INTRODUCTION

More usually called PCU (ph�nylc�tonurie) or    PKU (PhenylKetonUria),

It is a disease known as " orphan " bus not very current in Belgium, it touches only one new-born baby out of 15.000

pinocchio.jpg (6724 octets)

Is Ph�nylc�tonurie, what it?

                                                                     The ph�nylc�tonurie is a hereditary genetic metabolic disease. Discovered at the beginning of the years 1930, it is due to an enzymatic disorder preventing the degradation of an amino acid, the phenylalanine, which is in all proteins. This phenylalanine overload has a harmful action on the level of the brain which results in a severe mental deficit, behavioral problems (in particular of aggressiveness), by disorders of driving coordination and, sometimes of the epilepsy. A test carried out since 1968 as of the birth ( the TEST OF GUTHRIE ) makes it possible today to detect this disease systematically by controlling the phenylalanine rate in the blood of the new-born baby.  

The detected children will have, in order to have a normal life, to follow a Draconian mode, low in proteins in particular by excluding milk, the meat, the eggs, the fish... In practice, only the fruits and the vegetables are allowed, to which are added products of synthesis to maintain a growth and an optimal mental development. Each meal must be prepared by taking account of the age and the results of blood controls. No variation is authorized and one can imagine without sorrow the difficulties which encounter the phenylcetonuric young people in their everyday life.

<< synopsis >>                                                                           A-arrow03c.gif (3144 octets)       A-arrow03a.gif (3144 octets)

-102-

Hosted by www.Geocities.ws

1