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Early treatment with GH alone in Turner syndrome: prepubertal catch-up growth and waning effect
Malgorzata Wasniewska1, Filippo De Luca, Rosalba Bergamaschi
1Department of Pediatrics, University of Messina, Messina, Italy.
Insights
Early growth hormone (GH) treatment in girls with Turner syndrome (TS) significantly improves height gain before puberty. This strategy normalizes height, allowing for appropriate pubertal induction without accelerating bone maturation.
Area of Science:
- Pediatric Endocrinology
- Genetics and Genetic Diseases
- Growth Disorders
Background:
- Turner syndrome (TS) is a genetic condition affecting females, often characterized by short stature.
- Early intervention is crucial for optimizing growth outcomes in children with TS.
- Growth hormone (GH) therapy is a standard treatment for TS-related growth failure.
Purpose of the Study:
- To evaluate the benefits of early growth hormone (GH) treatment in Turner syndrome (TS).
- To assess prepubertal height gain in girls with TS receiving a consistent GH dosage.
- To determine the long-term effects of early GH therapy on projected adult height and pubertal timing.
Main Methods:
- A prospective study involving 29 prepubertal girls with TS (age < 6 years).
- Participants received biosynthetic GH at 1.0 IU/kg per week for at least 5 years before puberty.
- Regular measurements of height, height velocity (HV), and projected adult height (PAH) were conducted.
Main Results:
- Significant height acceleration was observed in the first year of GH treatment.
- Height deficiency decreased from -2.4 SDS to -1.0 SDS after 5 years.
- Projected adult height increased, though remained below target height; no accelerated bone maturation was noted.
Conclusions:
- Early GH treatment is an effective strategy for promoting growth in Turner syndrome.
- Prepubertal height normalization is achievable, facilitating appropriate pubertal induction.
- A GH dose of 1.0 IU/kg/week is suitable, with diminishing effects noted after the fourth year.
Objective:
In order to ascertain the advantages of early GH treatment in Turner syndrome (TS), we started a prospective study aimed at evaluating prepubertal height gain in a cohort of 29 girls who were treated with the same pro-kilo GH dose (1.0 IU/kg per week) since they were less than 6 years old and for at least 5 years before entering puberty.
Patients And Design:
Following a minimum of 6 months of baseline observations, 29 girls with TS were enrolled for this prospective study provided that they (a) were less than 6 years old, (b) were below -1.0 standard deviation score (SDS) for height, (c) had a projected adult height (PAH) lower than the respective target height (TH) and (d) had a height velocity (HV) lower than -1.0 SDS. All the selected girls underwent a 5-year treatment with biosynthetic GH at a stable dose of 1.0 IU/kg per week and were periodically measured during the treatment period in order to evaluate height, HV and PAH.
Results:
After a dramatic acceleration during the 1st year, HV was attenuated during the subsequent years, reaching its nadir at the 5th year. Height deficiency under therapy progressively decreased from entry onwards, shifting from -2.4+/-0.7 to -1.0+/-1.2 SDS. In the same period, mean PAH progressively increased, although after 5 years it remained lower than the average TH.
Conclusions:
(a) An effective growth-promoting strategy in TS should be based on early GH treatment, as suggested by our results. (b) This strategy could result in a prepubertal normalization of height, thus allowing the appropriate timing for the induction of puberty. (c) An initial GH dose of 1.0 IU/kg per week may be suitable during the first years of therapy, as shown by our data documenting an important waning effect of GH therapy only after the 4th year of treatment. (d) No acceleration of bone maturation was observed under this treatment regimen.
