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Table 3 Therapy management and satisfaction (single centre survey; n = 30; 17 females/13 males). A Clinic visits, laboratory controls and dietary treatment. B Transition satisfaction and coping with treatment

From: Ten years of specialized adult care for phenylketonuria – a single-centre experience

A: Clinic visits, laboratory controls and dietary treatment
Frequency of laboratory controls/dried blood samples n %
    Monthly 13 43.3
    Every two month 5 16.7
    Quarterly 5 16.7
    Biannually 3 10.0
    Annually 2 6.7
    No answer 2 6.7
Frequency of clinic visits   
    Quarterly 11 36.7
    Three times a year 2 6.7
    Biannually 12 40.0
    Annually 4 13.3
    No visits 1 3.3
Dietary treatment   
Phe/protein restricted diet 22 73.3
      Exact calculating Phe content 4 12.3
      Estimation of Phe content 16 53.3
      No strict diet regime/vegetarian diet 2 6.6
Amino acid mixture (AAM) 24 80.0
    BH4 0 0
Discontinuation of the PKU treatment   
    Never 14 46.7
    Once 7 23.3
    Several times 5 16.7
    No answer 4 13.3
Reasons for discontinuation of the PKU treatment (n = 12)   
    Stop of dietary treatment in adolescence     according to former procedures 3 25.0
    Weariness to follow the diet/Wish for     independence from treatment 5 41.7
    Good metabolic control following a     vegetarian diet 3 25.0
    No answer 1 8.3
Restart of the PKU treatment 10 83.3
Reasons for restart of the PKU treatment (n = 10)   
    Doctors recommendation 2 20.0
    More capability/long-term prevention 2 20.0
    Wish to become pregnant/pregnancy 4 40.0
    No answer 2 20.0
B: Transition satisfaction and coping with treatment.
Satisfaction with the actual individual metabolic control n %
    Very satisfied 10 33.3
    Satisfied 14 46.7
    Less satisfied 5 16.7
    Not satisfied 0 0
    No answer 1 3.3
Personal importance of good metabolic control   
    Very important 12 40.0
    Important 14 46.7
    Less important 3 10.0
    Not important 0 0
    No answer 1 3.3
Current coping with dietary therapy   
    Very well 10 33.3
    Well 13 43.3
    Moderately 2 6.7
    Badly 1 3.3
    No answer 4 12.3
Coping with dietary treatment in adulthood in comparison to childhood   
    Easier 13 43.3
    Indifferent 8 26.7
    More difficult 5 16.7
    No answer 4 12.3
Satisfaction with transition process and adult care   
    Very satisfied 10 33.3
    Satisfied 15 50.0
    Less satisfied 0 0
    Not satisfied 3 10.0
    No answer 2 6.7