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IgA deficiency in adults

Esther van de Vosse, Monique M van Ostaijen-Ten Dam, René Vermaire, Els M Verhard, Jacqueline L Waaijer, Jaap A Bakker, Sandra T Bernards, Hermann Eibel, Maarten J van Tol, Jaap T van Dissel, Margje H Haverkamp
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. We determined lymphocyte subsets, expression of BAFF receptors, serum immunoglobulins, complement pathways, Pneumovax-23 vaccination response and genetic variations in BAFFR and MBL2...
July 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
Yanghui Gu, Yu Wang, Chunlan Ji, Ping Fan, Zhiren He, Tao Wang, Xusheng Liu, Chuan Zou
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. Aim. To explore the feasibility of differentiation of TCM syndrome types among IgA nephropathy patients based on clinicopathological parameters. Materials and Methods. The cross-sectional study enrolled 464 biopsy-proven IgA nephropathy adult patients from 2010 to 2016...
2017: Evidence-based Complementary and Alternative Medicine: ECAM
Roger Chou, Christina Bougatsos, Ian Blazina, Katherine Mackey, Sara Grusing, Shelley Selph
Importance: Silent or subclinical celiac disease may result in potentially avoidable adverse health consequences. Objective: To review the evidence on benefits and harms of screening for celiac disease in asymptomatic adults, adolescents, and children 3 years and older for the US Preventive Services Task Force. Data Sources: Ovid MEDLINE, Cochrane Central Register of Controlled Trials, and Cochrane Database of Systematic Reviews, searched to June 14, 2016...
March 28, 2017: JAMA: the Journal of the American Medical Association
Thomas Rauen, Jürgen Floege
Immunoglobulin A nephropathy (IgAN) is the most frequently occurring primary glomerulonephritis in Caucasian and Asian populations. Nonetheless, therapeutic recommendations are based on weak evidence, large controlled trials are scarce and, in particular, the additional value of immunosuppression beyond comprehensive supportive measures is not well-established. The use of immunosuppressants is supported by experimental insights into IgAN pathogenesis that suggest an autoimmune component in disease development...
March 14, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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
Richard A Lafayette, Pietro A Canetta, Brad H Rovin, Gerald B Appel, Jan Novak, Karl A Nath, Sanjeev Sethi, James A Tumlin, Kshama Mehta, Marie Hogan, Stephen Erickson, Bruce A Julian, Nelson Leung, Felicity T Enders, Rhubell Brown, Barbora Knoppova, Stacy Hall, Fernando C Fervenza
IgA nephropathy frequently leads to progressive CKD. Although interest surrounds use of immunosuppressive agents added to standard therapy, several recent studies have questioned efficacy of these agents. Depleting antibody-producing B cells potentially offers a new therapy. In this open label, multicenter study conducted over 1-year follow-up, we randomized 34 adult patients with biopsy-proven IgA nephropathy and proteinuria >1 g/d, maintained on angiotensin-converting enzyme inhibitors or angiotensin receptor blockers with well controlled BP and eGFR<90 ml/min per 1...
April 2017: Journal of the American Society of Nephrology: JASN
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
Ankit Malik, Deepika Sharma, Qifan Zhu, Rajendra Karki, Clifford S Guy, Peter Vogel, Thirumala-Devi Kanneganti
Inflammatory bowel diseases (IBD) affect over 5 million individuals in the industrialized world, with an increasing incidence rate worldwide. IBD also predisposes affected individuals to development of colorectal cancer, which is a leading cause of cancer-related deaths in adults. Mutations in genes encoding molecules in the IL-33 signaling pathway are associated with colitis and colitis-associated cancer (CAC), but how IL-33 modulates gut homeostasis is unclear. Here, we have shown that Il33-deficient mice are highly susceptible to colitis and CAC...
December 1, 2016: Journal of Clinical Investigation
E Kamycheva, T Goto, C A Camargo
We investigated the association between celiac disease (CD) and bone mass density (BMD) and risk of osteoporotic fractures in the general US population. In children and men ≥18 years, CD was associated with reduced BMD, and in men ≥40 years, CD was associated with increased risk of osteoporotic fractures. INTRODUCTION: Celiac disease (CD) is an autoimmune condition, characterized by inflammation of the small intestine. CD has an increasing prevalence, and if unrecognized or untreated, CD can lead to complications from malabsorption and micronutrient deficiencies...
March 2017: Osteoporosis International
T V Latysheva, E A Latysheva, I A Martynova, G E Aminova
Primary immunodeficiencies (PIDs) are a group of congenital diseases of the immune system, which numbers more than 230 nosological entities associated with lost, decreased, or wrong function of its one or several components. Due to the common misconception that these are extremely rare diseases that occur only in children and lead to their death at an early age, PIDs are frequently ruled out by physicians of related specialties from the range of differential diagnosis. The most common forms of PIDs, such as humoral immunity defects, common variable immune deficiency, X-linked agammaglobulinemia, selective IgA deficiency, etc...
2016: Terapevticheskiĭ Arkhiv
Rafaela Cabral Gonçalves Fabiano, Sérgio Veloso Brant Pinheiro, Ana Cristina Simões E Silva
BACKGROUND AND AIM: IgA nephropathy is one of the leading causes of primary glomerulonephritis worldwide and an important etiology of renal disease in young adults. IgA nephropathy is considered an immune complex-mediated disease. METHODS: This review article summarizes recent evidence on the pathophysiology of IgA nephropathy. RESULTS: Current studies indicate an ordered sequence of multi-hits as fundamental to disease occurrence. Altered glycan structures in the hinge region of the heavy chains of IgA1 molecules act as auto-antigens, potentially triggering the production of glycan-specific autoantibodies...
October 2016: Inflammation Research: Official Journal of the European Histamine Research Society ... [et Al.]
Aaron Rohr, Ryan Ash, John Vadaparampil, Jacqueline Hill, Louis Wetzel
A 51-year-old man with history of undiagnosed pulmonary nodules 4 years prior, presented with right-sided chest pain. Acute cardiac workup was negative, and a chest computed tomography examination demonstrated marked improvement in bilateral pulmonary nodules. A concordant abdominal computed tomography examination showed new subcentimeter hypodense lesions throughout the liver and spleen, mild progressive abdominopelvic lymphadenopathy, and new small lytic lesions of T11 and L4 vertebrae. A positron emission tomography examination demonstrated hypermetabolic activity of these abdominopelvic lesions suggesting metastatic disease...
June 2016: Radiology case reports
P Mohammadinejad, S Pourhamdi, H Abolhassani, B Mirminachi, A Havaei, S N Masoom, B Sadeghi, A Ghajar, M Afarideh, N Parvaneh, B Mirsaeed-Ghazi, M Movahedi, M Gharagozlou, Z Chavoushzadeh, A Mahdaviani, F Zandieh, R Sherkat, M Sadeghi-Shabestari, R Faridhosseini, F Jabbari-Azad, H Ahanchian, M Zandkarimi, T Cherghi, A Fayezi, I Mohammadzadeh, R Amin, S Aleyasin, M Moghtaderi, J Ghaffari, M Bemanian, A Shafiei, N Kalantari, A Ahmadiafshar, H A Khazaei, J Mohammadi, M Nabavi, N Rezaei, A Aghamohammadi
BACKGROUND: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders (PID), with a broad spectrum of clinical features ranging from severe and recurrent infections to asymptomatic disease. OBJECTIVES: The current study was performed to evaluate and compare demographic and clinical data in the most common types of PAD. MATERIALS AND METHODS: We performed a retrospective review of the medical records of all PAD patients with a confirmed diagnosis of common variable immunodeficiency (CVID), hyper IgM syndrome (HIgM), selective IgA deficiency (SIgAD), and X-linked agammaglobulinemia (XLA) who were diagnosed during the last 30 years at the Children's Medical Center, Tehran, Iran...
2015: Journal of Investigational Allergology & Clinical Immunology
Gopala Krishna Koundinya Lanka, Jieh-Juen Yu, Siqi Gong, Rishein Gupta, Shamimunisa B Mustafa, Ashlesh K Murthy, Guangming Zhong, James P Chambers, M Neal Guentzel, Bernard P Arulanandam
Neonatal Chlamydia lung infections are associated with serious sequelae such as asthma and airway hyper-reactivity in children and adults. Our previous studies demonstrated the importance of Th-1 type cytokines, IL-12 and IFN-γ in protection against neonatal pulmonary chlamydial challenge; however, the role of the humoral arm of defense has not been elucidated. We hypothesized that B-cells and IgA, the major mucosal antibody, play a protective role in newborns against development of later life respiratory sequelae to Chlamydia infection...
April 2016: Pathogens and Disease
J M Lucena, S Burillo Sanz, A Núñez-Roldan, B Sánchez
No abstract text is available yet for this article.
2015: Journal of Investigational Allergology & Clinical Immunology
G Gualdi, V Lougaris, M Baronio, M Vitali, G Tampella, D Moratto, P Tanghetti, P Monari, P Calzavara-Pinton, A Plebani
No abstract text is available yet for this article.
2015: Journal of Investigational Allergology & Clinical Immunology
Guan Wee Wong, Michael A Heneghan
For many patients with autoimmune hepatitis (AIH), the presence of extrahepatic features is well recognised both at the time of presentation and during long-term follow-up. Concomitant 'autoimmune disorders' have been described in 20-50% of patients with AIH, both in adults and children. Indeed, the presence of these associated phenomena has been incorporated into both the original and revised International AIH group scoring systems as an aid to codifying the diagnosis. In acute index presentations, non-specific joint pains sometimes flitting in nature have been reported in 10-60% of patients, and while joint swelling is uncommon, rheumatoid arthritis and mixed connective tissue disease have been reported in 2-4% of patients with AIH...
2015: Digestive Diseases
F B Serinkan Cinemre, Hakan Cinemre, Cengiz Karacaer, Birsen Aydemir, Ahmet Nalbant, Tezcan Kaya, Ali Tamer
OBJECTIVES AND DESIGN: The growth factor midkine (MK) is a protein that is involved in cancer, inflammation, immunity. Vitamin D is a potent immunomodulator. Anti-Saccharomyces cerevisiae antibody (ASCA) is reported in autoimmune disorders, some of which are among the causes of vitamin D deficiency. The objective of this study was to investigate a possible association of MK and ASCA with vitamin D deficiency. MATERIALS AND METHODS: 208 adults presented to internal medicine outpatient clinic for history and physical examination has been studied...
February 2016: Inflammation Research: Official Journal of the European Histamine Research Society ... [et Al.]
Hanen Ouadani, Imen Ben-Mustapha, Meriem Ben-ali, Leila Ben-khemis, Beya Larguèche, Raoudha Boussoffara, Sonia Maalej, Ilhem Fetni, Saida Hassayoun, Abdelmajid Mahfoudh, Fethi Mellouli, Sadok Yalaoui, Hatem Masmoudi, Mohamed Bejaoui, Mohamed-Ridha Barbouche
Immunoglobulin class switch recombination deficiencies (Ig-CSR-D) are characterized by normal or elevated serum IgM level and absence of IgG, IgA, and IgE. Most reported cases are due to X-linked CD40L deficiency. Activation-induced cytidine deaminase deficiency is the most frequent autosomal recessive form, whereas CD40 deficiency is more rare. Herein, we present the first North African study on hyper IgM (HIGM) syndrome including 16 Tunisian patients. Phenotypic and genetic studies allowed us to determine their molecular basis...
January 2016: Immunogenetics
Diego S Fernández Romero, María Gabriela Torre, Blas J Larrauri, Eloisa Malbrán, María Cecilia Juri, Alejandro Malbrán
Rituximab, a chimeric monoclonal antibody against CD20, induces the depletion of B lymphocytes. It is used for the treatment of lymphoproliferative and autoimmune diseases. Antibody immunodeficiency associated to RTX treatment is a new motif for consultation to our service. We decided to study those patients that having been treated with RTX, consulted for hypogammaglobulinemia or recurrent infections between November 2010 and December 2014. We evaluated eight patients, seven female and one male. The average follow up time was 19...
2015: Medicina
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