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Revista chilena de pediatría

versión impresa ISSN 0370-4106

Resumen

LOPEZ, M; HUETE, I  y  HERNANDEZ, Marta. Acute Cerebrovascular events associated to hemolytic uremic syndrome: Description of two pediatric cases. Rev. chil. pediatr. [online]. 2017, vol.88, n.5, pp.640-646. ISSN 0370-4106.  http://dx.doi.org/10.4067/S0370-41062017000500011.

Diarrhea-associated Hemolytic Uremic Syndrome (D+HUS) is a multisystem disorder in which neurological involvement (35 to 50%) is associated to adverse outcome. An important cause of a permanent neurological impairment is the cerebrovascular pathology. Objective: To report two pediatric cases of D+HUS with severe neurological involvement due to cerebrovascular disease, and review available literature. Clinical cases: Two previously healthy 15- and 21-month-old children debuted with seizures and impairment of consciousness within the first week of a D+HUS. Both presented hypertension, severe acute renal failure, and focal motor deficit. One child showed significant improvement in neurologic status after five sessions of plasmapheresis. Brain magnetic resonance showed in the first child multiple bilateral infarcts of small vessels and lesions of white matter. In the second patient, large bilateral infarcts on both middle cerebral arteries territories were identified. One year after the acute event, both children showed functional impairment; The first patient evolved with language delay and spastic hemiparesis; the second patient with spastic quadriparesis, epilepsy with poor seizure control and marked functional impairment. Conclusion: Although most of the children with D+HUS and brain involvement do not have long-term sequelae, cerebrovascular disease in the acute period causes permanent damage, and in addition to the management of electrolyte disturbances, hypertension, and renal failure, therapies directed at specific pathophysiological mechanisms that trigger vascular compromise may improve prognosis.

Palabras clave : Pediatric stroke; acute renal failure; thrombotic microangiopathy; disability; Hemolytic Uremic Syndrome.

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