Analysis of daily energy, protein, fat, and carbohydrate intake in citrin-deficient patients: Towards prevention of adult-onset type II citrullinemia
Analysis of daily energy, protein, fat, and carbohydrate intake in citrin-deficient patients: Towards prevention of adult-onset type II citrullinemia
复制标题
DOI：
10.1016/j.ymgme.2021.03.004
复制
发表时间：
2021-04-20
影响因子：
3.8
通讯作者：
Takeshima, Yasuhiro
中科院分区：
文献类型：
--
作者：
Okano, Yoshiyuki;Okamoto, Miki;Takeshima, Yasuhiro
适应/代偿期Citrin缺乏症患者表现出不同的临床特征，并具有高蛋白、高脂肪和低碳水化合物的特征性饮食。日本料理通常含有高碳水化合物，但2008年对缺乏桔霉素患者的饮食评估显示，能量摄入量低，蛋白质：脂肪：碳水化合物（PFC）比例为19：44：37，这表明碳水化合物消耗率低。这些发现提示需要进行饮食干预，以预防成人II型瓜氨酸血症（CTLN 2）的发作。自该报告于约10年前发表以来，一般都建议病人在积极的饮食咨询和干预下吃他们想吃的东西。在这项研究中，生活在同一个家庭的桔霉素缺乏患者和对照组提供了一份问卷的答案，填写了最多6天的食物日记，并提供了身体数据和药物信息（如果有的话）。为了研究当前饮食的影响，该调查收集了62名患者和45名对照组的数据，比较了每日能量，蛋白质，脂肪和碳水化合物的摄入量。食物分析显示病人的能量摄入是日本标准的115%。患者PFC比值的置信区间为20-22：47-51：28-32，表明相对于以前的报道，蛋白质更高，脂肪更高，碳水化合物更低。女性患者的平均PFC比率（22：53：25）与男性患者的平均PFC比率（20：46：34）显著不同，这可能解释了女性CTLN 2的频率较低。将目前的数据与10年前发表的数据进行比较，能量，蛋白质和脂肪的摄入量显着增加，但碳水化合物的消耗量保持不变。不考虑年龄，大多数患者（青少年除外）消耗100-200 g/天碳水化合物，这符合健康个体估计的平均需求量100 g/天。最后，患者一般不超重，一些CTLN 2患者体重过轻，尽管他们的能量摄入量高于对照组。我们推测，在饮食干预下，高能量低碳水化合物饮食可能有助于Citrin缺乏患者获得正常生长并预防CTLN 2的发生。(c)2021作者爱思唯尔公司出版这是CC BY-NC-ND许可下的开放获取文章（http：//creativecommons.org/licenses/by-nc-nd/4.0/）。
Patients with citrin deficiency during the adaptation/compensation period exhibit diverse clinical features and have characteristic diet of high protein, high fat, and low carbohydrate. Japanese cuisine typically contains high carbohydrate but evaluation of diet of citrin-deficient patients in 2008 showed a low energy intake and a protein:fat:carbohydrate (PFC) ratio of 19:44:37, which indicates low carbohydrate consumption rate. These findings prompted the need for diet intervention to prevent the adult onset of type II citrullinemia (CTLN2). Since the publication of the report about 10 years ago, patients are generally advised to eatwhat theywish under active dietary consultation and intervention. In this study, citrin-deficient patients and control subjects living in the same household provided answers to a questionnaire, filled-up a maximum6-day food diary, and supplied physical data and information on medications if any. To study the effects of the current diet, the survey collected data from 62 patients and 45 controls comparing daily intakes of energy, protein, fat, and carbohydrate. Food analysis showed that patient's energy intakewas 115% compared to the Japanese standard. The confidence interval of the PFC ratio of patients was 20-22:47-51:28-32, indicating higher protein, higher fat and lower carbohydrate relative to previous reports. The mean PFC ratio of female patients (22:53:25) was significantly different from that of male patients (20:46:34), which may explain the lower frequency of CTLN2 in females. Comparison of the present data to those published 10 years ago, energy, protein, and fat intakes were significantly higher but the amount of carbohydrate consumption remained the same. Regardless of age, most patients (except for adolescents) consumed 100-200 g/day of carbohydrates, whichmet the estimated average requirement of 100 g/day for healthy individuals. Finally, patients were generally not overweight and some CTLN2 patients were underweight although their energy intake was higher compared with the control subjects. We speculate that highenergy of a low carbohydrate diet under dietary intervention may help citrin-deficient patients attain normal growth and prevent the onset of CTLN2. (c) 2021 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).