Immunological features in pediatric patients with recurrent and severe infection: Identification of Primary Immunodeficiency Diseases in Merida, Venezuela.

Autor:	Linares NA; Instituto de Inmunología Clínica, Facultad de Medicina, Universidad de Los Andes, Merida, Venezuela., Bouchard M; Instituto de Inmunología Clínica, Facultad de Medicina, Universidad de Los Andes, Merida, Venezuela., Gutiérrez NS; Instituto de Inmunología Clínica, Facultad de Medicina, Universidad de Los Andes, Merida, Venezuela., Colmenares M; Centro de Microscopía Electrónica 'Dr Ernesto Palacios Prü'. Universidad de Los Andes, Mérida, Venezuela., Cantor-Garcia A; Instituto de Inmunología Clínica, Facultad de Medicina, Universidad de Los Andes, Merida, Venezuela., Gabaldon-Figueira JC; Instituto de Inmunología Clínica, Facultad de Medicina, Universidad de Los Andes, Merida, Venezuela., Bellorin AV; Instituto de Inmunología Clínica, Facultad de Medicina, Universidad de Los Andes, Merida, Venezuela., Rujano B; Departamento de Pediatría, Instituto Autónomo Hospital Universitario de Los Andes, Merida, Venezuela., Peterson DL; Department of Biochemistry and Molecular Biology, Virginia Commonwealth University, Richmond, VA, USA., Salmen S; Instituto de Inmunología Clínica, Facultad de Medicina, Universidad de Los Andes, Merida, Venezuela. Electronic address: sihamsa@ula.ve.
Jazyk:	angličtina
Zdroj:	Allergologia et immunopathologia [Allergol Immunopathol (Madr)] 2019 Sep - Oct; Vol. 47 (5), pp. 437-448. Date of Electronic Publication: 2019 Jul 29.
DOI:	10.1016/j.aller.2019.02.002
Abstrakt:	Introduction and Objectives: Primary 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 Methods: Following 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 Conclusions: During 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. (Copyright © 2019 SEICAP. Published by Elsevier España, S.L.U. All rights reserved.)
Databáze:	MEDLINE
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