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  1. Frequently Asked Questions about PKU

    ... a mother affect her fetus during pregnancy? Can high phenylalanine enter the brain in PKU patients? What is the importance of ... day to day practice in PKU? What is the phenylalanine tolerance in PKU in patients on dietary treatment only? What are the ...

  2. SSIEM Congres report

    ... PKU Measurement of the standard deviation (SD) of blood phenylalanine ( Phe ) levels in patients with PKU may be a useful tool for assessing the Phe tolerance of patients, according to Ms Fiona White of the Willink Biochemical ...

  3. Oral communications

    ... Diet was liberalized in 25/30 (83.3%) of patients. Phe tolerance increased 2.26-fold (0.88-4.23) from a median of 47.5 mg/kg/day to a ... (İzmir, Turkey.) BH4 administration in HPA increased phenylalanine tolerance in BH4 responsive patients.   1 - Ms Sarah ...

  4. SSIEM 2014 and PKU: A dietician's perspective

    ... PKU who were working full-time that they had higher blood phenylalanine concentrations than those in part-time employment suggesting that ... responsive patients, its use increased phenylalanine tolerance by 30.5 mg/kg/day when compared with a low phenylalanine diet only ...

  5. Short Summary of the SSIEM Annual Symposium, 2018

    ... L-tryptophan and L-threonine) with or without partial phenylalanine ( Phe ) restrictions were compared with 1) Phe restrictions ... team from Portugal presented their examination of Phe tolerance in patients aged ≥12 years with PKU. In preparation for a ...

  6. 26th ESPKU Conference

    ... that Phase III clinical studies using PEGylated recombinant phenylalanine ammonia lyase (PEG-rPAL) would be going ahead in the USA in 2013. ... true long-term BH4-responsiveness by different methods, Phe tolerance has to be assessed and the amount of natural proteins balanced with ...

  7. Perspective interview - Nicola Longo (PEG-PAL)

    ... Work to evaluate clinically the use of pegylated recombinant phenylalanine ammonia lyase ( PEG-PAL ) as a potential therapy for PKU is ... We know from other cases that the human body can develop tolerance to the administration of foreign proteins over time but we don’t ...

  8. 2013 International Congress of Inborn Errors of Metabolism

    ... to sapropterin, and lead to a 30-80% reduction of blood phenylalanine ( Phe ) levels. To further support this approach, genotyping is ... discussing the correct modalities to perform the BH4 tolerance test and responsiveness, in order to get information on the proper ...

  9. XXV ESPKU Conference

    ... therapy by using recombinant pegylated Anabaena variabilis phenylalanine ammonia lyase (rAvPAL-PEG), a bacterial-derived Phe-ammonia ... for better efficacy; they are also verifying patients’ tolerance to the drug. Preliminary but incomplete data seem encouraging, ...