Phenylketonuria natural treatment with supplements by Ray Sahelian, M.D.
Phenylketonuria PKU requires a lifelong low-phenylalanine diet that provides the majority of protein from a phenylalanine-free amino acid formula.
Fish oil benefit
Effect of fish oil supplementation on fatty acid
status, coordination, and fine motor skills in children with phenylketonuria.
J Pediatr. 2007 May;150(5):479-84. Division of Metabolic Diseases and Nutrition,
Dr. von Haunersches Kinderspital, Ludwig-Maximilians-University, Munich,
Germany.
Thirty-six patients with Phenylketonuria (1-11 years of age, good metabolic
control: plasma phenylalanine < or = 360 micromol/L for > or = 6 months). We
determined plasma phospholipid fatty acids, and in patients > 4 years of age (N
= 24) the motometric Rostock-Oseretzky Scale (ROS), before and after
supplementation with fish oil for 3 months (15 mg docosahexaenoic acid [DHA]/kg
body weight daily). Supplementation increased phospholipid n-3 LC-PUFA,
decreased n-6 LC-PUFA. Patients tolerated fish oil well. Plasma phenylalanine
remained unchanged. In patients with Phenylketonuria, fish oil
supplementation enhances n-3 LC-PUFA levels and improves motor skills.
Benefit of Glycomacropeptide
Improved nutritional management of phenylketonuria by using a diet containing
glycomacropeptide compared with amino acids
American Journal of Clinical Nutrition, doi:10.3945/ajcn.2008.27280
Glycomacropeptide (GMP), an intact protein formed during cheese production,
contains minimal phenylalanine. The objective was to investigate the effects of
substituting GMP food products for the AA formula on acceptability, safety,
plasma AA concentrations, and measures of protein utilization in subjects with
PKU.
Eleven subjects participated in an inpatient metabolic study with two 4-d
treatments: a current AA diet (AA diet) followed by a diet that replaced the AA
formula with GMP (GMP diet) supplemented with limiting AAs. Plasma
concentrations of AAs, blood chemistries, and insulin were measured and compared
in AA (day 4) and GMP diets (day 8). The GMP diet was preferred to the AA diet
in 10 of 11 subjects with PKU, and there were no adverse reactions to GMP. There
was no significant difference in phenylalanine concentration in postprandial
plasma with the GMP diet compared with the AA diet. When comparing fasting with
postprandial plasma, plasma phenalyalanine concentration increased significantly
with the AA but not with the GMP diet. Blood urea nitrogen was significantly
lower, which suggests decreased ureagenesis, and plasma insulin was higher with
the GMP diet than with the AA diet. GMP, when supplemented with limiting AAs, is
a safe and highly acceptable alternative to synthetic AAs as the primary protein
source in the nutritional management of PKU. As an intact protein source, GMP
improves protein retention and phenylalanine utilization compared with AAs.