Kristel Vande Kerckhove1, Marianne Diels2, Sigrid Vanhaesebrouck2, Karin Luyten3, Nancy Pyck3, An De Meyer3, Marleen Van Driessche4, Martine Robert5, Karen Corthouts6, Ariane Caris7, Emilie Duchateau8, Martine Dassy9, Genevieve Bihet10. 1. Inherited Metabolic Disease Center, University Hospital Leuven, Leuven, Belgium. Electronic address: kristel.vandekerckhove@uzleuven.be. 2. Inherited Metabolic Disease Center, University Hospital Leuven, Leuven, Belgium. 3. Inherited Metabolic Disease Center, University Hospital Antwerpen, Antwerp, Belgium. 4. Inherited Metabolic Disease Center, University Hospital Gent, Gent, Belgium. 5. Inherited Metabolic Disease Center, Children's Hospital, Brussels, Belgium. 6. Inherited Metabolic Disease Center, University Hospital Brussel, Brussels, Belgium. 7. Service de Génétique Humaine, CHU Sart Tilman Liège, Liège, Belgium. 8. Institute de Pathologie et de Génétique, Gosselies, Belgium. 9. Cliniques Universitaires Saint- Luc Bruxelles -Woluwe, Brussels, Belgium. 10. Clinique de l'Espérance, Montegnée, Belgium.
Abstract
BACKGROUND AND AIM: Worldwide there is scientific discussion about the dietary management of galactosemia. The dietary management is very different in several countries among Europe, the US and Canada. The main points of discussion are related to the fact that i) despite a strict diet some patients still have poor outcomes; ii) there is lack of scientific knowledge about the role of endogenous production of galactose on disease evolution, with or without diet. The aim of the current work was the creation of a Belgian consensus on dietary guidelines for the management of galactosemia. METHODS: A step-wise approach was used to achieve a consensus, including: a workshop, a Delphi round, discussion groups and a round table of different Belgian experts. RESULTS: The consensus is an agreement between strict guidelines (strict limitation of fruits, vegetables and soybean products/French guidelines) and the more liberal guidelines (comparable with a diet free of lactose/guidelines of UK and the Netherlands). The consensus document consists of different modules, including the medical context, the theoretical background of dietary guidelines and the age-specific practical dietary guidelines. CONCLUSION: A Belgian consensus on the guidelines for the dietary management of classical galactosemia was developed despite the uncertainties of the efficacy and practical application of these guidelines. The final consensus is based on scientific knowledge and practical agreement among experts. In the future, regular revision of the guidelines is recommended and a uniform European guideline is desirable.
BACKGROUND AND AIM: Worldwide there is scientific discussion about the dietary management of galactosemia. The dietary management is very different in several countries among Europe, the US and Canada. The main points of discussion are related to the fact that i) despite a strict diet some patients still have poor outcomes; ii) there is lack of scientific knowledge about the role of endogenous production of galactose on disease evolution, with or without diet. The aim of the current work was the creation of a Belgian consensus on dietary guidelines for the management of galactosemia. METHODS: A step-wise approach was used to achieve a consensus, including: a workshop, a Delphi round, discussion groups and a round table of different Belgian experts. RESULTS: The consensus is an agreement between strict guidelines (strict limitation of fruits, vegetables and soybean products/French guidelines) and the more liberal guidelines (comparable with a diet free of lactose/guidelines of UK and the Netherlands). The consensus document consists of different modules, including the medical context, the theoretical background of dietary guidelines and the age-specific practical dietary guidelines. CONCLUSION: A Belgian consensus on the guidelines for the dietary management of classical galactosemia was developed despite the uncertainties of the efficacy and practical application of these guidelines. The final consensus is based on scientific knowledge and practical agreement among experts. In the future, regular revision of the guidelines is recommended and a uniform European guideline is desirable.