دورية أكاديمية
Normalizing Diet in Individuals with Phenylketonuria Treated with Pegvaliase: A Case Series and Patient Perspective
| العنوان: | Normalizing Diet in Individuals with Phenylketonuria Treated with Pegvaliase: A Case Series and Patient Perspective |
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| المؤلفون: | Bernstein L, Hansen J, Kogelmann C, Ellerbrok M, Gizewska M, Gaughan S, Rocha JC, Belanger A, Rohr F |
| المصدر: | Nutrition and Dietary Supplements, Vol Volume 13, Pp 145-154 (2021) |
| بيانات النشر: | Dove Medical Press, 2021. |
| سنة النشر: | 2021 |
| المجموعة: | LCC:Nutrition. Foods and food supply LCC:Nutritional diseases. Deficiency diseases |
| مصطلحات موضوعية: | pku, phenylketonuria, pegvaliase, medical nutrition therapy, Nutrition. Foods and food supply, TX341-641, Nutritional diseases. Deficiency diseases, RC620-627 |
| الوصف: | Laurie Bernstein,1,2 Joyanna Hansen,3 Christian Kogelmann,4 Margret Ellerbrok,4 Maria Gizewska,5 Sommer Gaughan,6 Julio Cesar Rocha,7– 9 Amaya Belanger,10 Fran Rohr2 1Section of Clinical Genetics and Metabolism, Department of Pediatrics, University of Colorado, Aurora, CO, USA; 2Met Ed Co, Boulder, CO, USA; 3Department of Molecular and Medical Genetics, Oregon Health & Science University, Portland, OR, USA; 4University Medical Center Hamburg-Eppendorf, Hamburg, Germany; 5Department of Pediatrics, Endocrinology, Diabetology, Metabolic Diseases and Cardiology of the Developmental Age, Pomeranian Medical University, Szczecin, Poland; 6Inherited Metabolic Diseases Clinic, Children’s Hospital Colorado, Aurora, CO, USA; 7Nutrition and Metabolism, NOVA Medical School | Faculdade de Ciências Médicas, Universidade NOVA de Lisboa, Lisboa, 1169-056, Portugal; 8CINTESIS - Center for Health Technology and Services Research, Porto, Portugal; 9Reference Centre of Inherited Metabolic Diseases, Centro Hospitalar Universitário de Lisboa Central, Lisboa, 1169-045, Portugal; 10Hospital Universitario Ramon y Cajal, Madrid, SpainCorrespondence: Fran Rohr Email fran.rohr@met-ed.netAbstract: Phenylketonuria (PKU) is one of the most common inherited metabolic disorders, and historically has required affected individuals to follow a severely protein-restricted diet with medical food for life. A novel enzyme substitution therapy, pegvaliase, allows many adults with PKU on therapy to have a reduction in blood phenylalanine (Phe) while on an unrestricted diet. However, there is limited information on optimal nutrition management of individuals with PKU on pegvaliase therapy. This manuscript summarizes a virtual European meeting focused on nutrition management of individuals on pegvaliase therapy, including three case studies and a patient perspective that together provide clinical insights into the real-world management of individuals with PKU on pegvaliase.Keywords: PKU, phenylketonuria, pegvaliase, medical nutrition therapy |
| نوع الوثيقة: | article |
| وصف الملف: | electronic resource |
| اللغة: | English |
| تدمد: | 1179-1489 |
| العلاقة: | https://www.dovepress.com/normalizing-diet-in-individuals-with-phenylketonuria-treated-with-pegv-peer-reviewed-fulltext-article-NDSTest; https://doaj.org/toc/1179-1489Test |
| الوصول الحر: | https://doaj.org/article/4f3c913f902d439ebbe6af5a03bcabeeTest |
| رقم الانضمام: | edsdoj.4f3c913f902d439ebbe6af5a03bcabee |
| قاعدة البيانات: | Directory of Open Access Journals |
Full Text Finder | تدمد: | 11791489 |
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