N Ramírez-Vargas1, S E Arablin-Oropeza1, D Mojica-Martínez2, M A Yamazaki-Nakashimada3, M de la Luz García-Cruz4, L M Terán-Juárez4, R M Cortés-Grimaldo5, C Torres-Lozano6, I Madrigal-Beas6, M Ortega-Cisneros6, M E Vargas-Camaño7, T Staines-Boone8, D Pietropaolo-Cienfuegos9, L Berrón-Ruiz1, F J Espinosa-Rosales10, M Guevara-Cruz11, L Blancas-Galicia12. 1. Unidad de Investigación en Inmunodeficiencias, Instituto Nacional de Pediatría, Mexico City, Mexico. 2. Centro Médico Nacional, La Raza, IMSS, Mexico City, Mexico. 3. Servicio de Inmunología, Instituto Nacional de Pediatría, Mexico City, Mexico. 4. Departamento de Alergia, Instituto Nacional de Enfermedades Respiratorias, Mexico City, Mexico. 5. Clínica 180 del IMSS, Guadalajara, Mexico. 6. Hospital de Especialidades del Centro Médico Nacional de Occidente, IMSS, Guadalajara, Mexico. 7. Centro Médico Nacional 20 de Noviembre, ISSSTE, Mexico City, Mexico. 8. Unidad Médica de Alta Especialidad #25 IMSS, Monterrey N.L., Mexico. 9. Servicio de Alergia e Inmunología, Hospital Infantil de Mexico, Mexico City, Mexico. 10. Unidad de Investigación en Inmunodeficiencias, Instituto Nacional de Pediatría, Mexico City, Mexico; Dirección de investigación, Instituto Nacional de Pediatría, Mexico City, Mexico. 11. Departamento de Fisiologia de la Nutrición, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubiran, Mexico. 12. Unidad de Investigación en Inmunodeficiencias, Instituto Nacional de Pediatría, Mexico City, Mexico. Electronic address: lbg73_2000@yahoo.com.
Abstract
BACKGROUND: Common variable immunodeficiency (CVID) is characterised by hypogammaglobulinaemia and a broad clinical spectrum, mainly showing recurrent bacterial infections accompanied sometimes by increased susceptibility to chronic lung disease, autoimmunity, and neoplastic diseases. OBJECTIVES: To evaluate the clinical and immunological characteristics of patients with CVID in Mexico. METHODS: This is a retrospective analysis of 43 patients with CVID from the Immunology Division of seven different reference centres in Mexico. Patients were diagnosed according to the diagnostic criteria of the European Society for Immunodeficiency Diseases. We collected demographics, clinical and immunological data from each patient and a statistical analysis was performed. RESULTS: There were 23 (53.5%) male and 20 (46.5%) female patients. Median age at onset of disease was 13.7 years, and median age at diagnosis was 19 years. Average delay in diagnosis was 12.5 years. The median total serum levels of IgG, IgM, and IgA at diagnosis were 175, 18, and 17.8mg/dL, respectively. The mean percentage of CD19+ B cells was 8.15%. Sinusitis (83%), pneumonia (83%), gastrointestinal infection (70%), and acute otitis media (49%) were the most common manifestations. Bronchiectasis was present in 51% of the patients, 44% manifested non-infectious chronic diarrhoea, and 70% experienced weight loss. Autoimmunity was present in 23% of the patients; haemolytic anaemia and autoimmune thrombocytopenic purpura were the most common presentations. Allergy was present in 30.2% of patients, with allergic rhinitis and asthma being the most frequent types. Two patients developed malignancy. All the patients received Intravenous immunoglobulin (IVIG) as a fundamental part of the treatment at a mean dose of 408mg/kg. CONCLUSION: This is the first cohort of CVID reported in Mexico We found that infection diseases were the most frequent presentations at onset. Moreover, patients had an average diagnosis delay of twelve years and thus a major prevalence of bronchiectasis. We suggest performing an extended analysis of patients with CVID patients in other Latin American countries.
BACKGROUND: Common variable immunodeficiency (CVID) is characterised by hypogammaglobulinaemia and a broad clinical spectrum, mainly showing recurrent bacterial infections accompanied sometimes by increased susceptibility to chronic lung disease, autoimmunity, and neoplastic diseases. OBJECTIVES: To evaluate the clinical and immunological characteristics of patients with CVID in Mexico. METHODS: This is a retrospective analysis of 43 patients with CVID from the Immunology Division of seven different reference centres in Mexico. Patients were diagnosed according to the diagnostic criteria of the European Society for Immunodeficiency Diseases. We collected demographics, clinical and immunological data from each patient and a statistical analysis was performed. RESULTS: There were 23 (53.5%) male and 20 (46.5%) female patients. Median age at onset of disease was 13.7 years, and median age at diagnosis was 19 years. Average delay in diagnosis was 12.5 years. The median total serum levels of IgG, IgM, and IgA at diagnosis were 175, 18, and 17.8mg/dL, respectively. The mean percentage of CD19+ B cells was 8.15%. Sinusitis (83%), pneumonia (83%), gastrointestinal infection (70%), and acute otitis media (49%) were the most common manifestations. Bronchiectasis was present in 51% of the patients, 44% manifested non-infectious chronic diarrhoea, and 70% experienced weight loss. Autoimmunity was present in 23% of the patients; haemolytic anaemia and autoimmune thrombocytopenic purpura were the most common presentations. Allergy was present in 30.2% of patients, with allergic rhinitis and asthma being the most frequent types. Two patients developed malignancy. All the patients received Intravenous immunoglobulin (IVIG) as a fundamental part of the treatment at a mean dose of 408mg/kg. CONCLUSION: This is the first cohort of CVID reported in Mexico We found that infection diseases were the most frequent presentations at onset. Moreover, patients had an average diagnosis delay of twelve years and thus a major prevalence of bronchiectasis. We suggest performing an extended analysis of patients with CVIDpatients in other Latin American countries.
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
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