title: How strict is galactose restriction in adults with galactosaemia? International practice.
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: Royal Hospital for Sick Children, Glasgow, UK.
authors: S
authors: S
authors: Adam
authors: National Metabolic Service, Starship Children's Health and Auckland City Hospital, Auckland, New Zealand.
authors: R
authors: R
authors: Akroyd
authors: Ospedale pediatrico Bambino Gesù, Rome, Italy.
authors: S
authors: S
authors: Bernabei
authors: UniversitätsSpital Zürich, Switzerland.
authors: S
authors: S
authors: Bollhalder
authors: University Hospitals Birmingham NHS Foundation Trust, UK.
authors: S
authors: S
authors: Boocock
authors: Division of Inherited Metabolic Diseases, Reference Centre Expanded Newborn Screening, Department of Pediatrics, University Hospital, Padova, Italy.
authors: A
authors: A
authors: Burlina
authors: National Metabolic Service, Starship Children's Health and Auckland City Hospital, Auckland, New Zealand.
authors: T
authors: T
authors: Coote
authors: University Hospitals Leuven, Center of Metabolic Diseases, Belgium.
authors: K
authors: K
authors: Corthouts
authors: Hospital La Fe, Valencia, Spain.
authors: J
authors: J
authors: Dalmau
authors: Royal Hospital for Sick Children Edinburgh, UK.
authors: S
authors: S
authors: Dawson
authors: Hôpital Universitaire des Enfants, Reine fabiola, Bruxelles, Belgium.
authors: S
authors: S
authors: Defourny
authors: Center of Metabolic Diseases, University Hospital, Antwerp, Belgium.
authors: A
authors: A
authors: De Meyer
authors: Universitair Ziekenhuis Gent, Belgium.
authors: A
authors: A
authors: Desloovere
authors: Royal Victoria Hospital, Newcastle, UK.
authors: Y
authors: Y
authors: Devlin
authors: University Hospitals Leuven, Center of Metabolic Diseases, Belgium.
authors: M
authors: M
authors: Diels
authors: Dr. von Hauner Children's Hospital, Munich, Germany.
authors: K
authors: K
authors: Dokoupil
authors: Addenbrookes, Cambridge, UK.
authors: S
authors: S
authors: Donald
authors: Birmingham Children's Hospital, Birmingham, UK.
authors: S
authors: S
authors: Evans
authors: Division of Inherited Metabolic Diseases, Reference Centre Expanded Newborn Screening, Department of Pediatrics, University Hospital, Padova, Italy.
authors: I
authors: I
authors: Fasan
authors: Royal Victoria Hospital, Newcastle, UK.
authors: C
authors: C
authors: Ferguson
authors: North Bristol NHS Trust Southmead and Frenchay, UK.
authors: S
authors: S
authors: Ford
authors: Hospital Clinic Barcelona, Spain.
authors: M
authors: M
authors: Forga
authors: Ospedale pediatrico Bambino Gesù, Rome, Italy.
authors: G
authors: G
authors: Gallo
authors: University Children's Hospital Freiburg, Germany.
authors: S C
authors: SC
authors: Grünert
authors: Universitätsklinikum Hamburg-Eppendorf, Germany.
authors: M
authors: M
authors: Heddrich-Ellerbrok
authors: Karolinska University Hospital Stockholm, Sweden.
authors: C
authors: C
authors: Heidenborg
authors: Academic Medical Hospital, Amsterdam, Netherlands.
authors: C
authors: C
authors: Jonkers
authors: Royal Melbourne Hospital, Melbourne, Australia.
authors: K
authors: K
authors: Lefebure
authors: Center of Metabolic Diseases, University Hospital, Antwerp, Belgium.
authors: K
authors: K
authors: Luyten
authors: Birmingham Children's Hospital, Birmingham, UK. Electronic address: anita.macdonald@bch.nhs.uk.
authors: A
authors: A
authors: MacDonald
authors: Clinic of Paediatric Kidney, Liver- and Metabolic Diseases Medical School Hannover, Germany.
authors: U
authors: U
authors: Meyer
authors: St Thomas' Hospital, London, UK.
authors: A
authors: A
authors: Micciche
authors: Children's Hospital Heidelberg, Germany.
authors: E
authors: E
authors: Müller
authors: Galactosemia Support Group, UK.
authors: P
authors: P
authors: Portnoi
authors: Salford Royal, Manchester, UK.
authors: S
authors: S
authors: Ripley
authors: Hôpital Universitaire des Enfants, Reine fabiola, Bruxelles, Belgium.
authors: M
authors: M
authors: Robert
authors: University Hospitals Birmingham NHS Foundation Trust, UK.
authors: L V
authors: LV
authors: Robertson
authors: University Children's Hospital Freiburg, Germany.
authors: S
authors: S
authors: Rosenbaum-Fabian
authors: Children's Hospital Heidelberg, Germany.
authors: K
authors: K
authors: Sahm
authors: Universitätsklinikum Hamburg-Eppendorf, Germany.
authors: S
authors: S
authors: Schultz
authors: University Hospital of Wales, Cardiff, UK.
authors: K
authors: K
authors: Singleton
authors: Children's Hospital, University Hospital Skåne, Sweden.
authors: E
authors: E
authors: Sjöqvist
authors: Oslo University Hospital Rikshospitalet, Norway.
authors: L
authors: L
authors: Stoelen
authors: Alderhey Children's Hospital, Liverpool, UK.
authors: A
authors: A
authors: Terry
authors: Children's Hospital, Westmead, Sydney, Australia.
authors: S
authors: S
authors: Thompson
authors: UMC Utrecht, Netherlands.
authors: C
authors: C
authors: Timmer
authors: University Hospitals Leuven, Center of Metabolic Diseases, Belgium.
authors: K
authors: K
authors: Vande Kerckhove
authors: University Hospital Maastricht, Netherlands.
authors: L
authors: L
authors: van der Ploeg
authors: Universitair Ziekenhuis Gent, Belgium.
authors: M
authors: M
authors: Van Driessche
authors: University of Groningen, University Medical Center Groningen, Netherlands.
authors: M
authors: M
authors: van Rijn
authors: Univ.-Kinderklinik, Pädiatrische Diätetik, Munster, Germany.
authors: A
authors: A
authors: van Teeffelen-Heithoff
authors: Hospital La Fe, Valencia, Spain.
authors: I
authors: I
authors: Vitoria
authors: Hôpital Antoine Béclère, France.
authors: C
authors: C
authors: Voillot
authors: CHU Bicëtre Hospital, Paris, France.
authors: J
authors: J
authors: Wenz
authors: Westmead Hospital, Sydney, Australia.
authors: M
authors: M
authors: Westbrook
authors: Bradford Teaching Hospital NHS Trust, UK.
authors: J
authors: J
authors: Wildgoose
authors: Radboud UMC, Netherlands.
authors: H
authors: H
authors: Zweers
mesh_terms: D000328:Adult; D004032:Diet; D005502:Food; D005638:Fruit; D005690:Galactose / Q000008:administration & dosage*; D005693:Galactosemias / Q000178:diet therapy*; D006801:Humans; D007785:Lactose / Q000008:administration & dosage; D011795:Surveys and Questionnaires; D014675:Vegetables
chemical_list: D007785:Lactose; D005690:Galactose
keywords: Diet; Galactosaemia; Galactose; Galactose-1-phosphate; Lactose; Monitoring