Studies on the pathophysiology of encephalopathy in Reye's syndrome; Hyperammonemia in Reye's syndrome.
The initial acid-base status of eight survivors of Reye's syndrome was characterized by acute respiratory alkalosis (Pco2=32 mm Hg; Hco3-=22.0 mEq/liter) while that of eight children who died was associated with metabolic acidosis as well (HCO3-=10.0 mEg/liter). Arterial-internal jugular venous ammonia concentration differences on day 1 (299 mg/100 ml) and day 2 (90 mg/100 ml) reflected cerebral uptake of ammonia while those on days 3 and 4 (-43 and -55 mg/100 ml) demonstrated cerebral release. Arterial blood hyperammonemia can be detoxified safely in the brain as long as the levels do not exceed approximately 300mug/100 ml. Beyond that level lactic acidosis is observed, particularly in cerebral venous drainage. Arterial blood hyperammonemia was also related to the extent of alveolar hyperventilation. These findings are very similar to those seen in experimental hyperammonemia and support the concept that neurotoxicity in children with Reye's syndrome is at least partly due to impaired oxidative metabolism secondary to hyperammonemia.
D000137:Acid-Base Imbalance / Q000097:blood; D000641:Ammonia / Q000097:blood* / Q000134:cerebrospinal fluid / Q000378:metabolism; D001921:Brain / Q000378:metabolism / Q000503:physiopathology*; D001927:Brain Diseases / Q000503:physiopathology*; D002648:Child; D006801:Humans; D006985:Hyperventilation / Q000097:blood; D007773:Lactates / Q000378:metabolism; D012202:Reye Syndrome / Q000097:blood / Q000134:cerebrospinal fluid / Q000503:physiopathology*
D007773:Lactates; D000641:Ammonia