{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,30]],"date-time":"2026-06-30T03:22:35Z","timestamp":1782789755904,"version":"3.54.5"},"reference-count":22,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2007,7,11]],"date-time":"2007-07-11T00:00:00Z","timestamp":1184112000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2007,10]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Treatment of phenylketonuria (PKU, OMIM 261600) means a diet restricted in natural protein and supplemented with phenylalanine (Phe)\u2010free <jats:sc>l<\/jats:sc>\u2010amino 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\u2010free 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 (<jats:italic>Z<\/jats:italic>\u2010) 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\u2013193%) of the RDA (DACH <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"#CR7\">2000<\/jats:ext-link>). 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\u2014rather than total protein\u2014indicates that the enhancement of tolerance to natural protein may be of value in PKU patients.<\/jats:p>","DOI":"10.1007\/s10545-007-0549-3","type":"journal-article","created":{"date-parts":[[2007,7,16]],"date-time":"2007-07-16T16:33:00Z","timestamp":1184603580000},"page":"694-699","source":"Crossref","is-referenced-by-count":72,"title":["Growth and body composition in children with classical phenylketonuria: Results in 34 patients and review of the literature"],"prefix":"10.1002","volume":"30","author":[{"given":"M.","family":"Huemer","sequence":"first","affiliation":[{"name":"Department of Pediatrics Landeskrankenhaus Bregenz Carl Pedenz Str. 2 Bregenz 6900 Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Huemer","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Landeskrankenhaus Bregenz Carl Pedenz Str. 2 Bregenz 6900 Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"M\u00f6slinger","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Huter","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"St\u00f6ckler\u2010Ipsiroglu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Biochemical Diseases University of British Columbia Vancouver Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2007,7,11]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005176-199809000-00003"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1080\/07315724.1999.10718836"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-8223(03)00983-0"},{"key":"e_1_2_1_5_1","first-page":"178","article-title":"Body protein in prepubertal children with phenylketonuria","volume":"50","author":"Allen JR","year":"1996","journal-title":"Eur J Clin Nutr"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2002.126455"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004310051008"},{"key":"e_1_2_1_8_1","first-page":"45","article-title":"The LMS method for constructing normalized growth standards","volume":"44","author":"Cole TJ","year":"1990","journal-title":"Eur J Clin Nutr"},{"key":"e_1_2_1_9_1","volume-title":"Referenzwerte f\u00fcr die N\u00e4hrstoffzufuhr","year":"2000"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/jn\/131.7.1965"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.74.5.386"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1024063726046"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2005.05.013"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01957005"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-005-0122-x"},{"key":"e_1_2_1_16_1","doi-asserted-by":"crossref","first-page":"700","DOI":"10.1542\/peds.63.5.700","article-title":"Physical growth in phenylketonuria. 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