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β-Hydroxybutyric acid, also known as 3-hydroxybutyric acid or BHB, is an organic compound and a beta hydroxy acid with the chemical formula CH 3 CH(OH)CH 2 CO 2 H; its conjugate base is β-hydroxybutyrate, also known as 3-hydroxybutyrate. β-Hydroxybutyric acid is a chiral compound with two enantiomers: D-β-hydroxybutyric acid and L-β-hydroxybutyric acid.
Both acetoacetate and beta-hydroxybutyrate are acidic, and, if levels of these ketone bodies are too high, the pH of the blood drops, resulting in ketoacidosis. Ketoacidosis is known to occur in untreated type I diabetes (see diabetic ketoacidosis ) and in alcoholics after prolonged binge-drinking without intake of sufficient carbohydrates (see ...
If beta-hydroxybutyrate levels continue to excessively rise or remain persistently elevated, additional workup can be performed to detect ketone transporter defects. [ 2 ] While ketotic hypoglycemia can be definitively diagnosed in the inpatient setting, once diagnosis is established, parents can monitor frequency and duration of episodes at ...
In the liver, therefore, oxaloacetate is unavailable for condensation with acetyl-CoA when significant gluconeogenesis has been stimulated by low (or absent) insulin and high glucagon concentrations in the blood. Under these circumstances, acetyl-CoA is diverted to the formation of acetoacetate and beta-hydroxybutyrate. [2]
Ketosis is a metabolic state characterized by elevated levels of ketone bodies in the blood or urine. Physiological ketosis is a normal response to low glucose availability. In physiological ketosis, ketones in the blood are elevated above baseline levels, but the body's acid–base homeostasis is maintained.
The most researched ketone ester, or ketone monoester, is called D-Beta Hydroxybutyrate/ R 1,3-butanediol monoester, which is a naturally derived compound through a fermentation process. It was created by Dr. Richard Veech and Todd King at the NIH, and then commercialized by companies including KetoneAid and TDeltaS, and previously by HVMN, a ...
An elevated anion gap metabolic acidosis and ketosis is the classic present. [3] However, a mixed acid-base disorder may be present especially if vomiting is contributing to a hypochloremic alkalosis. [2] The ketone which is present is mostly beta-hydroxybutyrate rather than acetoacetate resulting in only a weakly positive nitroprusside test. [2]
Detectable amounts of insulin are abnormal and indicate that hyperinsulinism is likely to be the cause. Other aspects of the person's metabolic state, especially low levels of free fatty acids, beta-hydroxybutyrate and ketones, and either high or low levels of C-peptide and proinsulin can provide confirmation.