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

Table 1 Height and weight of the proband

Age	Height		Weight
	cm	SD	kg	SD
8 mo	70.0	-0.5	8.8	-0.5
1 yr 1 mo	77.0	0.0	9.4	-0.8
1 yr 7 mo	82.5	0.2	11.6	0.1
1 yr 9 mo	84.0	-0.5	11.7	-0.4
2 yr 5 mo	92.0	-0.7	12.4	-0.4
3 yr 5 mo	100.0	0.0	13.5	-1.0
5 yr	108.5	-0.7	16.5	-1.2
5 yr 8 mo	113.7	-0.5	17.6	-1.3
6 yr 2 mo	117.4	-0.3	17.5	-1.7

Table 2 Concentrations of branched-chain amino acids of the proband

Amino acid	Leucine (μmol/L)	Isoleucine (μmol/L)	Valine (μmol/L)	Alanine (μmol/L)	Leucine/alanine ratio
Reference range	4.0-197.0	24.0-118.0	99.0-333.0	210.0-545.0
8 mo	468.6↑	258.8↑	574.2↑	229.0	2.12
1 yr 1 mo	184.1	92.8	292.7	208.5↓	0.88
1 yr 7 mo	327.0↑	165.8↑	460.7↑	252.3	1.29
2 yr	258.3↑	199.4↑	406.8↑	150.1↓	1.72
2 yr 5 mo	201.2↑	92.7	274.0	211.2	0.95
3 yr 3 mo	288.1↑	157.4↑	311.9↑	186.0↓	1.55
4 yr 10 mo	222.0↑	124.5↑	270.2	266.4	0.83
5 yr 7 mo	258.5↑	156.1↑	295.3	171.2↓	1.51
6 yr 2 mo	244.0↑	130.1↑	431.3↑	280.0	0.87