Motil K J, Schultz R, Brown B, Glaze D G, Percy A K
USDA/ARS Children's Nutrition Research Center, Rett Center, Department of Pediatrics, Baylor College of Medicine, Houston, TX.
J Child Neurol. 1994 Jul;9(3):315-9. doi: 10.1177/088307389400900319.
To determine whether alterations in energy balance account for growth failure in Rett syndrome, we measured dietary energy intakes, fecal fat losses, activity patterns, and sleeping as well as quietly and actively awake metabolic rates in Rett syndrome girls and healthy controls. Dietary energy intakes and fecal fat losses did not differ between the groups. Metabolic rates while sleeping and quietly awake were 23% lower (P < .05) in Rett syndrome girls than in controls; metabolic rates while actively awake did not differ between the groups. However, because of the 2.4-fold greater time (P < .001) spent in involuntary motor movement, energy expenditure associated with activity was twofold greater (P < .05) in Rett syndrome girls than in controls. Although total daily energy expenditure of the two groups did not differ significantly, energy balance was less positive in the Rett syndrome girls than in the controls. This small difference in energy balance, if sustained over months to years, is sufficient to account for growth failure in Rett syndrome girls.
为了确定能量平衡的改变是否是雷特综合征生长发育迟缓的原因，我们测量了雷特综合征女童和健康对照者的膳食能量摄入量、粪便脂肪流失、活动模式、睡眠情况以及安静和活跃清醒状态下的代谢率。两组之间的膳食能量摄入量和粪便脂肪流失没有差异。雷特综合征女童睡眠和安静清醒时的代谢率比对照组低23%（P < 0.05）；两组活跃清醒时的代谢率没有差异。然而，由于雷特综合征女童非自主运动的时间比对照组多2.4倍（P < 0.001），与活动相关的能量消耗是对照组的两倍（P < 0.05）。虽然两组的每日总能量消耗没有显著差异，但雷特综合征女童的能量平衡比对照组的更不积极。这种能量平衡的微小差异，如果持续数月至数年，足以解释雷特综合征女童的生长发育迟缓。
