artículo
Acute Cerebrovascular events associated to hemolytic uremic syndrome: Description of two pediatric cases
Fecha
2017Registro en:
10.4067/S0370-41062017000500011
0370-4106
MEDLINE:29546950
SCOPUS_ID:85035798559
WOS:000416507900011
Autor
Lopez, M.
Huete, Isidro
Hernández Chávez, Marta Isabel
Institución
Resumen
Introduction: 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.