RESUMO
OBJECTIVES: To examine relationships between dietary intake, growth and body composition patterns in patients with inborn errors of intermediary protein metabolism and to determine a safe protein:energy ratio (P:E ratio) associated with optimal growth outcomes. STUDY DESIGN: Retrospective longitudinal data of growth and dietary intake in patients (n = 75) with isovaleric acidemia (IVA; n = 7), methylmalonic acidemia/propionic acidemia (MMA/PA; n = 14), urea cycle defects (UCD; n = 44), classical maple syrup urine disease (MSUD; n = 10) were collected. Prospective longitudinal data of growth, dietary intake, and body composition from 21 patients: IVA (n = 5), MMA/PA (n = 6), UCD (n = 7), and MSUD (n = 3) were collected at clinic visits. RESULTS: Fifty-two of 75 (66%), 49 of 74 (68%), and 44 of 65 (68%) patients had a z-score of 0 (±1) for lifetime weight, height, and body mass index, respectively. Patients with MMA/PA had the lowest median height and weight z-scores, and MSUD patients had highest median body mass index z-score at all ages. In IVA, MMA/PA, and UCD, total natural protein intake met or exceeded the Food and Agriculture Organization of the United Nations (FAO)/World Health Organization (WHO)/United Nations University (UNU) recommended safe levels. Median percentage fat mass was 17.6% in IVA, 20.7% in MMA/PA, 19.4% in UCD, and 17.8% in MSUD. There was a significant negative correlation between percentage fat mass and total protein intake in IVA, MMA/PA, and UCD (r = -0.737; P = .010). The correlation between the P:E ratio and growth variables in IVA, MMA/PA, and UCD suggest a safe P:E ratio (>1.5 to < 2.9) g protein:100 kcal/day. CONCLUSION: Growth outcomes in inborn errors of intermediary protein metabolism are not always ideal. Most patients with IVA, MMA/PA, and UCD consume sufficient natural protein to meet FAO/WHO/UNU recommendations. A P:E ratio range of (>1.5 to < 2.9)g protein/100 kcal/day correlates with optimal growth outcomes.
Assuntos
Erros Inatos do Metabolismo dos Aminoácidos/fisiopatologia , Composição Corporal/fisiologia , Ingestão de Energia/fisiologia , Adolescente , Distribuição da Gordura Corporal , Estatura/fisiologia , Índice de Massa Corporal , Peso Corporal/fisiologia , Criança , Pré-Escolar , Proteínas Alimentares/administração & dosagem , Feminino , Humanos , Estudos Longitudinais , Masculino , Estudos RetrospectivosRESUMO
OBJECTIVE: To evaluate a commercially available, structured short-term weight management program designed for adolescents with obesity delivered by nonhealth professionals. STUDY DESIGN: A multisite parallel-group randomized controlled trial was conducted to evaluate a commercial 12-week lifestyle behavioral program in commercial weight management centers in Australia. Eligible participants (13-17 years, body mass index (BMI) z score ?1.282 with no presenting morbidities) were randomized (n?=?88) to intervention or wait-list, and the program was delivered by consultants at participating weight management centers. The primary outcome was change in BMI z score. Secondary outcomes included the psychometric variables quality of life, body-esteem, and self-esteem. Data was analyzed according to intention-to-treat principles. RESULTS: Of 74 participants who consented to enter the study, 66 provided baseline anthropometric data and 12-week data were available for 55 individuals (74%). A significantly greater decrease in BMI z score in the intervention group (n?=?32) was observed when compared with the wait-list control group, mean difference (MD)?=??0.27?kg/m2; 95% CI, ?0.37,?0.17; P?
Assuntos
Obesidade Infantil/terapia , Programas de Redução de Peso , Adolescente , Austrália , Imagem Corporal , Índice de Massa Corporal , Comércio , Ingestão de Energia , Exercício Físico , Feminino , Humanos , Estilo de Vida , Masculino , Qualidade de Vida , AutoimagemRESUMO
Introduction. Self-reported measures of habitual physical activity rely completely on the respondent's ability to provide accurate information on their own physical activity behaviours. Our aim was to investigate if obese adolescents could accurately report their physical activity levels (PAL) using self-reported diaries. Methods. Total energy expenditure (TEE) was measured using doubly labelled water (DLW) and resting energy expenditure (REE) was measured via indirect calorimetry. Activity energy expenditure (AEE) and PAL values were derived from measured TEE and REE. Self-reported, four-day activity diaries were used to calculate daily MET values and averaged to give an estimated PAL value (ePAL). Results. Twenty-two obese adolescents, mean age 13.2 ± 1.8 years, mean BMI 31.3 ± 4.6 kg/m(2), completed the study. No significant differences between mean measured and estimated PAL values were observed (1.37 ± 0.13 versus 1.40 ± 0.34, P = 0.74). Bland Altman analysis illustrated a significant relationship (r = -0.76, P < 0.05) between the two methods; thus the bias was not consistent across a range of physical activity levels, with the more inactive overreporting their physical activity. Conclusion. At an individual level, obese adolescents are unlikely to be able to provide an accurate estimation of their own activity.
RESUMO
OBJECTIVE: To determine whether a defect in energy metabolism exists in infants with cystic fibrosis (CF). DESIGN: Unselected, newly-diagnosed subjects with CF (n = 46) and 24 healthy infants aged <20 weeks had measurements of resting energy expenditure (REE), total energy expenditure (TEE) (n = 25), and body composition. Metabolizable energy intake (MEI) was calculated. Genotype, energy intake, and pancreatic status was determined in all subjects with CF, and 24 underwent bronchial lavage. RESULTS: At diagnosis, infants with CF detected by newborn screening had significant anthropometric deficits (mean [SD] z-weight = 0.5 [1.0], z-length = 0.7 [1.3]) associated with pancreatic insufficiency. Their REE, TEE, or MEI (absolute measurements, per unit body weight or fat-free mass) were not increased. No relationship between REE, TEE, or MEI and Delta F(508) genotype, and no proportional differences in individual components of MEI between subjects with CF and controls, or between subjects with CF who were homozygotes or compound heterozygotes for Delta F(508) were observed. REE and TEE were not correlated with bronchial infection or inflammation. CONCLUSION: Growth impairment during the first weeks of life in infants with CF is associated with pancreatic insufficiency. However, there is no evidence for a defect of energy metabolism related to Delta F(508), and in infants with CF, minimal lung disease is unaccompanied by increased energy expenditure.