bruton agammaglobulinemia, and common variable immunodeficiency (CVID). Read More

    Salivary IgA versus HIV and Dental Caries.
    J Clin Diagn Res 2016 Sep 1;10(9):ZC61-ZC64. Epub 2016 Sep 1.
    Professor, Department of Pedodontics and Preventive Dentistry Dr. R. Ahmed Dental College and Hospital , Kolkata, West Bengal, India .
    Introduction: The inter-relationship of Human Immunodeficiency Virus (HIV) infection and dental caries as well as Salivary Immunoglobulin-A (S-IgA) level appear to remain under explored while a manual and electronic search of the literature was made. Hence, the present study was undertaken to assess the relationship of S-IgA and dental caries status in HIV positive children.

    Aim: The aim of this study was to find out the relationship of S-IgA antibody with dental caries by measuring the concentration of IgA in saliva of HIV positive and negative children and determine the dental caries status in HIV positive and HIV negative children, which may help in treatment planning and prevention of the same. Read More

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