PSYCHOSOCIAL-ASPECTS IN PHENYLKETONURIA

Psychosocial aspects in phenylketonuric (PKU) patients are reported. I n two separate studies patients with PKU differing in age (children ve rsus adolescents), were assessed. The main message of the first prospe ctive study on 58 10-year-old patients is that normally intelligent PK U patients who were treated early and strictly did not show a higher r isk for severe emotional and behavioural maladjustment compared with h ealthy controls at the age of 10 years. The data were obtained in the course of the German PKU Collaborative Study by the ''Personality Ques tionnaire for Children (PFK 9-14)''. All patients received nutritional , medical, and psychological counselling every 6 months. In the second retrospective study, 34 early treated, normally intelligent adolescen ts with PKU (age: mean = 14.6, SD = 2.0, range 11-18 years) and their mothers were assessed with several psychometric personality inventorie s and self-developed questionnaires concerning their psychosocial situ ation and their disease- and diet-specific knowledge. Using the Mannhe imer Biographic Inventory (MBI), the Personality Questionnaire for Chi ldren (PFK 9-14), and the Freiburger Personality Inventory (FPI) the a dolescent patients described their social life and their emotional dev elopment as being distinctly restricted. Their knowledge concerning di sease and diet was alarmingly poor and the majority had great difficul ties in satisfactory dietetic management without parental help. In add ition to the burdensome diet, developmental crises like puberty may ca use more frequently emotional and behavioural problems in PKU patients .