ENERGY METABOLISM IN PATIENTS WITH HUNTINGTON'S DISEASE

亨廷顿病患者的能量代谢

• 批准号: 2573716
• 负责人: R PRATLEY
• 金额: --
• 依托单位: NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES
• 依托单位国家: 美国
• 资助国家: 美国
• 起止时间: 至
• 项目状态: 未结题
• 来源: https://reporter.nih.gov/project-details/2573716
• 关键词: Huntington's disease; basal metabolism; bioenergetics; body composition; body physical activity; clinical research; electrophysiology; human subject; medical complication; photon absorptiometry; weight loss

Huntington's Disease is an autosomal dominant neurologic disorder characterized by chorea, eye movement abnormalities, and dementia. Significant weight loss is a common finding early in the course of the disease despite reportedly normal or increased caloric intakes. The cause of this apparent increase in energy expenditure is not known. We hypothesize that energy expenditure is increased in patients with Huntigton's Disease as a consequence of the movement disorder. To test this, basal metabolic rate, 24-hour energy expenditure, and non- resting energy expenditure (all measured using the human respiratory chamber on the Research Ward of the Clinical Diabetes and Nutrition Section), and 7-day free-living energy expenditure (measured using the doubly-labeled water technique) will be compared in three groups of patients with Huntington's Disease with varying degrees of chorea (asymptomatic, mild, and moderate) and age, sex, and weight matched controls. To correct energy expenditure for differences in metabolically active (primarily lean) body mass among subjects, all individuals will have body composition measured by anthropometry, bioelectrical impedance analysis, and dual energy X-ray absorptiometry scanning. A total of 18 subjects (13 patients and 5 controls) have been studied to date. Twenty-four energy expenditure measured in the respiratory chamber was significantly higher in patients than controls (1903+/-101 v 1514+/-105 KCal/24h, P < 0.05) whereas BMR was not significantly different (1.15+/-0.09 v 1.01+/-0.04 Kcal/min). These preliminary results suggest that the increased energy expenditure among patients with Huntingtons Disease is attributable to the movement disorder. In addition to providing insight into the pathophysiologic abnormalities in Huntington's Disease, this study may also improve the care of patients with Huntington's Disease and other persons with movement disorders by providing more precise estimates of their caloric needs.

亨廷顿病是一种常染色体显性遗传性神经系统疾病 以舞蹈病、眼球运动异常和痴呆为特征。 显著的体重减轻是在治疗过程早期的常见发现。 尽管据报道热量摄入正常或增加，但仍患有疾病。 的 能量消耗明显增加的原因尚不清楚。 我们假设，患者的能量消耗增加， 运动障碍导致的亨氏病。 到 测试这个，基础代谢率，24小时能量消耗， 静息能量消耗（均使用人呼吸道测量） 临床糖尿病与营养研究病房的小室 部分）和7天自由生活能量消耗（使用 双标记水技术）将在三组中进行比较， 伴有不同程度舞蹈病的亨廷顿病患者 （无症状、轻度和中度）和年龄、性别和体重匹配 对照 为了纠正能量消耗的差异， 受试者的代谢活性（主要是瘦）体重，所有 个体将具有通过人体测量法测量的身体组成， 生物电阻抗分析和双能X线吸收法 扫描。 共有18名受试者（13名患者和5名对照） 至今被研究。 24个能量消耗测量 患者的呼吸腔明显高于对照组 (1903+/-101 v1514 +/-105 KCal/24 h，P < 0.05），而BMR不是 显著不同（1.15+/-0.09 v1. 01 +/-0.04 Kcal/min）。 这些 初步结果表明，增加的能量消耗 亨廷顿氏病患者中， 运动障碍 除了深入了解 亨廷顿病的病理生理异常，这项研究可能 还可以改善对患有亨廷顿病和其他疾病的患者的护理， 通过提供更精确的估计， 他们的卡路里需求。