{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,21]],"date-time":"2026-07-21T19:31:11Z","timestamp":1784662271128,"version":"3.55.0"},"reference-count":39,"publisher":"SAGE Publications","issue":"2","license":[{"start":{"date-parts":[[2024,4,23]],"date-time":"2024-04-23T00:00:00Z","timestamp":1713830400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Nutr Health"],"published-print":{"date-parts":[[2025,4]]},"abstract":"<jats:p>\n                    <jats:bold>Background:<\/jats:bold>\n                    Maternal phenylketonuria (mPKU) is a pathologic condition occurring in the fetus of a mother with PKU that is caused by prolonged elevated intrauterine blood phenylalanine (Phe) levels, which can lead to congenital abnormalities and mental retardation of newborns. Management of PKU during pregnancy can be challenging as protein substitutes may exacerbate nausea, vomiting, and gastrointestinal symptoms.\n                    <jats:bold>Aim:<\/jats:bold>\n                    To report the successful management of four PKU pregnant women.\n                    <jats:bold>Methods:<\/jats:bold>\n                    The patients were administered with prolonged-release amino acid supplementation and were recommended to follow a strict diet. Blood Phe concentration, adherence to diet, and occurrence of adverse events were monitored.\n                    <jats:bold>Results:<\/jats:bold>\n                    All patients achieved safe levels of blood Phe concentration (120\u2013360\u2009\u00b5mol\/L) since preconception and during pregnancy (mean Phe concentration values of 143.34\u2009\u00b1\u2009137.59, 226.48\u2009\u00b1\u2009194.57, 186.68\u2009\u00b1\u2009133.67, and 187.47\u2009\u00b1\u200942.59\u2009\u00b5mol\/L). During the first trimester of pregnancy, all patients manifested gastrointestinal symptoms such as nausea, gastrointestinal reflux, and abdominal bloating, which were managed by either changing protein substitute or extending the time window between different meals and amino acid mixtures administration. The four women continued their pregnancies without experiencing further complications and delivered neonates with normal growth parameters and no malformations.\n                    <jats:bold>Conclusion:<\/jats:bold>\n                    Findings of this case series suggest that the intake of a prolonged-release amino acid mixture in granules is well tolerated by pregnant PKU patients, eventually leading to good metabolic control and fetal growth within normal ranges.\n                  <\/jats:p>","DOI":"10.1177\/02601060241248522","type":"journal-article","created":{"date-parts":[[2024,4,23]],"date-time":"2024-04-23T08:27:46Z","timestamp":1713860866000},"page":"777-788","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":4,"title":["Benefits of a prolonged-release amino acid mixture in four pregnant women with phenylketonuria"],"prefix":"10.1177","volume":"31","author":[{"given":"Simona","family":"Sestito","sequence":"first","affiliation":[{"name":"UOC Pediatria Specialistica e Malattie Rare, Universit\u00e0 Magna Graecia, Catanzaro, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucia","family":"Brodosi","sequence":"additional","affiliation":[{"name":"SSD Nutrizione Clinica e Metabolismo - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefania","family":"Ferraro","sequence":"additional","affiliation":[{"name":"UOC Pediatria Specialistica e Malattie Rare, Universit\u00e0 Magna Graecia, Catanzaro, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosa","family":"Carella","sequence":"additional","affiliation":[{"name":"Department of Metabolic Diseases, Clinical Genetics and Diabetology, Giovanni XXIII Children Hospital, Azienda Ospedaliero-Universitaria Consorziale, Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donatella","family":"De Giovanni","sequence":"additional","affiliation":[{"name":"Department of Metabolic Diseases, Clinical Genetics and Diabetology, Giovanni XXIII Children Hospital, Azienda Ospedaliero-Universitaria Consorziale, Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorina","family":"Mita","sequence":"additional","affiliation":[{"name":"SSD Nutrizione Clinica e Metabolismo - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-5841-8202","authenticated-orcid":false,"given":"Michele","family":"Moretti","sequence":"additional","affiliation":[{"name":"SSD Nutrizione Clinica e Metabolismo - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Teresa","family":"Moricca","sequence":"additional","affiliation":[{"name":"UOC Pediatria Specialistica e Malattie Rare, Universit\u00e0 Magna Graecia, Catanzaro, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Concolino","sequence":"additional","affiliation":[{"name":"UOC Pediatria Specialistica e Malattie Rare, Universit\u00e0 Magna Graecia, Catanzaro, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8823-8061","authenticated-orcid":false,"given":"Albina","family":"Tummolo","sequence":"additional","affiliation":[{"name":"Department of Metabolic Diseases, Clinical Genetics and Diabetology, Giovanni XXIII Children Hospital, Azienda Ospedaliero-Universitaria Consorziale, Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2024,4,23]]},"reference":[{"key":"e_1_3_3_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(10)60961-0"},{"key":"e_1_3_3_3_1","doi-asserted-by":"publisher","DOI":"10.1080\/03007995.2020.1847717"},{"key":"e_1_3_3_4_1","doi-asserted-by":"publisher","DOI":"10.3390\/nu12072078"},{"key":"e_1_3_3_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.numecd.2020.02.003"},{"key":"e_1_3_3_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/clinchem\/39.1.66"},{"key":"e_1_3_3_7_1","doi-asserted-by":"publisher","DOI":"10.1079\/PNS19790054"},{"key":"e_1_3_3_8_1","doi-asserted-by":"publisher","DOI":"10.3390\/nu13020484"},{"issue":"1","key":"e_1_3_3_9_1","article-title":"Pathogenesis of cognitive dysfunction in phenylketonuria: Review of hypotheses","volume":"99","author":"De Groot MJ","year":"2010","unstructured":"De Groot MJ, Hoeksma M, Blau N, et al. 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