Akademik Veri Yönetim Sistemi
MOLECULAR GENETICS AND METABOLISM REPORTS, cilt.2, ss.85-88, 2015 (ESCI)
Lifelong low-phenylalanine (Phe) dietarymanagement is the foundation of care in phenylketonuria (PKU). However, strict monitoring of food intake places a burden on patients and their caregivers, and adherence to the required diet frequently decreases in later childhood and adolescence. Rarely, parents of children with PKU refuse to recognise the importance of treatment and follow-up for this chronic condition. Here, two case studies are presented that document consideration of placement of children into foster care or kinship homes as a last resort to improve persistently high Phe concentrations. In the first case, social service referral led to a 3-year-old girl being placed in a kinship homewith her grandparents, resulting in excellent Phe control thereafter. In the second case, discussion with the parents of possible placement of a 12-year-old child into foster care was sufficient to have a positive effect on Phe control. A staged approach for managing intractable non-adherence in PKU is proposed. (C) 2015 The Authors. Published by Elsevier Inc.