The truth of treating patients with phenylketonuria after childhood: The need for a new guideline
| Autor: | van FrancJan Spronsen, Peter Burgard |
|---|---|
| Rok vydání: | 2008 |
| Předmět: |
Adult
PLASMA PHENYLALANINE medicine.medical_specialty Pediatrics Nutritional Sciences Phenylalanine Concordance MEDLINE Guidelines as Topic METABOLIC-CONTROL Models Biological MATERNAL PHENYLKETONURIA Quality of life (healthcare) Bone Density QUALITY-OF-LIFE Phenylketonurias YOUNG-ADULTS NEUROLOGICAL DETERIORATION Genetics medicine Humans AMINO-ACIDS ADULT PATIENTS Young adult Child Intensive care medicine Genetics (clinical) Adult patients business.industry Dietary management Guideline Magnetic Resonance Imaging Blood-Brain Barrier SUSTAINED ATTENTION DIETARY-MANAGEMENT business Psychosocial Diet Therapy |
| Zdroj: | Journal of Inherited Metabolic Disease. 31:673-679 |
| ISSN: | 1573-2665 0141-8955 |
| Popis: | In recent years, an increasing number of national guidelines on the treatment of phenylketonuria (PKU) have emerged. Most of these guidelines are dedicated to the care of children, while less attention is paid to the care of adults, although all guidelines underline the importance of diet for life. This review aims to summarize issues that need to be addressed within a guideline on the treatment of PKU, especially when care for patients beyond childhood is concerned. In this respect, it is of importance that adult patients, both willing and unwilling to be treated, need a guideline for care and follow-up. In PKU there is certainly a need for an improved unified guideline, especially after childhood, although many of the considerations in this article also apply to recommendations for treatment of children. Such a guideline will be a tool to improve treatment in PKU patients but should also include recommendations for collecting data for clinical and research purposes. Guideline development should also focus on nutritional, neuropsychological and psychosocial issues and not only on target plasma phenylalanine concentrations. In addition, guidelines must address not only what has to be done but also how it can be done, thereby improving concordance with the recommendations for treatment and management. |
| Databáze: | OpenAIRE |
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