Table 3.
All SymX and AsymX patients on the diet are summarized in Table 3
| Groups | Patient#/study ID/diagnosis | Long chain fat intake | MCT | Protein Intake (g/kg/d) |
||
|---|---|---|---|---|---|---|
| Recommended intake | Actual intake | Recommended intake | Actual intake | |||
| SymX group | 6/6/CPTII def | 20% | 30% | NA | NA | 1.09 |
| 7/14/CPTII def | 20% | < 20% | 10–15% | 1% | 1.17 | |
| 8/15/CPTII def | 35% | 44% | 10–20% | 10–20% | 1.33 | |
| 9/37/LCHAD def | 10% | 8% | 10–15% | 0% | NA | |
| 10/42/VLCAD def | 10–15% | 6% | 10–15% | 21% | 2.12 | |
| 11/52/VLCAD def | 5% | 5% | 35% | 35% | 2.94 | |
| 14/48/MAD def | 25–30% | 33% | NA | NA | 2.32 | |
| AsymX group | 24/38/LCHAD def | 10% | 7% | 15–25% | 23% | 2.57 |
| 25/39/LCHAD def | 10–15% | 10% | 20–25% | 22% | 2.13 | |
| 26/53/LCHAD def | 10% | 10% | 30% | 32% | 2.30 | |
| 27/40/VCLAD def | 15–25% | 26% | 5% | 0% | 0.97 | |
| 28/43/VLCAD def | 15–25% | 13% | 13% | 13% | 1.51 | |
| 29/44/VLCAD def | 10–20% | 10–12% | 15–20% | 15% | 2.15 | |
CACT carnitine acylcarnitine translocase; CPT-I carnitine palmitoyltransferase I; CPT-II carnitine palmitoyltransferase II; CTD carnitine transporter defect; def deficiency; LCHAD long-chain 3-hydroxyacyl-CoA dehydrogenase; MAD multiple acyl-CoA dehydrogenase; VLCAD very long-chain acyl-CoA dehydrogenase