RESUMEN
OBJECTIVE: To determine whether increased total daily energy expenditure (TDEE) associated with repetitive, involuntary movements contributes to growth failure in girls with Rett syndrome (RS). STUDY DESIGN: Fourteen girls with RS and 11 healthy girls were studied for 10 days to obtain measurements of height, weight, body circumference, and skin-fold thickness with stadiometric and anthropometric methods; whole-body potassium by potassium 40 counting; 72-hour dietary energy intakes by test weighing; 24-hour activity patterns using observational methods; and TDEE using the doubly-labeled water technique. RESULTS: TDEE, when adjusted for differences in lean body mass, did not differ significantly between girls with RS and healthy girls. Although girls with RS spent more waking hours in physical activity than their healthy counterparts (85%+/-10% vs. 73%+/-11% awake time per day, p < 0.05), their repetitive movements were not sufficiently intense to increase TDEE. However, girls with RS had significantly less lean body mass, but not body fat, which contributed to their lower absolute TDEE in comparison with that of healthy girls (845+/-251 vs. 1453+/-534 kcal/day, p < 0.01). Dietary energy intake, when adjusted for differences in body weight, was not significantly different in girls with RS compared with healthy girls. CONCLUSIONS: Increased TDEE as a result of repetitive, involuntary movements does not explain the alterations in growth and body composition of girls with RS.
Asunto(s)
Metabolismo Energético , Trastornos del Crecimiento/fisiopatología , Síndrome de Rett/fisiopatología , Adolescente , Índice de Masa Corporal , Niño , Preescolar , Femenino , Trastornos del Crecimiento/etiología , Trastornos del Crecimiento/metabolismo , Humanos , Trastornos del Movimiento/complicaciones , Síndrome de Rett/complicaciones , Síndrome de Rett/metabolismoRESUMEN
The adaptive response of whole body mineral metabolism and collagen turnover to nutritional supplementation was determined in adolescent males with Crohn disease and growth failure. Body calcium, magnesium, phosphorus, and nitrogen status was characterized in six patients before and after 3 weeks of nutritional supplementation and in five healthy age- and sex-matched controls by the metabolic balance technique; collagen turnover was assessed by urinary hydroxyproline excretion. Fecal calcium (P less than 0.05), magnesium (P less than 0.01), and nitrogen (P less than 0.01) losses were significantly greater in the Crohn disease patients compared with the controls; after dietary supplementation, only fecal magnesium excretion increased further (P less than 0.001). Approximately twofold increases in phosphorus (P less than 0.001), nitrogen (P less than 0.02), and calcium (P less than 0.05) retention occurred with nutritional supplementation, whereas the marked renal conservation of phosphorus (P less than 0.001) suggested that this mineral was a limiting nutrient. Urinary hydroxyproline excretion was reduced (P less than 0.05) compared with the control values; however, with nutritional supplementation, urinary hydroxyproline excretion increased significantly (P less than 0.02), suggesting that the soluble fraction of tissue collagen was rapidly turning over during nutritional rehabilitation. These findings suggest that the mineral deficiencies present in adolescents with Crohn disease and growth failure can be reversed with nutritional supplementation. Furthermore, the improvement in nutritional status is reflected in the restoration of collagen-containing tissues of the body. However, nutrient imbalances may be present during nutritional therapy, thereby preventing optimal recovery from malnutrition.
Asunto(s)
Enfermedad de Crohn/metabolismo , Alimentos Fortificados , Crecimiento , Minerales/metabolismo , Adolescente , Calcio de la Dieta/administración & dosificación , Calcio de la Dieta/metabolismo , Enfermedad de Crohn/dietoterapia , Enfermedad de Crohn/fisiopatología , Metabolismo Energético , Humanos , Hidroxiprolina/orina , Magnesio/administración & dosificación , Magnesio/metabolismo , Masculino , Minerales/administración & dosificación , Nitrógeno/administración & dosificación , Nitrógeno/metabolismo , Fósforo/metabolismoRESUMEN
The effect of chronic inflammation, corticosteroid therapy, and nutritional supplementation on whole body protein metabolism and growth was determined in adolescent males with Crohn disease and growth failure. Body protein status was characterized in six patients and in five healthy, age-matched controls by nitrogen balance, whole body potassium (measured as 40K), urinary creatinine excretion, and [15N]glycine turnover studies. The patients subsequently received an intragastric nutritional supplement that increased dietary protein and energy intakes by 40% for seven months. Follow-up anthropometric measurements were obtained in both groups of adolescents. Our results demonstrated that lean body mass (40K) and muscle mass (creatinine) were significantly reduced in the patients compared to the controls, despite similar nitrogen balances between both groups. Whole body nitrogen flux, rates of protein synthesis and breakdown, and net protein retention were no different between those patients with chronic inflammation, while receiving corticosteroid therapy, and their controls. Long-term nutritional supplementation significantly improved linear and ponderal growth velocities in the patients such that growth rates were commensurate with those of the healthy controls. We conclude that chronic inflammation and corticosteroid therapy are not associated with detectable alterations in protein metabolic processes at the whole body level. Rather, dietary insufficiency appears to be primarily responsible for growth failure in adolescents with Crohn disease.
Asunto(s)
Enfermedad de Crohn/metabolismo , Trastornos del Crecimiento/metabolismo , Proteínas/metabolismo , Adolescente , Corticoesteroides/uso terapéutico , Niño , Enfermedad de Crohn/complicaciones , Enfermedad de Crohn/dietoterapia , Enfermedad de Crohn/tratamiento farmacológico , Proteínas en la Dieta/administración & dosificación , Ingestión de Energía , Nutrición Enteral , Trastornos del Crecimiento/etiología , Humanos , Masculino , Nitrógeno/metabolismoRESUMEN
Temperatures for rewarming neonates have been established for convection incubators. The purpose of this study was to evaluate the effects on neonates when the skin surface was rewarmed at one of four different levels with an infra-red warmer. A total of 42 normal term neonates were first divided into four groups according to deep rectal temperature on admission; infants in each of the respective groups were then allotted to one of the following skin surface temperature set-point groups: 35, 36, 37 or 38 degrees c for rewarming under a servo-controlled infra-red heat source. Although rewarming at 38 degrees (100.4 degrees F) surfacetemperature bears the theoretic risk of the effects of hyperthermic stress to the neonate, particularly of apnea, the 12 infants rewarmed at this temperature set-point all achieved normal rectal temperatures significantly sooner than the infants in the other three groups without evidence of ill effects. The attainment of a rectal temperature of 37 degrees C within the 4 hr study period and the absence of hypoglycemia in the 38 degrees C skin temperature group was statistically significant when compared to the frequency of occurrence in the other groups.