Immunological features in pediatric patients with recurrent and severe infection: Identification of Primary Immunodeficiency Diseases in Merida,Venezuela |
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| Affiliation: | 1. Instituto de Inmunología Clínica, Facultad de Medicina, Universidad de Los Andes, Merida, Venezuela;2. Centro de Microscopía Electrónica “Dr Ernesto Palacios Prü”. Universidad de Los Andes, Mérida, Venezuela;3. Departamento de Pediatría, Instituto Autónomo Hospital Universitario de Los Andes, Merida, Venezuela;4. Department of Biochemistry and Molecular Biology, Virginia Commonwealth University, Richmond, VA, USA;1. Istanbul Medipol University, Department of Pediatric Allergy and Immunology, Istanbul, Turkey;2. Yeditepe University Hospital, Department of Pediatrics, Istanbul, Turkey;3. Bagcilar Training and Research Hospital, Department of Pediatrics, Istanbul, Turkey;4. Bagcilar Training and Research Hospital, Department of Biochemistry, Istanbul, Turkey;1. CEUMA University, Rua Josué Montello, n: 1, Renascença II, CEP: 65.075-120 São Luís, Maranhão, Brazil;2. Laboratory of Environmental Microbiology, Master''s Program in Environment, CEUMA University, Rua Josué Montello, n: 1, Renascença II, CEP: 65.075-120 São Luís, Maranhão, Brazil;3. Federal University of Maranhão - UFMA, Department of Biology, Av dos Portugueses 1966, Bacanga, CEP: 65080-805 São Luís, Maranhão, Brazil;4. Laboratory of Immunophysiology, Federal University of Maranhão, Av dos Portugueses 1966, Bacanga, CEP: 65080-805 São Luís, Maranhão, Brazil;1. University of Groningen, the Netherlands;2. Lifelong Learning, Education and Assessment Research Network (LEARN), Centre for Education Development and Research in Health Professions, University of Groningen and University Medical Centre Groningen, Groningen, The Netherlands;3. Isala Academy, Department of Medical Education and Faculty Development, Isala Hospital, Zwolle, the Netherlands;4. School of Human and Community Development, University of the Witwatersrand, Johannesburg, South Africa |
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| Abstract: | ![]()
Introduction and objectivesPrimary immunodeficiency diseases (PIDs) are disorders associated mainly with recurrent and severe infection and an increase in susceptibility to autoimmune conditions and cancer. In Venezuela, PIDs are underdiagnosed and there is usually a delay in their diagnosis. Hence there are no data concerning the frequency and type of PIDs that occur. The aim of this study was to identify and quantify the types of PIDs that occur in Merida, a population within Venezuela.Patients or materials and methodsFollowing an informative program designed to alert local health professionals to the warning signs for PIDs, patients with a history of recurrent infections were referred to the Instituto de Inmunologia Clinica, Universidad de Los Andes.Results and conclusionsDuring the three-year period January 2014 to January 2017, thirty-two cases of PIDs were identified in pediatric patients, and 17 different types of PIDs, were identified. Predominantly antibody deficiencies were most frequent (40.6%), followed by immunodeficiencies affecting cellular and humoral immunity (21.8%), congenital defects of phagocyte (18.7%), CID with associated or syndromic features (9.3%), defects in intrinsic and innate immunity (6.4%) and diseases of immune dysregulation (3.2%). These results have important implications not only to the future approach for management of patients in our regions, but add important knowledge concerning PIDs in Latin America and worldwide. |
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| Keywords: | PIDs Immune response Antibody deficiencies Immunodeficiencies affecting cellular and humoral immunity Congenital defects of phagocyte Defects in intrinsic and innate immunity Combined immunodeficiency Immune dysregulation |
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