On Saturday April 27 Dr. Aldamiz we gave a talk on “Self Management of adult fenilcetonúrico” in the room Ganekogorta Cruces University Hospital.
The talk began about 11 h 45 min and 30 families attended the various pathologies. The talk was nice, pleasant and familiar.
We made a small introduction telling a little history of phenylketonuria.
Phenylketonuria or PKU its acronym in English, was identified in 1934 by Folling, which saw high levels of Phe cause of mental retardation. In 1949 Bickel began to treat these patients successfully with a low protein diet.
In 1963 Guthrie measure development technique impregnated cardboard Phe levels.
The consequences of increased levels cause white matter damage, oxidative damage, impaired brain LNAA incorporation and other unknown effects,
the prevalence is 1:20,000.
The diet is low in protein-free amino acids Phe, Tyr supplemented with vitamins, trace elements, essential fatty acids and low protein foods. Products are being seen as neutral amino acids that compete with Phe.
He spoke of BH4 to which all equal unresponsive. He also spoke of GMP which is extracted from whey, purified and treated and is being used in Chile and the U.S. It is used in pastries and baking and has a very small component of phenylalanine.
Among the forms of PKU, in our community, there is a higher percentage of moderate compared to the classical and mild. However, in Galicia is a greater incidence of severe.
In some countries this working self-dubbed as “MY PKU” www.ojrd.com/content/6/1/48.
? disease information
? Proficiency in blood collection
? Logistics Information
? Assistance cooking workshop
? rations exchanges Rating
? Availability of dietitians
? Do I use programs?
Then performed a series of questions from families.
The talk was very positive, not only for PKU families but to all who attended that opened many doors for us to work with adults regardless of their pathology.