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Agammaglobulinemia

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Asghar Aghamohammadi – 1st expert on this subject based on the ideXlab platform

  • Agammaglobulinemia: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management.
    Endocrine‚ Metabolic & Immune Disorders-Drug Targets, 2020
    Co-Authors: Salar Pashangzadeh, Reza Yazdani, Gholamreza Azizi, Farzad Nazari, Hassan Abolhassani, Asghar Aghamohammadi

    Abstract:

    : Agammaglobulinemia is a type of primary antibody deficiencies, characterized by severe reduction in serum level of all types of immunoglobulins level and absence of B cells in the peripheral blood. X-linked and various autosomal recessive/dominant mutations have been identified underlying the pathogenesis of this disorder. Affected patients present a broad range of clinical manifestations, including respiratory infections, gastrointestinal complications, Enterovirus infections, autoimmunity, and malignancies. This disease can be controlled via different therapeutic strategies. In this review, we describe different aspects of Agammaglobulinemia such as epidemiology, pathogenesis, clinical phenotype, diagnosis, management, and prognosis of congenital Agammaglobulinemia.

  • Autosomal Recessive Agammaglobulinemia: A Novel Non-sense Mutation in CD79a
    Journal of Clinical Immunology, 2014
    Co-Authors: Abbas Khalili, Alessandro Plebani, Hassan Abolhassani, Vassilios Lougaris, Nima Rezaei, Massimiliano Vitali, Babak Mirminachi, Asghar Aghamohammadi

    Abstract:

    This study describes the fifth case worldwide of autosomal recessive Agammaglobulinemia due to a novel non-sense mutation in CD79a gene with a severe unusual onset due to an invasive central nervous system infection.

  • Clinical, Immunological and Molecular Characteristics of 37 Iranian Patients with X-Linked Agammaglobulinemia
    International Archives of Allergy and Immunology, 2006
    Co-Authors: Asghar Aghamohammadi, Hirokazu Kanegane, Maurilia Fiorini, Mostafa Moin, Nima Parvaneh, Shahram Teimourian, Mehdi Yeganeh, Francesca Goffi, Ali Akbar Amirzargar, Zahra Pourpak

    Abstract:

    Background: X-linked Agammaglobulinemia (XLA) is a hereditary immunodeficiency characterized by an early onset of recurrent bacterial infections, a profound deficiency of all immuno

Nima Rezaei – 2nd expert on this subject based on the ideXlab platform

  • Autosomal Recessive Agammaglobulinemia: A Novel Non-sense Mutation in CD79a
    Journal of Clinical Immunology, 2014
    Co-Authors: Abbas Khalili, Alessandro Plebani, Hassan Abolhassani, Vassilios Lougaris, Nima Rezaei, Massimiliano Vitali, Babak Mirminachi, Asghar Aghamohammadi

    Abstract:

    This study describes the fifth case worldwide of autosomal recessive Agammaglobulinemia due to a novel non-sense mutation in CD79a gene with a severe unusual onset due to an invasive central nervous system infection.

  • efficacy of intravenous immunoglobulin on the prevention of pneumonia in patients with Agammaglobulinemia
    Fems Immunology and Medical Microbiology, 2004
    Co-Authors: Asghar Aghamohammadi, Zahra Pourpak, Mostafa Moin, A Farhoudi, Nima Rezaei, Masoud Movahedi, Mohammad Gharagozlou, Mohammad Nabavi, Amin Shahrokhi

    Abstract:

    Abstract Agammaglobulinemia is characterized by failure of B-cell differentiation (hypogammaglobulinemia) and increased susceptibility to bacterial infections. The present study was set up in order to evaluate the effectiveness of intravenous immunoglobulin (IVIG) treatment on the incidence of pneumonia in patients with Agammaglobulinemia. We carried out chart reviews of 23 patients with Agammaglobulinemia (mean age 11.5±5.4 years), who had been observed in a 22-year period (July 1981–January 2003) in Iran’s referral center for primary immunodeficiency disorders. Nineteen of these 23 (82.5%) had been infected with pneumonia at least once before receiving the immunoglobulin treatment and 11 of them had experienced multiple episodes. During treatment with γ-globulin – over a mean period of 6.8±4.1 years (range: 0.8–15.3 years) – the incidence of pneumonia requiring treatment or hospitalization decreased from 0.82 to 0.12 per patient per year (P=0.006). During IVIG replacement, hospitalization due to pneumonia decreased from 0.58 to 0.05 per patient per year (P=0.08) and the immunoglobulin G level (mean±S.D.) changed from 66.2±63.9 (range: 0–210 mg dl−1) to 552.4±199.1 (range: 136–942 mg dl−1) (P

Vassilios Lougaris – 3rd expert on this subject based on the ideXlab platform

  • Chapter 13 – Agammaglobulinemia
    Stiehm's Immune Deficiencies, 2020
    Co-Authors: Alessandro Plebani, Vassilios Lougaris

    Abstract:

    Agammaglobulinemia is a rare form of primary immune deficiency characterized by absence of circulating B cells and low serum levels of all immunoglobulin classes, in the presence of normal T cell counts and function. Affected patients are particularly susceptible to infections, frequently severe ones. The majority (85%) of affected patients are males (X-linked form), while a small percentage (15%) of patients of both sexes present with an autosomal recessive trait of inheritance. In this latter case, not all patients have a definite genetic diagnosis as yet. Although current therapeutic protocols have prolonged the lifespan of patients with Agammaglobulinemia, their prognosis is affected by the occurrence of long-term complications – mainly the development of chronic lung disease (CLD).

  • a possible role for b cells in covid 19 lesson from patients with Agammaglobulinemia
    The Journal of Allergy and Clinical Immunology, 2020
    Co-Authors: Isabella Quinti, Vassilios Lougaris, Cinzia Milito, Francesco Cinetto, Antonio Pecoraro, Ivano Mezzaroma, Claudio Maria Mastroianni, Ombretta Turriziani, Maria Pia Bondioni, Matteo Filippini

    Abstract:

    Summary COVID-19 had a mild clinical course in patients with Agammaglobulinemia lacking B lymphocytes, whereas it developed aggressively in Common Variable Immune Deficiency. Our data offer mechanisms for possible therapeutic targets.

  • Autosomal Recessive Agammaglobulinemia: A Novel Non-sense Mutation in CD79a
    Journal of Clinical Immunology, 2014
    Co-Authors: Abbas Khalili, Alessandro Plebani, Hassan Abolhassani, Vassilios Lougaris, Nima Rezaei, Massimiliano Vitali, Babak Mirminachi, Asghar Aghamohammadi

    Abstract:

    This study describes the fifth case worldwide of autosomal recessive Agammaglobulinemia due to a novel non-sense mutation in CD79a gene with a severe unusual onset due to an invasive central nervous system infection.