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Treatment of CVID

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https://www.readbyqxmd.com/read/28623282/a-nationwide-study-of-severe-and-protracted-diarrhoea-in-patients-with-primary-immunodeficiency-diseases
#1
Wen-I Lee, Chien-Chang Chen, Tang-Her Jaing, Liang-Shiou Ou, Chuen Hsueh, Jing-Long Huang
Diarrhoea lasting longer than 14 days and failing to respond to conventional management is defined as severe and protracted diarrhoea (SD). In this study, we investigated the prevalence, pathogens and prognosis of SD in primary immunodeficiency diseases (PIDs). Among 246 patients with predominantly paediatric-onset PIDs from 2003-2015, 21 [Btk (six), IL2RG (four), WASP, CD40L, gp91 (three each), gp47, RAG2 (one each)] and five [CVID (four), SCID (one)] without identified mutations had SD before prophylactic treatment...
June 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28606051/comparison-of-bone-mineral-density-in-common-variable-immunodeficiency-and-x-linked-agammaglobulinaemia-patients
#2
Ali Mohebbi, Gholamreza Azizi, Naeimeh Tavakolinia, Mehdi Karimipour, Fatemeh Kiaee, Reza Yazdani, Sareh Sadat Ebrahimi, Hosein Rafiemanesh, Vahid Ziaee, Hassan Abolhassani, Asghar Aghamohammadi, Farzaneh Abbasi, Fatemeh Sayarifard, Mehran Ebrahimi, Javad Tafaroji
BACKGROUND: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders, resulting from different defects in development and function of B cell lineage. Common variable immunodeficiency (CVID) and X-linked agammaglobulinemia (XLA) are two of the major types of PADs. Optimal growth and subsequently bone health could potentially compromise due to the interference of several factors in PAD with childhood onset. In the present study, our aim was to evaluate bone mineral density (BMD) of patients with CVID and XLA...
June 11, 2017: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/28600865/immune-deficiency-and-autoimmunity-in-patients-with-ctla-4-mutations
#3
REVIEW
Nisha Verma, Siobhan O Burns, Lucy Sk Walker, David M Sansom
Immune deficiency disorders are a heterogeneous group of diseases of variable genetic aetiology. Whilst the hallmark of immunodeficiency is susceptibility to infection, it is increasingly clear that autoimmunity is prevalent suggestive of a more general immune dysregulation in some cases. With the increasing use of genetic technologies the underlying causes of immune dysregulation are beginning to emerge. Here we provide a review of the heterozygous mutations found in the immune checkpoint protein CTLA-4, which was originally identified in cases of Common Variable Immunodeficiency Disorders (CVID) with accompanying autoimmunity...
June 10, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28554560/th1-phenotype-of-t-follicular-helper-cells-indicates-an-ifn%C3%AE-associated-immune-dysregulation-in-cd21low-cvid-patients
#4
Susanne Unger, Maximilian Seidl, Pauline van Schouwenburg, Mirzokhid Rakhmanov, Alla Bulashevska, Natalie Frede, Bodo Grimbacher, Jens Pfeiffer, Klaudia Schrenk, Luis Munoz, Leif Hanitsch, Ina Stumpf, Fabian Kaiser, Oliver Hausmann, Florian Kollert, Sigune Goldacker, Mirjam van der Burg, Baerbel Keller, Klaus Warnatz
BACKGROUND: A subgroup of patients with Common Variable Immunodeficiency (CVID) suffers from immune dysregulation, manifesting as autoimmunity, lymphoproliferation and organ inflammation, and thereby increasing morbidity and mortality. Therefore, treatment of these complications demands a deeper comprehension of their etiology and pathophysiology. OBJECTIVES: Based on the identification of an Interferon (IFN) signature in CVID patients with secondary complications and a skewed T follicular helper cell (TFH) differentiation in defined monogenic immunodeficiencies, we sought to determine the profile of CD4 memory T cells in blood and secondary lymphatic tissues of these patients...
May 26, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28536745/health-related-quality-of-life-in-adult-patients-with-common-variable-immunodeficiency-disorders-and-impact-of-treatment
#5
Nicholas L Rider, Carleigh Kutac, Joud Hajjar, Chris Scalchunes, Filiz O Seeborg, Marcia Boyle, Jordan S Orange
PURPOSE: Common variable immunodeficiency disorder (CVID) is a primary immunodeficiency disease (PIDD) often associated with severe and chronic infections. Patients commonly receive immunoglobulin (Ig) treatment to reduce the cycle of recurrent infection and improve physical functioning. However, how Ig treatment in CVID affects quality of life (QOL) has not been thoroughly evaluated. The purpose of a recent Immune Deficiency Foundation (IDF) mail survey was to assess the factors that are associated with QOL in patients with CVID receiving Ig treatment...
May 23, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28433201/humoral-primary-immunodeficiency-diseases-clinical-overview-and-chest-high-resolution-computed-tomography-hrct-features-in-the-adult-population
#6
REVIEW
L Cereser, R Girometti, P d'Angelo, M De Carli, A De Pellegrin, C Zuiani
Humoral primary immunodeficiency diseases (hPIDs) are a heterogeneous group of hereditary disorders resulting in abnormal susceptibility to infections of the sinopulmonary tract. Some of these conditions (e.g., common variable immunodeficiency disorders [CVID]) imply a number of non-infectious thoracic complications such as non-infectious airway disorders, diffuse lung parenchymal diseases, and neoplasms. Chest high-resolution computed tomography (HRCT) is a key imaging tool to characterise and quantify the extent of underlying thoracic involvement, as well as to direct and monitor treatment...
July 2017: Clinical Radiology
https://www.readbyqxmd.com/read/28345437/clinical-profile-dosing-and-quality-of-life-outcomes-in-primary-immune-deficiency-patients-treated-at-home-with-immunoglobulin-g-data-from-the-ideal-patient-registry
#7
Sean Kearns, Loretta Kristofek, William Bolgar, Luqman Seidu, Samantha Kile
BACKGROUND: Patients with primary immune deficiency (PID) often require immunoglobulin G (IgG, commonly referred to as Ig) replacement therapy to prevent infections and associated comorbidities. Ig therapy can be given either through intravenous or subcutaneous routes, and both can be done in the home setting. There is limited information available on the real-world diagnosis, management, and outcomes of this patient population, given the variable disease presentation and treatment options...
April 2017: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/28240614/diagnostic-and-therapeutic-considerations-in-patients-with-hypogammaglobulinemia-after-rituximab-therapy
#8
Ruba Kado, Georgiana Sanders, W Joseph McCune
PURPOSE OF REVIEW: There are no established guidelines for evaluating and treating hypogammaglobulinemia in patients with rheumatic disease who receive B-cell depleting agents. The purpose of this article is to review findings in the work-up and treatment of common variable immunodeficiency (CVID) that can guide our evaluation of patients with autoimmune disease who develop hypogammaglobulinemia after rituximab/B-cell depleting therapy. RECENT FINDINGS: Infection rates are higher in rheumatic disease patients who develop hypogammaglobulinemia than those who do not...
May 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28068853/chronic-lung-disease-in-common-variable-immune-deficiency-cvid-a-pathophysiological-role-for-microbial-and-non-b-cell-immune-factors
#9
Denver Mooney, David Edgar, Gisli Einarsson, Damian Downey, Stuart Elborn, Michael Tunney
One of the most common and most severe forms of primary antibody deficiency encountered in the clinical setting is a heterogeneous group of syndromes termed common variable immune deficiency (CVID). This disorder is characterized by reduced immunoglobulin production and increased susceptibility to infection, particularly of the respiratory tract. Infection and subsequent immunological/inflammatory processes may contribute to the development of pulmonary complications such as bronchiectasis and interstitial lung disease...
January 10, 2017: Critical Reviews in Microbiology
https://www.readbyqxmd.com/read/27896807/fdg-pet-ct-imaging-of-therapeutic-response-in-granulomatous-lymphocytic-interstitial-lung-disease-glild-in-common-variable-immunodeficiency-cvid
#10
S Jolles, E Carne, M Brouns, T El-Shanawany, P Williams, C Marshall, P Fielding
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...
January 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27856306/enhanced-formation-of-giant-cells-in-common-variable-immunodeficiency-relation-to-granulomatous-disease
#11
Timothy H Scott-Taylor, Karen Whiting, Ruth Pettengell, David A Webster
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...
February 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/27636680/autoimmunity-in-common-variable-immunodeficiency-epidemiology-pathophysiology-and-management
#12
REVIEW
Gholamreza Azizi, Hassan Abolhassani, Mohammad Hosein Asgardoon, Tina Alinia, Reza Yazdani, Javad Mohammadi, Nima Rezaei, Hans D Ochs, Asghar Aghamohammadi
Common variable immunodeficiency (CVID) comprises a large heterogeneous group of patients with primary antibody deficiency. Areas covered: The affected patients are characterized by increased susceptibility to infections and low levels of serum immunoglobulin. However, enteropathy, granulomatous organ infiltrates, malignancy, inflammatory and autoimmune conditions are also prevalent. The concomitance of immunodeficiency and autoimmunity appears to be paradoxical and creates difficulties in the management of autoimmune complications affecting these patients...
February 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/27614471/clonal-lymphoproliferations-in-a-patient-with-common-variable-immunodeficiency
#13
Sarah A Williams, Laura E Moench, Fatima Khan, Gregory Vercellotti, Michael A Linden
Common variable immunodeficiency (CVID) comprises a heterogeneous group of disorders of humoral immunity, characterized by marked deficiencies in serum immunoglobulins. Immune dysregulation causes susceptibility to recurrent bacterial infections, as well as autoimmune and lymphoproliferative disorders. Although the lymphoid cells comprising the atypical proliferations are often clonally related, their malignant potential and clinical significance differ from similar lesions in individuals with immunocompetence...
November 2016: Laboratory Medicine
https://www.readbyqxmd.com/read/27552393/immunoglobulin-replacement-therapy-reduces-chronic-rhinosinusitis-in-patients-with-antibody-deficiency
#14
Jarrett E Walsh, Jose G Gurrola, Scott M Graham, Sarah L Mott, Zuhair K Ballas
BACKGROUND: Patients with primary antibody deficiencies have an increased frequency of sinonasal and pulmonary infections. Immunoglobulin (Ig) replacement is a standard therapy for common variable immunodeficiency (CVID) and other antibody deficiency diseases. Although there is convincing evidence that Ig replacement reduces pulmonary infections, there is little evidence that it reduces sinus infections or abates chronic rhinosinusitis (CRS). This study aims to identify the impact of Ig replacement on CRS in antibody deficiencies...
January 2017: International Forum of Allergy & Rhinology
https://www.readbyqxmd.com/read/27511507/common-variable-immunodeficiency-in-adults-requires-reserved-protocols-for-long-term-follow-up
#15
Ahmet Zafer Çalişkaner, İsmail Reisli, Şevket Arslan, Ramazan Uçar, Hüseyin Ataseven, Nedim Yılmaz Selçuk
BACKGROUND/AIM: The aim of this study is to establish follow-up protocols for adult patients with common variable immunodeficiency (CVID) in a recently founded adult immunology clinic in the Central Anatolia Region of Turkey, where a clinical immunology center for adults was not available previously. MATERIALS AND METHODS: A total of 25 patients with CVID aged 18 years and older were included in this study. The file format consisted of 13 pages and was developed for the purpose of the study...
2016: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/27412077/anaphylaxis-to-ivig
#16
REVIEW
Sharon Julie Williams, Sudhir Gupta
Anaphylactic reactions are a known complication in some IgA-deficient patients receiving blood or plasma transfusions. It is of particular interest that anaphylaxis has been observed in patients with common variable immunodeficiency (CVID) who are receiving intravenous gammaglobulin (IVIG), and in that, although these patients have an impaired response to common vaccines, they retain the ability to produce autoantibodies. In this study, we review IgA antibodies (both IgG- and IgE-mediated reactions) in patients with CVID and hypogammaglobulinemia, anaphylaxis in antibody immunodeficient patients receiving IVIG, and proposed mechanisms of desensitization and prevention of anaphylactic reactions in immunodeficient patients receiving IVIG...
February 2017: Archivum Immunologiae et Therapiae Experimentalis
https://www.readbyqxmd.com/read/27379089/genetic-diagnosis-using-whole-exome-sequencing-in-common-variable-immunodeficiency
#17
Patrick Maffucci, Charles A Filion, Bertrand Boisson, Yuval Itan, Lei Shang, Jean-Laurent Casanova, Charlotte Cunningham-Rundles
Whole exome sequencing (WES) has proven an effective tool for the discovery of genetic defects in patients with primary immunodeficiencies (PIDs). However, success in dissecting the genetic etiology of common variable immunodeficiency (CVID) has been limited. We outline a practical framework for using WES to identify causative genetic defects in these subjects. WES was performed on 50 subjects diagnosed with CVID who had at least one of the following criteria: early onset, autoimmune/inflammatory manifestations, low B lymphocytes, and/or familial history of hypogammaglobulinemia...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27357411/comprehensive-activities-to-increase-recognition-of-primary-immunodeficiency-and-access-to-immunoglobulin-replacement-therapy-in-poland
#18
Małgorzata Pac, Ewa Bernatowska
UNLABELLED: The study presents an overview on current situation of primary immunodeficiency (PID) patients in Poland and the 2014 annual report of the Polish Working Group for Immunodeficiency (PWGID). The group was set up in 2005 to improve diagnosis, treatment, and care of patients with immunodeficiencies and currently includes 15 pediatric and 13 adult centers. According to PWGID report 4099, PID patients are recognized in Poland, with the prevalence 10.6/100,000. The majority of them (54...
August 2016: European Journal of Pediatrics
https://www.readbyqxmd.com/read/27338827/nf-%C3%AE%C2%BAb1-haploinsufficiency-causing-immunodeficiency-and-ebv-driven-lymphoproliferation
#19
Heidrun Boztug, Tatjana Hirschmugl, Wolfgang Holter, Karoly Lakatos, Leo Kager, Doris Trapin, Winfried Pickl, Elisabeth Förster-Waldl, Kaan Boztug
PURPOSE: NF-κB signaling is critically important for regulation of both innate and adaptive immune responses. While activation of NF-κB has been implicated in malignancies such as leukemia and lymphoma, loss-of-function mutations affecting different NF-κB pathway components have been shown to cause primary immunodeficiency disorders. Recently, haploinsufficiency of NF-κB1 has been described in three families with common variable immunodeficiency (CVID). METHODS AND RESULTS: We studied a patient with recurrent respiratory infections and bacterial parapharyngeal abscess...
August 2016: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/27335365/management-of-granulomatous-lymphocytic-interstitial-lung-disease-in-a-patient-with-common-variable-immune-deficiency
#20
REVIEW
Mohini Pathria, Daniel Urbine, Marc Stuart Zumberg, Juan Guarderas
A 61-year-old woman presented with longstanding cough and progressive dyspnoea. She underwent an extensive evaluation and was diagnosed with common variable immunodeficiency (CVID) with granulomatous lymphocytic interstitial lung disease (GLILD). She was initially treated with subcutaneous immunoglobulin therapy, having declined intravenous immunoglobulin (IVIG) therapy. She also declined treatment with oral glucocorticoids. Over several months, she became increasingly symptomatic and developed increased pulmonary infiltrates, pleural effusions, mediastinal adenopathy, splenomegaly, pancytopenia and ascites...
June 22, 2016: BMJ Case Reports
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