{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T15:33:21Z","timestamp":1786980801648,"version":"build-2736575974"},"reference-count":29,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2016,11,22]],"date-time":"2016-11-22T00:00:00Z","timestamp":1479772800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2016,11,22]],"date-time":"2016-11-22T00:00:00Z","timestamp":1479772800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2017,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Bone disease in patients with phenylketonuria (PKU) is incompletely characterized. We therefore analyzed, in a cross\u2010sectional study radius macroscopic bone architecture and forearm muscle size by peripheral quantitative computed tomography (pQCT) and muscle strength by hand dynamometry in a large cohort (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200956) of adolescent and adult patients with PKU aged 26.0\u2009\u00b1\u20098.9 (range, 11.8\u201341.5) years. Data were compared with a reference population (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u2009700) from the DONALD study using identical methodology. We observed a significant reduction of cortical thickness (z\u2010score \u22121.01\u2009\u00b1\u20090.79), Strength\u2013Strain Index (SSI) (z\u2010score \u22120.81\u2009\u00b1\u20091.03), and total bone mineral density (BMD) of the distal radius (z\u2010score \u22121.05\u2009\u00b1\u20091.00). Mean muscle cross\u2010sectional area (z\u2010score \u22120.98\u2009\u00b1\u20091.19) and muscle grip force (z\u2010score \u22120.64\u2009\u00b1\u20091.26) were also significantly reduced, indicating an impaired muscular system as part of the clinical phenotype of PKU. SSI positively correlated (\n                    <jats:italic>r<\/jats:italic>\n                    \u2009=\u20090.53,\n                    <jats:italic>P<\/jats:italic>\n                    \u2009&lt;\u20090.001) with the corresponding muscle cross\u2010sectional area in the reference population; however, the regression line slope in PKU patients was less steep (\n                    <jats:italic>P<\/jats:italic>\n                    \u2009&lt;\u20090.001), indicating that bone strength is not adequately adapted to muscle force. In conclusion, the radial bone in PKU patients is characterized by reduced bone strength in relation to muscular force, decreased cortical thickness, and impaired total BMD at the metaphyseal site. These alterations indicate a mixed bone defect in PKU, both of which are due to primary alterations of bone metabolism and to secondary alterations in response to neuromuscular abnormalities.\n                  <\/jats:p>","DOI":"10.1007\/s10545-016-0002-6","type":"journal-article","created":{"date-parts":[[2016,11,22]],"date-time":"2016-11-22T03:27:54Z","timestamp":1479785274000},"page":"219-226","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Analysis of the functional muscle\u2013bone unit of the forearm in patients with phenylketonuria by peripheral quantitative computed tomography"],"prefix":"10.1002","volume":"40","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1631-3883","authenticated-orcid":false,"given":"Daniela","family":"Choukair","sequence":"first","affiliation":[{"name":"Department of Pediatrics I University Children's Hospital Heidelberg Im Neuenheimer Feld 430 Heidelberg 69120 Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolin","family":"Kneppo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics I University Children's Hospital Heidelberg Im Neuenheimer Feld 430 Heidelberg 69120 Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reinhard","family":"Feneberg","sequence":"additional","affiliation":[{"name":"Department of Pediatrics I University Children's Hospital Heidelberg Im Neuenheimer Feld 430 Heidelberg 69120 Germany"},{"name":"ICON Clinical Research GmbH Langen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eckhard","family":"Sch\u00f6nau","sequence":"additional","affiliation":[{"name":"University Children's Hospital Cologne Cologne Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Lindner","sequence":"additional","affiliation":[{"name":"Division of Neuropediatrics and Metabolic Medicine, Department of Pediatrics I University Children's Hospital Heidelberg Heidelberg Germany"},{"name":"University Children's Hospital Frankfurt Frankfurt am Main Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"K\u00f6lker","sequence":"additional","affiliation":[{"name":"Division of Neuropediatrics and Metabolic Medicine, Department of Pediatrics I University Children's Hospital Heidelberg Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georg F.","family":"Hoffmann","sequence":"additional","affiliation":[{"name":"Department of Pediatrics I University Children's Hospital Heidelberg Im Neuenheimer Feld 430 Heidelberg 69120 Germany"},{"name":"Division of Neuropediatrics and Metabolic Medicine, Department of Pediatrics I University Children's Hospital Heidelberg Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Burkhard","family":"T\u00f6nshoff","sequence":"additional","affiliation":[{"name":"Department of Pediatrics I University Children's Hospital Heidelberg Im Neuenheimer Feld 430 Heidelberg 69120 Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,11,22]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00774-010-0216-x"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/59.2.419"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2008-1033752"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2012.01.006"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-015-0232-y"},{"key":"e_1_2_10_7_1","volume-title":"Introduction to a new skeletal physiology","author":"Frost HMF","year":"1995"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1515\/JPEM.2001.14.5.481"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1997.tb08882.x"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-014-9735-2"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1753-4887.2008.00104.x"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-010-9189-0"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1600-6135.ajt10301.x"},{"issue":"3","key":"e_1_2_10_14_1","first-page":"240","article-title":"pQCT provides better prediction of canine femur breaking load than does DXA","volume":"3","author":"Moisio KC","year":"2003","journal-title":"J Musculoskel Neuronal Interact"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S8756-3282(00)00429-4"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s001980170074"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-200109000-00003"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1359\/jbmr.2001.16.8.1547"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004150050301"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1523-1755.2004.00937.x"},{"key":"e_1_2_10_21_1","first-page":"141","volume-title":"Paediatric osteology: newdevelopments in diagnostics and therapy","author":"Schiessl HFJ","year":"1996"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1210\/jcem.85.3.6451"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1210\/jcem.86.2.7186"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004670050479"},{"issue":"1","key":"e_1_2_10_25_1","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1159\/000184834","article-title":"Influence of muscle strength on bone strength during childhood and adolescence","volume":"45","author":"Schonau E","year":"1996","journal-title":"Horm Res"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1998.tb01387.x"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0045165"},{"key":"e_1_2_10_28_1","volume-title":"Growth at adolescence","author":"Tanner JM","year":"1962"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2011.08.025"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1999.tb01049.x"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10545-016-0002-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s10545-016-0002-6\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10545-016-0002-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-016-0002-6","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T04:48:51Z","timestamp":1694753331000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-016-0002-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,11,22]]},"references-count":29,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2017,3]]}},"alternative-id":["10.1007\/s10545-016-0002-6"],"URL":"https:\/\/doi.org\/10.1007\/s10545-016-0002-6","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,11,22]]}}}