دورية أكاديمية
Preparing Enteral Formulas for Adult Patients with Phenylketonuria: A Minor Necessity but Major Challenge—A Case Report.
| العنوان: | Preparing Enteral Formulas for Adult Patients with Phenylketonuria: A Minor Necessity but Major Challenge—A Case Report. |
|---|---|
| المؤلفون: | Pané, Adriana1,2,3 (AUTHOR) pane@clinic.cat, Carrasco-Serrano, Marcos1,2 (AUTHOR) garrabou@clinic.cat, Milad, Camila1 (AUTHOR), Leyes, Pere1,2 (AUTHOR) jugarcia@clinic.cat, Moreno-Lozano, Pedro Juan2,4,5 (AUTHOR) rmlopez@clinic.cat, Ventura, Roser2,5 (AUTHOR) aribes@clinic.cat, Milisenda, José Cesar2,5,6,7 (AUTHOR), García-García, Francesc Josep2,6,7,8 (AUTHOR), Garrabou, Glòria2,6,7,8 (AUTHOR), García-Villoria, Judit2,4,6,7,9 (AUTHOR), López-Galera, Rosa Maria2,4,6,7,9 (AUTHOR), Ribes, Antonia2,4,6,7,9 (AUTHOR), Grau-Junyent, Josep Maria2,5,6,7 (AUTHOR), Forga-Visa, Maria de Talló1,2,4 (AUTHOR), Montserrat-Carbonell, Cristina1,2,4 (AUTHOR) pane@clinic.cat |
| المصدر: | Journal of Clinical Medicine. Dec2023, Vol. 12 Issue 23, p7452. 10p. |
| مصطلحات موضوعية: | *PHENYLKETONURIA, *TUBE feeding, *ARTIFICIAL feeding, *ENTERAL feeding, *ADULTS |
| مستخلص: | Phenylketonuria (PKU) is the most frequent of the congenital errors of amino acid (AA) metabolism worldwide. It leads to the accumulation of the essential AA phenylalanine (Phe) and it is associated with severe neurological defects. The early diagnosis and treatment of this rare disease, achieved through newborn screening and low-Phe diet, has profoundly changed its clinical spectrum, resulting in normal cognitive development. We face the first generation of PKU patients perinatally diagnosed and treated who have reached adulthood, whose special needs must be addressed, including feeding through enteral nutrition (EN). However, recommendations regarding EN in PKU constitute a gap in the literature. Although protein substitutes for patients with PKU are offered in multiple forms (Phe-free L-amino acid or casein glycomacropeptide supplements), none of these commercial formulas ensures the whole provision of daily total energy and protein requirements, including a safe amount of Phe. Consequently, the combination of different products becomes necessary when artificial nutrition via tube feeding is required. Importantly, the composition of these specific formulas may result in physicochemical interactions when they are mixed with standard EN products, leading to enteral feeding tubes clogging, and also gastrointestinal concerns due to hyperosmolality. Herein, we present the first reported case of EN use in an adult patient with PKU, where the separate administration of protein substitutes and the other EN products avoided physicochemical interactions. [ABSTRACT FROM AUTHOR] |
| قاعدة البيانات: | Academic Search Index |
Full Text Finder | ResultId |
1 |
|---|---|
| Header |
asx Academic Search Index 174113839 1410 6 Academic Journal academicJournal 1410.24304199219 |
| PLink |
https://search.ebscohost.com/login.aspx?direct=true&site=eds-live&scope=site&db=asx&AN=174113839&custid=s6537998&authtype=sso |
| FullText |
Array
(
[Availability] => 0
)
Array ( [0] => Array ( [Url] => https://resolver.ebscohost.com/openurl?custid=s6537998&groupid=main&authtype=ip,guest&sid=EBSCO:asx&genre=article&issn=20770383&ISBN=&volume=12&issue=23&date=20231201&spage=7452&pages=7452-7461&title=Journal of Clinical Medicine&atitle=Preparing%20Enteral%20Formulas%20for%20Adult%20Patients%20with%20Phenylketonuria%3A%20A%20Minor%20Necessity%20but%20Major%20Challenge%E2%80%94A%20Case%20Report.&id=DOI:10.3390/jcm12237452 [Name] => Full Text Finder (s6537998api) [Category] => fullText [Text] => Full Text Finder [Icon] => https://imageserver.ebscohost.com/branding/images/FTF.gif [MouseOverText] => Full Text Finder ) ) |
| Items |
Array
(
[Name] => Title
[Label] => Title
[Group] => Ti
[Data] => Preparing Enteral Formulas for Adult Patients with Phenylketonuria: A Minor Necessity but Major Challenge—A Case Report.
)
Array ( [Name] => Author [Label] => Authors [Group] => Au [Data] => <searchLink fieldCode="AR" term="%22Pané%2C+Adriana%22">Pané, Adriana</searchLink><relatesTo>1,2,3</relatesTo> (AUTHOR)<i> pane@clinic.cat</i><br /><searchLink fieldCode="AR" term="%22Carrasco-Serrano%2C+Marcos%22">Carrasco-Serrano, Marcos</searchLink><relatesTo>1,2</relatesTo> (AUTHOR)<i> garrabou@clinic.cat</i><br /><searchLink fieldCode="AR" term="%22Milad%2C+Camila%22">Milad, Camila</searchLink><relatesTo>1</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Leyes%2C+Pere%22">Leyes, Pere</searchLink><relatesTo>1,2</relatesTo> (AUTHOR)<i> jugarcia@clinic.cat</i><br /><searchLink fieldCode="AR" term="%22Moreno-Lozano%2C+Pedro+Juan%22">Moreno-Lozano, Pedro Juan</searchLink><relatesTo>2,4,5</relatesTo> (AUTHOR)<i> rmlopez@clinic.cat</i><br /><searchLink fieldCode="AR" term="%22Ventura%2C+Roser%22">Ventura, Roser</searchLink><relatesTo>2,5</relatesTo> (AUTHOR)<i> aribes@clinic.cat</i><br /><searchLink fieldCode="AR" term="%22Milisenda%2C+José+Cesar%22">Milisenda, José Cesar</searchLink><relatesTo>2,5,6,7</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22García-García%2C+Francesc+Josep%22">García-García, Francesc Josep</searchLink><relatesTo>2,6,7,8</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Garrabou%2C+Glòria%22">Garrabou, Glòria</searchLink><relatesTo>2,6,7,8</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22García-Villoria%2C+Judit%22">García-Villoria, Judit</searchLink><relatesTo>2,4,6,7,9</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22López-Galera%2C+Rosa+Maria%22">López-Galera, Rosa Maria</searchLink><relatesTo>2,4,6,7,9</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Ribes%2C+Antonia%22">Ribes, Antonia</searchLink><relatesTo>2,4,6,7,9</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Grau-Junyent%2C+Josep+Maria%22">Grau-Junyent, Josep Maria</searchLink><relatesTo>2,5,6,7</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Forga-Visa%2C+Maria+de+Talló%22">Forga-Visa, Maria de Talló</searchLink><relatesTo>1,2,4</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Montserrat-Carbonell%2C+Cristina%22">Montserrat-Carbonell, Cristina</searchLink><relatesTo>1,2,4</relatesTo> (AUTHOR)<i> pane@clinic.cat</i> ) Array ( [Name] => TitleSource [Label] => Source [Group] => Src [Data] => <searchLink fieldCode="JN" term="%22Journal+of+Clinical+Medicine%22">Journal of Clinical Medicine</searchLink>. Dec2023, Vol. 12 Issue 23, p7452. 10p. ) Array ( [Name] => Subject [Label] => Subject Terms [Group] => Su [Data] => *<searchLink fieldCode="DE" term="%22PHENYLKETONURIA%22">PHENYLKETONURIA</searchLink><br />*<searchLink fieldCode="DE" term="%22TUBE+feeding%22">TUBE feeding</searchLink><br />*<searchLink fieldCode="DE" term="%22ARTIFICIAL+feeding%22">ARTIFICIAL feeding</searchLink><br />*<searchLink fieldCode="DE" term="%22ENTERAL+feeding%22">ENTERAL feeding</searchLink><br />*<searchLink fieldCode="DE" term="%22ADULTS%22">ADULTS</searchLink> ) Array ( [Name] => Abstract [Label] => Abstract [Group] => Ab [Data] => Phenylketonuria (PKU) is the most frequent of the congenital errors of amino acid (AA) metabolism worldwide. It leads to the accumulation of the essential AA phenylalanine (Phe) and it is associated with severe neurological defects. The early diagnosis and treatment of this rare disease, achieved through newborn screening and low-Phe diet, has profoundly changed its clinical spectrum, resulting in normal cognitive development. We face the first generation of PKU patients perinatally diagnosed and treated who have reached adulthood, whose special needs must be addressed, including feeding through enteral nutrition (EN). However, recommendations regarding EN in PKU constitute a gap in the literature. Although protein substitutes for patients with PKU are offered in multiple forms (Phe-free L-amino acid or casein glycomacropeptide supplements), none of these commercial formulas ensures the whole provision of daily total energy and protein requirements, including a safe amount of Phe. Consequently, the combination of different products becomes necessary when artificial nutrition via tube feeding is required. Importantly, the composition of these specific formulas may result in physicochemical interactions when they are mixed with standard EN products, leading to enteral feeding tubes clogging, and also gastrointestinal concerns due to hyperosmolality. Herein, we present the first reported case of EN use in an adult patient with PKU, where the separate administration of protein substitutes and the other EN products avoided physicochemical interactions. [ABSTRACT FROM AUTHOR] ) |
| RecordInfo |
Array
(
[BibEntity] => Array
(
[Identifiers] => Array
(
[0] => Array
(
[Type] => doi
[Value] => 10.3390/jcm12237452
)
)
[Languages] => Array
(
[0] => Array
(
[Code] => eng
[Text] => English
)
)
[PhysicalDescription] => Array
(
[Pagination] => Array
(
[PageCount] => 10
[StartPage] => 7452
)
)
[Subjects] => Array
(
[0] => Array
(
[SubjectFull] => PHENYLKETONURIA
[Type] => general
)
[1] => Array
(
[SubjectFull] => TUBE feeding
[Type] => general
)
[2] => Array
(
[SubjectFull] => ARTIFICIAL feeding
[Type] => general
)
[3] => Array
(
[SubjectFull] => ENTERAL feeding
[Type] => general
)
[4] => Array
(
[SubjectFull] => ADULTS
[Type] => general
)
)
[Titles] => Array
(
[0] => Array
(
[TitleFull] => Preparing Enteral Formulas for Adult Patients with Phenylketonuria: A Minor Necessity but Major Challenge—A Case Report.
[Type] => main
)
)
)
[BibRelationships] => Array
(
[HasContributorRelationships] => Array
(
[0] => Array
(
[PersonEntity] => Array
(
[Name] => Array
(
[NameFull] => Pané, Adriana
)
)
)
[1] => Array
(
[PersonEntity] => Array
(
[Name] => Array
(
[NameFull] => Carrasco-Serrano, Marcos
)
)
)
[2] => Array
(
[PersonEntity] => Array
(
[Name] => Array
(
[NameFull] => Milad, Camila
)
)
)
[3] => Array
(
[PersonEntity] => Array
(
[Name] => Array
(
[NameFull] => Leyes, Pere
)
)
)
[4] => Array
(
[PersonEntity] => Array
(
[Name] => Array
(
[NameFull] => Moreno-Lozano, Pedro Juan
)
)
)
[5] => Array
(
[PersonEntity] => Array
(
[Name] => Array
(
[NameFull] => Ventura, Roser
)
)
)
[6] => Array
(
[PersonEntity] => Array
(
[Name] => Array
(
[NameFull] => Milisenda, José Cesar
)
)
)
[7] => Array
(
[PersonEntity] => Array
(
[Name] => Array
(
[NameFull] => García-García, Francesc Josep
)
)
)
[8] => Array
(
[PersonEntity] => Array
(
[Name] => Array
(
[NameFull] => Garrabou, Glòria
)
)
)
[9] => Array
(
[PersonEntity] => Array
(
[Name] => Array
(
[NameFull] => García-Villoria, Judit
)
)
)
[10] => Array
(
[PersonEntity] => Array
(
[Name] => Array
(
[NameFull] => López-Galera, Rosa Maria
)
)
)
[11] => Array
(
[PersonEntity] => Array
(
[Name] => Array
(
[NameFull] => Ribes, Antonia
)
)
)
[12] => Array
(
[PersonEntity] => Array
(
[Name] => Array
(
[NameFull] => Grau-Junyent, Josep Maria
)
)
)
[13] => Array
(
[PersonEntity] => Array
(
[Name] => Array
(
[NameFull] => Forga-Visa, Maria de Talló
)
)
)
[14] => Array
(
[PersonEntity] => Array
(
[Name] => Array
(
[NameFull] => Montserrat-Carbonell, Cristina
)
)
)
)
[IsPartOfRelationships] => Array
(
[0] => Array
(
[BibEntity] => Array
(
[Dates] => Array
(
[0] => Array
(
[D] => 01
[M] => 12
[Text] => Dec2023
[Type] => published
[Y] => 2023
)
)
[Identifiers] => Array
(
[0] => Array
(
[Type] => issn-print
[Value] => 20770383
)
)
[Numbering] => Array
(
[0] => Array
(
[Type] => volume
[Value] => 12
)
[1] => Array
(
[Type] => issue
[Value] => 23
)
)
[Titles] => Array
(
[0] => Array
(
[TitleFull] => Journal of Clinical Medicine
[Type] => main
)
)
)
)
)
)
)
|
| IllustrationInfo |