{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T06:26:02Z","timestamp":1772346362937,"version":"3.50.1"},"reference-count":0,"publisher":"Oxford University Press (OUP)","content-domain":{"domain":["eje.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[1998,4,1]]},"abstract":"<jats:p>The causes of growth retardation of children with thalassaemia major are multifactorial. We studied the GH response to provocation by clonidine and glucagon, measured the circulating concentrations of insulin, IGF-I, IGF-binding protein-3 (IGFBP-3) and ferritin, and evaluated IGF-I generation after a single dose of GH (0.1 mg\/kg per dose) in 15 prepubertal patients with thalassaemia, 15 age-matched children with constitutional short stature (CSS) (height standard deviation score less than -2, with normal GH response to provocation) and 11 children with isolated GH deficiency (GHD). Children with thalassaemia had significantly lower peak GH response to provocation by clonidine and glucagon (6.2 +\/- 2.3 and 6.8 +\/- 2.1 microg\/l respectively) than the CSS group (18.6 +\/- 2.7 and 16.7 +\/- 3.7 microg\/l respectively). They had significantly decreased circulating concentrations of IGF-I and IGFBP-3 (47.5 +\/- 19 ng\/ml and 1.2 +\/- 0.27 mg\/l respectively) compared with those with CSS (153 +\/- 42 ng\/ml and 2.06 +\/- 0.37 mg\/l respectively), but the IGF-I and IGFBP-3 concentrations were not different from those with GHD (56 +\/- 25 ng\/ml and 1.1 +\/- 0.32 mg\/l respectively). These data demonstrate that the GH-IGF-I-IGFBP-3 axis in thalassaemic children is defective. Serum ferritin concentration correlated significantly with GH peak response to provocation (r = -0.36, P &lt; 0.05) and circulating IGF-I (r = -0.47, P &lt; 0.01) and IGFBP-3 (r = -0.42, P &lt; 0.01) concentrations. In the IGF-I generation test, after GH injection, the thalassaemic children had significantly lower IGF-I and IGFBP-3 levels 86.7 +\/- 11.2 ng\/ml and 2.05 +\/- 0.51 mg\/l respectively) than those in the CSS group (226 +\/- 45.4 ng\/ml and 2.8 +\/- 0.43 mg\/l respectively). The IGF-I response was significantly higher in children with GHD (158 +\/- 50 ng\/ml) than in thalassaemic children. Six short (height standard deviation score less than -2) thalassaemic children who had defective GH response to provocation (&lt; 10 microg\/l), all the children with GHD and eight short normal children (CSS) were treated for 1 year with human GH (18 units\/m2 per week divided into daily s.c. doses). After 1 year of GH therapy there was a marked acceleration of growth velocity in both thalassaemic children (from 3.8 +\/- 0.6 cm\/year to 7.2 +\/- 0.8 cm\/year) and controls. However, the linear acceleration of growth velocity on GH therapy was significantly slower in thalassaemic children (3.3 +\/- 0.3 cm\/year increment) compared with those with CSS (5.3 +\/- 0.4 cm\/year increment) and GHD (6.9 +\/- 1.2 cm\/year increment) (P &lt; 0.05). Their circulating IGF-I concentration (105 +\/- 36 ng\/ml) was significantly lower than those for CSS (246 +\/- 58 ng\/ml) and GHD (189 +\/- 52 ng\/ml) after 1 year of GH therapy. These data prove that some children with beta-thalassaemia major have a defective GH-IGF-I-IGFBP-3 axis and suggest the presence of partial resistance to GH.<\/jats:p>","DOI":"10.1530\/eje.0.1380394","type":"journal-article","created":{"date-parts":[[2005,1,24]],"date-time":"2005-01-24T21:34:41Z","timestamp":1106602481000},"page":"394-400","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-1","source":"Crossref","is-referenced-by-count":28,"title":["GH response to provocation and circulating IGF-I and IGF-binding protein-3 concentrations, the IGF-I generation test and clinical response to GH therapy in children with beta-thalassaemia"],"prefix":"10.1093","author":[{"given":"AT","family":"Soliman","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N","family":"El Banna","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"BM","family":"Ansari","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","container-title":["European Journal of Endocrinology"],"original-title":[],"link":[{"URL":"https:\/\/eje.bioscientifica.com\/view\/journals\/eje\/138\/4\/394.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/eje.bioscientifica.com\/view\/journals\/eje\/138\/4\/394.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,2,14]],"date-time":"2023-02-14T16:25:07Z","timestamp":1676391907000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ejendo\/article\/138\/4\/394\/6759905"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1998,4,1]]},"references-count":0,"URL":"https:\/\/doi.org\/10.1530\/eje.0.1380394","relation":{},"ISSN":["0804-4643","1479-683X"],"issn-type":[{"value":"0804-4643","type":"print"},{"value":"1479-683X","type":"electronic"}],"subject":[],"published":{"date-parts":[[1998,4,1]]}}}