Nutrition and Metabolopathies Unit, La Fe University Hospital, 46025 Valencia, Spain.
National Technological Center for Food and Canning Industries, 30500 Murcia, Spain.
Nutrients. 2023 Jan 23;15(3):594. doi: 10.3390/nu15030594.
In galactosaemia, a strict galactose-free diet is necessary to prevent or resolve acute symptoms in infants. However, because the body produces up to 10 times more galactose than is found in a galactose-restricted diet, excessively restrictive diets should be avoided in children and adults to prevent nutritional deficiencies. Since cheese is a nutritional source of the calcium necessary for bone health, the latest international guidelines on the management of classical galactosaemia (2017) allow the consumption of cured cheeses with less than 25 mg of galactose/100 g and recommend that each country verifies the adequacy of the cheeses, since most mature cheeses do not always have a lower galactose content. In total, 32 cheese samples were purchased (19 Spanish and 13 international cheeses), and their lactose and galactose contents were analysed using ion chromatography with pulsed amperometric detection (IC-PAD). Five Spanish cheeses contained less than 25 mg of galactose/100 g: García Baquero semi-cured cheese; Hacendado, Gran Reserva and Mahón cured cheeses; and García Baquero Reserva 12-month cured cheese. In addition, eight international cheeses were confirmed as suitable: Comté, Gouda, Gruyere, Maasdam, Parmigiano, Edam, Emmental, and some samples of Cheddar. In addition to the well-known low-galactose Swiss and Dutch cheeses, according to the current results, five Spanish cheeses can be safely consumed. The greater availability of types of cheese favours better bone health in patients with galactosaemia.
在半乳糖血症中,严格的无半乳糖饮食是预防或缓解婴儿急性症状的必要措施。然而,由于人体产生的半乳糖比限制饮食中含有的半乳糖多 10 倍,因此儿童和成人应避免过度限制饮食,以防止营养缺乏。由于奶酪是营养来源,可为骨骼健康提供所需的钙,因此最新的经典半乳糖血症管理国际指南(2017 年)允许食用每 100 克含少于 25 毫克半乳糖的干酪,并建议每个国家都验证奶酪的充足性,因为大多数成熟奶酪并不总是具有较低的半乳糖含量。总共购买了 32 个奶酪样本(19 个西班牙奶酪和 13 个国际奶酪),并使用脉冲安培检测离子色谱法(IC-PAD)分析了它们的乳糖和半乳糖含量。五款西班牙奶酪的半乳糖含量低于 25 毫克/100 克:García Baquero 半干奶酪;Hacendado、Gran Reserva 和 Mahón 干奶酪;以及 García Baquero 12 个月陈酿奶酪。此外,有 8 种国际奶酪被证实是合适的:Comté、Gouda、Gruyere、Maasdam、Parmigiano、Edam、Emmental,以及一些切达奶酪样本。除了众所周知的低半乳糖瑞士和荷兰奶酪外,根据目前的结果,五种西班牙奶酪可以安全食用。奶酪种类的更多可获得性有利于半乳糖血症患者的骨骼健康。