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Article: Serum growth hormone (GH) binding protein, IGF-I and IGFBP-3 in patients with β-thalassaemia major and the effect of GH treatment

TitleSerum growth hormone (GH) binding protein, IGF-I and IGFBP-3 in patients with β-thalassaemia major and the effect of GH treatment
Authors
Issue Date1998
PublisherWiley-Blackwell Publishing Ltd.. The Journal's web site is located at http://www.wiley.com/bw/journal.asp?ref=0300-0664
Citation
Clinical Endocrinology, 1998, v. 48 n. 5, p. 641-646 How to Cite?
AbstractOBJECTIVE: Levels of IGFI have been shown to be low in transfusion- dependent thalassaemia and there is preliminary evidence to suggest that this may be reversed by GH treatment. In this further study we have evaluated serum growth hormone (GH) binding protein (GHBP), IGF-I and IGFBP-3 in patients with β-thalassaemia major and the effects of GH treatment on these various parameters. PATIENTS: Fifty-six transfusion dependent patients with β-thalassaemia major without GH deficiency between 2 and 20 years of age were studied. Thirteen non-GH deficient patients with heights of -1.5 SD or more were treated with GH at a dose of 0.14 IU/kg/day subcutaneously for 1 year. MEASUREMENTS: Serum GHBP, IGF-I and IGFBP-3 were measured in all the patients. In the 13 patients treated with GH, these serum parameters were measured before and after 3, 6 and 12 months of treatment. RESULTS: The mean serum GHBP concentrations were normal in both prepubertal and pubertal children but the serum IGF-I and IGFBP-3 concentrations were low throughout childhood and adolescence. There was a significant correlation between serum IGF-I and IGFBP-3 concentrations (r=0.79; P=0.0001) but there was no correlation between the height SDS of the patients with serum GHBP, IGF-I or IGFBP-3 levels. GH treatment in the 13 patients resulted in significant growth acceleration associated with a significant rise in the serum IGF-I and IGFBP-3 and a significant fall in serum GHBP concentrations. CONCLUSIONS: The low serum concentrations of IGFI and IGFBP-3 in the presence of normal GH reserve and serum GHBP concentrations in patients with β-thalassaemia suggest a state of partial GH insensitivity at the post-receptor level. This partial GH insensitivity state can be overcome by supraphysiological doses of exogenous GH. The lack of correlation of IGF-I, IGFBP-3 and GHBP with height SDS of the patients imply that the growth failure commonly observed in patients with β-thalassaemia major may not be specifically related to dysregulation of the GH-IGF-I axis. GH therapy resulted in significant increase in serum IGF-I and IGFBP-3 but a significant fall in GHBP.
Persistent Identifierhttp://hdl.handle.net/10722/170289
ISSN
2015 Impact Factor: 3.487
2015 SCImago Journal Rankings: 1.314
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorLow, LCKen_HK
dc.contributor.authorPostelVinay, MCen_HK
dc.contributor.authorKwan, EYWen_HK
dc.contributor.authorCheung, PTen_HK
dc.date.accessioned2012-10-30T06:07:15Z-
dc.date.available2012-10-30T06:07:15Z-
dc.date.issued1998en_HK
dc.identifier.citationClinical Endocrinology, 1998, v. 48 n. 5, p. 641-646en_HK
dc.identifier.issn0300-0664en_HK
dc.identifier.urihttp://hdl.handle.net/10722/170289-
dc.description.abstractOBJECTIVE: Levels of IGFI have been shown to be low in transfusion- dependent thalassaemia and there is preliminary evidence to suggest that this may be reversed by GH treatment. In this further study we have evaluated serum growth hormone (GH) binding protein (GHBP), IGF-I and IGFBP-3 in patients with β-thalassaemia major and the effects of GH treatment on these various parameters. PATIENTS: Fifty-six transfusion dependent patients with β-thalassaemia major without GH deficiency between 2 and 20 years of age were studied. Thirteen non-GH deficient patients with heights of -1.5 SD or more were treated with GH at a dose of 0.14 IU/kg/day subcutaneously for 1 year. MEASUREMENTS: Serum GHBP, IGF-I and IGFBP-3 were measured in all the patients. In the 13 patients treated with GH, these serum parameters were measured before and after 3, 6 and 12 months of treatment. RESULTS: The mean serum GHBP concentrations were normal in both prepubertal and pubertal children but the serum IGF-I and IGFBP-3 concentrations were low throughout childhood and adolescence. There was a significant correlation between serum IGF-I and IGFBP-3 concentrations (r=0.79; P=0.0001) but there was no correlation between the height SDS of the patients with serum GHBP, IGF-I or IGFBP-3 levels. GH treatment in the 13 patients resulted in significant growth acceleration associated with a significant rise in the serum IGF-I and IGFBP-3 and a significant fall in serum GHBP concentrations. CONCLUSIONS: The low serum concentrations of IGFI and IGFBP-3 in the presence of normal GH reserve and serum GHBP concentrations in patients with β-thalassaemia suggest a state of partial GH insensitivity at the post-receptor level. This partial GH insensitivity state can be overcome by supraphysiological doses of exogenous GH. The lack of correlation of IGF-I, IGFBP-3 and GHBP with height SDS of the patients imply that the growth failure commonly observed in patients with β-thalassaemia major may not be specifically related to dysregulation of the GH-IGF-I axis. GH therapy resulted in significant increase in serum IGF-I and IGFBP-3 but a significant fall in GHBP.en_HK
dc.languageengen_US
dc.publisherWiley-Blackwell Publishing Ltd.. The Journal's web site is located at http://www.wiley.com/bw/journal.asp?ref=0300-0664en_HK
dc.relation.ispartofClinical Endocrinologyen_HK
dc.rightsClinical Endocrinology. Copyright © Blackwell Publishing Ltd.-
dc.subject.meshAdolescenten_US
dc.subject.meshAdulten_US
dc.subject.meshCarrier Proteins - Blooden_US
dc.subject.meshChilden_US
dc.subject.meshChild, Preschoolen_US
dc.subject.meshFemaleen_US
dc.subject.meshGrowth Hormone - Blood - Therapeutic Useen_US
dc.subject.meshHumansen_US
dc.subject.meshInsulin-Like Growth Factor Binding Protein 3 - Analysisen_US
dc.subject.meshInsulin-Like Growth Factor I - Analysisen_US
dc.subject.meshMaleen_US
dc.subject.meshBeta-Thalassemia - Blood - Drug Therapyen_US
dc.titleSerum growth hormone (GH) binding protein, IGF-I and IGFBP-3 in patients with β-thalassaemia major and the effect of GH treatmenten_HK
dc.typeArticleen_HK
dc.identifier.emailLow, LCK: lcklow@hkucc.hku.hken_HK
dc.identifier.emailCheung, PT: ptcheung@hku.hken_HK
dc.identifier.authorityLow, LCK=rp00337en_HK
dc.identifier.authorityCheung, PT=rp00351en_HK
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.doi10.1046/j.1365-2265.1998.00470.xen_HK
dc.identifier.pmid9666877-
dc.identifier.scopuseid_2-s2.0-0031778827en_HK
dc.identifier.hkuros31881-
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-0031778827&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume48en_HK
dc.identifier.issue5en_HK
dc.identifier.spage641en_HK
dc.identifier.epage646en_HK
dc.identifier.isiWOS:000073762400014-
dc.publisher.placeUnited Kingdomen_HK
dc.identifier.scopusauthoridLow, LCK=7007049461en_HK
dc.identifier.scopusauthoridPostelVinay, MC=7006010106en_HK
dc.identifier.scopusauthoridKwan, EYW=7006484387en_HK
dc.identifier.scopusauthoridCheung, PT=7202595465en_HK

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