title: Clinical and immunological features of common variable immunodeficiency in Mexican patients.
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.
  
  
  OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Unidad de Investigación en Inmunodeficiencias, Instituto Nacional de Pediatría, Mexico City, Mexico.
authors.forename: N
authors.initials: N
authors.lastname: Ramírez-Vargas
authors.affiliation: Unidad de Investigación en Inmunodeficiencias, Instituto Nacional de Pediatría, Mexico City, Mexico.
authors.forename: S E
authors.initials: SE
authors.lastname: Arablin-Oropeza
authors.affiliation: Centro Médico Nacional, La Raza, IMSS, Mexico City, Mexico.
authors.forename: D
authors.initials: D
authors.lastname: Mojica-Martínez
authors.affiliation: Servicio de Inmunología, Instituto Nacional de Pediatría, Mexico City, Mexico.
authors.forename: M A
authors.initials: MA
authors.lastname: Yamazaki-Nakashimada
authors.affiliation: Departamento de Alergia, Instituto Nacional de Enfermedades Respiratorias, Mexico City, Mexico.
authors.forename: M
authors.initials: M
authors.lastname: de la Luz García-Cruz
authors.affiliation: Departamento de Alergia, Instituto Nacional de Enfermedades Respiratorias, Mexico City, Mexico.
authors.forename: L M
authors.initials: LM
authors.lastname: Terán-Juárez
authors.affiliation: Clínica 180 del IMSS, Guadalajara, Mexico.
authors.forename: R M
authors.initials: RM
authors.lastname: Cortés-Grimaldo
authors.affiliation: Hospital de Especialidades del Centro Médico Nacional de Occidente, IMSS, Guadalajara, Mexico.
authors.forename: C
authors.initials: C
authors.lastname: Torres-Lozano
authors.affiliation: Hospital de Especialidades del Centro Médico Nacional de Occidente, IMSS, Guadalajara, Mexico.
authors.forename: I
authors.initials: I
authors.lastname: Madrigal-Beas
authors.affiliation: Hospital de Especialidades del Centro Médico Nacional de Occidente, IMSS, Guadalajara, Mexico.
authors.forename: M
authors.initials: M
authors.lastname: Ortega-Cisneros
authors.affiliation: Centro Médico Nacional 20 de Noviembre, ISSSTE, Mexico City, Mexico.
authors.forename: M E
authors.initials: ME
authors.lastname: Vargas-Camaño
authors.affiliation: Unidad Médica de Alta Especialidad #25 IMSS, Monterrey N.L., Mexico.
authors.forename: T
authors.initials: T
authors.lastname: Staines-Boone
authors.affiliation: Servicio de Alergia e Inmunología, Hospital Infantil de Mexico, Mexico City, Mexico.
authors.forename: D
authors.initials: D
authors.lastname: Pietropaolo-Cienfuegos
authors.affiliation: Unidad de Investigación en Inmunodeficiencias, Instituto Nacional de Pediatría, Mexico City, Mexico.
authors.forename: L
authors.initials: L
authors.lastname: Berrón-Ruiz
authors.affiliation: Unidad de Investigación en Inmunodeficiencias, Instituto Nacional de Pediatría, Mexico City, Mexico; Dirección de investigación, Instituto Nacional de Pediatría, Mexico City, Mexico.
authors.forename: F J
authors.initials: FJ
authors.lastname: Espinosa-Rosales
authors.affiliation: Departamento de Fisiologia de la Nutrición, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubiran, Mexico.
authors.forename: M
authors.initials: M
authors.lastname: Guevara-Cruz
authors.affiliation: Unidad de Investigación en Inmunodeficiencias, Instituto Nacional de Pediatría, Mexico City, Mexico. Electronic address: lbg73_2000@yahoo.com.
authors.forename: L
authors.initials: L
authors.lastname: Blancas-Galicia
mesh_terms: D000293:Adolescent; D000328:Adult; D018941:Antigens, CD19 / Q000378:metabolism; D001249:Asthma / Q000276:immunology*; D015551:Autoimmunity; D001402:B-Lymphocytes / Q000276:immunology*; D001987:Bronchiectasis / Q000276:immunology*; D002648:Child; D015331:Cohort Studies; D017074:Common Variable Immunodeficiency / Q000276:immunology* / Q000503:physiopathology / Q000628:therapy; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000097:blood; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007239:Infections / Q000276:immunology*; D008297:Male; D008800:Mexico; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D018941:Antigens, CD19; D007136:Immunoglobulins; D016756:Immunoglobulins, Intravenous
keywords: Common variable immunodeficiency; Humoral deficiency; Primary antibody deficiency; Primary immunodeficiency