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How strict is galactose restriction in adults with galactosaemia? International practice.
Mol Genet Metab. 2015 May; 115(1):23-6.MG

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.

Authors+Show Affiliations

Royal Hospital for Sick Children, Glasgow, UK.National Metabolic Service, Starship Children's Health and Auckland City Hospital, Auckland, New Zealand.Ospedale pediatrico Bambino Gesù, Rome, Italy.UniversitätsSpital Zürich, Switzerland.University Hospitals Birmingham NHS Foundation Trust, UK.Division of Inherited Metabolic Diseases, Reference Centre Expanded Newborn Screening, Department of Pediatrics, University Hospital, Padova, Italy.National Metabolic Service, Starship Children's Health and Auckland City Hospital, Auckland, New Zealand.University Hospitals Leuven, Center of Metabolic Diseases, Belgium.Hospital La Fe, Valencia, Spain.Royal Hospital for Sick Children Edinburgh, UK.Hôpital Universitaire des Enfants, Reine fabiola, Bruxelles, Belgium.Center of Metabolic Diseases, University Hospital, Antwerp, Belgium.Universitair Ziekenhuis Gent, Belgium.Royal Victoria Hospital, Newcastle, UK.University Hospitals Leuven, Center of Metabolic Diseases, Belgium.Dr. von Hauner Children's Hospital, Munich, Germany.Addenbrookes, Cambridge, UK.Birmingham Children's Hospital, Birmingham, UK.Division of Inherited Metabolic Diseases, Reference Centre Expanded Newborn Screening, Department of Pediatrics, University Hospital, Padova, Italy.Royal Victoria Hospital, Newcastle, UK.North Bristol NHS Trust Southmead and Frenchay, UK.Hospital Clinic Barcelona, Spain.Ospedale pediatrico Bambino Gesù, Rome, Italy.University Children's Hospital Freiburg, Germany.Universitätsklinikum Hamburg-Eppendorf, Germany.Karolinska University Hospital Stockholm, Sweden.Academic Medical Hospital, Amsterdam, Netherlands.Royal Melbourne Hospital, Melbourne, Australia.Center of Metabolic Diseases, University Hospital, Antwerp, Belgium.Birmingham Children's Hospital, Birmingham, UK. Electronic address: anita.macdonald@bch.nhs.uk.Clinic of Paediatric Kidney, Liver- and Metabolic Diseases Medical School Hannover, Germany.St Thomas' Hospital, London, UK.Children's Hospital Heidelberg, Germany.Galactosemia Support Group, UK.Salford Royal, Manchester, UK.Hôpital Universitaire des Enfants, Reine fabiola, Bruxelles, Belgium.University Hospitals Birmingham NHS Foundation Trust, UK.University Children's Hospital Freiburg, Germany.Children's Hospital Heidelberg, Germany.Universitätsklinikum Hamburg-Eppendorf, Germany.University Hospital of Wales, Cardiff, UK.Children's Hospital, University Hospital Skåne, Sweden.Oslo University Hospital Rikshospitalet, Norway.Alderhey Children's Hospital, Liverpool, UK.Children's Hospital, Westmead, Sydney, Australia.UMC Utrecht, Netherlands.University Hospitals Leuven, Center of Metabolic Diseases, Belgium.University Hospital Maastricht, Netherlands.Universitair Ziekenhuis Gent, Belgium.University of Groningen, University Medical Center Groningen, Netherlands.Univ.-Kinderklinik, Pädiatrische Diätetik, Munster, Germany.Hospital La Fe, Valencia, Spain.Hôpital Antoine Béclère, France.CHU Bicëtre Hospital, Paris, France.Westmead Hospital, Sydney, Australia.Bradford Teaching Hospital NHS Trust, UK.Radboud UMC, Netherlands.

Pub Type(s)

Comparative Study
Journal Article
Multicenter Study

Language

eng

PubMed ID

25873073

Citation

Adam, S, et al. "How Strict Is Galactose Restriction in Adults With Galactosaemia? International Practice." Molecular Genetics and Metabolism, vol. 115, no. 1, 2015, pp. 23-6.
Adam S, Akroyd R, Bernabei S, et al. How strict is galactose restriction in adults with galactosaemia? International practice. Mol Genet Metab. 2015;115(1):23-6.
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., ... Zweers, H. (2015). How strict is galactose restriction in adults with galactosaemia? International practice. Molecular Genetics and Metabolism, 115(1), 23-6. https://doi.org/10.1016/j.ymgme.2015.03.008
Adam S, et al. How Strict Is Galactose Restriction in Adults With Galactosaemia? International Practice. Mol Genet Metab. 2015;115(1):23-6. PubMed PMID: 25873073.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - How strict is galactose restriction in adults with galactosaemia? International practice. AU - Adam,S, AU - Akroyd,R, AU - Bernabei,S, AU - Bollhalder,S, AU - Boocock,S, AU - Burlina,A, AU - Coote,T, AU - Corthouts,K, AU - Dalmau,J, AU - Dawson,S, AU - Defourny,S, AU - De Meyer,A, AU - Desloovere,A, AU - Devlin,Y, AU - Diels,M, AU - Dokoupil,K, AU - Donald,S, AU - Evans,S, AU - Fasan,I, AU - Ferguson,C, AU - Ford,S, AU - Forga,M, AU - Gallo,G, AU - Grünert,S C, AU - Heddrich-Ellerbrok,M, AU - Heidenborg,C, AU - Jonkers,C, AU - Lefebure,K, AU - Luyten,K, AU - MacDonald,A, AU - Meyer,U, AU - Micciche,A, AU - Müller,E, AU - Portnoi,P, AU - Ripley,S, AU - Robert,M, AU - Robertson,L V, AU - Rosenbaum-Fabian,S, AU - Sahm,K, AU - Schultz,S, AU - Singleton,K, AU - Sjöqvist,E, AU - Stoelen,L, AU - Terry,A, AU - Thompson,S, AU - Timmer,C, AU - Vande Kerckhove,K, AU - van der Ploeg,L, AU - Van Driessche,M, AU - van Rijn,M, AU - van Teeffelen-Heithoff,A, AU - Vitoria,I, AU - Voillot,C, AU - Wenz,J, AU - Westbrook,M, AU - Wildgoose,J, AU - Zweers,H, Y1 - 2015/04/07/ PY - 2015/02/12/received PY - 2015/03/29/revised PY - 2015/03/30/accepted PY - 2015/4/16/entrez PY - 2015/4/16/pubmed PY - 2016/1/28/medline KW - Diet KW - Galactosaemia KW - Galactose KW - Galactose-1-phosphate KW - Lactose KW - Monitoring SP - 23 EP - 6 JF - Molecular genetics and metabolism JO - Mol Genet Metab VL - 115 IS - 1 N2 - 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. SN - 1096-7206 UR - https://www.unboundmedicine.com/medline/citation/25873073/How_strict_is_galactose_restriction_in_adults_with_galactosaemia_International_practice_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1096-7192(15)00088-8 DB - PRIME DP - Unbound Medicine ER -