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GRADE 1 REYE'S SYNDROME (RS)-OUTCOME AND PREDICTORS OF PROGRESSION TO DEEPER COMA GRADES

Recent studies (N Engl J Med 309:133, 1983) suggest that non-comatose RS (so-called Grade 1) may comprise as many as 75% of all cases. We sought to determine the outcome of all cases of hospitalized Grade 1 RS and determine if any clinical or laboratory characteristics might predict progression to deeper coma grades. Since 1969, 84 patients had liver biopsy proven Grade 1 RS. Group A included 79 patients with no coma grade change and in Group B, 5 (5.9%) progressed to deeper coma grades (1 to Grade 2, 3 to Grade 3 and 1 to Grade 4). All patients survived without sequelae except one Group B patient with severe brain damage. There were no differences between groups in age at presentation, sex, admitting SGOT, glucose, CPK, BUN, salicylate level, uric acid, or hours of emesis prior to admission; however, admitting NH3 was significantly higher (p = .005) in Group B (291 ± 42 μg/dl, [xmacr ] ± SEM) vs. Group A (52 ± 5 μg/dl) and the prothrombin time was significantly more prolonged (p = .003) in Group B (3.86 ± .49 seconds) vs. Group A (1.63 ± .16 seconds). Liver ultrastructural changes were severe in all Group B subjects, and varied from mild to severe in Group A. Conclusions: 1) The prognosis is excellent for survival without sequelae in Grade 1 Reye's Syndrome (98.8%) when management includes hospital surveillance and intravenous glucose-electrolyte infusion. 2) A small number (6%) progress to deeper coma grades and the admitting NH3 and prothrombin time may predict progression.