Infections in patients with primary immunodeficiencies in Pinar del Rio

Authors

Keywords:

INFECTIONS, DIAGNOSIS, PRIMARY IMMUNODEFICIENCY, INBORN ERRORS OF IMMUNITY.

Abstract

Introduction: inborn errors of immunity are disorders whose cause is a genetic defect in one or more components of the immune system. Although the form of presentation varies according to the genetic defect, most of them present infectious diseases with recurrent and persistent characteristics.

Objective: to determine the prevalence of infectious processes in patients registered with diagnosis of Primary Immunodeficiency in the Immunology Service at Pepe Portilla Provincial Pediatric Teaching Hospital, Pinar del Rio in the period from 1994 to March 2021.

Methods: descriptive, cross-sectional study. The medical records of 125 patients diagnosed with immunodeficiencies registered in the database of the Immunology Service at Pepe Portilla Provincial Pediatric Teaching Hospital of Pinar del Rio were analyzed.

Results: the 85,6 % of patients presented diagnosis of some predominant antibody deficit. The predominant sex was male and the age range was from 6 to 10 years old. The most frequent infections were acute catarrhal pharyngotonsillitis (77,6 %), followed by pneumonia (58,4 %) and tonsillitis (56 %). Of the alarm signs related to infections only 4 were found in the patients in the registry.

Conclusions: susceptibility to infections is not necessarily the predominant clinical feature in primary immunodeficiencies, but the infectious phenotype is still the best suggestive indicator for a defect in immunity. Although it is necessary to propose new criteria for infectious manifestations that facilitate the probable diagnosis of immunodeficiency in general and by groups.  

 

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References

1. Franco-Paredes C, Rodríguez-morales A. El comodín de la historia universal : las enfermedades infecciosas. RevHospNiños(BAires) [Internet]. 2020 [citado 11/03/2022]; 62(276): 15-21. Disponible en: http://revistapediatria.com.ar/edicion-276-comodin-la-historia-universal-las-enfermedades-infecciosas/

2. del Rosal Rabes T. Curso de formación: Inmunodeficiencias 2019. [Internet] Madrid: Asociación Española de Pediatría; 2019 [citado 11/03/2022]. Disponible en: http://continuum.aeped.es/courses/info/659

3. Valle AA. Inmunodeficiencias Primarias : un reto para la inmuno-genética. Rev Cuba Reumatol [Internet]. 2020 [citado 11/03/2022]; 22(2): e828. Disponible en: http://scielo.sld.php?script=sci_abstract&pid=S1817-59962020000200009

4. Bousfiha A, Jeddane L, Picard C, Al-Herz W, Ailal F, Chatila T, et al. Human Inborn Errors of Immunity: 2019 Update of the IUIS Phenotypical Classification. J Clin Immunol [Internet]. 2020 [citado el 11/03/2022]; 40(1): 66-81. Disponible en: https://doi.org/10.1007/s10875-020-00758-x

5. Tangye SG, Al-herz W, Bousfiha A, Chatila T, Cunningham-rundles C. Human Inborn Errors of Immunity: 2019 Update on the Classification from the International Union of Immunological Societies Expert Committee. J Clin Immunol [Internet]. 2020 [citado 11/03/2022]; 40: 24–64. Disponible en: http://doi.org/10.1007/s10875-019-00737-x

6. Abraham CM. Una mirada al diagnóstico y tratamiento de las inmunodeficiencias primarias en Cuba. Rev Cuba Hematol Inmunol y Hemoter [Internet]. 2019 [citado 11/03/2022]; 35(4): 1-5. Disponible en: http://www.revhematologia.sld.cu/index.php/hih/article/view/1178/1040

7. Martín FDJ. Enfermedades infecciosas en pediatría : cambios y avances en los últimos 50 años. Boletín la Soc Pediatría Aragón, la Rioja y Soria [Internet]. 2020 [citado 11/03/2022]; 50(1): 34-8. Disponible en: http://dialnet.unirioja.es/servlet/articulo?codigo=7387277

8. Elva S, Rosa A, Hernández-martínez C, Espinosa-rosales F, Elva S, Hernández-martínez AR, et al. Conceptos básicos de las inmunodeficiencias primarias. Rev Alerg México [Internet]. 2016 [citado 11/03/2022]; 63(2): 180-9. Disponible en: http://doi.org/10.29262/ram.v63i2.146

9. Jeffrey Modell Foundation. Know the 10 Warning Signs of PI. 2013. Disponible en: http://www.info4pi.org

10. García-torres DA, Macías-robles AP, Pérez-jiménez L, Rodríguez-saldaña MB, Albores-chávez YF, Tlacuilo-parra A, et al. Características clínicas de las inmunodeficiencias primarias en niños de un hospital de tercer nivel. Rev Alerg Mex [Internet]. 2020 [citado 11/03/2022]; 67(3): 202-13. Disponible en: https://revistaalergia.mx/ojs/index.php/ram/article/view/721/1299

11. Soler-Palacín P. Inmunodeficiencias primarias. Málaga : 17 Congreso Actualización Pediatría [Internet]. 2020 [citado 11/03/2022]. p. 311-20. Disponible en: https://www.aepap.org/sites/default/files/documento/archivos-adjuntos/congreso2020/311-320_inmunodeficiencias.pdf

12. Abolhassani H, Azizi G, Sharifi L, Yazdani R, Mahdaviani SA, Kalantari A, et al. Global systematic review of primary immunodeficiency registries. Expert Rev Clin Immunol [Internet]. 2020 [citado 11/03/2022]; 16(7): 717-32. Disponible en: https://pubmed.ncbi.nlm.nih.gov/32720819/

13. Shillitoe B, Bangs C, Guzman D, Gennery AR, Longhurst HJ, Slatter M, et al. The United Kingdom Primary Immune Deficiency ( UKPID ) registry 2012 to 2017. Clin Exp Immmunology [Internet]. 2018 [citado 11/03/2022]; 192(3): 284-91. Disponible en: https://doi.org/10.1111/cei.13125

14. Fernandez J. Deficiencia selectiva de anticuerpos con inmunoglobulinas normales (DSAIN) [Internet]. Manual Msd; 2019 [citado 11/03/2022]. Disponible en: https://www.msdmanuals.com/es/professional/inmunología-y-trastornos-alérgicos/inmunodeficiencias/deficiencia-selectiva-de-anticuerpos-con-inmunoglobulinas-normales-dsain#

15. Wu J, Zhong W, Yin Y, Zhang H. Primary immunodeficiency disease : a retrospective study of 112 Chinese children in a single tertiary care center. BMC Pediatr [Internet]. 2019 [citado 11/03/2022]; 19(1): 1-7. Disponible en: https://www.researchgate.net/publication/337015977_Primary_immunodeficiency_disease_A_retrospective_study_of_112_Chinese_children_in_a_single_tertiary_care_center

16. Morales Pamela CS. DEFICIENCIA DE ANTICUERPOS ESPECÍFICOS EN PACIENTES PEDIÁTRICOS CON INFECCIONES RESPIRATORIAS RECURRENTES. Neumol Pediatr [Internet]. 2019 [citado 11/03/2022]; 14(3): 154-8. Disponible en: https://www.neumologia-pediatrica.cl/index.php/NP/article/view/102

17. Soler-palacín P, Gracia J De, González-granado LI, Martín C, Rodríguez-Gallego C, Sánchez-ramón S, et al. Primary immunodeficiency diseases in lung disease : warning signs , diagnosis and management. Respir Res [Internet]. 2018 [citado 11/03/2022]; 19(1): 219. Disponible en: https://pubmed.ncbi.nlm.nih.gov/30419907/

18. Bahrami A, Sayyahfar S, Soltani Z, Khodadost M, Moazzami B, Rezaei N. Evaluation of the frequency and diagnostic delay of primary immunodeficiency disorders among suspected patients based on the 10 warning sign criteria: A cross-sectional study in Iran. Allergol Immunopathol (Madr) [Internet]. 2020 [citado 11/03/2022]; 48(6): 711-9. Disponible en: https://all-imm.com/index.php/aei/article/view/122/98

Published

2022-07-03

How to Cite

1.
Lugo-Hernández A, Orraca-Castillo O, Miló-Valdés CA, Barreras-Sixto D, Valdés-Lanza L, Peláez-Yáñez LA. Infections in patients with primary immunodeficiencies in Pinar del Rio. Rev Ciencias Médicas [Internet]. 2022 Jul. 3 [cited 2025 Aug. 1];26(4):e5487. Available from: https://revcmpinar.sld.cu/index.php/publicaciones/article/view/5487

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ORIGINAL ARTICLES