[Serratia infections, should we think about primary immunodeficiencies?]
Chronic granulomatous disease (CGD) is a primary immunodeficiency with an incidence of 1/200,000-250,000 live births. CGD affects mainly male patients, most of the mutations being X-linked, and autosomal recessive forms occur more frequently in communities with greater numbers of consanguineous marriages. CGD is characterized by sensitivity to recurrent and severe bacterial and fungal infections, with formation of granulomas due to the inability of phagocytes to generate reactive oxygen compounds, necessary for the intracellular death of phagocytic microorganisms. We report three cases of CGD in which Serratia marcescens was isolated, and after detailed anamnesis and performance of neutrophil function tests, a molecular diagnosis of the disease was reached. CGD can be manifested in a wide variety of ways, so that high suspicion and a meticulous anamnesis are essential to reach a diagnosis.
Hospital Infantil Miguel Servet, Zaragoza, España. alicia87mr@hotmail.com.
Alicia
A
Montaner Ramón
Hospital Infantil Miguel Servet, Zaragoza, España.
Laura
L
Murillo Sanjuán
Hospital Infantil Miguel Servet, Zaragoza, España.
Cristina
C
Martínez Faci
Hospital Infantil Miguel Servet, Zaragoza, España.
Carmelo
C
Guerrero Laleona
Hospital Infantil Miguel Servet, Zaragoza, España.
Carmen
C
Rodríguez-Vigil Iturrate
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D006105:Granulomatous Disease, Chronic / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008297:Male; D016868:Serratia Infections / Q000276:immunology*
Serratia marcescens; granuloma; pediatrics; phagocytes; primary immunodeficiencies