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A new, low‐volume protein substitute for teenagers and adults with phenylketonuria
Some older patients with phenylketonuria (PKU) fail to consume their protein substitute (with or without vitamin and mineral supplements) in prescribed amounts, which contributes to poor blood phenylalanine control. PKU Express (Vitaflo), is a new low‐volume (amino acids 72 g/100 g), low‐carbohydrat...
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| Published in: | Journal of inherited metabolic disease 2004-01, Vol.27 (2), p.127-135 |
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| container_end_page | 135 |
| container_issue | 2 |
| container_start_page | 127 |
| container_title | Journal of inherited metabolic disease |
| container_volume | 27 |
| creator | MacDonald, A. Lilburn, M. Cochrane, B. Davies, P. Daly, A. Asplin, D. Hall, S. K. Cousins, A. Chakrapani, A. Robinson, P. Lee, P. |
| description | Some older patients with phenylketonuria (PKU) fail to consume their protein substitute (with or without vitamin and mineral supplements) in prescribed amounts, which contributes to poor blood phenylalanine control. PKU Express (Vitaflo), is a new low‐volume (amino acids 72 g/100 g), low‐carbohydrate, phenylalanine‐free protein substitute with added vitamins and minerals designed for people with PKU over 8 years of age. In an open intervention study, the aim was to investigate its acceptability and effectiveness in a group of teenagers and adults with PKU. Twenty‐three subjects (15 female; 8 male) with PKU, who had a median age of 17 years (range 8–37 years) took the substitute for 8weeks. A 3‐day prospective diet diary, height, weight, plasma amino acids, biochemical and haematological nutritional analytes were measured at weeks 0 and 8. Skin‐puncture bloods for plasma phenylalanine were collected every 2 weeks. The median weight of protein substitute (with or without vitamin and mineral supplements) consumed decreased by 33% from 150 g (range 140–180) daily to 100 g (range 100–125) daily (p |
| doi_str_mv | 10.1023/B:BOLI.0000028784.11080.9b |
| format | article |
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K. ; Cousins, A. ; Chakrapani, A. ; Robinson, P. ; Lee, P.</creator><creatorcontrib>MacDonald, A. ; Lilburn, M. ; Cochrane, B. ; Davies, P. ; Daly, A. ; Asplin, D. ; Hall, S. K. ; Cousins, A. ; Chakrapani, A. ; Robinson, P. ; Lee, P.</creatorcontrib><description>Some older patients with phenylketonuria (PKU) fail to consume their protein substitute (with or without vitamin and mineral supplements) in prescribed amounts, which contributes to poor blood phenylalanine control. PKU Express (Vitaflo), is a new low‐volume (amino acids 72 g/100 g), low‐carbohydrate, phenylalanine‐free protein substitute with added vitamins and minerals designed for people with PKU over 8 years of age. In an open intervention study, the aim was to investigate its acceptability and effectiveness in a group of teenagers and adults with PKU. Twenty‐three subjects (15 female; 8 male) with PKU, who had a median age of 17 years (range 8–37 years) took the substitute for 8weeks. A 3‐day prospective diet diary, height, weight, plasma amino acids, biochemical and haematological nutritional analytes were measured at weeks 0 and 8. Skin‐puncture bloods for plasma phenylalanine were collected every 2 weeks. The median weight of protein substitute (with or without vitamin and mineral supplements) consumed decreased by 33% from 150 g (range 140–180) daily to 100 g (range 100–125) daily (p<0.001). Median change in energy intake decreased by a median of 10% (95% CI 2.0 to 18.0) when compared to intake on original protein substitute. On PKU Express, the intakes of all nutrients exceeded the dietary reference values but none was excessively high. Blood phenylalanine decreased by a mean of 37 µmol/L (95% CI‐27 to 102) during the trial. Body mass index decreased in 40% of subjects. Changes in blood phenylalanine or body mass index were not statistically significant. Most of the nutritional, haematological and biochemical indices stayed within normal reference ranges for the analytes studied. Sixteen (70%) of the subjects had low plasma selenium at the start, but only 13 (57%) at the study end. Plasma vitamin B12 was high in 8 subjects at the start of the study and 9 at the end. Twenty‐one subjects (96%) stated that the product was convenient and easy to prepare. However, 7 (32%) described the smell and 9 (46%) the texture as the same as or worse than those of previous protein substitutes. Because of the use of the premeasured sachets, some subjects were able to prepare their own protein substitute for the first time. PKU Express is a safe, efficacious, protein substitute that significantly reduces the daily volume of prescribed protein substitute.</description><identifier>ISSN: 0141-8955</identifier><identifier>EISSN: 1573-2665</identifier><identifier>DOI: 10.1023/B:BOLI.0000028784.11080.9b</identifier><identifier>PMID: 15159643</identifier><identifier>CODEN: JIMDDP</identifier><language>eng</language><publisher>Dordrecht: Kluwer Academic Publishers</publisher><subject>Adolescent ; Adult ; Biological and medical sciences ; Body Height ; Body Weight ; Child ; Dietary Supplements ; Electrolytes - blood ; Feeding. Feeding behavior ; Female ; Fundamental and applied biological sciences. Psychology ; Genetics of eukaryotes. Biological and molecular evolution ; Hemoglobins ; Humans ; Male ; Molecular and cellular biology ; Phenylalanine - blood ; Phenylketonurias - blood ; Phenylketonurias - diet therapy ; Phenylketonurias - drug therapy ; Prospective Studies ; Proteins - administration & dosage ; Trace Elements - blood ; Vertebrates: anatomy and physiology, studies on body, several organs or systems ; Vitamins - blood</subject><ispartof>Journal of inherited metabolic disease, 2004-01, Vol.27 (2), p.127-135</ispartof><rights>2004 SSIEM</rights><rights>2004 INIST-CNRS</rights><rights>Copyright Kluwer Academic Publishers 2004</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4297-2ebb4191a28cf6f1e50ea28f30b030e7a955e74d5b2a9edf61f244364fcba2813</citedby><cites>FETCH-LOGICAL-c4297-2ebb4191a28cf6f1e50ea28f30b030e7a955e74d5b2a9edf61f244364fcba2813</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27901,27902</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=15868640$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/15159643$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>MacDonald, A.</creatorcontrib><creatorcontrib>Lilburn, M.</creatorcontrib><creatorcontrib>Cochrane, B.</creatorcontrib><creatorcontrib>Davies, P.</creatorcontrib><creatorcontrib>Daly, A.</creatorcontrib><creatorcontrib>Asplin, D.</creatorcontrib><creatorcontrib>Hall, S. K.</creatorcontrib><creatorcontrib>Cousins, A.</creatorcontrib><creatorcontrib>Chakrapani, A.</creatorcontrib><creatorcontrib>Robinson, P.</creatorcontrib><creatorcontrib>Lee, P.</creatorcontrib><title>A new, low‐volume protein substitute for teenagers and adults with phenylketonuria</title><title>Journal of inherited metabolic disease</title><addtitle>J Inherit Metab Dis</addtitle><description>Some older patients with phenylketonuria (PKU) fail to consume their protein substitute (with or without vitamin and mineral supplements) in prescribed amounts, which contributes to poor blood phenylalanine control. PKU Express (Vitaflo), is a new low‐volume (amino acids 72 g/100 g), low‐carbohydrate, phenylalanine‐free protein substitute with added vitamins and minerals designed for people with PKU over 8 years of age. In an open intervention study, the aim was to investigate its acceptability and effectiveness in a group of teenagers and adults with PKU. Twenty‐three subjects (15 female; 8 male) with PKU, who had a median age of 17 years (range 8–37 years) took the substitute for 8weeks. A 3‐day prospective diet diary, height, weight, plasma amino acids, biochemical and haematological nutritional analytes were measured at weeks 0 and 8. Skin‐puncture bloods for plasma phenylalanine were collected every 2 weeks. The median weight of protein substitute (with or without vitamin and mineral supplements) consumed decreased by 33% from 150 g (range 140–180) daily to 100 g (range 100–125) daily (p<0.001). Median change in energy intake decreased by a median of 10% (95% CI 2.0 to 18.0) when compared to intake on original protein substitute. On PKU Express, the intakes of all nutrients exceeded the dietary reference values but none was excessively high. Blood phenylalanine decreased by a mean of 37 µmol/L (95% CI‐27 to 102) during the trial. Body mass index decreased in 40% of subjects. Changes in blood phenylalanine or body mass index were not statistically significant. Most of the nutritional, haematological and biochemical indices stayed within normal reference ranges for the analytes studied. Sixteen (70%) of the subjects had low plasma selenium at the start, but only 13 (57%) at the study end. Plasma vitamin B12 was high in 8 subjects at the start of the study and 9 at the end. Twenty‐one subjects (96%) stated that the product was convenient and easy to prepare. However, 7 (32%) described the smell and 9 (46%) the texture as the same as or worse than those of previous protein substitutes. Because of the use of the premeasured sachets, some subjects were able to prepare their own protein substitute for the first time. PKU Express is a safe, efficacious, protein substitute that significantly reduces the daily volume of prescribed protein substitute.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Biological and medical sciences</subject><subject>Body Height</subject><subject>Body Weight</subject><subject>Child</subject><subject>Dietary Supplements</subject><subject>Electrolytes - blood</subject><subject>Feeding. Feeding behavior</subject><subject>Female</subject><subject>Fundamental and applied biological sciences. Psychology</subject><subject>Genetics of eukaryotes. Biological and molecular evolution</subject><subject>Hemoglobins</subject><subject>Humans</subject><subject>Male</subject><subject>Molecular and cellular biology</subject><subject>Phenylalanine - blood</subject><subject>Phenylketonurias - blood</subject><subject>Phenylketonurias - diet therapy</subject><subject>Phenylketonurias - drug therapy</subject><subject>Prospective Studies</subject><subject>Proteins - administration & dosage</subject><subject>Trace Elements - blood</subject><subject>Vertebrates: anatomy and physiology, studies on body, several organs or systems</subject><subject>Vitamins - blood</subject><issn>0141-8955</issn><issn>1573-2665</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2004</creationdate><recordtype>article</recordtype><recordid>eNqV0MtuEzEUBmALgWgovAKyKsGKCb7O2F3RlFtQUDdlbdkzx3TKxJPaY6LseASekSfBIZGK2OHN8eI79q8foTNK5pQw_npxvrhaLedkf5hqlJhTShSZa_cAzahseMXqWj5EM0IFrZSW8gQ9Sem2cK2kfIxOqKRS14LP0PUFDrB9hYdx--vHz-_jkNeAN3GcoA84ZZemfsoTYD9GPAEE-xViwjZ02HZ5mBLe9tMN3txA2A3fYBpDjr19ih55OyR4dpyn6Mv7d9eXH6vV1Yfl5cWqagXTTcXAOUE1tUy1vvYUJIFy95w4wgk0tiSHRnTSMauh8zX1TAheC9-64ig_RS8P75bAdxnSZNZ9amEYbIAxJ9NQLbnSe3j2D7wdcwwlm2FUKcYb1RR0fkBtHFOK4M0m9msbd4YSsy_eLMy-eHNfvPlTvNGuLD8__pDdGrr71WPTBbw4AptaO_hoQ9unv5yqVS1IcW8ObtsPsPuPCObT8vNbQlnDfwO16aB5</recordid><startdate>20040101</startdate><enddate>20040101</enddate><creator>MacDonald, A.</creator><creator>Lilburn, M.</creator><creator>Cochrane, B.</creator><creator>Davies, P.</creator><creator>Daly, A.</creator><creator>Asplin, D.</creator><creator>Hall, S. K.</creator><creator>Cousins, A.</creator><creator>Chakrapani, A.</creator><creator>Robinson, P.</creator><creator>Lee, P.</creator><general>Kluwer Academic Publishers</general><general>Springer</general><general>Blackwell Publishing Ltd</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7QP</scope><scope>7TK</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope></search><sort><creationdate>20040101</creationdate><title>A new, low‐volume protein substitute for teenagers and adults with phenylketonuria</title><author>MacDonald, A. ; Lilburn, M. ; Cochrane, B. ; Davies, P. ; Daly, A. ; Asplin, D. ; Hall, S. 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Biological and molecular evolution</topic><topic>Hemoglobins</topic><topic>Humans</topic><topic>Male</topic><topic>Molecular and cellular biology</topic><topic>Phenylalanine - blood</topic><topic>Phenylketonurias - blood</topic><topic>Phenylketonurias - diet therapy</topic><topic>Phenylketonurias - drug therapy</topic><topic>Prospective Studies</topic><topic>Proteins - administration & dosage</topic><topic>Trace Elements - blood</topic><topic>Vertebrates: anatomy and physiology, studies on body, several organs or systems</topic><topic>Vitamins - blood</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>MacDonald, A.</creatorcontrib><creatorcontrib>Lilburn, M.</creatorcontrib><creatorcontrib>Cochrane, B.</creatorcontrib><creatorcontrib>Davies, P.</creatorcontrib><creatorcontrib>Daly, A.</creatorcontrib><creatorcontrib>Asplin, D.</creatorcontrib><creatorcontrib>Hall, S. K.</creatorcontrib><creatorcontrib>Cousins, A.</creatorcontrib><creatorcontrib>Chakrapani, A.</creatorcontrib><creatorcontrib>Robinson, P.</creatorcontrib><creatorcontrib>Lee, P.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni)</collection><collection>ProQuest Central</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of inherited metabolic disease</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>MacDonald, A.</au><au>Lilburn, M.</au><au>Cochrane, B.</au><au>Davies, P.</au><au>Daly, A.</au><au>Asplin, D.</au><au>Hall, S. K.</au><au>Cousins, A.</au><au>Chakrapani, A.</au><au>Robinson, P.</au><au>Lee, P.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>A new, low‐volume protein substitute for teenagers and adults with phenylketonuria</atitle><jtitle>Journal of inherited metabolic disease</jtitle><addtitle>J Inherit Metab Dis</addtitle><date>2004-01-01</date><risdate>2004</risdate><volume>27</volume><issue>2</issue><spage>127</spage><epage>135</epage><pages>127-135</pages><issn>0141-8955</issn><eissn>1573-2665</eissn><coden>JIMDDP</coden><abstract>Some older patients with phenylketonuria (PKU) fail to consume their protein substitute (with or without vitamin and mineral supplements) in prescribed amounts, which contributes to poor blood phenylalanine control. PKU Express (Vitaflo), is a new low‐volume (amino acids 72 g/100 g), low‐carbohydrate, phenylalanine‐free protein substitute with added vitamins and minerals designed for people with PKU over 8 years of age. In an open intervention study, the aim was to investigate its acceptability and effectiveness in a group of teenagers and adults with PKU. Twenty‐three subjects (15 female; 8 male) with PKU, who had a median age of 17 years (range 8–37 years) took the substitute for 8weeks. A 3‐day prospective diet diary, height, weight, plasma amino acids, biochemical and haematological nutritional analytes were measured at weeks 0 and 8. Skin‐puncture bloods for plasma phenylalanine were collected every 2 weeks. The median weight of protein substitute (with or without vitamin and mineral supplements) consumed decreased by 33% from 150 g (range 140–180) daily to 100 g (range 100–125) daily (p<0.001). Median change in energy intake decreased by a median of 10% (95% CI 2.0 to 18.0) when compared to intake on original protein substitute. On PKU Express, the intakes of all nutrients exceeded the dietary reference values but none was excessively high. Blood phenylalanine decreased by a mean of 37 µmol/L (95% CI‐27 to 102) during the trial. Body mass index decreased in 40% of subjects. Changes in blood phenylalanine or body mass index were not statistically significant. Most of the nutritional, haematological and biochemical indices stayed within normal reference ranges for the analytes studied. Sixteen (70%) of the subjects had low plasma selenium at the start, but only 13 (57%) at the study end. Plasma vitamin B12 was high in 8 subjects at the start of the study and 9 at the end. Twenty‐one subjects (96%) stated that the product was convenient and easy to prepare. However, 7 (32%) described the smell and 9 (46%) the texture as the same as or worse than those of previous protein substitutes. Because of the use of the premeasured sachets, some subjects were able to prepare their own protein substitute for the first time. PKU Express is a safe, efficacious, protein substitute that significantly reduces the daily volume of prescribed protein substitute.</abstract><cop>Dordrecht</cop><pub>Kluwer Academic Publishers</pub><pmid>15159643</pmid><doi>10.1023/B:BOLI.0000028784.11080.9b</doi><tpages>9</tpages></addata></record> |
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| identifier | ISSN: 0141-8955 |
| ispartof | Journal of inherited metabolic disease, 2004-01, Vol.27 (2), p.127-135 |
| issn | 0141-8955 1573-2665 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_71953891 |
| source | SpringerLINK Contemporary 1997-Present; Wiley-Blackwell Read & Publish Collection |
| subjects | Adolescent Adult Biological and medical sciences Body Height Body Weight Child Dietary Supplements Electrolytes - blood Feeding. Feeding behavior Female Fundamental and applied biological sciences. Psychology Genetics of eukaryotes. Biological and molecular evolution Hemoglobins Humans Male Molecular and cellular biology Phenylalanine - blood Phenylketonurias - blood Phenylketonurias - diet therapy Phenylketonurias - drug therapy Prospective Studies Proteins - administration & dosage Trace Elements - blood Vertebrates: anatomy and physiology, studies on body, several organs or systems Vitamins - blood |
| title | A new, low‐volume protein substitute for teenagers and adults with phenylketonuria |
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