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Denmark LNAA STUDY 1 tablet/kg/day PowerPoint Presentation
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UkraineProf. Elena Grechanina, Kharkov Genetic & Neonatal Screening Centre RussiaProf. Peter Novikov, Moscow Central Genetic Center DenmarkFlemming Güttler, M.D., PhD., Glostrup, J. F. Kennedy Genetic InstituteUSAReuben Matalon, M.D., Ph.D., Kim Matalon, the University of Texas Medical Branch

neophe 0 5 g kg in pku subjects
NeoPhe 0.5 g/kg in PKU Subjects
  • 13 subjects
  • Mean age 26.6 years
  • 7 males, 6 females
  • Mean decrease in blood Phe after one week 243 µmol/L
  • Average decrease in blood Phe 22 %.
neophe 1 0 g kg in pku subjects
NeoPhe 1.0 g/kg in PKU Subjects
  • 7 subjects
  • Mean age 25.2 years
  • 5 males, 2 females
  • Mean decrease in blood Phe after one week 377 µmol/L
  • Average decrease in blood Phe 25 %.
conclusions
CONCLUSIONS:
  • For the first time mixture of LNAA can lower blood phenylalanine
  • Using NeoPhe avoids lysine deficiency and negative nitrogen balance
  • NeoPhe offers new options to treat PKU
  • Diet can be more liberal with NeoPhe
  • Still blood phe- levels need to be controlled