Blood Spot Branched Chain Amino Acids


Measurement of branched chain amino acids leucine, isoleucine, and valine is indicated in maple syrup urine disease. A disorder due to deficiency of the branched-chain a-keto (2-oxo) acid dehydrogenase complex.

Also known as


Request a test

To request this test please send sample with a request providing patient ID (three identifiers), specimen information, assay required, relevant clinical details and sender information. Before sending sample please read details on requesting and labelling by clicking on the link. Please also refer to any additional information provided for this test.

Additional information

Spot must completely fill circle and fully soak through the card with no multi-spotting.

Sending address

Chemical Pathology Reception
Level 1, Camelia Botnar Building
Great Ormond Street Hospital
Great Ormond Street

Laboratory service

Chemical Pathology

Sample requirements

A minimum of two adequate blood spots on a Guthrie Card.

Reference range

Please click on the link to view reference range

Turnaround time

2 days

Disease / group

Maple syrup urine disease


Upon request

Call in advance?

Not required

Chemical Pathology

The Chemical Pathology Laboratory provides an extensive range of services covering both routine biochemistry and specialised analysis and interpretation.

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