Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test
Beta-hydroxybutyrate (BHB) is 1 of 3 sources of ketone bodies. Its relative proportion in the blood (78%) is greater than the other 2 ketone bodies, acetoacetate (20%) and acetone (2%). During carbohydrate deprivation (starvation, digestive disturbances, frequent vomiting), decreased carbohydrate utilization (diabetes mellitus), glycogen storage diseases, and alkalosis, acetoacetate production increases. The increase may exceed the metabolic capacity of the peripheral tissues. As acetoacetate accumulates in the blood, a small amount is converted to acetone by spontaneous decarboxylation. The remaining and greater portion of acetoacetate is converted to BHB.
Monitoring therapy for diabetic ketoacidosis
Investigating the differential diagnosis of any patient presenting to the emergency room with hypoglycemia, acidosis, suspected alcohol ingestion, or an unexplained increase in the anion gap
In pediatric patients, the presence or absence of ketonemia/uria is an essential component in the differential diagnosis of inborn errors of metabolism
Serum beta-hydroxybutyrate is a key parameter monitored during controlled 24-hour fasts
The beta-hydroxybutyrate (BHB)/acetoacetate ratio is typically between 3:1 and 7:1 in severe ketotic states.
Serum BHB increases in response to fasting, but should not exceed 0.4 mmol/L following an overnight fast (up to 12 hours).
In pediatric patients, a hypo- or hyper-ketotic state (with or without hypoglycemia) may suggest specific groups of metabolic disorders.
Cautions Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
Twenty four-hour fasting tests should not be performed in patients <2 years of age.
Dipstick serum ketone determination using nitroprusside reagent is often used to estimate ketone body status, but that method has inherent problems. The dipstick does not measure beta-hydroxybutyrate, the most abundant of the physiological ketone bodies; the nitroprusside reagent only reacts with acetoacetate and acetone.
Reference Values Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.
Clinical References Provides recommendations for further in-depth reading of a clinical nature
1. Tietz Textbook of Clinical Chemistry. Edited by CA Burtis, ER Ashwood. Philadelphia, WB Saunders Co. 1999
2. Vassault A, Bonnefont JP, Specola N, et al: Lactate, pyruvate, and ketone bodies. In Techniques in Diagnostic Human Biochemical Genetics - A Laboratory Manual. Edited by F Hommes. New York, Wiley-Liss, 1991
3. Bonnefont JP, Specola NB, Vassault A, et al: The fasting test in paediatrics: application to the diagnosis of pathological hypo- and hyperketotic states. Eur J Pediatr 1990;150:80-85