Growth and body composition in children with classical phenylketonuria: Results in 34 patients and review of the literature
Treatment of phenylketonuria (PKU, OMIM 261600) means a diet restricted in natural protein and supplemented with phenylalanine (Phe)-free l-amino acid mixtures. Growth impairment has been described even in patients with a total protein intake at or above the recommended dietary allowance (RDA). In t...
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| Veröffentlicht in: | Journal of inherited metabolic disease 2007-10, Vol.30 (5), p.694-699 |
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| creator | Huemer, M Huemer, C Möslinger, D Huter, D Stöckler-Ipsiroglu, S |
| description | Treatment of phenylketonuria (PKU, OMIM 261600) means a diet restricted in natural protein and supplemented with phenylalanine (Phe)-free l-amino acid mixtures. Growth impairment has been described even in patients with a total protein intake at or above the recommended dietary allowance (RDA). In the present study, growth and body composition (fat-free mass (FFM) and fat) were recorded over 12 months in 34 treated PKU patients (mean age 8.7 years at baseline). Measurements were compared with those of healthy peers and with general population standard (Z-) scores calculated using the LMS method. In 28 PKU patients, data on birth weight and birth length were available and related to measurements at baseline of the study. Mean total protein intake in PKU patients was 124% (range 77-193%) of the RDA (DACH 2000). No significant differences in growth and body composition were present between PKU patients and healthy populations either at birth or during the study period. The significant correlation of FFM (representing muscle mass) with intake of natural protein—rather than total protein—indicates that the enhancement of tolerance to natural protein may be of value in PKU patients. |
| doi_str_mv | 10.1007/s10545-007-0549-3 |
| format | Article |
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Growth impairment has been described even in patients with a total protein intake at or above the recommended dietary allowance (RDA). In the present study, growth and body composition (fat-free mass (FFM) and fat) were recorded over 12 months in 34 treated PKU patients (mean age 8.7 years at baseline). Measurements were compared with those of healthy peers and with general population standard (Z-) scores calculated using the LMS method. In 28 PKU patients, data on birth weight and birth length were available and related to measurements at baseline of the study. Mean total protein intake in PKU patients was 124% (range 77-193%) of the RDA (DACH 2000). No significant differences in growth and body composition were present between PKU patients and healthy populations either at birth or during the study period. The significant correlation of FFM (representing muscle mass) with intake of natural protein—rather than total protein—indicates that the enhancement of tolerance to natural protein may be of value in PKU patients.</description><identifier>ISSN: 0141-8955</identifier><identifier>EISSN: 1573-2665</identifier><identifier>DOI: 10.1007/s10545-007-0549-3</identifier><identifier>PMID: 17628756</identifier><identifier>CODEN: JIMDDP</identifier><language>eng</language><publisher>Dordrecht: Dordrecht : Springer Netherlands</publisher><subject>Adolescent ; Amino Acids - administration & dosage ; Aminoacid disorders ; Biological and medical sciences ; Body Composition ; Body Height ; Body Mass Index ; Body Weight ; Case-Control Studies ; Child ; Child, Preschool ; Cross-Sectional Studies ; Diet, Protein-Restricted - adverse effects ; Errors of metabolism ; Female ; Follow-Up Studies ; General aspects ; Growth Disorders - etiology ; Growth Disorders - physiopathology ; Humans ; Infant ; Male ; Medical genetics ; Medical sciences ; Metabolic diseases ; Nutrition Policy ; Phenylketonurias - diet therapy ; Phenylketonurias - physiopathology ; Time Factors ; Treatment Outcome</subject><ispartof>Journal of inherited metabolic disease, 2007-10, Vol.30 (5), p.694-699</ispartof><rights>2007 SSIEM</rights><rights>2007 INIST-CNRS</rights><rights>SSIEM and Springer 2007</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c429H-6e6844ce8d1a350df9f668b73addd26e67927cf5f2a44e6f11dde867541af7833</citedby><cites>FETCH-LOGICAL-c429H-6e6844ce8d1a350df9f668b73addd26e67927cf5f2a44e6f11dde867541af7833</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1007%2Fs10545-007-0549-3$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1007%2Fs10545-007-0549-3$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27903,27904,45553,45554</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=19147374$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/17628756$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Huemer, M</creatorcontrib><creatorcontrib>Huemer, C</creatorcontrib><creatorcontrib>Möslinger, D</creatorcontrib><creatorcontrib>Huter, D</creatorcontrib><creatorcontrib>Stöckler-Ipsiroglu, S</creatorcontrib><title>Growth and body composition in children with classical phenylketonuria: Results in 34 patients and review of the literature</title><title>Journal of inherited metabolic disease</title><addtitle>J Inherit Metab Dis</addtitle><description>Treatment of phenylketonuria (PKU, OMIM 261600) means a diet restricted in natural protein and supplemented with phenylalanine (Phe)-free l-amino acid mixtures. Growth impairment has been described even in patients with a total protein intake at or above the recommended dietary allowance (RDA). In the present study, growth and body composition (fat-free mass (FFM) and fat) were recorded over 12 months in 34 treated PKU patients (mean age 8.7 years at baseline). Measurements were compared with those of healthy peers and with general population standard (Z-) scores calculated using the LMS method. In 28 PKU patients, data on birth weight and birth length were available and related to measurements at baseline of the study. Mean total protein intake in PKU patients was 124% (range 77-193%) of the RDA (DACH 2000). No significant differences in growth and body composition were present between PKU patients and healthy populations either at birth or during the study period. The significant correlation of FFM (representing muscle mass) with intake of natural protein—rather than total protein—indicates that the enhancement of tolerance to natural protein may be of value in PKU patients.</description><subject>Adolescent</subject><subject>Amino Acids - administration & dosage</subject><subject>Aminoacid disorders</subject><subject>Biological and medical sciences</subject><subject>Body Composition</subject><subject>Body Height</subject><subject>Body Mass Index</subject><subject>Body Weight</subject><subject>Case-Control Studies</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Cross-Sectional Studies</subject><subject>Diet, Protein-Restricted - adverse effects</subject><subject>Errors of metabolism</subject><subject>Female</subject><subject>Follow-Up Studies</subject><subject>General aspects</subject><subject>Growth Disorders - etiology</subject><subject>Growth Disorders - physiopathology</subject><subject>Humans</subject><subject>Infant</subject><subject>Male</subject><subject>Medical genetics</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Nutrition Policy</subject><subject>Phenylketonurias - diet therapy</subject><subject>Phenylketonurias - physiopathology</subject><subject>Time Factors</subject><subject>Treatment Outcome</subject><issn>0141-8955</issn><issn>1573-2665</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2007</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><recordid>eNqFkc2OFCEUhStG47SjD-BGiYnuSvmHcmdGnR4zxkSdNaGLi81YDSVU2en48lLpTiZx44pD-M65wGmapwS_JhirN4VgwUVbZVtF17J7zYoIxVoqpbjfrDDhpNWdEGfNo1JuMcadFuJhc0aUpFoJuWr-XOa0n7bIRoc2yR1Qn3ZjKmEKKaIQUb8Ng8sQ0T5Uqh9sKaG3Axq3EA_DT5hSnHOwb9FXKPMwlcXDOBrtFCDW7ZKb4XeAPUoeTVtAQ5gg22nO8Lh54O1Q4MlpPW9uPn74frFur79cXl28u257Trt1K0FqznvQjlgmsPOdl1JvFLPOOVpPVUdV74WnlnOQnhDnQEslOLFeacbOm1fH3DGnXzOUyexC6WEYbIQ0FyM1k7LyFXzxD3ib5hzr3QwlWlPF6JJGjlCfUykZvBlz2Nl8MASbpRZzrMUscqnFLJ5np-B5swN35zj1UIGXJ8CW-r0-29iHcsd1hCumeOX0kduHAQ7_n2w-XX1-jzlR62p9frR6m4z9kWv8zTeKCcNEUdzVl_0Fd4KwMA</recordid><startdate>200710</startdate><enddate>200710</enddate><creator>Huemer, M</creator><creator>Huemer, C</creator><creator>Möslinger, D</creator><creator>Huter, D</creator><creator>Stöckler-Ipsiroglu, S</creator><general>Dordrecht : Springer Netherlands</general><general>Springer Netherlands</general><general>Springer</general><general>Blackwell Publishing Ltd</general><scope>FBQ</scope><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>200710</creationdate><title>Growth and body composition in children with classical phenylketonuria: Results in 34 patients and review of the literature</title><author>Huemer, M ; Huemer, C ; Möslinger, D ; Huter, D ; Stöckler-Ipsiroglu, S</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c429H-6e6844ce8d1a350df9f668b73addd26e67927cf5f2a44e6f11dde867541af7833</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2007</creationdate><topic>Adolescent</topic><topic>Amino Acids - administration & dosage</topic><topic>Aminoacid disorders</topic><topic>Biological and medical sciences</topic><topic>Body Composition</topic><topic>Body Height</topic><topic>Body Mass Index</topic><topic>Body Weight</topic><topic>Case-Control Studies</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Cross-Sectional Studies</topic><topic>Diet, Protein-Restricted - adverse effects</topic><topic>Errors of metabolism</topic><topic>Female</topic><topic>Follow-Up Studies</topic><topic>General aspects</topic><topic>Growth Disorders - etiology</topic><topic>Growth Disorders - physiopathology</topic><topic>Humans</topic><topic>Infant</topic><topic>Male</topic><topic>Medical genetics</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>Nutrition Policy</topic><topic>Phenylketonurias - diet therapy</topic><topic>Phenylketonurias - physiopathology</topic><topic>Time Factors</topic><topic>Treatment Outcome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Huemer, M</creatorcontrib><creatorcontrib>Huemer, C</creatorcontrib><creatorcontrib>Möslinger, D</creatorcontrib><creatorcontrib>Huter, D</creatorcontrib><creatorcontrib>Stöckler-Ipsiroglu, S</creatorcontrib><collection>AGRIS</collection><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>ProQuest 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 UK/Ireland</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>PML(ProQuest Medical Library)</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>Huemer, M</au><au>Huemer, C</au><au>Möslinger, D</au><au>Huter, D</au><au>Stöckler-Ipsiroglu, S</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Growth and body composition in children with classical phenylketonuria: Results in 34 patients and review of the literature</atitle><jtitle>Journal of inherited metabolic disease</jtitle><addtitle>J Inherit Metab Dis</addtitle><date>2007-10</date><risdate>2007</risdate><volume>30</volume><issue>5</issue><spage>694</spage><epage>699</epage><pages>694-699</pages><issn>0141-8955</issn><eissn>1573-2665</eissn><coden>JIMDDP</coden><abstract>Treatment of phenylketonuria (PKU, OMIM 261600) means a diet restricted in natural protein and supplemented with phenylalanine (Phe)-free l-amino acid mixtures. Growth impairment has been described even in patients with a total protein intake at or above the recommended dietary allowance (RDA). In the present study, growth and body composition (fat-free mass (FFM) and fat) were recorded over 12 months in 34 treated PKU patients (mean age 8.7 years at baseline). Measurements were compared with those of healthy peers and with general population standard (Z-) scores calculated using the LMS method. In 28 PKU patients, data on birth weight and birth length were available and related to measurements at baseline of the study. Mean total protein intake in PKU patients was 124% (range 77-193%) of the RDA (DACH 2000). No significant differences in growth and body composition were present between PKU patients and healthy populations either at birth or during the study period. The significant correlation of FFM (representing muscle mass) with intake of natural protein—rather than total protein—indicates that the enhancement of tolerance to natural protein may be of value in PKU patients.</abstract><cop>Dordrecht</cop><pub>Dordrecht : Springer Netherlands</pub><pmid>17628756</pmid><doi>10.1007/s10545-007-0549-3</doi><tpages>6</tpages></addata></record> |
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| ispartof | Journal of inherited metabolic disease, 2007-10, Vol.30 (5), p.694-699 |
| issn | 0141-8955 1573-2665 |
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| source | Wiley-Blackwell Journals; MEDLINE; SpringerLink Journals - AutoHoldings |
| subjects | Adolescent Amino Acids - administration & dosage Aminoacid disorders Biological and medical sciences Body Composition Body Height Body Mass Index Body Weight Case-Control Studies Child Child, Preschool Cross-Sectional Studies Diet, Protein-Restricted - adverse effects Errors of metabolism Female Follow-Up Studies General aspects Growth Disorders - etiology Growth Disorders - physiopathology Humans Infant Male Medical genetics Medical sciences Metabolic diseases Nutrition Policy Phenylketonurias - diet therapy Phenylketonurias - physiopathology Time Factors Treatment Outcome |
| title | Growth and body composition in children with classical phenylketonuria: Results in 34 patients and review of the literature |
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