Glucose Metabolism in Ullrich Turner Syndrome: Long-Term Effects of Therapy with Human Growth Hormone

Weise, M.; James, David F.; Leitner, Chr.; Hartmann, K.; Böhles, H.; Attanasio, A.

doi:10.1159/000182692

Cited by 31 publications

(21 citation statements)

References 15 publications

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“…Our 4-yr results of the effects of GH treatment on lipid metabolism showed no unwanted side effects (22). As described in other studies, GH treatment had no adverse effect on glucose metabolism, but the insulin levels had increased significantly after 4 yr of GH treatment compared to baseline levels (22)(23)(24). In the present study, we showed that during 7 yr of treatment glycosylated hemoglobin levels stayed within the normal range; an extensive evaluation of the effects of long-term GH treatment on carbohydrate metabolism is described elsewhere (24a).…”

Section: Discussionsupporting

confidence: 82%

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

Sas¹

1999

Journal of Clinical Endocrinology & Metabolism

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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/ m 2 ⅐day (Ϸ0.045 mg/kg⅐day); group B, first yr 4, thereafter 6 IU/m 2 ⅐day (Ϸ0.0675 mg/kg/day); group C, first yr 4, second yr 6, thereafter 8 IU/m 2 ⅐day (Ϸ0.090 mg/kg⅐day). In the first 4 yr of GH treatment, no estrogens for pubertal induction were given to the girls. Thereafter, girls started with 17␤-estradiol (5 g/kg bw⅐day, 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 growthpromoting 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. (J Clin Endocrinol Metab 84: 4607-4612, 1999)

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Section: Discussionsupporting

confidence: 82%

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

Sas¹

1999

Journal of Clinical Endocrinology & Metabolism

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“…The participants were well matched for weight and body mass index (BMI), although dual densitometry x-ray (DEXA) scans showed significant differences in regional adiposity (142). Most studies have also found a relative impairment of glucose tolerance or found insulin resistance during GH treatment (154,157,165,166), while others have not (167). However, most agree that GH treatment induces insulin resistance, an effect that is reversible with discontinuation of treatment (166).…”

Section: Growth Promoting Treatment In Turner Syndrome -Growth Hormonmentioning

confidence: 99%

Epidemiological, endocrine and metabolic features in Turner syndrome

2004

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Turner syndrome is one of the more common genetic disorders, associated with abnormalities of the X chromosome, and occurring in about 50 per 100 000 liveborn girls. Turner syndrome is usually associated with reduced adult height, gonadal dysgenesis and thus insufficient circulating levels of female sex steroids, and infertility. A number of other signs and symptoms are seen more frequently with the syndrome. Morbidity and mortality are increased. The average intellectual performance is within the normal range. A number of recent studies have provided new insights with respect to epidemiology, cardiology, endocrinology and metabolism. Treatment with GH during childhood and adolescence allows a considerable gain in adult height, although verylong-term consequences of this treatment are not clear. Puberty has to be induced in most cases, and female sex hormone replacement therapy is given during the adult years. The proper dose of hormone replacement therapy (HRT) has not been established, and, likewise, benefits and/or drawbacks from HRT have not been thoroughly evaluated. Since the risk of cardiovascular and endocrinological disease is clearly elevated, proper care during adulthood is emphasized. In summary, Turner syndrome is a condition associated with a number of diseases and conditions which are reviewed in the present paper.

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“…Girls with TS have an even higher incidence of impaired glucose tolerance [2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12]and, therefore, an increased risk of developing diabetes mellitus in adulthood [2, 3, 5, 6, 13]. Stoppoloni et al [14]showed an increase in insulin resistance in 4 patients with TS (age 13–21 years) using an euglycaemic hyperinsulinaemic glucose clamp technique.…”

Section: Introductionmentioning

confidence: 99%

Effect of Growth Hormone and Oxandrolone Treatment on Glucose Metabolism in Turner Syndrome

Joss

Zurbrügg²,

Tönz³

et al. 2000

Horm Res Paediatr

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In 18 girls with Turner syndrome, glucose tolerance was studied before treatment and after 6 and 24 months of growth-promoting treatment with recombinant human growth hormone (24 IU/m²/week; 8 mg/m²/week) and oxandrolone (0.06 mg/kg/day), as well as after termination of the treatment. One girl developed an overt non-ketotic diabetes mellitus 50 months after termination of treatment. The results of the remaining 17 girls in whom the effect of treatment on glucose metabolism was reversible are presented as a group. Their median age at the beginning of the treatment was 10.4 years (range 6.9–15.9), and 15.0 years (range 12.1–19.9) at the final assessment. There was a moderate, but not significant rise in fasting glucose throughout the course of the longitudinal study. At oral glucose tolerance testing (oGTT), the area under the curve for glucose rose significantly (p = 0.013) during the period of treatment and returned to the basic value thereafter. Fasting insulin and especially the integrated insulin values (AUCi; area under the curve for insulin) during oGTT increased continuously during treatment, declined after termination of treatment but were still significantly (p = 0.04) higher than before treatment. Considering the fact that in untreated girls with Turner syndrome the fasting insulin and the AUCi increase with age, one can conclude that the insulinaemia returned to age-specific norm after termination of treatment. Thus the effect of a combined growth hormone and oxandrolone growth-promoting treatment on glucose metabolism was fully reversible in these 17 girls with Turner syndrome.

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Glucose Metabolism in Ullrich Turner Syndrome: Long-Term Effects of Therapy with Human Growth Hormone

Cited by 31 publications

References 15 publications

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

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

Epidemiological, endocrine and metabolic features in Turner syndrome

Effect of Growth Hormone and Oxandrolone Treatment on Glucose Metabolism in Turner Syndrome

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