Journal article
Dietary practices in propionic acidemia: A European survey.
- Daly A Birmingham Women's and Children's Hospital, Birmingham, UK.
- Pinto A Birmingham Women's and Children's Hospital, Birmingham, UK.
- Evans S Birmingham Women's and Children's Hospital, Birmingham, UK.
- Almeida MF Centro de Genética Médica, Centro Hospitalar do Porto - CHP, Porto, Portugal.
- Assoun M Centre de référence des maladies héréditaires du métabolisme, Hôpital Necker Enfants Malades, Paris, France.
- Belanger-Quintana A Unidad de Enfermedades Metabolicas, Servicio de Pediatria, Hospital Ramon y Cajal Madrid, Spain.
- Bernabei SM Children Hospital Bambino Gesù, Division of Artificial Nutrition, Rome, Italy.
- Bollhalder S Univerisity Hospital Zurich, Switzerland.
- Cassiman D Metabolic Center, University Hospitals Leuven and KU Leuven, Belgium.
- Champion H Addenbrooke's Hospital, Cambridge, UK.
- Chan H Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
- Dalmau J Unit of Nutrition and Metabolopathies, Hospital La Fe, Valencia, Spain.
- de Boer F University of Groningen, University Medical Center Groningen, Netherlands.
- de Laet C Hôpital Universitaire des Enfants, Reine Fabiola, Bruxelles, Belgium.
- de Meyer A Center of Metabolic Diseases, University Hospital, Antwerp, Belgium.
- Desloovere A University Hospital Ghent, Belgium.
- Dianin A Department of Pediatrics, Regional Centre for Newborn Screening, Diagnosis and Treatment of Inherited Metabolic Diseases and Congenital Endocrine Diseases, University Hospital of Verona, Italy.
- Dixon M Great Ormond Street Hospital for Children NHS FoundationTrust, London, UK.
- Dokoupil K Dr. von Hauner Children's Hospital, Munich, Germany.
- Dubois S Centre de référence des maladies héréditaires du métabolisme, Hôpital Necker Enfants Malades, Paris, France.
- Eyskens F Center of Metabolic Diseases, University Hospital, Antwerp, Belgium.
- Faria A Hospital Pediátrico, Centro Hospitalar e Universitário de Coimbra, EPE, Portugal.
- Fasan I Division of Inherited Metabolic Diseases, Department of Pediatrics, University Hospital of Padova, Italy.
- Favre E Reference center for Inborn Errors of Metabolism, Department of Pediatrics, Children's University Hospital, Nancy, France.
- Feillet F Reference center for Inborn Errors of Metabolism, Department of Pediatrics, Children's University Hospital, Nancy, France.
- Fekete A Metabolic Centre of Vienna, Austria.
- Gallo G Children Hospital Bambino Gesù, Division of Artificial Nutrition, Rome, Italy.
- Gingell C Nottingham University Hospitals, UK.
- Gribben J Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
- Kaalund Hansen K Charles Dent Metabolic Unit National Hospital for Neurology and Surgery, London, UK.
- Ter Horst NM Emma Children's Hospital, AMC, Amsterdam, Netherlands.
- Jankowski C Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, UK.
- Janssen-Regelink R Radboud University Medical Center Nijmegen, Netherlands.
- Jones I Center of Metabolic Diseases, University Hospital, Antwerp, Belgium.
- Jouault C CHU Angers, France.
- Kahrs GE Haukeland University Hospital, Bergen, Norway.
- Kok IL Wilhelmina Children's Hospital, University Medical Centre Utrecht, Netherlands.
- Kowalik A Institute of Mother & Child, Warsaw, Poland.
- Laguerre C Centre de Compétence de L'Hôpital des Enfants de Toulouse, France.
- Le Verge S Centre de référence des maladies héréditaires du métabolisme, Hôpital Necker Enfants Malades, Paris, France.
- Lilje R Oslo University Hospital, Norway.
- Maddalon C University Children's Hospital Zurich, Switzerland.
- Mayr D Ernährungsmedizinische Beratung, Universitätsklinik für Kinder- und Jugendheilkunde, Salzburg, Austria.
- Meyer U Clinic of Paediatric Kidney, Liver- and Metabolic Diseases, Medical School Hannover, Germany.
- Micciche A Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
- Och U University Children's Hospital, Munster, Germany.
- Robert M Hôpital Universitaire des Enfants, Reine Fabiola, Bruxelles, Belgium.
- Rocha JC Centro de Genética Médica, Centro Hospitalar do Porto - CHP, Porto, Portugal.
- Rogozinski H Bradford Teaching Hospital NHS Foundation Trust, UK.
- Rohde C Hospital of Children's & Adolescents, University of Leipzig, Germany.
- Ross K Royal Aberdeen Children's Hospital, Scotland.
- Saruggia I Centre de Reference des Maladies Héréditaires du Métabolisme du Pr. B. Chabrol CHU Timone Enfant, Marseille, France.
- Schlune A Department of General Pediatrics, Neonatology and Pediatric Cardiology, University Children's Hospital, Heinrich Heine University, Moorenstr. 5, 40225 Düsseldorf, Germany.
- Singleton K University Hospital of Wales, Cardiff, UK.
- Sjoqvist E Children's Hospital, University Hospital, Lund, Sweden.
- Skeath R Great Ormond Street Hospital for Children NHS FoundationTrust, London, UK.
- Stolen LH Oslo University Hospital, Norway.
- Terry A Alder Hey Children's Hospital NHS Foundation Trust Liverpool, UK.
- Timmer C Academisch Medisch Centrum, Amsterdam, Netherlands.
- Tomlinson L University Hospitals Birmingham NHS Foundation Trust, UK.
- Tooke A Nottingham University Hospitals, UK.
- Vande Kerckhove K Metabolic Center, University Hospitals Leuven and KU Leuven, Belgium.
- van Dam E University of Groningen, University Medical Center Groningen, Netherlands.
- van den Hurk T Wilhelmina Children's Hospital, University Medical Centre Utrecht, Netherlands.
- van der Ploeg L Maastricht University Medical Centre + (MUMC +), Netherlands.
- van Driessche M University Hospital Ghent, Belgium.
- van Rijn M University of Groningen, University Medical Center Groningen, Netherlands.
- van Wegberg A Radboud University Medical Center Nijmegen, Netherlands.
- Vasconcelos C Centro Hospitalar São João - Unidade de Doenças Metabólicas, Porto, Portugal.
- Vestergaard H National University Hospital, Copenhagen, Denmark.
- Vitoria I Unit of Nutrition and Metabolopathies, Hospital La Fe, Valencia, Spain.
- Webster D Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, UK.
- White FJ Central Manchester University Hospitals NHS Foundation Trust, Manchester, UK.
- White L Sheffield Children's Hospital, UK.
- Zweers H Radboud University Medical Center Nijmegen, Netherlands.
- MacDonald A Birmingham Women's and Children's Hospital, Birmingham, UK.
- 2017-10-13
Published in:
- Molecular genetics and metabolism reports. - 2017
English
BACKGROUND
The definitive dietary management of propionic acidaemia (PA) is unknown although natural protein restriction with adequate energy provision is of key importance.
AIM
To describe European dietary practices in the management of patients with PA prior to the publication of the European PA guidelines.
METHODS
This was a cross-sectional survey consisting of 27 questions about the dietary practices in PA patients circulated to European IMD dietitians and health professionals in 2014.
RESULTS
Information on protein restricted diets of 186 PA patients from 47 centres, representing 14 European countries was collected. Total protein intake [PA precursor-free L-amino acid supplements (PFAA) and natural protein] met WHO/FAO/UNU (2007) safe protein requirements for age in 36 centres (77%). PFAA were used to supplement natural protein intake in 81% (n = 38) of centres, providing a median of 44% (14-83%) of total protein requirement. Seventy-four per cent of patients were prescribed natural protein intakes below WHO/FAO/UNU (2007) safe levels in one or more of the following age groups: 0-6 m, 7-12 m, 1-10 y, 11-16 y and > 16 y. Sixty-three per cent (n = 117) of patients were tube fed (74% gastrostomy), but only 22% received nocturnal feeds.
CONCLUSIONS
There was high use of PFAA with intakes of natural protein commonly below WHO/FAO/UNU (2007) safe levels. Optimal dietary management can only be determined by longitudinal, multi-centre, prospective case controlled studies. The metabolic instability of PA and small patient cohorts in each centre ensure that this is a challenging undertaking.
The definitive dietary management of propionic acidaemia (PA) is unknown although natural protein restriction with adequate energy provision is of key importance.
AIM
To describe European dietary practices in the management of patients with PA prior to the publication of the European PA guidelines.
METHODS
This was a cross-sectional survey consisting of 27 questions about the dietary practices in PA patients circulated to European IMD dietitians and health professionals in 2014.
RESULTS
Information on protein restricted diets of 186 PA patients from 47 centres, representing 14 European countries was collected. Total protein intake [PA precursor-free L-amino acid supplements (PFAA) and natural protein] met WHO/FAO/UNU (2007) safe protein requirements for age in 36 centres (77%). PFAA were used to supplement natural protein intake in 81% (n = 38) of centres, providing a median of 44% (14-83%) of total protein requirement. Seventy-four per cent of patients were prescribed natural protein intakes below WHO/FAO/UNU (2007) safe levels in one or more of the following age groups: 0-6 m, 7-12 m, 1-10 y, 11-16 y and > 16 y. Sixty-three per cent (n = 117) of patients were tube fed (74% gastrostomy), but only 22% received nocturnal feeds.
CONCLUSIONS
There was high use of PFAA with intakes of natural protein commonly below WHO/FAO/UNU (2007) safe levels. Optimal dietary management can only be determined by longitudinal, multi-centre, prospective case controlled studies. The metabolic instability of PA and small patient cohorts in each centre ensure that this is a challenging undertaking.
- Language
-
- English
- Open access status
- gold
- Identifiers
-
- DOI 10.1016/j.ymgmr.2017.09.002
- PMID 29021961
- Persistent URL
- https://sonar.ch/global/documents/33018
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