Academic Journal
Development of a practical dietitian road map for the nutritional management of phenylketonuria (PKU) patients on pegvaliase
| العنوان: | Development of a practical dietitian road map for the nutritional management of phenylketonuria (PKU) patients on pegvaliase |
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| المؤلفون: | Júlio César Rocha, Heather Bausell, Amaya Bélanger-Quintana, Laurie Bernstein, Hülya Gökmen-Özel, Alexandra Jung, Anita MacDonald, Fran Rohr, Esther van Dam, Margret Heddrich-Ellerbrok |
| المصدر: | Molecular Genetics and Metabolism Reports, Vol 28, Iss , Pp 100771- (2021) |
| بيانات النشر: | Elsevier, 2021. |
| سنة النشر: | 2021 |
| المجموعة: | LCC:Medicine (General) LCC:Biology (General) |
| مصطلحات موضوعية: | Phenylketonuria, PKU, Phenylalanine, Pegvaliase, Nutrition, Dietitian, Medicine (General), R5-920, Biology (General), QH301-705.5 |
| الوصف: | Background: The metabolic dietitian/nutritionist (hereafter ‘dietitian’) plays an essential role in the nutritional management of patients with phenylketonuria (PKU), including those on pegvaliase. Currently, more educational support and clinical experience is needed to ensure that dietitians are prepared to provide optimal nutritional management and counselling of pegvaliase-treated patients. Methods: Via a face-to-face data-review meeting, followed by a virtual consolidation meeting, a group of expert dietitians and one paediatrician discussed and developed a series of recommendations on the nutritional evaluation and management of patients receiving pegvaliase. The consensus group consisted of 10 PKU experts: six dietitians and one paediatrician from Europe and three dietitians from the US. One European and three US dietitians had experience with pegvaliase-treated patients. Results: The consensus group recommended that a physician, dietitian and nurse are part of the pegvaliase treatment team. Additionally, a psychologist/counsellor should be included if available. Practical proposals for the nutritional evaluation of pegvaliase-treated patients at baseline, during the induction and titration phases and for long-term maintenance were developed. The consensus group suggested assessment of blood Phe at least monthly or every 2 weeks in the event of low blood Phe (i.e., blood Phe |
| نوع الوثيقة: | article |
| وصف الملف: | electronic resource |
| اللغة: | English |
| تدمد: | 2214-4269 |
| Relation: | http://www.sciencedirect.com/science/article/pii/S2214426921000653; https://doaj.org/toc/2214-4269 |
| DOI: | 10.1016/j.ymgmr.2021.100771 |
| URL الوصول: | https://doaj.org/article/7da1310c6578481e9e90c62904657daa |
| رقم الانضمام: | edsdoj.7da1310c6578481e9e90c62904657daa |
| قاعدة البيانات: | Directory of Open Access Journals |
Full Text Finder | تدمد: | 22144269 |
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| DOI: | 10.1016/j.ymgmr.2021.100771 |