SciELO - Scientific Electronic Library Online

vol.76 número2Hendidura esternal total en un recién nacido y parcial en una niña de 4 años: 2 clinical casesEmpleo del DDAVP en el manejo de la enfermedad de Von Willebrand índice de autoresíndice de materiabúsqueda de artículos
Home Pagelista alfabética de revistas  

Revista chilena de pediatría

versión impresa ISSN 0370-4106


GONZALEZ M., Benito; ROA A., Johanna  y  SCHMIDT S., Nadia. Activated macrophage syndrome in paediatrics: 4 clinical cases. Rev. chil. pediatr. [online]. 2005, vol.76, n.2, pp.183-192. ISSN 0370-4106.

Introduction: The macrophage activation syndrome (MAS) is a rare paediatric condition characterized by enhanced activation of the macrophage and T-cell system, with increased liberation of T-cell cytokines. Clinically it resembles a multiorgan failure syndrome of infectious aetiology. There is still some debate about the syndromes nomenclature, hemato-oncologists usually classify this syndrome as a histiocytic disorder, while rheumatologists define it as MAS. The objective of this publication is to describe 4 patients in which the diagnosis of MAS was made in the early stages of the disease. Two patients had juvenile idiopathic artritis (systemic presentation), 1 with probable immunodeficiency, and in the last case, a familial histiocytosis was suspected. Trigger factors were drugs in 2 patients and 2 infections in the remaining cases. Conclusions: The relevance of this report is to show that the early diagnosis and aggressive treatment with steroids and cyclosporine can improve the prognosis of this rare syndrome

Palabras clave : MAS; juvenile idiopathic artritis; systemic juvenile idiopathic artritis; immunodeficienc; histiocytosis.

        · resumen en Español     · texto en Español


Creative Commons License Todo el contenido de esta revista, excepto dónde está identificado, está bajo una Licencia Creative Commons