Good's syndrome and common variable immune deficiency (CVID) are associated with chronic rhinosinusitis. Good's syndrome is characterized by hypogammaglobulinemia, B-cell depletion, variable defects in cellular immunity and thymoma. Immunodeficiency and recurrent infections can initially present after thymectomy. The pathogenesis can involve cytokines from bone marrow along with genetic defects. Intravenous gamma globulin (IVIG) restores defective signaling and can reestablish immune homeostasis. IVIG at regular intervals is the most effective way to improve the clinical symptoms and reduce patient mortality. Increased awareness of the clinical and immunological profile of this syndrome may increase its early recognition. CVID patients have hypogammaglobulinemia, respond to IVIG and have a dysregulated antimicrobial peptide response to pathogenic bacteria in the upper respiratory tract. This article reviewed selected literature on Good's syndrome, described an unusual case of Good's syndrome, CVID including SAD related to chronic rhinosinusitis.
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http://dx.doi.org/10.1007/s11882-014-0438-4 | DOI Listing |
Gastroenterology
December 2024
Department of Gastroenterology, Sir Run Run Hospital, Nanjing Medical University, Nanjing, China. Electronic address:
Respir Investig
November 2024
Department of Respiratory Medicine, Kyoto Katsura Hospital, 17 Yamadahirao-cho, Nishikyo-ku, Kyoto, 615-8256, Japan.
A 69-year-old man with a history of thymoma resection was diagnosed with COVID-19 pneumonia. Initially, the patient responded well to molnupiravir, but he experienced a relapse. Subsequent steroids for COVID-19-related organizing pneumonia (OP) led to temporary improvement, but his condition deteriorated when the steroids were tapered off.
View Article and Find Full Text PDFIran J Allergy Asthma Immunol
July 2024
Clinical Tuberculosis and Epidemiology Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Cureus
September 2024
General Medicine, King's College Hospital NHS Foundation Trust, London, GBR.
Good's syndrome is a rare secondary immunodeficiency characterised by recurrent infection and hypogammaglobulinemia associated with thymus tumour, and symptoms of recurrent infection may persist even after thymectomy. It is characterised by increased susceptibility to bacterial, viral, and fungal infections, as well as autoimmunity. Immunoglobulin replacement therapy has been reported to decrease infection rate, hospitalization, and the rate of antibiotic use in these patients.
View Article and Find Full Text PDFDiscov Oncol
October 2024
Department of Clinical Laboratory, South China Hospital, Medical School, Shenzhen University, 1 Fuxin Road, Shenzhen, 518111, China.
Objectives: Good's syndrome (GS) is a rare secondary immunodeficiency which is characterized by hypogammaglobulinemia and thymoma. This study aims to investigate the expression and distribution of B cells in thymoma tissue, given that B cells had been found to be reduced or absent in peripheral blood or bone marrow.
Methods: This study retrospectively analyzed thymoma tissues from 5 GS patients at the First Affiliated Hospital of Wenzhou Medical University and Zhejiang Provincial People's Hospital.
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