SNDT WOMEN'S UNIVERSITY
BMK Knowledge Resource Centre
Vithaldas Vidyavihar, Juhu Tara Road,
Santacruz (West) Mumbai - 400049
Benefits of a prolonged-release amino acid mixture in four pregnant women with phenylketonuria (Record no. 133248)
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| 000 -LEADER | |
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| fixed length control field | 02301nam a2200157 4500 |
| 008 - FIXED-LENGTH DATA ELEMENTS--GENERAL INFORMATION | |
| fixed length control field | 251107b |||||||| |||| 00| 0 eng d |
| 100 ## - MAIN ENTRY--PERSONAL NAME | |
| Personal name | Simona Sestito |
| 245 ## - TITLE STATEMENT | |
| Title | Benefits of a prolonged-release amino acid mixture in four pregnant women with phenylketonuria |
| 300 ## - PHYSICAL DESCRIPTION | |
| Extent | pp777-787 |
| 520 ## - SUMMARY, ETC. | |
| Summary, etc. biblio.abstract | Background: 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. Aim: To report the successful management of four PKU pregnant women. Methods: 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. Results: All patients achieved safe levels of blood Phe concentration (120–360 µmol/L) since preconception and during pregnancy (mean Phe concentration values of 143.34 ± 137.59, 226.48 ± 194.57, 186.68 ± 133.67, and 187.47 ± 42.59 µmol/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. Conclusion: 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. |
| 654 ## - SUBJECT ADDED ENTRY--FACETED TOPICAL TERMS | |
| Subject | <a href="Maternal phenylketonuria">Maternal phenylketonuria</a> |
| -- | <a href="PKU">PKU</a> |
| -- | <a href="dietary control">dietary control</a> |
| -- | <a href="metabolic control">metabolic control</a> |
| -- | <a href="pregnancy">pregnancy</a> |
| 700 ## - ADDED ENTRY--PERSONAL NAME | |
| Personal name | Lucia Brodosi |
| 773 0# - HOST ITEM ENTRY | |
| Host Biblionumber | 133247 |
| Host Itemnumber | 114441 |
| Main entry heading | Andressa Silva Costa |
| Title | Effect of cashew nut flour on hematological parameters of children living with HIV |
| 942 ## - ADDED ENTRY ELEMENTS (KOHA) | |
| Koha item type | Journal Article |
| Withdrawn status | Lost status | Source of classification or shelving scheme | Damaged status | Not for loan | Location (home branch) | Sublocation or collection (holding branch) | Date acquired | Koha issues (times borrowed) | Piece designation (barcode) | Koha date last seen | Price effective from | Koha item type |
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| Dewey Decimal Classification | SNDT Juhu | SNDT Juhu | 07/11/2025 | jp963.38 | 07/11/2025 | 07/11/2025 | Journal Article |