BACKGROUND: Common variable immunodeficiency (CVID) is the most common form of symptomatic primary immunodeficiency disease. It is characterized by hypogammaglobulinemia, increased predisposition to infections, autoimmunity, and cancer. OBJECTIVES: This study was performed to evaluate the clinical and immunological features of a group of pediatric patients with CVID. METHODS: The study population comprised 69 individuals with CVID diagnosed during childhood. RESULTS: The patients were followed up for a mean (SD) period of 5.2 (4.3) years. The mean diagnostic delay was 4.4 (3.6) years, which was significantly lower in patients who were diagnosed recently. Children were classified according to 5 clinical phenotypes: infections only (n=39), polyclonal lymphocytic infiltration (n=17), autoimmunity (n=12), malignancy (n=7), and enteropathy (n=3). Postdiagnosis survival (10-year) was 71%. CONCLUSIONS: The high percentages of pediatric patients with CVID in Iran may be due to the considerable prevalence of parental consanguinity in the region and an underlying genetic background.
BACKGROUND: Common variable immunodeficiency (CVID) is the most common form of symptomatic primary immunodeficiency disease. It is characterized by hypogammaglobulinemia, increased predisposition to infections, autoimmunity, and cancer. OBJECTIVES: This study was performed to evaluate the clinical and immunological features of a group of pediatric patients with CVID. METHODS: The study population comprised 69 individuals with CVID diagnosed during childhood. RESULTS: The patients were followed up for a mean (SD) period of 5.2 (4.3) years. The mean diagnostic delay was 4.4 (3.6) years, which was significantly lower in patients who were diagnosed recently. Children were classified according to 5 clinical phenotypes: infections only (n=39), polyclonal lymphocytic infiltration (n=17), autoimmunity (n=12), malignancy (n=7), and enteropathy (n=3). Postdiagnosis survival (10-year) was 71%. CONCLUSIONS: The high percentages of pediatric patients with CVID in Iran may be due to the considerable prevalence of parental consanguinity in the region and an underlying genetic background.
Authors: Monica Piquer Gibert; Laia Alsina; María Teresa Giner Muñoz; Ofelia Cruz Martínez; Karen Ruiz Echevarria; Olga Dominguez; Ana María Plaza Martín; Juan I Arostegui; Guillem de Valles; Manel Juan Otero; Maria Anunciacion Martin-Mateos Journal: Eur J Pediatr Date: 2015-03-10 Impact factor: 3.183
Authors: Eduardo Guaní-Guerra; Ulises Noel García-Ramírez; Ana Isabel Jiménez-Romero; José Manuel Velázquez-Ávalos; Gabriela Gallardo-Martínez; Francisco-Javier Mendoza-Espinoza Journal: Biomed Res Int Date: 2013-09-01 Impact factor: 3.411
Authors: Linde N Nijhof; Marco van Brussel; Esther M Pots; Raphaële R L van Litsenburg; Elise M van de Putte; Joris M van Montfrans; Sanne L Nijhof Journal: J Clin Immunol Date: 2021-03-17 Impact factor: 8.317