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IgA deficiency in adults

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...
October 24, 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...
October 6, 2016: 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
Christina S Yee, Michel J Massaad, Wayne Bainter, Toshiro K Ohsumi, Niko Föger, Andrew C Chan, Nurten A Akarsu, Caner Aytekin, Deniz Çagdas Ayvaz, Ilhan Tezcan, Özden Sanal, Raif S Geha, Janet Chou
BACKGROUND: Coronin-1A (CORO1A) is a regulator of actin dynamics important for T-cell homeostasis. CORO1A deficiency causes T(-)B(+) natural killer-positive severe combined immunodeficiency or T-cell lymphopenia with severe viral infections. However, because all known human mutations in CORO1A abrogate protein expression, the role of the protein's functional domains in host immunity is unknown. OBJECTIVE: We sought to identify the cause of the primary immunodeficiency in 2 young adult siblings with a history of disseminated varicella, cutaneous warts, and CD4(+) T-cell lymphopenia...
March 2016: Journal of Allergy and Clinical Immunology
Pavlína Králíčková, Eva Malá, Doris Vokurková, Ondřej Souček, Irena Krčmová, Zbyněk Hrnčíř
INTRODUCTION: Systemic lupus erythematosus (SLE) is a chronic autoimmune multisystem disease. The aim of our study was to clarify the frequency of decreased serum immunoglobulin levels in SLE patients. There were evaluated 799 results of serum immunoglobulin levels gained from 157 patients fulfilling revised ACR criteria in the retrospective study. RESULTS: The immunoglobulin levels under the normal range were found in 29/157 (18.5 %) patients. The most frequent was isolated reduction of IgG 12/157 (7...
September 2015: Vnitr̆ní Lékar̆ství
B G Jones, C M Oshansky, R Bajracharya, L Tang, Y Sun, S S Wong, R Webby, P G Thomas, J L Hurwitz
Vitamin A supports the induction of immunoglobulin (Ig)A responses at mucosal surfaces in mice, but much less is known about the influence of vitamins on antibody isotype expression in humans. To address this knowledge gap, we examined 46 residual blood samples from adults and children, some of whom were experiencing influenza virus infections of the respiratory tract. Assays were performed for retinol binding protein (RBP, a surrogate for vitamin A), vitamin D (a related vitamin) and antibody isotypes. Results showed that all but two tested samples exhibited RBP and/or vitamin D insufficiencies or deficiencies...
February 2016: Clinical and Experimental Immunology
Kumar Pallav, Hua Xu, Daniel A Leffler, Toufic Kabbani, Ciaran P Kelly
BACKGROUND AND AIMS: Multiple European studies report increased prevalence of selective immunoglobulin A deficiency (SIgAD) and partial immunoglobulin A deficiency (PIgAD) in patients with celiac disease (CD). However; prospective data representing North American adults are lacking. While SIgAD precludes the use of IgA-tissue-transglutaminase antibody (IgA-tTG), the effect of PIgAD on IgA-tTG sensitivity is not well documented. We aim to determine the prevalence and impact of IgA deficiency on CD presentation and diagnosis in North American adult patients...
January 2016: Journal of Gastroenterology and Hepatology
Gul Javid, Shaheen Nazir Lone, Abid Shoukat, Bashir Ahmed Khan, Gulam Nabi Yattoo, Altaf Shah, Jaswinder Singh Sodi, Mushtaq Ahmed Khan, Showkat Ali Zarger
OBJECTIVES: The purpose of this study was to evaluate the prevalence of celiac disease in adult patients with iron-deficiency anemia of obscure origin. METHODS: One hundred and sixty-one consecutive patients with iron-deficiency anemia of obscure origin were evaluated. Tissue transglutaminase antibodies levels were done and duodenal biopsies were scored according to Marsh classification. Diagnosis was based on positive IgA anti-tissue transglutaminase antibodies and abnormal histopathology...
July 2015: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
Martin W Laass, Roma Schmitz, Holm H Uhlig, Klaus-Peter Zimmer, Michael Thamm, Sibylle Koletzko
BACKGROUND: Untreated celiac disease is associated with increased morbidity and mortality. Until now, no up-to-date figures have been available on the prevalence of celiac disease among children and adolescents in Germany, or on the percentage of undiagnosed cases. METHODS: To estimate the prevalence of celiac disease, serum samples obtained from 2003 to 2006 from participants in the German Health Interview and Examination Survey for Children and Adolescents (KiGGS) were studied for celiac disease-specific autoantibodies and total IgA...
August 17, 2015: Deutsches Ärzteblatt International
M Seven, A Güven, T M Bozoğlu, A Tolun
Focal dermal hypoplasia (FDH), an X-linked dominant disease with a highly variable phenotype, presents mainly with congenital linear pigmentation of the skin, herniation of fat through the dermal defects and multiple papillomas. PORCNmicrodeletions are identified in a total of 12 FDH patients to date. Routine molecular methods for detecting microdeletions have proven not to be effective, as patients also carry a normal allele. Additionally, methods using copy number estimations are labor-intensive, time-consuming and require expensive equipment...
2015: Genetic Counseling
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