Metabolic Diseases Unit, Neonatology Department, Clinical University Hospital of Santiago de Compostela, 15706 Santiago de Compostela, Spain.
IDIS-Health Research Institute of Santiago de Compostela, 15706 Santiago de Compostela, Spain.
Nutrients. 2024 Jan 31;16(3):423. doi: 10.3390/nu16030423.
Strict adherence to a diet is an essential pillar of long-term treatment for many inborn errors of metabolism (IEMs). Tools that educate patients about dietary management can positively condition adherence and prevent morbidity. We designed a free online dietary calculation program (Odimet, version 2.1.) for IEMs patients in 2008, updated in 2022, that provides detailed information on the content of amino acids, protein, lipids, carbohydrates, vitamins and minerals in >3000 food products, including specific medical foods for IEM. We analyzed the statistics on visits to Odimet to evaluate its usefulness for long-term dietary management during a 5-year period focusing on three periods: pre-pandemic (15 March 2018-14 March 2020); pandemic 1 (15 March 2020-14 March 2021); and pandemic 2 period (15 March 2021-15 March 2023), in 120 patients with the following distribution: 84 patients with phenylketonuria (PKU); 12 with maple syrup urine disease (MSUD); 11 with urea cycle disorders (UCDs); and 13 with classical galactosemia. The evolutionary levels of their specific metabolic markers were evaluated, showing that globally, both pediatric and adult patients maintain a good metabolic control, even during a pandemic (median levels of phenylalanine in pediatric PKU patients 213.4 µmol/L and 482.3 µmol/L in adults; of leucine in MSUD patients: 144.2 µmol/L; of glutamine in UCDs: 726.8 µmol/L; and of galactose 1-phosphate levels in galactosemia: 0.08 µmol/L). The proportion of patients using Odimet ranges from 78-100%. An increase in the number of diets being calculated was observed during COVID-19 pandemic. Currently, 14,825 products have been introduced (3094 from the general database, and 11,731 added by users to their own profiles). In 2023 63 emergency dietary adjustments in the studied intoxication-type pathologies were calculated in Odimet. Our results suggest that its regular use contributes to maintaining metabolic stability in IEMs patients, allowing them to adapt their menus to their lifestyle, and represents a powerful complementary tele-health tool which can be used to perform remote real-time dietary follow-up.
严格遵循饮食是许多先天性代谢错误（IEM）长期治疗的重要支柱。教育患者进行饮食管理的工具可以积极地影响患者的依从性并预防发病。我们于 2008 年设计了一个免费的在线饮食计算程序（Odimet，版本 2.1.），用于 IEM 患者，该程序于 2022 年更新，提供了 3000 多种食品中氨基酸、蛋白质、脂肪、碳水化合物、维生素和矿物质的详细信息，包括用于 IEM 的特定医疗食品。我们分析了访问 Odimet 的统计数据，以评估其在 5 年内用于长期饮食管理的有用性，重点关注三个时期：大流行前（2020 年 3 月 15 日至 2021 年 3 月 14 日）；大流行 1 期（2020 年 3 月 15 日至 2021 年 3 月 14 日）；和大流行 2 期（2021 年 3 月 15 日至 2023 年 3 月 15 日），涉及 120 名患者，分布如下：84 名苯丙酮尿症（PKU）患者；12 名枫糖尿症（MSUD）患者；11 名尿素循环障碍（UCD）患者；和 13 名经典半乳糖血症患者。评估了他们特定代谢标志物的进化水平，结果表明，全球范围内，儿科和成年患者都保持着良好的代谢控制，即使在大流行期间也是如此（儿科 PKU 患者苯丙氨酸中位数水平为 213.4 μmol/L 和成人 482.3 μmol/L；MSUD 患者亮氨酸中位数水平为 144.2 μmol/L；UCD 患者谷氨酰胺中位数水平为 726.8 μmol/L；半乳糖血症患者 1-磷酸半乳糖中位数水平为 0.08 μmol/L）。使用 Odimet 的患者比例为 78-100%。在 COVID-19 大流行期间，计算的饮食数量有所增加。目前，已经引入了 14825 种产品（普通数据库中有 3094 种，用户在自己的个人资料中添加了 11731 种）。2023 年，在研究的中毒型病理中，在 Odimet 中计算了 63 种紧急饮食调整。我们的结果表明，它的定期使用有助于维持 IEM 患者的代谢稳定性，使他们能够根据自己的生活方式调整饮食，并代表一种强大的补充远程医疗工具，可用于进行远程实时饮食随访。
