Growth hormone - insulin-like growth factor-I axis and bone mineral density in adults with thalassemia major
- PMID: 24701427
- PMCID: PMC3968729
- DOI: 10.4103/2230-8210.126525
Growth hormone - insulin-like growth factor-I axis and bone mineral density in adults with thalassemia major
Abstract
Introduction: Bone disease and short stature are frequent clinical features of patients with beta-thalassaemia major. Dysfunction of the GH-IGF-1 axis has been described in many thalassemics children and adolescents with short stature and reduced growth velocity. Assessment of the GH-IGF-1 axis in short adults with TM after attainment of final height may be required to select those who are candidates for replacement therapy and to prevent the development of bone disease. The aim of our study was to investigate GH secretion in adult thalassemic patients in relation to their bone mineral density (BMD) and serum ferritin concentrations.
Materials and methods: We performed clonidine stimulation test in 30 thalassemic patients (18 males, 12 females) with a mean age of 31.5± 7.2 years. The cut-off level for GH response was set at 7ug/l, according to the literature. Serum ferritin, IGF-I, liver enzymes, alkaline phosphatase (ALP) and type 1 Collagen Carboxy Telopeptide (CCT1) were also determined.
Results: We diagnosed GH deficiency (GHD) in 12 patients (40%) and IGF-I deficiency (IGF-I SDS <-2) was diagnosed in 20 patients (67%). Adult patients with TM had significantly decreased IGF-I concentrations and bone mineral density (BMD) at the femur neck and lumbar spine compared to normal controls. Thalassemic patients with GHD and IGF-I deficiency had significantly lower BMD T score at the lumbar spine compared to patients with normal GH and IGF-I levels. Thalassemic patients had higher serum CCT1 concentrations compared to normal controls. Peak GH levels were correlated significantly with IGF- I concentrations and IGF-I levels were correlated significantly with the height SDS (HtSDS) of thalassemic patients. Neither GH peak nor IGF-I concentrations were correlated to serum ferritin concentrations.
Conclusions: We conclude that GH status should be tested in adult thalassemic patients especially those with short stature and/or decreased BMD. Clonidine test appears to be effective and safe in adults with TM. If the diagnosis of adult GHD is established, GH treatment may be considered for possible improvement of bone mineral density and heart function in patients with TM.
Keywords: Bone mineral density; GH deficiency GHD; ferritin; growth; growth hormone; insulin like growth factor –I; prevalence; thalassemia.
Conflict of interest statement
Figures
Similar articles
-
Final adult height and endocrine complications in young adults with β-thalassemia major (TM) who received oral iron chelation (OIC) in comparison with those who did not use OIC.Acta Biomed. 2018 Feb 16;89(2-S):27-32. doi: 10.23750/abm.v89i2-S.7084. Acta Biomed. 2018. PMID: 29451226 Free PMC article.
-
Growth hormone secretion in adult patients with thalassaemia.Clin Endocrinol (Oxf). 2005 Jun;62(6):667-71. doi: 10.1111/j.1365-2265.2005.02276.x. Clin Endocrinol (Oxf). 2005. PMID: 15943827
-
Bone turnover and mineral density in adult thalassemic patients: relationships with growth hormone secretory status and circulating somatomedins.Endocrine. 2016 Aug;53(2):551-7. doi: 10.1007/s12020-016-0865-1. Epub 2016 Jan 29. Endocrine. 2016. PMID: 26825070
-
Growth and factors affecting it in thalassemia major.Hemoglobin. 2009;33 Suppl 1:S116-26. doi: 10.3109/03630260903347781. Hemoglobin. 2009. PMID: 20001614 Review.
-
New Entity-Thalassemic Endocrine Disease: Major Beta-Thalassemia and Endocrine Involvement.Diagnostics (Basel). 2022 Aug 9;12(8):1921. doi: 10.3390/diagnostics12081921. Diagnostics (Basel). 2022. PMID: 36010271 Free PMC article. Review.
Cited by
-
An ICET-A survey on occult and emerging endocrine complications in patients with β-thalassemia major: Conclusions and recommendations.Acta Biomed. 2019 Jan 15;89(4):481-489. doi: 10.23750/abm.v89i4.7774. Acta Biomed. 2019. PMID: 30657116 Free PMC article.
-
Genotypic and Clinical Analysis of a Thalassemia Major Cohort: An Observational Study.Adv Exp Med Biol. 2021;1339:65-76. doi: 10.1007/978-3-030-78787-5_10. Adv Exp Med Biol. 2021. PMID: 35023092
-
Final adult height and endocrine complications in young adults with β-thalassemia major (TM) who received oral iron chelation (OIC) in comparison with those who did not use OIC.Acta Biomed. 2018 Feb 16;89(2-S):27-32. doi: 10.23750/abm.v89i2-S.7084. Acta Biomed. 2018. PMID: 29451226 Free PMC article.
-
The diagnosis of GH deficiency in adult β-thalassemic patients: are two different stimulation tests necessary to improve specificity?Pituitary. 2025 May 29;28(3):67. doi: 10.1007/s11102-025-01540-1. Pituitary. 2025. PMID: 40442554 Free PMC article.
-
Approach to the management of β thalassemia major associated osteoporosis - A long-standing relationship revisited.Acta Biomed. 2022 Oct 26;93(5):e2022305. doi: 10.23750/abm.v93i5.13668. Acta Biomed. 2022. PMID: 36300213 Free PMC article. Review.
References
-
- Toumba M, Sergis A, Kanaris C, Skordis N. Endocrine complications in patients with Thalassaemia Major. Pediatr Endocrinol Rev. 2007;5:642–8. - PubMed
-
- Delvecchio M, Cavallo L. Growth and endocrine function in thalassemia major in childhood and adolescence. J Endocrinol Invest. 2010;33:61–8. - PubMed
-
- Vidergor G, Goldfarb AW, Glaser B, Dresner-Pollak R. Growth hormone reserve in adult beta thalassemia patients. Endocrine. 2007;31:33–7. - PubMed
-
- Scacchi M, Danesi L, Cattaneo A, Valassi E, Pecori Giraldi F, Argento C, et al. Growth hormone deficiency in adult thalassaemic patients. Clin Endocrinol (Oxf) 2007;67:790–5. - PubMed
-
- La Rosa C, De Sanctis V, Mangiagli A, Mancuso M, Guardabasso V, Galati MC, et al. Growth hormone secretion in adult patients with thalassaemia. Clin Endocrinol (Oxf) 2005;62:667–71. - PubMed
LinkOut - more resources
Full Text Sources
Other Literature Sources