{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T19:31:19Z","timestamp":1791487879774,"version":"4.3.4"},"reference-count":55,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T00:00:00Z","timestamp":1769990400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100017490","name":"Arla Foods Ingredients Group","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100017490","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>Background\/Objectives: Casein glycomacropeptide (cGMP) has been modified to enable its suitability as a low phenylalanine (Phe) protein substitute (PS) in phenylketonuria (PKU). No data is available about its global usage. Methods: A 60-item multiple choice and short answer\/extended response questionnaire examining the use of modified cGMP in PKU was distributed globally to dietitians and physicians via web-based professional inherited metabolic disorder groups. Results: Respondents (n = 208) from 45 countries across 6 continents completed the questionnaire. Of these, 83.7% (n = 174) were dietitians\/nutritionists, 14.9% (n = 31) medical doctors\/physicians and 1.4% (n = 3) other health professionals, caring for both paediatric and adult patients (59.1%), paediatrics only (25.0%) or adults only (15.9%). cGMP PS were reported as not available in their centre\/hospital by 19.7% (n = 41), mostly in Africa, South America, and southern and western Asia. The main reasons included lack of regulatory approval (65.8%), not promoted by manufacturers (41.5%), and cost (29.3%). An estimated 25% of represented patients globally were using cGMP PS; 78.4% (n = 163) following refusal\/poor adherence with Phe-free amino acids and 54.8% (n = 114) for adult patients recommencing dietary treatment. There were concerns about the residual Phe in cGMP negatively impacting blood Phe levels in children &lt;12y (66.3%), adolescents (48.0%), adults (34.6%), and the first trimester of pregnancy (53.1%). Sixty nine percent (n = 145) adjusted dietary Phe prescription according to the cGMP Phe content, particularly in regions with a higher percentage of severe PKU variants. Commonly perceived clinical advantages with cGMP were improved taste\/palatability (93.2%, n = 194) and fewer gastrointestinal symptoms (55.8%, n = 116). Perceived clinical disadvantages were residual Phe (72.1%, n = 150), lack of data in children &lt; 3 years (48.1%, n = 100), and the high energy content of some brands (45.2%, n = 94). There were concerns that cGMP PS were too high in sugar (34.1%, n = 71) and dissatisfaction or uncertainty about the adequacy of its Phe (66.3%) and amino acid (34.1%) content. Conclusions: There is global inconsistency in access to cGMP PS suitable for PKU, and in the interpretation of evidence-based research. Some professionals have significant concerns about its nutritional composition particularly residual Phe, limiting its estimated use to approximately 25% of PKU patients globally.<\/jats:p>","DOI":"10.3390\/nu18030488","type":"journal-article","created":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T15:38:12Z","timestamp":1770046692000},"page":"488","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Global Use of Casein Glycomacropeptide Protein Substitutes for Phenylketonuria (PKU): Health Professional Perspectives"],"prefix":"10.3390","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7654-3621","authenticated-orcid":false,"given":"Sharon","family":"Evans","sequence":"first","affiliation":[{"name":"Birmingham Children\u2019s Hospital, Steelhouse Lane, Birmingham B4 6NH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2096-2592","authenticated-orcid":false,"given":"Rani","family":"Singh","sequence":"additional","affiliation":[{"name":"Department of Human Genetics, Emory University School of Medicine, Atlanta, GA 30322, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kirsten","family":"Ahring","sequence":"additional","affiliation":[{"name":"The PKU Clinic, Kennedy Centre, Centre for Paediatric and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, 2100 Copenhagen, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Ashmore","sequence":"additional","affiliation":[{"name":"Birmingham Children\u2019s Hospital, Steelhouse Lane, Birmingham B4 6NH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2579-8699","authenticated-orcid":false,"given":"Anne","family":"Daly","sequence":"additional","affiliation":[{"name":"Birmingham Children\u2019s Hospital, Steelhouse Lane, Birmingham B4 6NH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1041-2116","authenticated-orcid":false,"given":"Suzanne","family":"Ford","sequence":"additional","affiliation":[{"name":"National Society for Phenylketonuria (NSPKU), Sheffield S12 9ET, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Ines","family":"Gama","sequence":"additional","affiliation":[{"name":"Birmingham Children\u2019s Hospital, Steelhouse Lane, Birmingham B4 6NH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8433-0864","authenticated-orcid":false,"given":"Maria","family":"Gi\u017cewska","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Rare Diseases and Metabolic Medicine, Pomeranian Medical University in Szczecin, 71-252 Szczecin, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melanie","family":"Hill","sequence":"additional","affiliation":[{"name":"Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S5 7AU, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6518-0255","authenticated-orcid":false,"given":"Fatma","family":"Ilgaz","sequence":"additional","affiliation":[{"name":"Department of Nutrition and Dietetics, Faculty of Health Sciences, Hacettepe University, Ankara 06100, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Jackson","sequence":"additional","affiliation":[{"name":"Department of Health Data Science, University of Liverpool, Liverpool L69 3GJ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Camille","family":"Newby","sequence":"additional","affiliation":[{"name":"Bristol Royal Hospital for Children, Upper Maudlin St, Bristol BS2 8BJ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alex","family":"Pinto","sequence":"additional","affiliation":[{"name":"Birmingham Children\u2019s Hospital, Steelhouse Lane, Birmingham B4 6NH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4859-5288","authenticated-orcid":false,"given":"Martina","family":"Tosi","sequence":"additional","affiliation":[{"name":"Department of Health Sciences, University of Milan, 20142 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ozlem Yilmaz","family":"Nas","sequence":"additional","affiliation":[{"name":"Department of Nutrition and Dietetics, Ankara University, Ankara 06760, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juri","family":"Zuvadelli","sequence":"additional","affiliation":[{"name":"Clinical Department of Pediatrics, San Paolo Hospital, ASST Santi Paolo e Carlo, 20142 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anita","family":"MacDonald","sequence":"additional","affiliation":[{"name":"Birmingham Children\u2019s Hospital, Steelhouse Lane, Birmingham B4 6NH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,2,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"101289","DOI":"10.1016\/j.gim.2024.101289","article-title":"Phenylalanine hydroxylase deficiency diagnosis and management: A 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