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Pediatrics, Reye Syndrome: Differential Diagnoses & Workup
Updated: Feb 3, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Differential Diagnoses
Other Problems to Be Considered
Differential diagnosis includes conditions that can cause vomiting and altered level of consciousness. The distinction between Reye and Reye-like syndrome is often unclear.
Workup
Laboratory Studies
- Ammonia: Ammonia level as high as 1.5 times normal (up to 1200 mcg/dL) 24-48 hours after the onset of mental status changes is the most frequent laboratory abnormality. Ammonia level may return to normal in stages 4 and 5.
- Transaminases levels: ALT and AST levels increase to 3 times normal but may return to normal by stages 4 or 5.
- Bilirubin: Bilirubin levels are >2 mg/dL (usually <3 mg/dL) in 10-15% of patients. If direct bilirubin level is >15% of total or total is >3 mg/dL, consider other diagnoses.
- Prothrombin time (PT) and activated partial thromboplastin time (aPTT) are prolonged >1.5-fold in more than 50% of patients.
- Lipase and amylase: These levels are elevated.
- Serum bicarbonate: This level is decreased secondary to vomiting.
- BUN and creatinine: These levels are elevated.
- Glucose: Expect hypoglycemia, particularly in children younger than 1 year.
- Lactic dehydrogenase (LDH): This level may be high or low.
- Anion gap and venous blood gas: Determine anion gap and venous blood gas level to evaluate for metabolic acidosis.
- Urine specific gravity and ketones: Specific gravity is increased; 80% of patients have ketonuria.
Imaging Studies
- Head CT scanning may reveal cerebral edema, but the results are usually normal.
Other Tests
- Free fatty acids and amino acids (eg, glutamine, alanine, lysine): These levels may be elevated.
- Factors II, VII, IX, X, fibrinogen: These levels may be low due to the disruption of synthetic activities in the liver. Consumption may also contribute to low levels of coagulation factors. Platelets are usually normal.
- Electroencephalogram (EEG) may reveal slow-wave activity in the early stages and flattened waves in advanced stages.
- CSF opening pressure, CSF WBCs: Lumbar puncture (LP) should only be performed in hemodynamically stable patients. Opening pressure may or may not be increased; WBCs (usually lymphocytes) are 8/mm3 or fewer (8 X 109/L or fewer).
- Workup to exclude IEM must be performed and should include evaluation for defects of fatty-acid oxidation, amino and organic acidurias, urea-cycle defects, and disorders of carbohydrate metabolism. For additional information, see the eMedicine article Pediatrics, Inborn Errors of Metabolism.
Procedures
- Vascular access - Arterial and/or central venous
- Lumbar puncture if patient is hemodynamically stable and no signs of increased ICP
- Intubation to maintain airway and ventilation and to manage intracranial pressure
- Nasogastric tube placement to decompress the abdomen
- Bladder catheterization to monitor urine output
- Percutaneous liver biopsy may be indicated to exclude IEM or toxic liver disease.
- Placement of an intracranial device for intracranial pressure monitoring is indicated for patients with increased intracranial pressure.
- Coagulopathy must be corrected before invasive procedures.
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References
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Further Reading
Keywords
Reye's syndrome, Reye syndrome, acute noninflammatory encephalopathy, inborn error of metabolism, IEM, Reye syndrome in children, hepatic failure, upper respiratory tract infection, URTI, influenza, varicella, gastroenteritis, use of aspirin, aspirin use in children
Differential Diagnoses & Workup: Pediatrics, Reye Syndrome