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*Turner Syndrome
The Journal of Clinical Endocrinology & Metabolism Vol. 84, No. 12 4607-4612
Copyright © 1999 by The Endocrine Society

Original Studies

Normalization of Height in Girls with Turner Syndrome after Long-Term Growth Hormone Treatment: Results of a Randomized Dose-Response Trial1

Theo C. J. Sas, Sabine M. P. F. de Muinck Keizer-Schrama, Theo Stijnen, Maarten Jansen, Barto J. Otten, J. J. Gera Hoorweg-Nijman, Thomas Vulsma, Guy G. Massa, Catrienus W. Rouwé, H. Maarten Reeser, Willem-Jan Gerver, Jos J. Gosen, Ciska Rongen-Westerlaken and Stenvert L. S. DROP

Department of Pediatrics, Division of Endocrinology (T.C.J.S., S.M.P.F.d.M.K-S., S.L.S.D.) Sophia Children’s Hospital, 3015 GJ Rotterdam; Department of Epidemiology and Biostatistics (T.S.), Erasmus University Rotterdam, 3015 GJ Rotterdam; Wilhelmina Children’s Hospital (M.J.), 3584 EA Utrecht; Sint Radboud University Hospital (B.J.O.), 6525 GA Nijmegen; Free University Hospital (J.J.G.H-N.), 1081 HV Amsterdam; Emma Children’s Hospital, Academic Medical Center (T.V.), 7105 AZ Amsterdam; Medical University Center (G.G.M.), 2333 AA Leiden; Beatrix Children’s Hospital (C.W.R.), 9713 EZ Groningen; Juliana Children’s Hospital (H.M.R.), 2566 ER The Hague; Academic Hospital (W.J.G.), 6202 AZ Maastricht; Rijnland Hospital (J.J.G.), 2353 GA Leiderdorp; Canisius-Wilhelmina Hospital (C.R-W.), 6532 SZ Nijmegen, the Netherlands

Address correspondence and requests for reprints to: Theo Sas, M.D., Division of Endocrinology, Sophia Children’s Hospital, Dr. Molewaterplein 60, 3015 GJ Rotterdam, the Netherlands. E-mail: sas{at}alkg.azr.nl

Short stature and ovarian failure are the main features in Turner syndrome (TS). To optimize GH and estrogen treatment, we studied 68 previously untreated girls with TS, age 2–11 yr, who were randomly assigned to one of three GH dosage groups: group A, 4 IU/m2·day ({approx}0.045 mg/kg·day); group B, first yr 4, thereafter 6 IU/m2·day ({approx}0.0675 mg/kg/day); group C, first yr 4, second yr 6, thereafter 8 IU/m2·day ({approx}0.090 mg/kg·day). In the first 4 yr of GH treatment, no estrogens for pubertal induction were given to the girls. Thereafter, girls started with 17ß-estradiol (5 µg/kg bw·day, orally) when they had reached the age of 12 yr. Subjects were followed up until attainment of adult height or until cessation of treatment because of satisfaction with the height achieved.

Seven-year data of all girls were evaluated to compare the growth-promoting effects of three GH dosages during childhood. After 7 yr, 85% of the girls had reached a height within the normal range for healthy Dutch girls. The 7-yr increment in height SD-score was significantly higher in groups B and C than in group A. In addition, we evaluated the data of 32 of the 68 girls who had completed the trial after a mean duration of treatment of 7.3 yr (range, 5.0 - 8.75). Mean (SD) height was 158.8 cm (7.1), 161.0 cm (6.8), and 162.3 cm (6.1) in groups A, B, and C, respectively. The mean (SD) difference between predicted adult height before treatment and achieved height was 12.5 cm (2.1), 14.5 cm (4.0), and 16.0 cm (4.1) for groups A, B, and C, respectively, being significantly different between group A and group C. GH treatment was well tolerated in all three GH dosage groups.

In conclusion, GH treatment starting in relatively young girls with TS results in normalization of height during childhood, as well as of adult height, in most of the individuals. With this GH and estrogen treatment regimen, most girls with TS can grow and develop much more in conformity with their healthy peers.




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