Amino Acid Medical Foods Provide a High Dietary Acid Load and Increase Urinary Excretion of Renal Net Acid, Calcium, and Magnesium Compared with Glycomacropeptide Medical Foods in Phenylketonuria

Table 1

Characteristics of enrolled participants ().

	Males				Females
Participant number	1	2	3	4	5	6	7	8
Age	34	34	19	17	35	35	28	16
Classical/variant^2	Classical	Variant	Classical	Variant	Variant	Classical	Classical	Variant
Mutation^4	IVS1nt5G>T; IVS12nt1G>A	R408W; IVS12nt1G>A	R408W; Y356X	p.R68S; IVS12+1G>T	E280K; E390G	L242F; R408W	p.F55>Lfs; R408W	p.R157N; p.L348V
Total body
Total fat mass, %	35	34	36	11	42	39	26	30
ALM/ht^2, kg/m^2	9.27	7.41	7.80	7.28	7.49	7.17	5.95	6.54
BMD, g/cm^2	1.131	0.938	1.113	1.078	1.233	1.077	1.035	1.166
Z-score	−1.6	−2.3	−0.7	−1.0	1.0	−0.3	0.0	0.8
Spine L1–L4
BMD, g/cm^2	1.026	0.800	1.145	1.134	1.375	1.059	1.114	1.106
Z-score	−2.4	−3.3	−0.6	−0.6	1.2	−1.2	−0.2	−0.6
Trabecular bone score	1.273	1.233	1.389	1.508	1.420	1.320	1.490	1.425
Femoral neck
BMD, g/cm^2	0.925	X^1	1.020	1.075	X	1.008	0.973^2	1.025
Z-score	−1.5	X	−0.7	−0.3	X	−0.1	−0.1^2	0.2
Femoral trochanter
BMD, g/cm^2	0.731	X	0.745	0.989	X	0.811	0.852^2	0.962
Z-score	−2.4	X	−1.8	0.3	X	−0.3	0.4^2	1.4
Total dual femur
BMD, g/cm^2	0.896	X	0.999	1.135	X	1.080	1.081^2	1.134
Z-score	−1.8	X	−0.9	0.1	X	0.6	0.9^2	0.9
Radius 33%
BMD, g/cm^2	0.892	X	0.982	0.730	X	0.812	0.779	0.838
Z-score	−1.0	X	N/A^3	N/A^3	X	−0.7	−1.1	N/A^3

Symbol represents data that was unable to be obtained at the time of DXA scan completion.
Femoral DXA parameters for one participant are based on the right femur only due to presence of metal in the left hip.
Z-Scores were unable to be calculated for 3 participants because reference population data for the 33% radius for individuals < 20 y were not in the GE-Healthcare Lunar database. AA-MF, amino acid medical food; ALM, appendicular lean mass; BMD, bone mineral density; DXA, dual X-ray absorptiometry; Spine L1–4, Spine Lumbar 1–4.