Abstract

LEMA, Fabián; POLO, María L.; ABADIE, Yamila  and  SCRIGNI, Adriana. Síndrome de tórax agudo: episodio vasoclusivo contra neumonía en un paciente con enfermedad drepanocítica. Arch. argent. pediatr. [online]. 2006, vol.104, n.5, pp.435-438. ISSN 0325-0075.

Sickle cell disease is caused by the substitution of glutamic acid for valine in the 6th position of the β-hemoglobin chain. This mutation causes a distortion in red blood cell shape and affects its morphology, structure and function. Vaso-occlusive crises, in different sites, are responsible for most of the severe complications of this disease. The acute chest syndrome, one of the clinical manifestations of this condition, affects about 40% to 50% of patients with sickle cell disease. We report an 11-year old girl with sickle cell disease, who had an acute chest syndrome. We describe her clinical presentation, outcome and treatment.

Keywords : Sickle cell disease; Acute chest syndrome.

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