Reye Syndrome Workup

  • Author: Debra L Weiner, MD, PhD; Chief Editor: Richard G Bachur, MD   more...
 
Updated: Feb 1, 2011
 

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.
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Imaging Studies

  • Head CT scanning may reveal cerebral edema, but the results are usually normal.
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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.
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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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Contributor Information and Disclosures
Author

Debra L Weiner, MD, PhD  Attending Physician, Division of Emergency Medicine, Children's Hospital, Boston; Assistant Professor, Department of Pediatrics, Harvard Medical School

Disclosure: Nothing to disclose.

Specialty Editor Board

Garry Wilkes, MBBS, FACEM  Director of Emergency Medicine, Bunbury Hospital; Medical Consultant, St John Ambulance, WA Ambulance Service; Adjunct Associate Professor, Edith Cowan University; Clinical Associate Professor, Rural Clinical School, University of Western Australia

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Grace M Young, MD  Associate Professor, Department of Pediatrics, University of Maryland Medical Center

Grace M Young, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Emergency Physicians

Disclosure: Nothing to disclose.

John D Halamka, MD, MS  Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center

John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

Richard G Bachur, MD  Associate Professor of Pediatrics, Harvard Medical School; Associate Chief and Fellowship Director, Attending Physician, Division of Emergency Medicine, Children's Hospital of Boston

Richard G Bachur, MD is a member of the following medical societies: American Academy of Pediatrics, Society for Academic Emergency Medicine, and Society for Pediatric Research

Disclosure: Nothing to disclose.

References

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