The Latin American Society for Immunodeficiencies Registry.

Autor: Seminario G; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Centro de Inmunología Clínica, Buenos Aires, Argentina., Gonzalez-Serrano ME; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Laboratorio de Inmunodeficiencias, Instituto Nacional de Pediatría, Ciudad del Mexico, Mexico., Aranda CS; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Disciplina de Alergia, Imunologia Clínica e Reumatologia, Universidade Federal de Sao Paulo, São Paulo, Brazil., Grumach AS; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Faculdade de Medicina ABC, Santo André, Brazil., Segundo GRS; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Federal University of Uberlandia, Uberlandia, Brazil., Regairaz L; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Unidad de Inmunologia, Hospital de Niños Sor Maria Ludovica, La Plata, Argentina., Cardona AA; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Hospital Star Médica Aguascalientes, Aguas Calientes, Mexico., Becerra JCA; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Allergy and immunology Division, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru., Poli C; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Program of immunogenetics and translational immunology, Faculty of Medicine Clinica Alemana- Universidad del Desarrollo, Santiago, Chile., King A; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Unidad de Inmunologia, Hospital de Niños Luis Calvo Mackenna, Santiago, Chile., Fernandes FR; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Instituto Pensi, São Paulo, Brazil., Leiva L; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Health Science Center, Louisiana State University, New Orleans, USA., Franco JL; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Inborn Errrors of Immunity Group, Department of Microbiology & Parasitology, School of Medicine, University of Antioquia, Medelin, Colombia., Espinosa-Rosales FJ; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Centro de inmunología, alergia y pediatría, Hospital Ángeles Lomas, Mexico City, Mexico., Sorensen R; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Health Science Center, Louisiana State University, New Orleans, USA., Costa Carvalho BT; Disciplina de Alergia, Imunologia Clínica e Reumatologia, Universidade Federal de Sao Paulo, São Paulo, Brazil., Bezrodnik L; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.; Centro de Inmunología Clínica, Buenos Aires, Argentina., Condino-Neto A; Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil. antoniocondino@gmail.com.; Institute of Biomedical Sciences, University of São Paulo, São Paulo, Brazil. antoniocondino@gmail.com.
Jazyk: angličtina
Zdroj: Journal of clinical immunology [J Clin Immunol] 2024 Oct 22; Vol. 45 (1), pp. 28. Date of Electronic Publication: 2024 Oct 22.
DOI: 10.1007/s10875-024-01822-6
Abstrakt: Purpose - The Latin American Society of Immunodeficiencies (LASID) Registry was established in 2009 to collect data on Inborn Errors of Immunity (IEI) patients in the region. Although several reports have been published regarding LASID data, this is the first report of the entire dataset. Methods - The European Society of Immunodeficiencies (ESID) donated the online platform in 2008. Data was collected from participating centers from Apr 13, 2009, to Dec 31, 2022, and included demographic, clinical, and follow-up information. Results - A total of 9307 patients were included in the database. At the end of the study period, 8,805 patients were alive or lost to follow-up, and 502 were deceased. The most common type of IEI was predominantly antibody deficiency (PAD, 60.35%), and selective IgA deficiency was the most frequent diagnosis (1627 patients, 17.48%), followed by Common Variable Immune Deficiency (CVID, 1191 patients). Most patients (78.16%) were ≤ 18 years old at inclusion, and the median age at diagnosis was 4.77 years. The median time to diagnosis was 5.04 years. Antibiotics were prescribed in 32.3% of visits, followed by immunoglobulins (29.49% ). Hematopoietic stem cell transplantation was performed in 5.03% of patients. Omenn syndrome was the most common disease in deceased patients, with a mortality rate of 52.63%. Conclusion - This study contributes to our understanding of IEIs in Latin America and highlights the importance of early diagnosis, appropriate treatments, and improved data collection to optimize patient outcome.
(© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
Databáze: MEDLINE
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