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European Journal of Pediatrics

, Volume 153, Issue 2, pp 72–77 | Cite as

Growth hormone and 17β-oestradiol treatment of Turner girls — 2-year results

  • Rune W. Naeraa
  • Johannes Nielsen
  • Knud W. Kastrup
Endocrinology Original Paper
  • 23 Downloads

Abstract

Girls with Turner syndrome are mainly characterized by growth retardation and gonadal insufficiency. In order to evaluate the effect of growth hormone (GH) and/or low dose 17β-oestradiol (E2) on growth and pubertal development, 39 Turner girls with a chronological age (CA) of 7.6–18.1 years were divided into three groups depending on pretreatment bone age (BA). They were treated with either GH 0.1 IE/kg per day (n=13, BA 7.1–10.2), peroral E2 0.01 mg/kg per day (n=8, BA 8.5–12.7) or both (n=18, BA 10.5–15.3). In the 2nd year the E2 group also received GH, while the E2 dose was reduced 30%. In the 1st year height velocity (HV) expressed as standard deviation scores (SDS) increased in all groups (mean): from −0.4 to 3.3 (P<0.01) in the GH group, −0.5 to 2.7 (P<0.01) in the E2 group, and −0.8 to 4.6 (P<0.001) in the GH +E2 group. A possible synergistic effect from combination therapy was seen, as HV increase was higher in group 3 than groups 1 and 2 (P<0.05). In the 2nd year HV was unchanged in groups 1 and 2, while a clear decrease was seen in the GH+E2 group (P<0.001). In the 1st year BA progression in the E2 group was rapid (1.9 BA/CA year) and higher than in the other groups (P<0.05). In the 2nd year progression slowed down-particularly in the E2 group (0.7 BA/CA year,P=0.07). Predicted final height (PFH) increased gradually over the 2 years in the GH group up to 4.1 cm (P<0.01), while in the GH+E2 group PFH increased 2.6 cm (P=0.001) after 1 year, but progressed no further during the 2nd year. In the E2 group PFH decreased −3.1 cm (P=0.055) during the 1st year, while in the 2nd year PFH returned to pretreatment levels. Insulin-like growth factor (IGF-I) increased during the 1st year in the GH and the GH+E2 groups (P<0.01 andP<0.05), while it was unchanged in the E2 group (P>0.4). Except for some of the youngest and oldest girls, breast development was appropriate. No major side-effects were noted. In one girl growth of naevi was noted, but they disappeared spontaneously within 3 months. We conclude that GH and peroral E2 both stimulate HV and appear to have a synergistic effect. The effect of peroral E2 is not mediated by increasing IGF-I. When BA is below 11 years, E2 leads to an accelerated BA maturation, which potentially decreases final height. An initial dose lower than 10 μg E2/kg per day and a minimum BA of 11 years are recommended to initiate treatment.

Key words

Turner syndrome Growth stimulation Oestradiol Growth hormone IGF-I 

Abbreviations

BA

bone age

CA

chronological age

E2

17β-oestradiol

EE

ethinyl-oestradiol

GH

human growth hormone

HV

height velocity

IGF-I

insulin-like growth factor

PFH

predicted final height

SDS

standard deviation scores

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References

  1. 1.
    Consensus Development Conference (1987) Prophylaxis and treatment of osteoporosis Br Med J 295:914–915Google Scholar
  2. 2.
    Grever WJM, Drayer NM, Es A van (1992) Does growth hormone treatment of patients with Turner's syndrome cause an abnormal body shape? Acta Paediatr 81:691–694Google Scholar
  3. 3.
    Hall JG (1989) The management of the adult with Turner syndrome. In: Rosenfeld RG, Grumbach MM (eds) Turner syndrome, Marcel Dekker Inc, New York, pp 393–403Google Scholar
  4. 4.
    Illig R, DeCampo C, Lang-Muritano MR, Prader A, Torresani T, Werder EA, Willi U, Schenkel L (1990) A physiological mode of puberty induction in hypogonadal girls by low dose transdermal 17-β-oestradiol. Eur J Pediatr 150:86–91Google Scholar
  5. 5.
    Joss EE (1988) Anabolic steroids in girls with Turner's syndrome. Acta Paediatr Scand [Suppl 343]:38–42Google Scholar
  6. 6.
    Karlberg J, Albertsson-Wikland K, Naeraa RW (1991) The infancy-childhoodpuberty (ICP) model of growth for Turner girls. In: Ranke M, Rosenfeld RG (eds) Turner syndrome: growth promoting therapies. Proceedings of an International Workshop. Excerpta Medica, Amsterdam, pp 89–94Google Scholar
  7. 7.
    Kastrup KW and the Turner Study Group (1988) Oestrogen therapy in Turner's syndrome. Acta Paediatr Scank [Suppl 343]:43–46Google Scholar
  8. 8.
    Naeraa RW, Nielsen J (1990) Standards for growth and final height in Turner's syndrome. Acta Paediatr Scand 79:182–190Google Scholar
  9. 9.
    Naeraa RW, Eiken M, Legarth EG, Nielsen J (1990) Prediction of final height in Turner's syndrome — a comparative study. Acta Paediatr Scand 79:776–783Google Scholar
  10. 10.
    Naeraa RW, Nielsen J, Pedersen IL, Sørensen K (1990) Effect of Oxandrolone on growth and final height in Turner's syndrome. Acta Paediatr Scand 79:784–789Google Scholar
  11. 11.
    Nielsen J, Sillesen I (1981) Turner's syndrome in 115 Danish girls born between 1955 and 1966. Acta Jutlandica 59, Aarhus University - Det l≸rde Selskab, AarhusGoogle Scholar
  12. 12.
    Nilsson KO, Wattsgård C, Alberttson-Wikland K, Aronson S, Gustafsson J, Hagenäs L, Häger A, Ivarsson SA, Moell C, Ritzén M, Tuvemo T, Westgren U, Westphal O (1991) Growth promoting treatment in Turner's syndrome — three year experience in Sweden. In: Ranke M, Rosenfeld RG (eds) Turner syndrome: growth promoting therapies. Proceedings of an International Workshop. Excerpta Medica, Amsterdam, pp 215–220Google Scholar
  13. 13.
    Ranke MB, Pflüger H, Rosendahl W, Stubbe P, Enders H, Bierich JR, Majewski F (1983) Turner's syndrome: spontaneous growth in 150 cases and review of the literature. Eur J Pediatr 141:81–88Google Scholar
  14. 14.
    Rosenfield RL (1983) Toward optimal estrogen replacement therapy [editorial]. N Engl J Med 309:1120–1121Google Scholar
  15. 15.
    Rosenfeld RG, Hintz RL, Johanson AJ, Sherman B, Brasel JA, Burstein S, Chernausek S, Compton P, Frane J, Gotlin RW, Kuntze J, Lippe BM, Mahoney PC, Moore WV, New MI, Saenger P, Sybert V (1988) Three-year results of a randomized prospective trial of methionyl human growth hormone and oxandrolone in Turner syndrome. J Pediatr 113:393–400Google Scholar
  16. 16.
    Ross JL, LM Long, M Skerda, Cassorla RG, Kurtz D, Loriaux DL, Cutler GB Jr (1986) Effect of low doses of estradiol on 6-month growth rates and predicted height in patients with Turner syndrome. J Pediatr 109:950–953Google Scholar
  17. 17.
    Vanderschueren-Lodeweyckx M, Massa G, Maes M, Craen M, Vliet G van, Heinrich C, Malvaux P (1990) Growth-promoting effect of growth hormone and low dose ethinyl estradiol in girls with Turner's syndrome. J Clin Endocrinol Metab 70:122–126Google Scholar
  18. 18.
    Walker JM, Bond SA, Voss LD, Betts PR, Wootton SA, Jackson AA (1990) Treatment of short normal children with growth hormone — a cautionary tale. Lancet 336:1331–1334Google Scholar
  19. 19.
    Werther G (1991) A multi-centre double-blind study of growth hormone and low-dose estrogen in Turner syndrome: an interim analysis. In: Ranke M, Rosenfeld RG (eds) Turner syndrome: growth promoting therapies. Preceedings of an International Workshop. Excerpta Medica, Amsterdam, pp 263–268Google Scholar

Copyright information

© Springer-Verlag 1994

Authors and Affiliations

  • Rune W. Naeraa
    • 1
  • Johannes Nielsen
    • 2
    • 3
  • Knud W. Kastrup
    • 4
  1. 1.Department of Internal Medicine M (Endocrinology and Diabetes), KommunehospitaletAarhus University HospitalAarhusDenmark
  2. 2.The Turner Centre, Aarhus Psychiatric HospitalAarhus University HospitalRisskovDenmark
  3. 3.Cytogenetic Laboratory, Aarhus Psychiatric HospitalAarhus University HospitalRisskovDenmark
  4. 4.Department of PaediatricsCopenhagen County HospitalGlostrupDenmark

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