Table 2.

Urine organic acid panel. We see a massive excretion of 3-OHbutyric and acetoacetic acids, with elevated branched-chain keto acids and severe lactic/pyruvic aciduria suggesting a severe catabolic state. The elevated excretion of adipic acid is most likely dietary in origin. Increased excretion of 4-OH-phenyllactic/phenylpyruvic acids suggests impaired hepatocellular function (OH) = hydroxy.

Urine Organic Acid Panel
Organic acid	Level (mmol/mol creatinine)	Normal range
3-OH-Butyric Acid	12,288	0–4
Lactic Acid	5547	0–50
Acetoacetic Acid	4316	0–4
Adipic Acid	274	0–35
4-OH-Phenyllactic Acid	59	0–4
2-Keto-3-Methylvaleric Acid	32	0–10
2-Keto-Isovaleric Acid	29	0–4
Fumaric Acid	27	0–4
2-Keto-Isocaproic Acid	26	0–4
4-OH-Phenylpyruvic Acid	5	0–2
Ethylmalonic Acid	5	0–4
Suberic Acid	5	0–3