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

•All centres treating adults with galactosaemia restrict lactose•Some centres still restrict galactose in fruit, vegetables, lentils & offal in adults.•Patient led modest lactose relaxation (trace amounts) occurs in some centres.•32% of adult patients were lost to dietetic follow-up.

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.