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Hepatorenal Tyrosinaemia: Impact of a Simplified Diet on Metabolic Control and Clinical Outcome
Friederike Bärhold
Uta Meyer
Anne-Kathrin Neugebauer
Eva Thimm
Dinah Lier
Stefanie Rosenbaum-Fabian
Ulrike Och
Anna Fekete
Dorothea Möslinger
Carmen Rohde
Skadi Beblo
Michel Hochuli
Nina Bogovic
Vanessa Korpel
Stephan Vom Dahl
Sebene Mayorandan
Aleksandra Fischer
Peter Freisinger
Katharina Dokoupil
Margret Heddrich-Ellerbrok
Monika Jörg-Streller
Agnes van Teeffelen-Heithoff
Janina Lahl
Anibh Martin Das
出版
Universität
, 2021
URL
http://books.google.com.hk/books?id=P5BwzgEACAAJ&hl=&source=gbs_api
註釋
Abstract: Background: Tyrosinaemia type 1 is a rare inherited metabolic disease caused by an enzyme defect in the tyrosine degradation pathway. It is treated using nitisinone and a low-protein diet. In a workshop in 2013, a group of nutritional specialists from Germany, Switzerland and Austria agreed to advocate a simplified low-protein diet and to allow more natural protein intake in patients with tyrosinaemia type 1. This retrospective study evaluates the recommendations made at different treatment centers and their impact on clinical symptoms and metabolic control. Methods: For this multicenter study, questionnaires were sent to nine participating treatment centers to collect data on the general therapeutic approach and data of 47 individual patients treated by those centers. Results: Dietary simplification allocating food to 3 categories led to increased tyrosine and phenylalanine blood concentrations without weighing food. Phenylalanine levels were significantly higher in comparison to a strict dietary regimen whereas tyrosine levels in plasma did not change. Non-inferiority was shown for the simplification and liberalization of the diet. Compliance with dietary recommendations was higher using the simplified diet in comparison to the stricter approach. Age correlates negatively with compliance. Conclusions: Simplification of the diet with increased natural protein intake based on three categories of food may be implemented in the diet of patients with tyrosinaemia type 1 without significantly altering metabolic control. Patient compliance is strongly influencing tyrosine blood concentrations. A subsequent prospective study with a larger sample size is necessary to get a better insight into the effect of dietary recommendations on metabolic control