A 2 year old with Reye’s syndrome

A 2 year old with Reye’s syndrome

Dr Ira Shah.
Medical Sciences Department, Pediatric Oncall, Mumbai.

Medical Sciences Department, Pediatric Oncall, Mumbai
Clinical Problem
Case :- A 2 year old girl born of non-consanguineous marriage presented with fever and cough since 20 days, vomiting, breathlessness and 4 episodes of generalized tonic clonic convulsion since 1 day followed by altered sensorium. She had no history of jaundice or contact with tuberculosis. On examination, she had a heart rate of 90/min with poor peripheral pulses, hypotension (84/52 mm of Hg), no spontaneous respiration, severe pallor and hepatosplenomegaly. She had hypotonia with brisk reflexes and was unconscious. She was intubated and started on positive pressure ventilation, ionotropic support and antibiotics. She was suspected to have Reye’s syndrome. Her investigations showed:
 Hemoglobin = 8.9 gm/dl
 WBC count = 27,000/cumm [68% polymorphs, 28% lymphocytes]
 Platelet count = 4,50,000/cumm
 S. electrolytes, Renal function tests = Normal
 Liver enzymes initially were normal and increased to SGOT of 2635 IU/L & SGPT of 6040 IU/L within 3 days of illness.
 Total proteins = 4.5 gm/dl
 Albumin = 2.7 gm/dl
 Venous blood gas = Metabolic acidosis [pH = 7.34, bicarbonate = 11 mmol/L]
 Random blood sugar = 84 mg/dl
 S.Bilirubin = 2.1 mg/dl
 HIV, HBsAg = Negative
 Leptospira tridot, Dengue, Anti Hepatitis A Antibody = Negative
 S. ammonia = 210 mg/dl
 Prothrombin Time & Partial Thromboplastin Time = deranged
 Malarial test = Negative
 USG Abdomen = Fatty liver
 Blood Bactec culture = No growth.
 CSF= normal

How should this child be managed further ?
Expert's opinion: Dr Ira Shah

This child had an episode of acute encephalopathy with anicteric hepatitis with hyperammonemia suggestive of Reye’s syndrome. Her predominant viral markers are negative. However one needs to rule out Hepatitis E in the child. If that too is negative, one must rule out an underlying metabolic disorder.
Fatty acid oxidation defects, lactic acidosis and urea cycle defects are known to come with Reye’s syndrome. This child should thus be investigated on those lines especially since the child had hyperammonemiia, fatty liver and metabolic acidosis. She needs to do the following baseline tests:
- MRI of the brain
- Urinary and plasma aminoacidogram (UAA and PAA)
- Urinary organic acids
- Urine dichloroacetic acid
- Serum lactate/pyruvate/carnitine

Depending on the results of these tests, specific enzyme analysis can be done.
This child’s lactate was 26.2 and pyruvate was 0.48 with a lactate: pyruvate ratio of 54 suggestive of lactic acidosis.
Urine organic acids, UAA, PAA were normal. S. carnitine was also normal and MRI brain was normal. Child was treated with Riboflavin, Carnitine and Biotin.
Compliance with ethical standards
Funding:  None  
Conflict of Interest:  None
Cite this article as:
Shah I. A 2 years old with Reye`s syndrome. Pediatr Oncall J. 2007;4: 54.
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