Reye's Syndrome

náhled|upright=1.8|Video v angličtině, definice, patogeneze, příznaky, komplikace, léčba. Reye syndrome is a rare encephalopathy (1:100 000 ) in small children. He gets on the virus. It causes damage to mitochondria, leading to steatosis liver, kidney, heart, and brain edema. The disease is triggered by salicylate treatment (+ genetic predisposition). The syndrome was discovered due to the massive administration of Aspirin to children "for everything" in the 1950s. It was described in 1963 by the Australian pathologist Douglas Rey. After the ban on the administration of salicylates to children in the 1980s, its incidence decreased significantly.

Etiology and pathogenesis
Etiology unknown. Demonstrated acyl-CoA dehydrogenase defect leading to carnitine deficiency (which we test for children with Reye's syndrome).

Pathological-anatomical image
Fat infiltration liver, heart and kidney, neurons and glia are edematous.

Clinical signs
Common prodromal symptoms of virosis are followed by a long latent period, which ends with a sharp onset of vomiting, delirium, and seizures escalating to coma here. Bearing symptoms are missing. It increases intracranial hypertension and can lead to death due to cerebral herniation.

Auxiliary examinations

 * Elevated values liver enzymes,
 * higher level fatty acids,
 * hypoglycaemia,
 * extended prothrombin time,
 * hyperammonemia.

Differential diagnosis
Encephalopathy in lead poisoning, lateral sinus thrombosis.

Treatment and prognosis
Normalization of intracranial pressure. Efforts to maintain glycemia and treat any coagulopathy.

Early diagnosis and supportive care reduced mortality from 80% to 30%. With intracranial hypertension, mortality is 50%. Survival is usually associated with residual cognitive impairment.

Související články

 * Virové infekce nervového systému

Externí odkazy

 * Reyeův syndrom – Podcast