Table 2.
Randomized controlled trials on the effect of long-chain polyunsaturated fatty acid supplementation in inborn errors of metabolism. *: % DHA/total fatty acid composition; a: mean (SD); b: mean (range); ↑: significant increase (p < 0.05) in the supplemented group compared with the control group at the end of the intervention; ↓: significant decrease (p < 0.05) in the supplemented group compared with the control group at the end of the intervention; —: no significant difference between the treatment and the control group at the end of the intervention.
| Study | Number of participants; age | Short description of intervention | Biomarker | DHA * (treatment vs. control group) | Clinical outcomes |
|---|---|---|---|---|---|
| [29] | 42 infants with PKU (21 in treatment and 21 in control group); 8–39 days | Supplemented formula (0.7 g AA and 0.3 g DHA/100 g fatty acids) for 1 yr | EPL | 3.60 (1.06) vs. 1.40 (0.44) a↑ | — P1 and P100 latency; — mental and physical development (Bayley Test); no adverse reactions |
| [30] | 21 infants with PKU (10 in treatment and 11 in control group); <4 wk | Supplemented formula (0.46 g AA and 0.27 g DHA/100 g fatty acids) for 1 yr | PPL | 3.08 (0.10) vs. 1.52 (0.19) a↑ | no adverse reactions |
| [31] | 21 children with PKU (10 in treatment and 11 in control group); 5–10 yr | 2.5–4 g fish oil (18 g EPA, 4 g DPA and 12 g DHA/100 g fatty acid) daily for 6 mo | P | 2.94 (0.88) vs. 0.73 (0.08) a↑ | ↓ plasma triacylglycerol; no adverse reactions |
| [16,32,33] | 20 children with HPA (10 in treatment and 10 in control group); 10 ± 7 yr | 1 capsule (37 mg AA, 27.5 mg EPA, 20 mg DPA and 40 mg DHA/0.5 g capsule) per 4 kg body weight for 1 yr | P | 2.3 (1.1) vs. 1.1 (0.3) a↑ | ↓ P100 wave latency; — plasma triacylglycerol; no adverse reactions |
| PPL | 3.1 (1.6) vs. 1.6 (0.4) a↑ | ||||
| PTG | 0.6 (0.5) vs. 0.3 (0.3) a— | ||||
| PCE | 0.5 (0.2) vs. 0.2 (0.1) a↑ | ||||
| E | 2.8 (1.5) vs. 1.5 (0.5) a— | ||||
| EPC | 0.9 (0.3) vs. 0.4 (0.2) a↑ | ||||
| EPEA | 3.7 (1.7) vs. 1.3 (0.9) a↑ | ||||
| [34] | 44 children with PKU (24 in treatment and 20 in control group); 1–10 yr | EFA supplemented protein substitute (17.2 g LA and 4.5 g ALA/100 g fatty acid) for 20 wk | EPL | 2.07 (0.8) vs. 1.64 (0.4) a↑ | no adverse reactions |
| [35] | 4 children with MMA; 9–16 yr; crossover design | 25 mg/kg DHA daily for 3 mo | P | 5.14 (2.72–7.94) vs. 1.89 (1.12–2.31) b↑ | ↓ plasma triacylglycerol; no adverse reactions |