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  1. Frequently Asked Questions about PKU

    ... called Phenylalanine ( Phe ) found in foods containing protein. High Phe may trigger brain-related problems throughout life. In ... symptoms. Treatment usually consists of a diet that limits natural protein intake in order to avoid Phe intake. This diet needs to be ...

  2. Phenylketonuria: blood brain and... Body (van Spronsen)

    ... bones and kidney, as well as the metabolic syndrome. High protein intake is associated with kidney dysfunction and metabolic syndrome at ... acid mixtures), more fat and carbohydrates (due to the low natural protein food regimen) did not result in such problems. Even so, the ...

  3. SSIEM Congres report

    ... with a low Phe SD were able to markedly increase their natural protein intake while still maintaining satisfactory Phe control. These ...

  4. Short Summary of the SSIEM Annual Symposium, 2018

    ... work. The latest reports on glycomacropeptide, slow release protein substitutes and PKU gut microbiota were featured but three studies ... a sapropterin trial, 40 patients were challenged with extra natural protein over a few months prior to sapropterin testing. Patients were ...

  5. 26th ESPKU Conference

    ... Anita MacDonald3 challenged current formulations of Phe-free protein substitutes in the dietary treatment of PKU. In order to reduce blood ... stably, protein substitutes should include a wide range of natural nutrients and novel protein sources. Prof MacDonald suggested: ...

  6. 2014 Annual Multidisciplinary European Phenylketonuria Symposium: PKU a lifetime of challenges

    ... intake of 114 mg/kg/day which equates to over 2g/kg/day of natural protein.  They concluded that sapropterin was safe and efficacious. The USA ...

  7. SSIEM Annual Symposium 2012

    ... on the use of glycomacropeptide (GMP) as a supplement in protein-restricted diets were encouraging. GMP, a natural whey protein extracted from milk and a by-product of cheese-making, has ...

  8. 3rd Dietitians’ Workshop

    ... levels of supplements may not appear high compared to natural foods, but in what form are these carbohydrates? How much is sugar? ... maintaining good Phe control and the influence of energy on protein utilization and nitrogen balance. Such foods promote anabolism and ...

  9. Perspective interview - François Feillet

    ... Where once patients had a choice of just a couple of low protein foods, today there are many more supplements available with different ... is quite different. ‘You are not eating the same natural proteins in France, in Japan, in the USA or in the UK, or wherever. We ...

  10. Perspective interview - Nicole Pallone

    ... The Pallones have had to take care to introduce natural protein without normalising Rosie’s diet. They felt this was essential in ...