Urinary dicarboxylic acids in Reye syndrome

Area of Science:

• Biochemistry
• Pediatric Medicine
• Metabolic Disorders

Background:

• Reye syndrome is a severe illness affecting children and teenagers, often triggered by viral infections and aspirin use.
• The underlying metabolic derangements in Reye syndrome are not fully understood, particularly concerning fatty acid metabolism.

Purpose of the Study:

• To investigate the profile of organic acids in the urine of patients with Reye syndrome.
• To assess the relationship between urinary organic acid excretion and clinical parameters like plasma lactate and ammonia levels.

Main Methods:

• Gas-liquid chromatography was used to analyze urine samples from Reye syndrome patients and healthy controls.
• Urinary dicarboxylic acid concentrations were quantified and compared between groups.
• Correlations between urinary metabolites and plasma markers were examined.

Main Results:

• Significantly elevated levels of lactic acid, dicarboxylic acids (adipic, suberic, sebacic), and 3-OH butyric acid were found in Reye syndrome patients' urine.
• Urinary dicarboxylic acid levels were markedly higher in Reye syndrome patients compared to controls, especially before treatment.
• These levels decreased following therapeutic interventions.
• Urinary dicarboxylic acid and ketone excretion correlated strongly with plasma lactate and blood ammonia levels.

Conclusions:

• Reye syndrome is characterized by significant dicarboxylic aciduria.
• Fatty acid metabolism is more profoundly impaired in Reye syndrome than previously recognized.
• Urinary organic acid analysis may serve as a valuable indicator of metabolic disturbance severity in Reye syndrome.