Autoinflammatory diseases. A challenge for the pediatrician

Authors

DOI:

https://doi.org/10.5281/zenodo.14876416

Keywords:

Autoinflammatory diseases, pediatrician

Abstract

The term autoinflammatory was used by doctors Daniel Kastner and John O'shea in 1999 to describe periodic fever syndromes, which corresponded to diseases inherited in an autosomal dominant manner due to mutations in the innate immune system, specifically in tumor necrosis factor alpha (TNF-α).

Currently there are countless autoinflammatory diseases, mainly in pediatric age, so it is necessary to have basic knowledge about these diseases; This contributes to improving the quality of life of these patients.

Autoinflammatory diseases (AID) are disorders characterized by episodic or continuous inflammation, without evidence of autoimmunity, usually related to defects in components of innate immunity.

This type of immunity constitutes the first line of defense against pathogens and harmful stimuli, through the recognition of molecular markers associated with infectious pathogens (PAMP) or damage (DAMP), with the subsequent activation of multiple inflammatory signaling cascades and their main effector cells: macrophages, neutrophils, mast cells and natural killer (NK) cells.

References

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Hernández Ostiz S, Prieto Torres L, Xirotagaros G, Noguera Morel L, Hernández Martín A, Torrelo A. Enfermedades autoinflamatorias en dermatología pediátrica. Parte 1: síndromes urticariformes, síndromes pustulosos y síndromes con ulceraciones cutáneo-mucosas. Actas Dermosifiliogr.2017 [citado 28/03/2020]; 108(7): 609-619. Disponible en: https://www.sciencedirect.com/science/article/abs/pii/S0001731017301771

Llobet Agulló MP, Moure González JD. Diagnóstico y manejo de las enfermedades autoinflamatorias en Pediatría. Protoc Diagn Ter Pediatr. 2019 [citado 13/02/2021]; 2: 453-469. Disponible en: https://www.aeped.es/sites/default/files/documentos/30_enfermedades_autoinflamatorias.pdf

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Published

2021-03-12 — Updated on 2021-03-12

How to Cite

1.
de la Rosa Santana JD, Calas Torres JJ. Autoinflammatory diseases. A challenge for the pediatrician. Rev. cient. estud. HolCien [Internet]. 2021 Mar. 12 [cited 2025 Apr. 4];2(1):e69. Available from: https://revholcien.sld.cu/index.php/holcien/article/view/69

Issue

Vol. 2 No. 1 (2021)

Section

EDITORIAL

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Copyright (c) 2021 Revista HolCien

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Centro Provincial de Información de Ciencias Médicas. Universidad de Ciencias Médicas de Holguín. Cuba.

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