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Semiquantitation of leucine, isoleucine, and valine by thin-layer chromatography in management of maple-syrup urine diseases.

A few microliters of serum suffice to estimate the leucine, isoleucine, and valine present. The three branched-chain amino acids are separated by unidirectional thin-layer chromatography, and estimated by visual comparison with co-chromatographed individual standards in graduated concentrations. The method is especially applicable to the frequent monitoring of serum branchedchain amino acids needed in treating patients with maple-syrup urine disease, and may also be used for rapid diagnosis of the disease in suspected infants.