Diagnosis of an intermediate case of maple syrup urine disease: A case report

Figure 2 The urinary organic acid chromatograms of the proband. A: The urinary organic acid chromatogram of a normal control; B-J: The urinary organic acid chromatograms of the proband at 8 mo, 1 year and 1 mo, 1 year and 7 mo, 2 years, 2 years and 5 mo, 3 years and 3 mo, 4 years and 10 mo, 5 years and 7 mo, and 6 years and 2 mo of age, respectively. Peak 1, internal standard; Peak 2, external standard; Peak 3, 2-OH-isovaleric acid; Peak 4 and 9, 3-OH-isovaleric acid; Peak 5, 2-OH-isocaproic acid; Peak 6, 2-keto-isocaproic acid; Peak 7 and 8, 2-keto-3-methylvaleric acid.