Cubo Esther, Rivadeneyra Jessica, Mariscal Natividad, Martinez Asunción, Armesto Diana, Camara Rafael J
Neurology Department Burgos University Hospital Burgos Spain.
Research Unit Burgos University Hospital Burgos Spain.
Mov Disord Clin Pract. 2016 Jan 18;3(5):452-459. doi: 10.1002/mdc3.12304. eCollection 2016 Sep-Oct.
Patients with Huntington's disease (HD) are at risk for body weight loss and increased risk for institutionalization, morbidity, and mortality. The aim of this study was to determine the factors associated with low body mass index (BMI) in patients with HD.
In this national, observational, cross-sectional study of the European Huntington's Disease Network, the frequency of food consumption, calories, and nutrient intake in patients with HD was assessed using questionnaires validated for the Spanish population and were calculated using the software package (), version 2.0. Nutritional status was estimated using the BMI, and disease severity was assessed using the Unified Huntington's Disease Rating Scale and a total functional capacity (TFC) score. Linear regression models were performed using BMI as the dependent variable and using energy balance (energy caloric intake - energy expenditure); the TFC score; the presence of a caregiver; dysphagia; cytosine, adenine, guanine (CAG) repeats; comorbidities; intake of supplements; pharmacologic treatments; age; gender; education; and physical activity as the independent variables.
Two hundred twenty-four patients with HD were included (59% women), and their mean age was 47.41 ± 14.26 years, a median TFC score of 9 (range, 3-13), normal BMI in 124 patients (55.4%), and low BMI in 13 patients (6.7%). In the linear regression model, older age (β = 0.003; = 0.01), male gender (β = 0.13; = 0.003), and lower energy balance (β = -0.0001; = 0.0003) were associated with a higher log-transformed BMI.
Younger female HD patients are at risk for low BMI. To counteract the influence of the HD gene mutation on decreased BMI, an increase in kilocalories per day should be encouraged.
亨廷顿舞蹈症（HD）患者存在体重减轻的风险，且被收容、发病和死亡风险增加。本研究的目的是确定与HD患者低体重指数（BMI）相关的因素。
在欧洲亨廷顿舞蹈症网络的这项全国性观察性横断面研究中，使用针对西班牙人群验证的问卷评估HD患者的食物消费频率、卡路里和营养摄入量，并使用软件包（）2.0版进行计算。使用BMI评估营养状况，使用统一亨廷顿舞蹈症评定量表和总功能能力（TFC）评分评估疾病严重程度。以BMI作为因变量，以能量平衡（能量卡路里摄入量 - 能量消耗）、TFC评分、是否有护理人员、吞咽困难、胞嘧啶、腺嘌呤、鸟嘌呤（CAG）重复序列、合并症、补充剂摄入量、药物治疗、年龄、性别、教育程度和身体活动作为自变量进行线性回归模型分析。
纳入了224例HD患者（59%为女性），他们的平均年龄为47.41±14.26岁，TFC评分中位数为9（范围3 - 13），124例患者（55.4%）BMI正常，13例患者（6.7%）BMI低。在线性回归模型中，年龄较大（β = 0.003； = 0.01）、男性（β = 0.13； = 0.003）和较低的能量平衡（β = -0.0001； = 0.0003）与较高的对数转换BMI相关。
年轻女性HD患者存在BMI低的风险。为抵消HD基因突变对BMI降低的影响，应鼓励每日增加千卡摄入量。
