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What are the current guidelines for the management of growth hormone therapy in children with Turner Syndrome?
Answer
Current guidelines recommend initiating growth hormone (GH) therapy in children with Turner syndrome as early as possible, ideally in early childhood, to optimise final adult height. GH therapy is considered the mainstay of treatment to improve growth velocity and adult stature in these patients 1.
Dosage and monitoring: The recommended GH dose is typically higher than that used for GH deficiency, often around 0.045–0.05 mg/kg/day, administered via daily subcutaneous injections. Treatment response should be monitored regularly through growth velocity measurements and IGF-1 levels to adjust dosing and ensure efficacy and safety 1.
Timing of therapy: GH therapy is usually started once growth failure is evident, often before the age of 4 years, but can be considered even earlier if growth is significantly impaired. Early initiation maximises height gain potential 1.
Adjunctive therapies and considerations: Estrogen replacement therapy, which is necessary for pubertal development, should be carefully timed to avoid premature epiphyseal closure that could limit height gain. Estrogen is typically introduced at a low dose around 11–12 years of age, after a period of GH therapy, to balance growth and pubertal development 1 (Warren and Chua, 2006; Gonzalez and Witchel, 2012).
Safety and long-term management: Regular monitoring for potential side effects such as glucose intolerance, intracranial hypertension, and scoliosis is advised. Multidisciplinary care including endocrinology, cardiology, and psychology is essential for comprehensive management 1 (Kriksciuniene et al., 2016).
In summary, current UK guidelines emphasise early initiation of GH therapy at appropriate doses with careful monitoring, combined with timed estrogen replacement to optimise growth and pubertal outcomes in children with Turner syndrome 1. Recent literature supports these recommendations and highlights the importance of individualising therapy based on growth response and pubertal timing (Warren and Chua, 2006; Gonzalez and Witchel, 2012; Kriksciuniene et al., 2016).
Key References
- NG145 - Thyroid disease: assessment and management
- (Warren and Chua, 2006): Appropriate use of estrogen replacement therapy in adolescents and young adults with Turner syndrome and hypopituitarism in light of the Women's Health Initiative.
- (Gonzalez and Witchel, 2012): The patient with Turner syndrome: puberty and medical management concerns.
- (Kriksciuniene et al., 2016): The current management of Turner Syndrome.
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