Clinical and immunological features of common variable immunodeficiency in Mexican patients

被引:31
|
作者
Ramirez-Vargas, N. [1 ]
Arablin-Oropeza, S. E. [1 ]
Mojica-Martinez, D. [2 ]
Yamazaki-Nakashimada, M. A. [3 ]
de la Luz Garcia-Cruz, M. [4 ]
Teran-Juarez, L. M. [4 ]
Cortes-Grimaldo, R. M. [5 ]
Torres-Lozano, C. [6 ]
Madrigal-Beas, I. [6 ]
Ortega-Cisneros, M. [6 ]
Vargas-Camano, M. E. [7 ]
Staines-Boone, T. [8 ]
Pietropaolo-Cienfuegos, D. [9 ]
Berron-Ruiz, L. [1 ]
Espinosa-Rosales, F. J. [1 ,10 ]
Guevara-Cruz, M. [11 ]
Blancas-Galicia, L. [1 ]
机构
[1] Inst Nacl Pediat, Unidad Invest Inmunodeficiencias, Mexico City, DF, Mexico
[2] IMSS, La Raza, Ctr Med Nacl, Mexico City, DF, Mexico
[3] Inst Nacl Pediat, Serv Inmunol, Mexico City, DF, Mexico
[4] Inst Nacl Enfermedades Resp, Dept Alergia, Mexico City, DF, Mexico
[5] IMSS, Guadalajara, Mexico
[6] IMSS, Hosp Especialidades, Ctr Med Nacl Occidente, Guadalajara, Mexico
[7] ISSSTE, Ctr Med Nacl, Mexico City, DF, Mexico
[8] Unidad Med Alta Especialidad, Monterrey, NL, Mexico
[9] Hosp Infantil Mexico Dr Federico Gomez, Serv Alergia & Inmunol, Mexico City, DF, Mexico
[10] Inst Nacl Pediat, Direcc Invest, Mexico City, DF, Mexico
[11] Inst Nacl Ciencias Med Nutr Salvador Zubiran, Dept Fisiol Nutr, Mexico City, DF, Mexico
关键词
Common variable immunodeficiency; Primary immunodeficiency; Primary antibody deficiency; Humoral deficiency; CHILDREN;
D O I
10.1016/j.aller.2013.01.007
中图分类号
R392 [医学免疫学];
学科分类号
100102 ;
摘要
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 17.8 mg/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 408 mg/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. (C) 2012 SEICAP. Published by Elsevier Espana, S.L. All rights reserved.
引用
收藏
页码:235 / 240
页数:6
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