Growth in children with 45,Xo Turner's syndrome

Brook, C. G. D.; Mürset, G.; Zachmann, M.; Prader, A.

doi:10.1136/adc.49.10.789

Cited by 157 publications

(42 citation statements)

References 10 publications

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“…Since, however, there are no references available based on BA, we decided to calculate a ‘developmental age’ (DA). This was calculated as the average CA for a given BA in TS [23, 24]. HT SDS for DA at start of GH, onset of puberty, at end of GH treatment and at final height was calculated for the group observed to adult height.…”

Section: Methodsmentioning

confidence: 99%

Experience with Growth Hormone Therapy in Turner Syndrome in a Single Centre: Low Total Height Gain, No Further Gains after Puberty Onset and Unchanged Body Proportions

Schweizer

Ranke²,

Binder³

et al. 2000

Horm Res Paediatr

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The experience gained since 1987, through observation of 85 girls with Turner syndrome under growth hormone (GH) treatment, has enabled the analysis of one of the largest cohorts. Our results show that age, karyotype and height reflect the heterogeneity of the patients examined at our growth centre. In 47 girls, followed over 4 years on GH (median dose 0.72 IU/kg/week), the median age was 9.4 years and mean height SDS was –3.55 (Prader) and –0.14 (Turner-specific), while height and other anthropometrical parameters [weight, body mass index, sitting height (SH), leg length (LL) SH/LL, head circumference, arm span] were documented and compared to normative data as well as to Turner-specific references established on the basis of a larger (n = 165) untreated cohort from Tübingen. The latter data are also documented in this article. Although there was a trend towards normalization of these parameters during the observation period, no inherent alterations in the Turner-specific anthropometric pattern occurred. In 42 girls who started GH treatment at a median age of 11.8 years, final height (bone age >15 years) was achieved at 16.7 years. The overall gain in height SDS (Turner) from start to end of GH therapy was 0.7 (± 0.8) SD, but 0.9 (± 0.6) SD from GH start to onset of puberty (spontaneous 12.2 years, induced 13.9 years) and –0.2 (± 0.8) from onset of puberty to end of growth. Height gain did not occur in 12 patients (29%) and a gain of > 5 cm was only observed in 16 patients (38%). Height gain correlated positively with age at puberty onset, duration, and dose of GH, and negatively with height and bone age at the time GH treatment started. Final height correlated positively with height SDS at GH start and negatively with the ratio of SH/LL (SDS). We conclude that, in the future, GH should be given at higher doses, but oestrogen substitution should be done cautiously, owing to its potentially harmful effect on growth. LL appears to determine height variation in Turner syndrome and the potential to treat short stature successfully with GH.

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Section: Methodsmentioning

confidence: 99%

Experience with Growth Hormone Therapy in Turner Syndrome in a Single Centre: Low Total Height Gain, No Further Gains after Puberty Onset and Unchanged Body Proportions

Schweizer

Ranke²,

Binder³

et al. 2000

Horm Res Paediatr

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“…This may be viewed as a consequence of the cellular hypoplasia of intrauterine growth retardation combined with a postnatal skeletal growth disturbance which is most marked between the ages of 3-13 years (20). Mean adult stature varies among series, but tends to range between 142.5 and 147 cm (21,22). Some series suggest a relationship to karyotype [with the mosaic patients being taller than the 45,X patients (23)] while others, including ourselves, do not see this relationship (22,24).…”

Section: Short Staturementioning

confidence: 98%

Management of Gonadal Dysgenesis

Lippe¹

1985

Journal of Pediatric Endocrinology and Metabolism

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“…Short stature, already present at birth, becomes gradually more apparent throughout childhood, and is exaggerated during adolescence due to an attenuated pubertal growth spurt [2,22]. Thus, spontaneous mean adult height has always been reported to be around 20 cm less than normal women [23,24].…”

Section: Introductionmentioning

confidence: 98%

Final height outcome in girls with Turner syndrome treated with a combination of low dose oestrogen and oxandrolone

Bareille¹,

Massarano

Stanhope³

1997

European Journal of Pediatrics

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Final stature in girls with Turner syndrome treated with combination of low dose oestrogen and oxandrolone. Nineteen prepubertal girls with Turner syndrome (mean age 10.9 years, range, 8.9-14.2 years) were randomly assigned to receive either oxandrolone (0.05 mg/kg/day) or ethinyloestradiol (40 ng/kg/day) for 1 year. Subsequently the alternate therapy was added and the combination given until attainment of final height. Ethinyloestradiol was gradually increased at the age of 12.5 years in order to induce secondary sexual characteristics. The duration of treatment was a mean of 5.2 years (range, 3.7 years) when the 1st year of monotherapy was included. Therapy produced a sustained acceleration in growth rate for a duration of 4 years and eventually has resulted in an increment of mean adult height of 3 cm relative to pre-treatment projected height with mean values of 146.5 cm versus 143.5 cm respectively. The moderate side-effects observed did not cause any of the girls to discontinue treatment. Nevertheless, amelioration of adult height appears to be modest, notably in comparison to published data of growth hormone treatment and 4 girls had a decrease in final height prediction. CONCLUSION Combination of low dose of oxandrolone and oestrogen may have a moderate but positive impact on final height in girls with Turner syndrome. However, some girls do worse than predicted in term of final height using this regimen. Oestrogen therapy started at low dose around the age of 10 years and increased gradually at approximately 12.5 years to induce secondary sexual characteristics does not have a deleterious effect on adult height in Turner syndrome. In summary, low dose oxandrolone-oestrogen treatment was found to accelerate the tempo of growth in girls with Turner syndrome, but did not appear to have a consistent effect on final height.

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Growth in children with 45,Xo Turner's syndrome

Cited by 157 publications

References 10 publications

Experience with Growth Hormone Therapy in Turner Syndrome in a Single Centre: Low Total Height Gain, No Further Gains after Puberty Onset and Unchanged Body Proportions

Experience with Growth Hormone Therapy in Turner Syndrome in a Single Centre: Low Total Height Gain, No Further Gains after Puberty Onset and Unchanged Body Proportions

Management of Gonadal Dysgenesis

Final height outcome in girls with Turner syndrome treated with a combination of low dose oestrogen and oxandrolone

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