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    2956 results match your criteria Common Variable Immunodeficiency

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    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

    The Hotel Study-Clinical and Health Service Effectiveness in a Cohort of Homeless or Marginally Housed Persons.
    Can J Psychiatry 2017 Jan 1:706743717693781. Epub 2017 Jan 1.
    1 Department of Psychiatry, University of British Columbia, Vancouver, British Columbia.
    Objective: The Hotel Study was initiated in Vancouver's Downtown East Side (DTES) neighborhood to investigate multimorbidity in homeless or marginally housed people. We evaluated the clinical effectiveness of existing, illness-specific treatment strategies and assessed the effectiveness of health care delivery for multimorbid illnesses.

    Method: For context, we mapped the housing locations of patients presenting for 552,062 visits to the catchment hospital emergency department (2005-2013). Read More

    Diagnosis of primary antibody and complement immunodeficiencies in young adults after a first invasive bacterial infection.
    Clin Microbiol Infect 2017 Feb 10. Epub 2017 Feb 10.
    Univ. Lille, INSERM U995 - LIRIC - Lille Inflammation Research International Center, F-59000 Lille, France; CHU Lille, Département de Médecine Interne et Immunologie Clinique, Centre National de Référence Maladies Systémiques et Auto-immunes Rares, F-59000 Lille, France; CHU Lille, Institut d'Immunologie, F-59000 Lille, France. Electronic address:
    Objectives: Screening for primary immunodeficiencies (PIDs) in adults is recommended after two severe bacterial infections. We aimed to evaluate if screening should be performed after the first invasive infection in young adults.

    Methods: Eligible patients were retrospectively identified using hospital discharge and bacteriology databases in 3 centers during a 3-year period. Read More

    Autoimmune and inflammatory manifestations occur frequently in primary immunodeficiencies.
    J Allergy Clin Immunol 2017 Feb 9. Epub 2017 Feb 9.
    Centre de Référence Déficits Immunitaires Héréditaires (CEREDIH), Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France; Unité d'Immuno-Hématologie et Rhumatologie pédiatrique, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France; Paris Descartes - Sorbonne Paris Cité University, Imagine Institute, Paris, France; Laboratory of Human Genetics of Infectious Diseases, INSERM UMR 1163, Paris, France.
    Background: Primary immunodeficiencies (PIDs) are inherited diseases associated with a considerably increase in susceptibility to infections. It is known that PIDs can also predispose to cancer and immune diseases including allergy, autoimmunity and inflammation.

    Objective: We aimed at determining the incidence of autoimmunity and inflammation in PID patients. Read More

    [Hematopoietic stem cells transplant in patients with common variable immunodeficiency. Is a therapeutic option?]
    Rev Alerg Mex 2017 Jan-Mar;64(1):121-125
    Instituto Mexicano del Seguro Social, Centro Médico Nacional Siglo XXI, Hospital de Especialidades, Servicio de Alergia e Inmunología Clínica. Ciudad de México, México.
    Background: Patients with common variable immunodeficiency show higher incidence of sinopulmonary and gastrointestinal infections, as well as lymphoproliferative and autoimmune diseases. The treatment of choice is replacement therapy with human gamma-globulin. Hematopoietic stem cell transplantation is a non-conventional therapeutic modality. Read More

    [Immunological alterations in common variable immunodeficiency].
    Rev Alerg Mex 2017 Jan-Mar;64(4):87-108
    Secretaría de Salud, Instituto Nacional de Pediatría, Unidad de Investigación en Inmunodeficiencias. Ciudad de México, México.
    Common variable immunodeficiency (CVID) is the largest group of symptomatic primary immune deficiencies; it is characterized by hypogammaglobulinemia, poor response to vaccines and increased susceptibility to infections. Cellular phenotypes and abnormalities have been described both in adaptive and innate immune response. Several classifications of common variable immunodeficiency are based on defects found on T and B cells, which have been correlated with clinical manifestations. Read More

    Pairwise diversity and tMRCA as potential markers for HIV infection recency.
    Medicine (Baltimore) 2017 Feb;96(6):e6041
    aDivision of Medical Virology, Stellenbosch University, Tygerberg, South Africa bBotswana-Harvard AIDS Institute Partnership, Gaborone, Botswana cAfrica Health Research Institute, School of Nursing and Public Health, University of KwaZulu-Natal, Durban, Republic of South Africa dHarvard T.H. Chan School of Public Health, Boston, MA, USA eNational Health Laboratory Services (NHLS), Tygerberg Coastal, South Africa fResearch Department of Infection, University College London, London, United Kingdom gCollege of Health Sciences, University of KwaZulu-Natal, Durban, Republic of South Africa hDivision of Sleep Medicine, Brigham and Women's Hospital, Boston, Massachusetts.
    Intrahost human immunodeficiency virus (HIV)-1 diversity increases linearly over time. We assessed the extent to which mean pairwise distances and the time to the most recent common ancestor (tMRCA) inferred from intrahost HIV-1C env sequences were associated with the estimated time of HIV infection. Data from a primary HIV-1C infection study in Botswana were used for this analysis (N = 42). Read More

    Inflammatory Duodenal Polyposis Associated with Primary Immunodeficiency Disease: A Novel Case Report.
    Case Rep Med 2017 10;2017:6206085. Epub 2017 Jan 10.
    Department of Sports Medicine, Safdarjung Hospital, New Delhi, India.
    Agammaglobulinemia is a rare form of B-cell primary immunodeficiency disease characterized by reduced levels of IgG, IgA, or IgM and recurrent bacterial infections. Agammaglobulinemia is most commonly associated with diffuse nodular lymphoid hyperplasia. Duodenal polyps are a rare entity; however, due to wide use of esophagogastroduodenoscopy, incidental diagnosis of duodenal polyps appears to be increasing. Read More

    Identifying functional defects in patients with immune dysregulation due to LRBA and CTLA-4 mutations.
    Blood 2017 Feb 3. Epub 2017 Feb 3.
    Institute of Immunity and Transplantation, Division of Infection & Immunity, School of Life and Medical Sciences, University College London, Royal Free Hospital, London, United Kingdom;
    Heterozygous CTLA-4 deficiency has been reported as a monogenic cause of common variable immune deficiency (CVID) with features of immune dysregulation. Direct mutation in CTLA-4 leads to defective regulatory T cell function associated with impaired ability to control levels of the CTLA-4 ligands, CD80 and CD86. However, additional mutations affecting the CTLA-4 pathway, such as those recently reported for LRBA, indirectly affect CTLA-4 expression resulting in clinically similar disorders. Read More

    Diffuse Nodular Lymphoid Hyperplasia of the Intestine Caused by Common Variable Immunodeficiency and Refractory Giardiasis.
    Intern Med 2017 1;56(3):283-287. Epub 2017 Feb 1.
    Department of Internal Medicine, Hanyang University College of Medicine, Korea.
    Diffuse nodular lymphoid hyperplasia of the gastrointestinal tract is a rare disease characterized by numerous small polypoid nodules in the small intestine, large intestine, or both. It is associated with immunodeficiency and infection, such as Giardia lamblia and Helicobacter pylori. Although diffuse nodular lymphoid hyperplasia associated with common variable immunodeficiency (CVID) and giardiasis is already known, a few studies have reported a regression of the lymphoid nodules after the eradication of infection. Read More

    [Retrospective study of 48 cases of primary central nervous system lymphoma].
    Medicina (B Aires) 2017 ;77(1):17-23
    Servicio de Neuroncología, Instituto de Investigaciones Neurológicas Raúl Carrea (FLENI), Buenos Aires, Argentina.
    Primary central nervous system lymphoma (PCNSL) is an infrequent form of non-Hodgkin lymphoma restricted to the CNS. More than 90% are type B and mainly affect patients aged 50-70 years. Immunodeficiency is the most important risk factor. Read More

    Evolving spectrum of LRBA deficiency-associated chronic arthritis: is there a causative role in juvenile idiopathic arthritis?
    Clin Exp Rheumatol 2017 Jan 27. Epub 2017 Jan 27.
    Department of Paediatrics, Allergy and Immunology, King Faisal Specialist Hospital and Research Centre; and Alfaisal University, Riyadh, Saudi Arabia.
    Lipopolysaccharide-responsive, beige-like anchor protein (LRBA) deficiency causes common variable immunodeficiency (CVID) disorders and autoimmunity. LRBA deficiency has become a clinically variable syndrome with a wide spectrum of clinical manifestations. We report a patient with LRBA deficiency associated chronic non-erosive arthritis. Read More

    IgG trough levels and progression of pulmonary disease in pediatric and adult CVID patients.
    J Allergy Clin Immunol 2017 Jan 23. Epub 2017 Jan 23.
    Division of Internal Medicine and Dermatology/ Department of Infectious diseases. University Medical Center Utrecht, Heidelberglaan 100, 3508 GA, Utrecht, the Netherlands. Electronic address:
    Capsule Summary: Common variable immunodeficiency patients show silent progression of airway disease which was inversely correlated to IgG trough levels. A randomized controlled study into optimal IgG through levels to prevent pulmonary disease progression is warranted. Read More

    Increased Incidence of Fatigue in Patients with Primary Immunodeficiency Disorders: Prevalence and Associations Within the US Immunodeficiency Network Registry.
    J Clin Immunol 2017 Jan 26. Epub 2017 Jan 26.
    Section of Immunology, Allergy and Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA.
    Introduction: Patients with primary immunodeficiency (PID) often report fatigue, yet this symptom has not been studied in PID. Fatigue affects 6-7.5% of healthy adults. Read More

    Pattern recognitions receptors in immunodeficiency disorders.
    Eur J Pharmacol 2017 Jan 14. Epub 2017 Jan 14.
    Mycobacteriology Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.
    Pattern recognition receptors (PRRs) recognize common microbial or host-derived macromolecules and have important roles in early activation and response of the immune system. Initiation of the innate immune response starts with the recognition of microbial structures called pathogen associated molecular patterns (PAMPs). Recognition of PAMPs is performed by germline-encoded receptors expressed mainly on immune cells termed pattern recognition receptors (PRRs). Read More

    NEIL1 is a candidate gene associated with common variable immunodeficiency in a patient with a chromosome 15q24 deletion.
    Clin Immunol 2017 Jan 14;176:71-76. Epub 2017 Jan 14.
    Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at Karolinska University Hospital Huddinge, SE-14186 Stockholm, Sweden. Electronic address:
    We report the first patient with an interstitial deletion of chromosome 15q24.1-q24.3 associated with common variable immunodeficiency (CVID). Read More

    Immunopathogenesis of granulomas in chronic autoinflammatory diseases.
    Clin Transl Immunology 2016 Dec 16;5(12):e118. Epub 2016 Dec 16.
    Department of Immunology and Pathology, Central Clinical School, Monash University , Melbourne, VIC, Australia.
    Granulomas are clusters of immune cells. These structures can be formed in reaction to infection and display signs of necrosis, such as in tuberculosis. Alternatively, in several immune disorders, such as sarcoidosis, Crohn's disease and common variable immunodeficiency, non-caseating granulomas are formed without an obvious infectious trigger. 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

    Thymoma-associated immunodeficiency: a diagnostic challenge for the clinician.
    Asian Cardiovasc Thorac Ann 2017 Feb 9;25(2):146-149. Epub 2017 Jan 9.
    1 Department of Respiratory Medicine, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.
    Good's syndrome or thymoma-associated immunodeficiency is a rare clinical entity that is often presumed to be common variable immunodeficiency, due to lack of awareness and recognition of this syndrome. This syndrome more often goes unrecognized if a thymoma is not detected. An appropriate immunological work-up that aids timely diagnosis and adequate therapy with antimicrobials and intravenous immunoglobulins are mandatory to prevent the long-term complications and mortality associated with this syndrome. Read More

    Common Variable Immunodeficiency patients with a phenotypic profile of immunosenescence present with thrombocytopenia.
    Sci Rep 2017 Jan 5;7:39710. Epub 2017 Jan 5.
    CLIP - Childhood Leukemia Investigation Prague, Department of Paediatric Haematology and Oncology, Second Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic.
    Common variable immunodeficiency (CVID) is a heterogeneous group of diseases. Our aim was to define sub-groups of CVID patients with similar phenotypes and clinical characteristics. Using eight-color flow cytometry, we analyzed both B- and T-cell phenotypes in a cohort of 88 CVID patients and 48 healthy donors. Read More

    Effect of a Primary Care-Based Psychological Intervention on Symptoms of Common Mental Disorders in Zimbabwe: A Randomized Clinical Trial.
    JAMA 2016 12;316(24):2618-2626
    London School of Hygiene and Tropical Medicine, London, United Kingdom.
    Importance: Depression and anxiety are common mental disorders globally but are rarely recognized or treated in low-income settings. Task-shifting of mental health care to lay health workers (LHWs) might decrease the treatment gap.

    Objective: To evaluate the effectiveness of a culturally adapted psychological intervention for common mental disorders delivered by LHWs in primary care. Read More

    Microbial Dysbiosis in Common Variable Immune Deficiencies: Evidence, Causes, and Consequences.
    Trends Immunol 2016 Dec 22. Epub 2016 Dec 22.
    Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, the Netherlands. Electronic address:
    Common variable immunodeficiency (CVID) is an immune disorder that not only causes increased susceptibility to infection, but also to inflammatory complications such as autoimmunity, lymphoid proliferation, malignancy, and granulomatous disease. Recent findings implicate the microbiome as a driver of this systemic immune dysregulation. Here, we critically review the current evidence for a role of the microbiome in the pathogenesis of CVID immune dysregulation, and describe the possible immunologic mechanisms behind causes and consequences of microbial dysbiosis in CVID. Read More

    Mucosal-associated invariant T cells are depleted and functionally altered in patients with common variable immunodeficiency.
    Clin Immunol 2016 Dec 21;176:23-30. Epub 2016 Dec 21.
    Discipline of Immunology, School of Medicine, Trinity College Dublin, Dublin, Ireland. Electronic address:
    Common variable immunodeficiency (CVID) is a primary immunoglobulin deficiency characterized by recurrent infections and complications, including autoimmunity, enteropathy, polyclonal lymphocytic infiltration or lymphoid malignancy. Innate T cells can support B cell maturation and antibody production. We investigated the numbers, phenotypes and functions of circulating B cell, γδ T cell, invariant natural killer T (iNKT) cell and mucosal-associated invariant T (MAIT) cell subsets in 23 CVID patients and 27 healthy controls. Read More

    Impact of Sexual Orientation Identity on Medical Morbidities in Male-to-Female Transgender Patients.
    LGBT Health 2017 Feb 22;4(1):11-16. Epub 2016 Dec 22.
    1 Department of Urology, San Francisco General Hospital, University of California San Francisco , San Francisco, California.
    Purpose: We aim to describe the relationship between sexual orientation identity and medical morbidities in a large sample of male-to-female (MTF) transgender patients.

    Methods: We reviewed medical records of patients presenting for MTF sex reassignment surgery (SRS) by a single, high-volume surgeon from 2011 to 2015. Sexual attraction to men (heterosexual), women (lesbian), or both (bisexual) was asked of each patient. Read More

    [Common variable immunodeficiency in adults].
    Ter Arkh 2016 ;88(11):94-98
    I.I. Mechnikov North-Western State Medical University, Ministry of Health of Russia, Saint Petersburg, Russia.
    The paper analyzes 7 cases of common variable immune deficiency (CVID), a primary immunodeficiency disease. All the cases were detected in outpatients over the age of 40 years. The diagnosis was based on their history data and general clinical findings with due regard for the results of previously conducted functional studies, expert opinions, and the results of immunological studies including the quantitative and functional indices of T and B cells, phagocytes and the levels of immunoglobulins. Read More

    When to initiate immunoglobulin replacement therapy (IGRT) in antibody deficiency: a practical approach.
    Clin Exp Immunol 2016 Dec 21. Epub 2016 Dec 21.
    Department of Clinical Immunology and Allergology, St Annes's University Hospital, Faculty of Medicine, Masaryk University, Brno, Czech Republic.
    Primary antibody deficiencies (PAD) constitute the majority of all primary immunodeficiency diseases (PID) and immunoglobulin replacement forms the mainstay of therapy for many patients in this category. Secondary antibody deficiencies (SAD) represent a larger and expanding number of patients resulting from the use of a wide range of immunosuppressive therapies, in particular those targeting B cells, and may also result from renal or gastrointestinal immunoglobulin losses. While there are clear similarities between primary and secondary antibody deficiencies, there are also significant differences. Read More

    Resistance to First-Line Antituberculosis Drugs in Washington State by Region of Birth and Implications for Latent Tuberculosis Treatment among Foreign-Born Individuals.
    Am J Trop Med Hyg 2016 Dec 19. Epub 2016 Dec 19.
    Washington State Department of Health Tuberculosis Program, Olympia, Washington.
    The United States has a low burden of drug resistance among tuberculosis (TB) cases compared with other world regions. TB is increasingly concentrated among foreign-born individuals who have higher rates of drug resistance than U.S. Read More

    Salivary Immunoglobulins in Individuals with Common Variable Immunodeficiency.
    Braz Dent J 2016 Oct-Dec;27(6):641-645
    Stomatology Department, Dental School of São Paulo, USP - Universidade de São Paulo, SP, Brazil.
    Oral manifestations of common variable immunodeficiency (CVID) are rare, have rarely been studied and have given controversial results. There are few data about IgA, IgG, and IgM antibody salivary levels in the literature, and there are few papers about the clinical impact of antibody deficiencies and CVID on the oral health of such patients. The aim of this study was to measure serum and salivary IgA, IgG, and IgM levels in CVID participants and controls, and to associate immunoglobulin levels with caries and periodontal disease. Read More

    Gene mutations responsible for primary immunodeficiency disorders: A report from the first primary immunodeficiency biobank in Iran.
    Allergy Asthma Clin Immunol 2016 2;12:62. Epub 2016 Dec 2.
    Acquired Immunodeficiency Research Center, Isfahan University of Medical Science, Khoram St, Isfahan, Iran.
    Background: Primary immunodeficiency (PID) is a heterogeneous group of inheritable genetic disorders with increased susceptibility to infections, autoimmunity, uncontrolled inflammation and malignancy. Timely precise diagnosis of these patients is very essential since they may not be able to live with their congenital immunity defects; otherwise, they could survive with appropriate treatment. DNA biobanks of such patients could be used for molecular and genetic testing, facilitating the detection of underlying mutations in known genes as well as the discovery of novel genes and pathways. Read More

    Advances in clinical immunology in 2015.
    J Allergy Clin Immunol 2016 Dec;138(6):1531-1540
    Immunology, Allergy and Rheumatology Section, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, Tex.
    Advances in clinical immunology in the past year included the report of practice parameters for the diagnosis and management of primary immunodeficiencies to guide the clinician in the approach to these relatively uncommon disorders. We have learned of new gene defects causing immunodeficiency and of new phenotypes expanding the spectrum of conditions caused by genetic mutations such as a specific regulator of telomere elongation (RTEL1) mutation causing isolated natural killer cell deficiency and mutations in ras-associated RAB (RAB27) resulting in immunodeficiency without albinism. Advances in diagnosis included the increasing use of whole-exome sequencing to identify gene defects and the measurement of serum free light chains to identify secondary hypogammaglobulinemias. Read More

    Idiopathic CD4 lymphocytopenia.
    Allergy Asthma Proc 2016 Nov;37(6):501-504
    1Department of Medical Education, Heart of Lancaster Regional Medical Center, Lititz, Pennsylvania, USA.
    Background: Idiopathic CD4 lymphocytopenia (ICL) is a rare disorder of unknown etiology. Diagnostic criteria include a persistent CD4 T-cell lymphopenia with no underlying primary or secondary immune deficiencies and a CD4 T-cell count of 300 cells/mL or 20% total lymphocyte on multiple occasions.

    Objective: To increase awareness of ICL and to provide a review of the clinical characteristics, diagnosis, and management of this disease process. Read More

    Patterns of constitutively phosphorylated kinases in B cells are associated with disease severity in common variable immunodeficiency.
    Clin Immunol 2017 Feb 3;175:69-74. Epub 2016 Dec 3.
    K.G. Jebsen Centre for Cancer Immunotherapy, Institute of Clinical Medicine, University of Oslo, Oslo, Norway; K.G. Jebsen Inflammation Research Centre, Institute of Clinical Medicine, University of Oslo, Oslo, Norway; Research Institute of Internal Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway; Section of Clinical Immunology and Infectious Diseases, Oslo University Hospital, Rikshospitalet, Oslo, Norway. Electronic address:
    Patients with common variable immunodeficiency (CVID) constitute a clinically and immunologically heterogeneous group characterized by B-cell dysfunction with hypogammaglobulinemia and defective immunoglobulin class switch of unknown etiology. Current classification systems are insufficient to achieve precise disease management. Characterization of signaling pathways essential for B-cell differentiation and class switch could provide new means to stratify patients. Read More

    Limitation of Simultaneous Analysis of T-Cell Receptor and κ-Deleting Recombination Excision Circles Based on Multiplex Real-Time Polymerase Chain Reaction in Common Variable Immunodeficiency Patients.
    Int Arch Allergy Immunol 2016 1;171(2):136-140. Epub 2016 Dec 1.
    Department of Clinical Immunology and Rheumatology, Hannover Medical School, Hannover, Germany.
    Aim Of Study: We used a triplex real-time polymerase chain reaction (PCR) to classify our common variable immunodeficiency (CVID) patients into distinct groups according to the amount of their T-cell receptor excision circles (TRECs) and κ-deleting recombination excision circles (KRECs).

    Materials And Methods: TREC and KREC analysis was performed using a multiplex real-time PCR assay. The T- and B-lymphocyte subsets were measured by flow cytometry. Read More

    FDG PET-CT imaging of therapeutic response in granulomatous lymphocytic interstitial lung disease (GLILD) in common variable immunodeficiency (CVID).
    Clin Exp Immunol 2017 Jan 28;187(1):138-145. Epub 2016 Nov 28.
    Department of Radiology, PETIC, University Hospital of Wales, Cardiff, UK.
    Common variable immunodeficiency (CVID) is the most common severe adult primary immunodeficiency and is characterized by a failure to produce antibodies leading to recurrent predominantly sinopulmonary infections. Improvements in the prevention and treatment of infection with immunoglobulin replacement and antibiotics have resulted in malignancy, autoimmune, inflammatory and lymphoproliferative disorders emerging as major clinical challenges in the management of patients who have CVID. In a proportion of CVID patients, inflammation manifests as granulomas that frequently involve the lungs, lymph nodes, spleen and liver and may affect almost any organ. Read More

    Immunodeficiency Presenting as an Undiagnosed Disease.
    Pediatr Clin North Am 2017 Feb;64(1):27-37
    Department of Pediatrics, Children's Corporate Center, Children's Research Institute, Medical College of Wisconsin, Suite C465, 9000 West Wisconsin Avenue, Milwaukee, WI 53226-4874, USA.
    Although primary immunodeficiencies typically present with recurrent, chronic, or severe infections, autoimmune manifestations frequently accompany these disorders and may be the initial clinical manifestations. The presence of 2 or more autoimmune disorders, unusual severe atopic disease, or a combination of these disorders should lead a clinician to consider primary immunodeficiency disorders. Read More

    [Primary immunodeficiency and autoimmunity].
    Rev Med Interne 2016 Nov 23. Epub 2016 Nov 23.
    CNRS UPR 3572 « immunopathologie et chimie thérapeutique », laboratoire d'excellence Medalis, institut de biologie moléculaire et cellulaire (IBMC), 67000 Strasbourg, France; Service d'immunologie clinique et de médecine interne, Centre national de référence des maladies auto-immunes rares, hôpitaux universitaires de Strasbourg, 1, place de l'Hôpital, 67091 Strasbourg, France.
    Many evidences highlight that immunodeficiency and autoimmunity are two sides of a same coin. Primary immune deficiencies (PIDs), which are rare mono- or multigenic defects of innate or adaptative immunity, frequently associate with autoimmunity. Analyses of single-gene defects in immune pathways of families with PIDs, by new tools of molecular biology (next genome sequencing technologies), allowed a better understanding of the ways that could both drive immune defect with immune deficiency and autoimmunity. Read More

    LPS-Responsive Beige-Like Anchor Gene Mutation Associated With Possible Bronchiolitis Obliterans Organizing Pneumonia Associated With Hypogammaglobulinemia and Normal IgM Phenotype and Low Number of B Cells.
    Acta Med Iran 2016 Oct;54(10):620-623
    Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. AND Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran. AND Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Boston, MA, USA.
    LPS-Responsive Beige-like Anchor (LRBA) deficiency is a disease which has recently been described in a group of patients with common variable immunodeficiency (CVID) in association with autoimmunity and/or inflammatory bowel disease (IBD)-like phenotype. We here describe a 10-year-old boy who experienced recurrent infections, mainly in the respiratory system, associated with thrombocytopenia and anemia. Immunological workup showed low numbers of B cells and low IgG, but normal IgM levels. Read More

    Enhanced formation of giant cells in common variable immunodeficiency: Relation to granulomatous disease.
    Clin Immunol 2017 Feb 14;175:1-9. Epub 2016 Nov 14.
    Department of Immunology, University College London, Royal Free Hospital NHS Trust, Pond Street, Hampstead NW3 2QG, London.
    Peripheral monocytes from patients with common variable immunodeficiency (CVID) had on average a 2 fold greater tendency to form giant cells in medium without additional cytokines. Giant cell formation was faster and 3 to 5 fold higher in most CVID cells compared to normal. Addition of IL4, GMCSF, IFNγ, TNFa and both T cell and monocyte conditioned media promoted monocyte fusion of some CVID individuals over 5 fold the normal average level, with combinations of cytokines and monokines acting synergistically. Read More

    Use of Cidofovir for Cytomegalovirus Disease Refractory to Ganciclovir in Solid Organ Recipients.
    Surg Infect (Larchmt) 2017 Feb/Mar;18(2):128-136. Epub 2016 Nov 16.
    6 Department of Pulmonology, Klinikum Klagenfurt , Klagenfurt, Austria .
    Background: Solid organ transplantation (SOT) frequently is complicated by cytomegalovirus (CMV) infections. Cidofovir (CDV) is active against CMV, including many ganciclovir (GCV)-resistant mutants, but often is considered to be too nephrotoxic for use after organ transplantation.

    Patients And Methods: Seven males and two females (median age 50. Read More

    Lung Disease in Primary Antibody Deficiencies.
    J Allergy Clin Immunol Pract 2016 Nov - Dec;4(6):1039-1052
    Division of Clinical Immunology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY. Electronic address:
    Primary antibody deficiencies (PADs) are the most common form of primary immunodeficiency and predispose to severe and recurrent pulmonary infections, which can result in chronic lung disease including bronchiectasis. Chronic lung disease is among the most common complications of PAD and a significant source of morbidity and mortality for these patients. However, the development of lung disease in PAD may not be solely the result of recurrent bacterial infection or a consequence of bronchiectasis. Read More

    Evaluation of ColdZyme® Mouth Spray on prevention of upper respiratory tract infections in a boy with primary immunodeficiency: a case report.
    J Med Case Rep 2016 Oct 31;10(1):302. Epub 2016 Oct 31.
    The Unit for Clinical Nursing Research and Clinical Research in Immunotherapy, Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at Karolinska University Hospital, Huddinge, Stockholm, 141 86, Stockholm, Sweden.
    Background: Primary immunodeficiencies include a variety of disorders that render patients more susceptible to infections. If left untreated, these infections may be fatal. Patients with primary antibody deficiencies are therefore given prophylactic immunoglobulin G replacement therapy. Read More

    Systematic review of case reports of antiphospholipid syndrome following infection.
    Lupus 2016 Dec 7;25(14):1520-1531. Epub 2016 Apr 7.
    Section of Rheumatology and Clinical Immunology, Department of General Internal Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
    Objective: The objective of this study was to conduct a systematic review of case reports documenting the development of antiphospholipid syndrome or antiphospholipid syndrome-related features after an infection.

    Methods: We searched Medline, EMBASE, Web of Science, PubMed ePubs, and The Cochrane Library - CENTRAL through March 2015 without restrictions. Studies reporting cases of antiphospholipid syndrome or antiphospholipid syndrome-related features following an infection were included. 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

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