Compromiso cerebrovascular agudo en sindrome hemolítico urémico: descripción de dos casos pediátricos

Introduction: Diarrhea-associated Hemolytic Uremic Syndrome (D+HUS) is a multisystem disorder  in  which  neurological  involvement  (35  to  50%)  is  associated  with  adverse functional  and  survival  outcomes.  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 with a marked 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.