{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T09:12:04Z","timestamp":1780391524674,"version":"3.54.1"},"reference-count":28,"publisher":"S. Karger AG","issue":"2","license":[{"start":{"date-parts":[[2010,1,1]],"date-time":"2010-01-01T00:00:00Z","timestamp":1262304000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2010,1,1]],"date-time":"2010-01-01T00:00:00Z","timestamp":1262304000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Horm Res Paediatr"],"published-print":{"date-parts":[[2010]]},"abstract":"<i>Background\/Aims:<\/i> Growth hormone insensitivity syndrome (GHIS) is a rare cause of growth retardation characterized by high serum GH levels, and low serum insulin-like growth factor I (IGF-I) levels associated with a genetic defect of the GH receptor (GHR) as well post-GHR signaling pathway. Based on clinical, as well as biochemical characteristics, GHIS can be genetically classified as classical\/Laron\u2019s syndrome and nonclassical\/atypical GHIS. Recombinant human IGF-I (rhIGF-I) treatment is effective in promoting growth in subjects who have GHIS. Further, pharmacological studies of a IGF-I compound containing a 1:1 molar complex of rhIGF-I and rhIGF-binding protein-3 (BP-3) demonstrated that the complex was effective in increasing levels of circulating total and free IGF-I and that the administration in patients with GHIS should be safe, well-tolerated and more effective than rhIGF-I on its own. <i>Patient\/Methods:<\/i> We describe the long-term effect of various IGF-I preparations (rhIGF; rhIGF-I\/rhIGFBP-3) in a single subject treated for more than 14 years while focusing on height, height velocity as well as on additional auxological and laboratory data. <i>Results:<\/i> This study confirms that rhIGF-I is effective in promoting growth in children with GHIS. However, on the combined rhIGF-I\/rhIGFBP-3 treatment as well as off rhIGF-I therapy the height velocity decreased drastically (2 and 1.8 cm vs. overall 6.5 cm\/year on rhIGF-I, respectively). On rhIGF-I treatment, serum IGF-I was found to be well within the normal range, whereas serum IGFBP-3 remained low. On the rhIGF-I\/rhIGFBP-3 compound therapy, however, serum IGFBP-3 increased into the normal range, which was not the case for serum IGF-I. Importantly, the increase of the serum IGFBP-3 level excludes noncompliance. In addition, body mass index as well as dual-energy X-ray absorptiometry analysis underlined the positive effect of rhIGF-I treatment on body composition. <i>Conclusions:<\/i>The rhIGF-I\/rhIGFBP-3 compound therapy seems to be not efficient in treating this individual patient with GHIS when compared with rhIGF-I alone.<\/jats:p>","DOI":"10.1159\/000277660","type":"journal-article","created":{"date-parts":[[2010,2,8]],"date-time":"2010-02-08T08:31:50Z","timestamp":1265617910000},"page":"140-147","source":"Crossref","is-referenced-by-count":8,"title":["Insulin-Like Growth Factor-I Treatment in Primary Growth Hormone Insensitivity: Effect of Recombinant Human IGF-I (rhIGF-I) and rhIGF-I\/rhIGF-Binding Protein-3 Complex"],"prefix":"10.1159","volume":"73","author":[{"given":"P.","family":"Tonella","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.E.","family":"Fl\u00fcck","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.-E.","family":"Mullis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2010,2,9]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1159%2F000063471"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1210%2Fjc.82.11.3529"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1210%2Fjc.2005-1017"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1530%2Feje.0.1500635"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1111%2Fj.1365-2362.1995.tb01689.x"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1097%2F01.mpg.0000189373.31697.15"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1007%2FBF00280883"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1038%2Fncpendmet0195"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1159%2F000184637"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1016%2F0140-6736%2892%2991594-X"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1210%2Fjc.80.4.1393"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1210%2Fjc.86.4.1504"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1210%2Fjc.82.2.629"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1046%2Fj.1365-2265.1999.00887.x"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1159%2F000023345"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1210%2Fjc.2006-1610"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1111%2Fj.1651-2227.1993.tb12918.x"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.1016%2F8756-3282%2895%2900078-R"},{"key":"ref19","doi-asserted-by":"publisher","DOI":"10.1210%2Fer.16.1.3"},{"key":"ref20","doi-asserted-by":"publisher","DOI":"10.1210%2Fjc.76.3.574"},{"key":"ref21","doi-asserted-by":"publisher","DOI":"10.2337%2Fdiabetes.50.5.1110"},{"key":"ref22","doi-asserted-by":"publisher","DOI":"10.1097%2FMOP.0b013e3282094126"},{"key":"ref23","doi-asserted-by":"publisher","DOI":"10.1074%2Fjbc.M005088200"},{"key":"ref24","doi-asserted-by":"publisher","DOI":"10.1093%2Fcarcin%2Fbgm088"},{"key":"ref25","doi-asserted-by":"publisher","DOI":"10.1677%2Fjoe.0.1750033"},{"key":"ref26","doi-asserted-by":"publisher","DOI":"10.1210%2Fen.142.5.1958"},{"key":"ref27","doi-asserted-by":"publisher","DOI":"10.1016%2FS0140-6736%2806%2969205-2"},{"key":"ref28","doi-asserted-by":"publisher","DOI":"10.1016%2Fj.jpeds.2006.09.040"}],"container-title":["Hormone Research in Paediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/277660","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/277660","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T20:50:48Z","timestamp":1745441448000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000277660"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010]]},"references-count":28,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2010,2,1]]}},"URL":"https:\/\/doi.org\/10.1159\/000277660","archive":["Portico"],"relation":{},"ISSN":["1663-2818","1663-2826"],"issn-type":[{"value":"1663-2818","type":"print"},{"value":"1663-2826","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010]]}}}