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

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.

The lactose content of Mini Babybel and suitability for galactosaemia.

UNLABELLED: The range of cheeses analysed for their lactose and galactose content to verify their suitability for a low galactose diet in galactosaemia continue to expand. No assumptions should be made about the suitability of any cheese for galactosaemia until reputable and repeated analysis has been conducted. AIM: To analyse the lactose content of a range of Mini Babybel cheeses. METHODS: The lactose content of 10 samples of Mini Babybel (five Mini Babybel Original and five Mini Babybel Emmental) was analysed by Leatherhead Food international Laboratory. RESULTS: The lactose content of Mini Babybel Original varied between <2 mg to 12 mg 100g(-1) and Mini Babybel Emmental was between 6 and 7 mg 100g(-1). CONCLUSIONS: The lactose content of Mini Babybel Original and five Mini Babybel Emmental is too high to render them suitable for the treatment of galactosaemia.

Determination of the lactose and galactose content of cheese for use in the galactosaemia diet.

BACKGROUND: Treatment of galactosaemia requires a low galactose diet. In the UK, traditionally, all cheeses have been excluded from the diet, although some types of mature hard cheese are likely to be low in lactose and galactose. The present study aimed to determine the lactose and galactose content of mature cheeses. METHODS: Over 6 years, the UK Galactosaemia Support Group commissioned the analysis of 109 samples (by two laboratories) of 12 cheese types, in eight batches throughout the year. Cheeses, obtained from retail outlets, were homogenised, sugars were extracted using water or 40% alcohol for fatty samples, and samples were deproteinised. Enzymatic analysis using measuring light absorbance was conducted on filtered extracts. RESULTS: Cheeses containing undetectable quantities of lactose (<2.8 mg 100 g(-1), Leatherhead Food International, Leatherhead, UK (LFI) analysis; <10 mg 100 g(-1), Laboratory of Government Chemist, Teddington, UK (LGC Limited) analysis) and galactose were: Gruyere (five samples); Emmental (block, sliced and grated) (16 samples); Jarlsberg (six samples); Parmigiano Reggiano and Grana Padano Italian Parmesan (block and grated) (16 samples); and mature Cheddar cheese from the UK West Country Farmhouse Cheese Makers Association (35 samples) only. Lactose containing cheeses included other mature Cheddar cheeses, Gouda and Edam. CONCLUSIONS: Gruyere, Emmental, Jarlsberg, Italian Parmesan (Parmigiano Reggiano and Grana Padano), and mature Cheddar cheese produced in one area of England where the manufacturing process is standardised and guaranteed are now allowed in the UK galactosaemia diet.

The Lactose and Galactose Content of Cheese Suitable for Galactosaemia: New Analysis.

INTRODUCTION: The UK Medical Advisory Panel of the Galactosaemia Support Group report the lactose and galactose content of 5 brands of mature Cheddar cheese, Comte and Emmi Emmental fondue mix from 32 cheese samples. The Medical Advisory Panel define suitable cheese in galactosaemia to have a lactose and galactose content consistently below 10 mg/100 g. METHODS: A total of 32 samples (5 types of mature Cheddar cheese, Comte and "Emmi Swiss Fondue", an emmental fondue mix) were analysed by high-performance anion exchange chromatography with pulsed amperometric detection (HPAEC-PAD) technology used to perform lactose and galactose analysis. RESULTS: Cheddar cheese types: Valley Spire West Country, Parkham, Lye Cross Vintage, Lye Cross Mature, Tesco West Country Farmhouse Extra Mature and Sainsbury's TTD West Country Farmhouse Extra Mature had a lactose and galactose content consistently below 10 mg/100 g (range <0.05 to 12.65 mg). All Comte samples had a lactose content below the lower limit of detection (<0.05 mg) with galactose content from <0.05 to 1.86 mg/100 g; all samples of Emmi Swiss Fondue had lactose below the lower limit of detection (<0.05 mg) and galactose between 2.19 and 3.04 mg/100 g. CONCLUSIONS: All of these cheese types were suitable for inclusion in a low galactose diet for galactosaemia. It is possible that the galactose content of cheese may change over time depending on its processing, fermentation time and packaging techniques.

The lactose and galactose content of milk fats and suitability for galactosaemia.

INTRODUCTION: Butter oil, ghee and butter are theoretically low in lactose. All three products are high in milk fat; butter oil and ghee contain approximately 99.3% fat and butter around 80% fat. We report the lactose and galactose content of butter, ghee, and butter oil and assess their suitability in a low galactose diet. METHODS: A total of 12 samples (butter oil n = 5, ghee n = 5, butter n = 2) were analysed by High-Performance Anion Exchange Chromatography with Pulsed Amperometric Detection (HPAEC-PAD) technology used to perform lactose and galactose analyses. RESULTS: Butter oil and ghee were consistently low in lactose and galactose. Butter oil analysis: lactose, 0.47 to 3.08 mg/100 g; galactose, < 0.05 to 2.28 mg/100 g. Ghee analysis: lactose, < 0.05 to 2.9 mg/100 g; galactose, < 0.05 mg to < 1.0 mg/100 g. Butter analysis: lactose 685 to 688 mg/100 g; galactose, 1.3 mg to 1.6 mg/100 g. CONCLUSIONS: Butter oil (commonly used in food industry) and ghee contain minimal lactose and galactose and so are permitted in a UK galactosaemia diet. Butter is considered too high in lactose and is unsuitable in a low galactose diet.