Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspective.

BACKGROUND: In phenylketonuria (PKU), modified casein glycomacropeptide supplements (CGMP-AA) are used as an alternative to the traditional phenylalanine (Phe)-free L-amino acid supplements (L-AA). However, studies focusing on the long-term nutritional status of CGMP-AA are lacking. This retrospective study evaluated the long-term impact of CGMP-AA over a mean of 29 months in 11 patients with a mean age at CGMP-AA onset of 28 years (range 15-43) [8 females; 2 hyperphenylalaninaemia (HPA), 3 mild PKU, 3 classical PKU and 3 late-diagnosed]. Outcome measures included metabolic control, anthropometry, body composition and biochemical parameters. RESULTS: CGMP-AA, providing 66% of protein equivalent intake from protein substitute, was associated with no significant change in blood Phe with CGMP-AA compared with baseline (562 ± 289 µmol/L vs 628 ± 317 µmol/L; p = 0.065). In contrast, blood tyrosine significantly increased on CGMP-AA (52.0 ± 19.2 µmol/L vs 61.4 ± 23.8 µmol/L; p = 0.027). CONCLUSIONS: Biochemical nutritional markers remained unchanged which is an encouraging finding in adults with PKU, many of whom are unable to maintain full adherence with nutritionally fortified protein substitutes. Longitudinal, prospective studies with larger sample sizes are necessary to fully understand the metabolic impact of using CGMP-AA in PKU.

Anthropometric changes in the Brazilian cohort of older adults: SABE survey (health, well-being, and aging).

The aim of the present study was to analyze the anthropometric changes in a home-based cohort of Brazilian older adults who participated in the SABE Survey, conducted in 2000 and 2006. A total of 1030 men and women were examined by age group: 60-69, 70-79, and ≥80 years. This representative sample consists of the survivors of the 2000 cohort. The following anthropometric variables were assessed: body mass, arm muscle, waist and calf circumferences, triceps skinfold thickness, body mass index, waist-hip ratio, and arm muscle area according to mean values and percentile distribution. Except for body mass and body mass index, a significant difference (P < 0.05) was observed among the assessed anthropometric variables during the follow-up period. The older adults ≥80 years presented the lowest values. The reduction in the mean values of triceps skinfold thickness was greater (30%) than that of waist circumference (9%) and was more pronounced in women (21%) than in men (9%). Arm muscle circumference and area reduced by 8% and 19%, respectively, in men and 1% and 3%, correspondingly, in women. Our findings revealed reductions in the mean values for all anthropometric variables in the follow-up period from 2000 to 2006 among older adults.