RESUMEN
OBJECTIVE: To study the relationship of fat mass, extracellular-to-intracellular-water ratio, and bone mineral density with growth hormone function and physical activity in Prader-Willi syndrome. STUDY DESIGN: There were 17 patients with PWS (10 women, ages 7.5-19.8 years) and 17 obese control patients, matched for gender and bone age. FM and extracellular-to-intracellular-water ratio were measured by bromide-deuterium dilution, BMD by dual-energy x-ray absorptiometry, GH function by fasted serum insulin-like growth factor-I concentration, and physical activity by doubly-labeled water in combination with basal metabolic rate by a ventilated hood. RESULTS: The PWS group had a similar fat mass, but a lower fat-free mass, whereas the extracellular-to-intracellular-water ratio was higher compared with the control group (0.87 +/- 0.07 l/l and 0.80 +/- 0.06 l/l, respectively [P <.01]). Fat mass was inversely related with PA in the PWS group, whereas IGF-I was positively correlated with FFM, ICW, and BMD of the limbs. BMD tended to be lower in patients with PWS. CONCLUSIONS: In children and adolescents with PWS, adiposity is associated with a reduced fat-free mass and extracellular-to-intracellular-water ratio is increased. Both findings are related to GH function and physical activity. BMD, especially in the limbs, tends to be reduced in patients with PWS and is related to GH function.
Asunto(s)
Composición Corporal , Obesidad/fisiopatología , Síndrome de Prader-Willi/fisiopatología , Adolescente , Adulto , Agua Corporal/metabolismo , Densidad Ósea , Niño , Óxido de Deuterio , Ejercicio Físico/fisiología , Espacio Extracelular/metabolismo , Humanos , Técnicas de Dilución del Indicador , Membranas Intracelulares/metabolismoRESUMEN
To determine the influence of the prenatal long-chain polyunsaturated fatty acid (LCP) supply on prenatal growth and on postnatal LCP levels, we studied 52 preterm infants and assessed the relations between the LCP status at birth (reflecting the prenatal LCP supply), gestational age and prenatal growth, and the relation between the LCP status at birth and at 37 to 42 weeks of gestational age. After a correction for gestational age at birth, significant relations (p < or = 0.05) were observed between anthropometric measurements at birth (weight, head circumference, and length) and LCP levels in the umbilical artery wall, the LCP content of which reflects the long-term fetal LCP status. Independent of the neonatal diet (human milk or formula), LCP levels in erythrocyte phospholipids at term were positively related to levels in the umbilical artery wall (docosahexaenoic acid (22:6n-3): p < or = 0.0003; arachidonic acid (20:4n-6): p = 0.02). Postnatal diet significantly influenced LCP levels in plasma phospholipids at term (docosahexaenoic acid: p < or = 0.004; arachidonic acid: p = 0.02); formula-fed infants had lower values. We conclude that the LCP status of preterm infants at birth is related to prenatal growth. Moreover, next to the postnatal enteral diet, the LCP status at birth significantly affects LCP levels at term postconceptional age. This finding may warrant further studies of the effects of essential fatty acid-enriched maternal diets during pregnancy on the neonatal LCP status at birth.