对于患有半乳糖血症的成年人，半乳糖限制有多严格？国际惯例。

How strict is galactose restriction in adults with galactosaemia? International practice.

作者信息

Adam S, Akroyd R, Bernabei S, Bollhalder S, Boocock S, Burlina A, Coote T, Corthouts K, Dalmau J, Dawson S, Defourny S, De Meyer A, Desloovere A, Devlin Y, Diels M, Dokoupil K, Donald S, Evans S, Fasan I, Ferguson C, Ford S, Forga M, Gallo G, Grünert S C, Heddrich-Ellerbrok M, Heidenborg C, Jonkers C, Lefebure K, Luyten K, MacDonald A, Meyer U, Micciche A, Müller E, Portnoi P, Ripley S, Robert M, Robertson L V, Rosenbaum-Fabian S, Sahm K, Schultz S, Singleton K, Sjöqvist E, Stoelen L, Terry A, Thompson S, Timmer C, Vande Kerckhove K, van der Ploeg L, Van Driessche M, van Rijn M, van Teeffelen-Heithoff A, Vitoria I, Voillot C, Wenz J, Westbrook M, Wildgoose J, Zweers H

机构信息

Royal Hospital for Sick Children, Glasgow, UK.

National Metabolic Service, Starship Children's Health and Auckland City Hospital, Auckland, New Zealand.

出版信息

Mol Genet Metab. 2015 May;115(1):23-6. doi: 10.1016/j.ymgme.2015.03.008. Epub 2015 Apr 7.

DOI:10.1016/j.ymgme.2015.03.008

PMID:25873073

Abstract

Dietary management of 418 adult patients with galactosaemia (from 39 centres/12 countries) was compared. All centres advised lactose restriction, 6 restricted galactose from galactosides ± fruits and vegetables and 12 offal. 38% (n=15) relaxed diet by: 1) allowing traces of lactose in manufactured foods (n=13) or 2) giving fruits, vegetables and galactosides (n=2). Only 15% (n=6) calculated dietary galactose. 32% of patients were lost to dietetic follow-up. In adult galactosaemia, there is limited diet relaxation.

摘要

对来自39个中心/12个国家的418例成年半乳糖血症患者的饮食管理进行了比较。所有中心均建议限制乳糖摄入，6个中心限制来自半乳糖苷±水果和蔬菜以及12种内脏的半乳糖。38%（n = 15）的患者放宽了饮食限制，方式如下：1）允许加工食品中含有微量乳糖（n = 13）或2）食用水果、蔬菜和半乳糖苷（n = 2）。只有15%（n = 6）的患者计算了饮食中的半乳糖含量。32%的患者失去了饮食随访。在成年半乳糖血症患者中，饮食放宽的情况有限。