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https://www.readbyqxmd.com/read/28212436/antibody-deficiency-in-patients-with-frequent-exacerbations-of-chronic-obstructive-pulmonary-disease-copd
#1
Brian N McCullagh, Alejandro P Comellas, Zuhair K Ballas, John D Newell, M Bridget Zimmerman, Antoine E Azar
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...
2017: PloS One
https://www.readbyqxmd.com/read/28188711/-clinical-symptoms-in-iga-deficiency
#2
Flavio Augusto De Oliveira-Serra, Tainá Mosca, Maria da Conceição Santos de Menezes, Wilma Carvalho-Neves Forte
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. OBJECTIVE: To determine IgA deficiency clinical manifestations. METHODS: Cross-sectional, retrospective, exploratory study, where the medical records of 39 patients with IgA deficiency were analyzed...
January 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/28162005/the-clinical-significance-of-complete-class-switching-defect-in-ataxia-telangiectasia-patients
#3
Saleh Ghiasy, Leila Parvaneh, Gholamreza Azizi, Ghazal Sadri, Majid Zaki Dizaji, Hassan Abolhassani, Asghar Aghamohammadi
OBJECTIVES: 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). RESULTS: Serum immunoglobulin profile in 66 AT patients showed normal immunoglobulin level (22...
February 4, 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28090187/recurrent-intestinal-obstruction-in-a-patient-with-selective-iga-deficiency
#4
Chee-Kin Hui
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. Multiple biopsies taken from the terminal ileum and colon on colonoscopy were all unremarkable. She represented one-year later with a recurrence of intestinal obstruction...
November 2016: Malaysian Journal of Medical Sciences: MJMS
https://www.readbyqxmd.com/read/28079608/mortality-and-differential-diagnoses-of-villous-atrophy-without-coeliac-antibodies
#5
Annalisa Schiepatti, Federico Biagi, Giacomo Fraternale, Claudia Vattiato, Davide Balduzzi, Simona Agazzi, Claudia Alpini, Catherine Klersy, Gino R Corazza
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. PATIENTS AND METHODS: Clinical and laboratory data of all the patients with VA directly diagnosed in our centre in the last 15 years were collected and statistically analysed...
January 11, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28052804/prevalence-of-dermatologic-diseases-among-patients-with-selective-immunoglobulin-a-deficiency
#6
Eli Magen, Atheer Masalha, Dan Andrei Waitman, Natan Kahan, Igor Viner, Leonora Klassov, Daniel Vardy
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...
January 1, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/27940199/nodular-lymphoid-hyperplasia-complicated-with-ileal-burkitt-s-lymphoma-in-an-adult-patient-with-selective-iga-deficiency
#7
Toni Hanich, Ljiljana Majnarić, Dragan Janković, Šefket Šabanović, Aleksandar Včev
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. PRESENTATION OF CASE: We present a 38-year-old male patient in a state of surgical emergency, suspected of Crohn's disease, who had an unusual combination of NLH and BL of the proximal ileum...
November 22, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27920422/population-based-screening-for-selective-immunoglobulin-a-iga-deficiency-in-lithuanian-children-using-a-rapid-antibody-based-fingertip-test
#8
Vaidotas Urbonas, Jolita Sadauskaite, Rimante Cerkauskiene, Arvydas Kaminskas, Markku Mäki, Kalle Kurppa
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. MATERIAL AND METHODS Altogether, 1000 children aged 11-13 years from randomly selected Lithuanian schools were enrolled. A point-of-care test with a fingertip sample was used to screen for the presence of IgA deficiency in children whose parents gave consent...
December 6, 2016: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/27884168/ataxia-telangiectasia-a-review
#9
REVIEW
Cynthia Rothblum-Oviatt, Jennifer Wright, Maureen A Lefton-Greif, Sharon A McGrath-Morrow, Thomas O Crawford, Howard M Lederman
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. CLINICAL DESCRIPTION: A-T is a complex disorder with substantial variability in the severity of features between affected individuals, and at different ages...
November 25, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27800605/b-cell-activation-with-cd40l-or-cpg-measures-the-function-of-b-cell-subsets-and-identifies-specific-defects-in-immunodeficient-patients
#10
Emiliano Marasco, Chiara Farroni, Simona Cascioli, Valentina Marcellini, Marco Scarsella, Ezio Giorda, Eva Piano Mortari, Lucia Leonardi, Alessia Scarselli, Diletta Valentini, Caterina Cancrini, Marzia Duse, Ola Grimsholm, Rita Carsetti
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. Here, we report our experience on the study of B-cell functions in infancy and throughout childhood. We show that T-independent stimulation with CpG measures proliferation and differentiation potential of memory B cells. Switched memory B cells respond better than IgM memory B cells...
November 1, 2016: European Journal of Immunology
https://www.readbyqxmd.com/read/27795213/-clinic-of-humoral-primary-immunodeficiencies-in-adults-experience-in-a-tertiary-hospital
#11
Julio César Cambray-Gutiérrez, Diana Andrea Herrera-Sánchez, Lizbeth Blancas-Galicia, Patricia María O'Farrill-Romanillos
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). OBJECTIVE: To describe the epidemiological and clinical characteristics of adults with humoral PID, cared for in a Primary Humoral Immunodeficiencies Clinic. METHODS: A descriptive cross-sectional study that included a year of analysis, including 35 patients with humoral PID, 31 with CVID, and 4 with Bruton agammaglobulinemia...
October 2016: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/27790582/salivary-iga-versus-hiv-and-dental-caries
#12
Pradip Kumar Mandal, Malay Mitra, Sonu Acharya, Chiranjit Ghosh, Susant Mohanty, Subrata Saha
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...
September 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27776452/the-long-and-winding-road-to-iga-deficiency-causes-and-consequences
#13
D T Laura Vo Ngoc, Lizette Krist, Frans J van Overveld, Ger T Rijkers
The most common humoral immunodeficiency is IgA deficiency. One of the first papers addressing the cellular and molecular mechanisms underlying IgA deficiency indicated that immature IgA-positive B-lymphocytes are present in these patients. This suggests that the genetic background for IgA is still intact and that class switching can take place. At this moment, it cannot be ruled out that genetic as well as environmental factors are involved. Areas covered: A clinical presentation, the biological functions of IgA, and the management of IgA deficiency are reviewed...
October 25, 2016: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/27763681/selective-iga-deficiency-epidemiology-pathogenesis-clinical-phenotype-diagnosis-prognosis-and-management
#14
REVIEW
R Yazdani, G Azizi, H Abolhassani, A Aghamohammadi
Selective immunoglobulin A deficiency (SIgAD) is the most common primary antibody deficiency. Although more patients with SIgAD are asymptomatic, selected patients suffer from different clinical complications such as pulmonary infections, allergies, autoimmune diseases, gastrointestinal disorders and malignancy. Pathogenesis of SIgAD is still unknown; however, a defective terminal differentiation of B cells and defect in switching to IgA-producing plasma cells are presumed to be responsible. Furthermore, some cytogenic defects and monogenic mutations are associated with SIgAD...
January 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/27739086/genetic-loss-of-immunoglobulin-a-does-not-influence-development-of-alcoholic-steatohepatitis-in-mice
#15
Tatsuo Inamine, An-Ming Yang, Lirui Wang, Kuei-Chuan Lee, Cristina Llorente, Bernd Schnabl
BACKGROUND: Chronic alcohol abuse is associated with intestinal dysbiosis and bacterial translocation. Translocated commensal bacteria contribute to alcoholic liver disease. Secretory immunoglobulin A (IgA) in the intestine binds bacteria and prevents bacterial translocation. METHODS: To investigate the functional role of IgA in ethanol (EtOH)-induced liver disease in mice, we subjected wild type (WT) and IgA-deficient littermate mice to Lieber-DeCarli models of chronic EtOH administration and the model of chronic and binge EtOH feeding (the NIAAA model)...
December 2016: Alcoholism, Clinical and Experimental Research
https://www.readbyqxmd.com/read/27737870/miller-fisher-syndrome
#16
Suresh Kumar Gupta, Kunal Kishor Jha, Mhd Diaa Chalati, Losan Tareq Alashi
A man aged 30 years presented to the emergency department (ED) with ataxia, areflexia, facial weakness, ophthalmoplegia, extremity weakness and back pain for 4 days. 4 days prior to attending the ED, the patient had suffered from diarrhoea for 2 weeks. The diagnosis of Miller Fisher syndrome was performed on the dual basis of clinical features in addition to an investigations report. Nerve conduction studies and anti-GQ1b IgG antibody analysis were requested. Once IgA deficiency was ruled out, the patient was started on intravenous immunoglobulin (400 mg/kg/day)...
October 13, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27734646/cavitary-lung-lesions-in-a-difficult-to-treat-asthma-patient
#17
R Ragesh, Animesh Ray, Agrima Mian, Surabhi Vyas, S K Sharma
We describe an interesting case of severe asthma who was not showing satisfactory response to standard treatment. Investigations revealed him to be suffering from allergic bronchopulmonary aspergillosis (ABPA). After starting systemic steroids he showed marked improvement initially only to have recurrent symptoms within a year. He was investigated further and found to have chronic pulmonary aspergillosis in the form of chronic cavitary pulmonary aspergillosis (CCPA) and aspergilloma as also the presence of selective IgA deficiency...
April 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27725814/monitoring-perinatal-gut-microbiota-in-mouse-models-by-mass-spectrometry-approaches-parental-genetic-background-and-breastfeeding-effects
#18
Stefano Levi Mortera, Federica Del Chierico, Pamela Vernocchi, Maria M Rosado, Agnese Cavola, Marco Chierici, Luisa Pieroni, Andrea Urbani, Rita Carsetti, Isabella Lante, Bruno Dallapiccola, Lorenza Putignani
At birth, contact with external stimuli, such as nutrients derived from food, is necessary to modulate the symbiotic balance between commensal and pathogenic bacteria, protect against bacterial dysbiosis, and initiate the development of the mucosal immune response. Among a variety of different feeding patterns, breastfeeding represents the best modality. In fact, the capacity of breast milk to modulate the composition of infants' gut microbiota leads to beneficial effects on their health. In this study, we used newborn mice as a model to evaluate the effect of parental genetic background (i...
2016: Frontiers in Microbiology
https://www.readbyqxmd.com/read/27723758/common-variants-at-pvt1-atg13-ambra1-ahi1-and-clec16a-are-associated-with-selective-iga-deficiency
#19
Paola G Bronson, Diana Chang, Tushar Bhangale, Michael F Seldin, Ward Ortmann, Ricardo C Ferreira, Elena Urcelay, Luis Fernández Pereira, Javier Martin, Alessandro Plebani, Vassilios Lougaris, Vanda Friman, Tomáš Freiberger, Jiri Litzman, Vojtech Thon, Qiang Pan-Hammarström, Lennart Hammarström, Robert R Graham, Timothy W Behrens
Selective immunoglobulin A deficiency (IgAD) is the most common primary immunodeficiency in Europeans. Our genome-wide association study (GWAS) meta-analysis of 1,635 patients with IgAD and 4,852 controls identified four new significant (P < 5 × 10(-8)) loci and association with a rare IFIH1 variant (p.Ile923Val). Peak new variants (PVT1, P = 4.3 × 10(-11); ATG13-AMBRA1, P = 6.7 × 10(-10); AHI1, P = 8.4 × 10(-10); CLEC16A, P = 1.4 × 10(-9)) overlapped with autoimmune markers (3/4) and correlated with 21 putative regulatory variants, including expression quantitative trait loci (eQTLs) for AHI1 and DEXI and DNase hypersensitivity sites in FOXP3(+) regulatory T cells...
November 2016: Nature Genetics
https://www.readbyqxmd.com/read/27681397/salivary-iga-and-dental-caries-in-hiv-patients-a-pilot-study
#20
Sonu Acharya, Pradip Kumar Mandal
INTRODUCTION: The interrelationship of human immunodeficiency virus (HIV) infection and dental caries, as well as Salivary IgA (S-IgA) level, appear to remain underexplored while a manual and electronic search of the literature was made. Hence, this study was undertaken to assess the relationship of S-IgA and dental caries status in HIV +ve 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 +ve and HIV -ve children and to determine the dental caries status in HIV +ve and HIV -ve children, which may help in treatment planning and prevention of the same...
October 2016: Journal of the Indian Society of Pedodontics and Preventive Dentistry
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