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We report on a 3\u2010year\u2010old girl with typical manifestations of Proteus syndrome, including local, asymmetric hypertrophy of various parts of the body. Total body length was reduced. Serum levels of IGF\u2010I and especially IGF\u2010II and their major growth hormone dependent binding protein (IGFBP\u20103) were significantly reduced, although growth hormone secretion after a pharmacological stimulus was normal. In vitro studies of fibroblasts derived from hypertrophied tissue showed normal IGF\u2010I production and somewhat reduced IGF\u2010II and IGFBP\u20103 production as compared to normal human skin fibroblasts.<\/jats:p><jats:p>Affinity cross\u2010linking experiments showed that fibroblasts of the affect tissue in Proteus syndrome produced an unusual pattern of IGF bindings proteins containing large amounts of an IGFBP with high affinity to IGF\u2010II. The data suggest that IGF production is generally disturbed in Proteus syndrome with imbalanced levels of specific IGFBP in affected tissue. \u00a9 1994 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.1320500213","type":"journal-article","created":{"date-parts":[[2005,6,11]],"date-time":"2005-06-11T17:02:30Z","timestamp":1118509350000},"page":"204-210","source":"Crossref","is-referenced-by-count":21,"title":["Growth hormone (GH), insulin\u2010like growth factors (IGFs), and IGF\u2010binding protein\u20103 (IGFBP\u20103) in a child with Proteus syndrome"],"prefix":"10.1002","volume":"50","author":[{"given":"G\u00fcnter","family":"Rudolph","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Werner F.","family":"Blum","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Enno W.","family":"Jenne","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin","family":"Sch\u00f6ning","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Herbert","family":"Enders","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Meitinger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jan D.","family":"Murken","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anselm","family":"Kampik","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,6,7]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02388283"},{"key":"e_1_2_1_3_1","first-page":"299","volume-title":"Klinische Genetik in der P\u00e4diatrie: 3. 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