Shakerdi Loai A, Wallace Leonie, Smyth Georgina, Madden Nora, Clark Anne, Hendroff Una, McGovern Marianne, Connellan Sarah, Gillman Barbara, Treacy Eileen P
National Centre for Inherited Metabolic Disorders Mater Misericordiae University Hospital Dublin 7 Ireland.
Public Analyst's Laboratory Galway Ireland.
Food Sci Nutr. 2022 Jul 19;10(11):3789-3800. doi: 10.1002/fsn3.2976. eCollection 2022 Nov.
Classical galactosemia (CG) is a disorder of galactose metabolism which results from deficiency of the enzyme galactose-1-phosphate uridylyl transferase (GALT). Treatment consists of immediately eliminating galactose from the diet in the new-born and lifelong restriction of dietary galactose. The inclusion of a wider variety of foods for people with CG may provide many benefits, including improved nutritional adequacy and quality of life. Galactose plays an important role in glycosylation of glycoproteins and glycolipids. Moderate liberalization of galactose restriction has been shown to improve immunoglobulin G (IgG) glycosylation for some individuals with CG. Moreover, recent outcome research suggests that strict restriction of nondairy galactose may have more unfavorable outcomes than moderate liberalization in CG patients. In the current work, based on patient feedback, we have analyzed the lactose and galactose content of different foods available in Ireland. These include a range of cheeses, yogurts, pizzas, soups, biscuits, cakes, pastries, crackers, mayonnaises, salad creams, fat spreads, crisps, corn chips, salamis, and gravies. This work provides information to support the development of a practical food-based approach to facilitate analysis of dietary galactose intake and to possibly increase overall variety of food choices for people with CG.
经典型半乳糖血症（CG）是一种半乳糖代谢紊乱疾病，由半乳糖-1-磷酸尿苷酰转移酶（GALT）缺乏所致。治疗方法包括在新生儿期立即从饮食中去除半乳糖，并终身限制膳食半乳糖摄入。为CG患者提供更多种类的食物可能会带来诸多益处，包括改善营养充足状况和生活质量。半乳糖在糖蛋白和糖脂的糖基化过程中发挥着重要作用。对于一些CG患者，适度放宽半乳糖限制已被证明可改善免疫球蛋白G（IgG）的糖基化。此外，最近的结果研究表明，在CG患者中，严格限制非乳制品半乳糖可能比适度放宽限制产生更不利的结果。在当前的工作中，基于患者反馈，我们分析了爱尔兰市场上不同食物中的乳糖和半乳糖含量。这些食物包括一系列奶酪、酸奶、披萨、汤、饼干、蛋糕、糕点、薄脆饼干、蛋黄酱、沙拉酱、涂抹酱、薯片、玉米片、意大利腊肠和肉汁。这项工作提供了相关信息，以支持开发一种基于食物的实用方法，便于分析膳食半乳糖摄入量，并可能增加CG患者的食物选择总体种类。
