European Journal of Pediatrics

, Volume 154, Issue 6, pp 445–449 | Cite as

Circadian growth hormone secretion in short multitransfused prepubertal children with thalassaemia major

  • G. Katzos
  • F. Harsoulis
  • M. Papadopoulou
  • M. Athanasiou
  • K. Sava
Endocrinology Original Paper


Growth hormone (GH) secretion was determined by evaluating circadian GH profiles for 24 h and GH responses to clonidine stimulation test and insulin-stimulated hypoglycaemia (ITT), in nine prepubertal children with β-thalassaemia major (TM) and 17 with non-GH deficient short stature (NGHDSS). The TM children were multitransfused and had early and intensive chelation therapy. All patients had normal hypoglycaemia to ITT, with peak GH levels of 15.71±5.86 ng/ml for children with NGHDSS and 13.91±7.20 ng/ml for children with TM. Peak GH levels during a clonidine test were 17.54±5.30 and 17.15±1.38 ng/ml, respectively. The GH peak parameters such as the number of peaks, the integrated GH concentration and the area under the curve (AUC) were similar in both groups of children and reflected the total 24-h secretion and the daily and nocturnal secretion separately. An abnormal 24-h GH profile compatible with the diagnosis of endogenous neurosecretory GH dysfunction was found in only two out of nine children with TM and in four out of seven children with NGHDSS.


Our data suggest that growth hormone neurosecretory dysfunction is not a universal finding in children with thalassaemia major but might depend on the degree of iron deposit in the pituitary.

Key words

Circadian growth hormone secretion β-Thalassaemia major Non growth hormone deficient short stature 



area under the curve


growth hormone


growth hormone neurosecretory dysfunction


integrated concentration of GH


insulin-like growth factor-I


insulin tolerance test


non GH deficient short stature


thalassaemia major


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  1. 1.
    Bercu BB, Shulman D, Root AW, Spiliotis BE (1986) Growth hormone (GH) provocative testing frequently does not reflect endogenous GH secretion. J Clin Endocrinol Metab 63:709–716PubMedGoogle Scholar
  2. 2.
    Borgna-Pignatti C, De Stefano P, Zonta L, Vullo C, De Sanctis V, Melevendi C, Naselli A, Masera G, Terzoli S, Gabutti V, Piga A (1985) Growth and sexual maturation in thalassemia major. J Pediatr 106:150–155PubMedGoogle Scholar
  3. 3.
    Chatterjee R, Katz M, Cox T, Bantock H (1993) Evaluation of growth hormone in thalassaemic boys with failed puberty: spontaneous versus provocative tests. Eur J Pediatr 152:721–726CrossRefPubMedGoogle Scholar
  4. 4.
    Fink HE (1984) Transfusion hemochromatosis in Cooley's anemia. Ann NY Acad Sci 119:680–683Google Scholar
  5. 5.
    Flynn DM, Fairney A, Jackson D, Clayton BE (1976) Hormonal changes in thalassaemia major. Arch Dis Child 51:828–836PubMedGoogle Scholar
  6. 6.
    Frasier SD, Lippe B (1991) The rational use of growth hormone during childhood. J Clin Endocrinol Metab 71:269–273Google Scholar
  7. 7.
    Greulich WW, Pyle SI (1959) Radiographic atlas of skeletal development of the hand and the wrist. Stanford University Press, StanfordGoogle Scholar
  8. 8.
    Herington AC, Werther GA, Matthews RN, Burger HG (1981) Studies on the possible mechanism for deficiency of non suppressible insuline-like activity in thalassaemia major. J Clin Endocrinol Metab 52:393–398PubMedGoogle Scholar
  9. 9.
    Hintz RL, Suskind R, Amatayakul K, Thanangkul O, Olson R (1978) Plasma somatomedin and growth hormone values in children with protein-calorie malnutrition. J Pediatr 92:153–156PubMedGoogle Scholar
  10. 10.
    Hunder FE, Gebisti JM, Mofftem PE (1983) Swelling and lysis of rat liver mitochondria induced by serious ions. J Biol Chem 238:828–832Google Scholar
  11. 11.
    Kattamis C, Liakopoulou T, Kattamis A (1990) Growth and development in children with thalassaemia major. Acta Paediatr Scand [Suppl] 366:111–117Google Scholar
  12. 12.
    Lassman MN, O'Brien RT, Pearson HA, Wise JK, Donabedian RK, Felig P, Cenel M (1974) Endocrine evaluation in thalassaemia major. Ann NY Acad Sci 232:226–237PubMedGoogle Scholar
  13. 13.
    Loche S (1985) Growth and sexual maturation in thalassaemia. J Pediatr 107:642–643Google Scholar
  14. 14.
    Masala A, Meloni T, Gallisai D, Alagna S, Rovasio PP, Rassu S, Milia AF (1984) Endocrine functioning in multitransfused prepubertal patients with homozygous β-thalassaemia. J Clin Endocrinol Metab 58:667–670PubMedGoogle Scholar
  15. 15.
    McIntosh N (1976) Endocrinopathy in thalassaemia major. Arch Dis Child 51:195–201PubMedGoogle Scholar
  16. 16.
    Merriam GR, Wachter KW (1982) Algorithms for the study of episodic hormone secretion. Am J Physiol 243: 310–318Google Scholar
  17. 17.
    Pintor C, Cella SG, Manso P, Corda R, Dessi C, Locatelli V, Muller EE (1986) Impaired growth hormone (GH) response to GH-releasing hormone in thalassaemia major. J Clin Endocrinol Metab 62:263–267PubMedGoogle Scholar
  18. 18.
    Postel-Vinay MC, Girot R, Leger J, Hocquette JF, Mc Kelvie P, Amar-Costesec A, Rappaport R (1988) No evidence for a defect in GH binding to liver membranes in thalassemia major. J Clin Endocrinol Metab 68:94–98Google Scholar
  19. 19.
    Saenger P, Schwartz F, Markenson AL, Graziano JH, Levine LS, New MI, Hilgartner MW (1980) Depressed serum somatomedin activity in β-thalassemia. J Pediatr 96:214–218PubMedGoogle Scholar
  20. 20.
    Shehadeh N, Hazani A, Rudolf MCJ, Peleg I, Benderly A, Hochberg Z (1990) Neurosecretory dysfunction of growth hormone secretion in thalassemia major. Acta Paediatr Scand 79:790–795PubMedGoogle Scholar
  21. 21.
    Spiliotis BE, August GP, Hung W, Sonis W, Mendelson W, Bercu BB (1984) Growth hormone neurosecretory dysfunction: a treatable cause of short stature. JAMA 251:2223–2230CrossRefPubMedGoogle Scholar
  22. 22.
    Takano K, Hall K, Kastrup KW, Hizuka N, Shizume K, Kawai K, Akimoto M, Takumo T, Sugino N (1979) Serum somatomedin A in chronic renal failure. J Clin Endocrinol Metab 48:371–376PubMedGoogle Scholar
  23. 23.
    Tanner JM, Whitehouse RH, Hughes RCR, Vince FP (1971) Effect of human growth hormone treatment for 1 to 7 years on growth of 100 children with growth hormone deficiency, low birthweight inherited smallness, Turner's syndrome and other complaints. Arch Dis Child 46:745–782PubMedGoogle Scholar
  24. 24.
    Tanner JM, Whitehouse RH, Marshall WA, Healy JR, Goldstein H (1975) Assessment of skeletal maturity and prediction of adult height: TW 2 method. Academic Press, New YorkGoogle Scholar
  25. 25.
    Theochari M, Stamoyannou L, Kafourou A, Nounopoulos H, Bouloukos A, Bartsokas CS (1993) Specific binding of insulin-like growth factor I by erythrocytes of children with thalassemia. Paediatr N Gr 5: 233–237Google Scholar
  26. 26.
    Vannasaeng S, Ploybutr S, Visutcul P, Tandhanand S, Suwanik P, Wasi P (1981) Endocrine function in thalassaemia. Clin Endocrinol 14:165–173Google Scholar
  27. 27.
    Werther GA, Matthews RN, Burger HG, Herington AC (1981) Deficiency of non-supressible insuline-like activity in children with thalassemia major. Arch Dis Child 56:855–860PubMedGoogle Scholar
  28. 28.
    Zadik Z, Kowarski A (1989) Incidence of neurosecretory dysfunction among children aged 6–14 years in Rehovot Israel. Acta Paediatr Scand 349:77–80Google Scholar

Copyright information

© Springer-Verlag 1995

Authors and Affiliations

  • G. Katzos
    • 1
  • F. Harsoulis
    • 2
    • 3
  • M. Papadopoulou
    • 1
  • M. Athanasiou
    • 1
  • K. Sava
    • 1
  1. 1.Department of PaediatricsAristotle University, Hippokration General HospitalThessalonikiGreece
  2. 2.Department of MedicineAristotle University, Hippokration General HospitalThessalonikiGreece
  3. 3.ThessalonikiGreece

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