Effect of Growth Hormone Therapy in Patients with Noonan Syndrome: A Retrospective Study
| Author | Louise Jayne Apperley | en |
| Author | Renuka Ramakrishnan | en |
| Author | Poonam Dharmaraj | en |
| Author | Urmi Das | en |
| Author | Mohammed Didi | en |
| Author | Jo Blair | en |
| Author | Senthil Senniappan | en |
| Orcid | Louise Jayne Apperley [0000-0002-6325-146X] | en |
| Orcid | Renuka Ramakrishnan [0000-0002-9864-5303] | en |
| Orcid | Poonam Dharmaraj [0000-0002-2249-1862] | en |
| Orcid | Urmi Das [0000-0003-3624-8780] | en |
| Orcid | Mohammed Didi [0000-0003-3729-3276] | en |
| Orcid | Jo Blair [0000-0003-3128-5574] | en |
| Orcid | Senthil Senniappan [0000-0002-8001-0342] | en |
| Issued Date | 2020-10-31 | en |
| Abstract | Background: Noonan syndrome is an autosomal dominant condition with an incidence of 1:1000 to 1:2500. The disorder is associated with distinct dysmorphic features, cardiac anomalies, developmental delay and delayed puberty. Short stature is a recognised feature of Noonan syndrome. Objectives: The aim of this study is to assess the effect of growth hormone treatment in patients with Noonan syndrome. Methods: Retrospective data was collected from patients with Noonan syndrome treated with growth hormone. The results were analysed with variables expressed as mean values and standard deviation scores. Results: Twelve Noonan syndrome patients (M: F = 10:2) treated with growth hormone were identified. The mean age of starting growth hormone was 8 years, with baseline height standard deviation score of -2.96 (range: -1.64 to -5.54). The height standard deviation score significantly improved to -2.50 (P = 0.0035) and then -2.22 (P = 0.0025), following one and two years of treatment, respectively. The average height velocity for the patients prior to starting treatment was 5.16cm/year (range: 2.4 - 8.2 cm/year), which significantly improved to 7.76cm/year (ranging from 4.1 to 12.8 cm/year) after one year of growth hormone treatment (P = 0.020) and to 6.51cm/year at the end of two years. Conclusions: Our study has shown that growth hormone treatment significantly improves the height standard deviation score of patients with Noonan syndrome over a two-year course of growth hormone therapy without any side effects. Further research is required to analyse the long-term effect of growth hormone therapy in patients with Noonan syndrome, including the impact on final adult height. | en |
| DOI | https://doi.org/10.5812/ijem.107292 | en |
| Keyword | Growth Hormone | en |
| Keyword | Noonan Syndrome | en |
| Keyword | Short stature | en |
| Publisher | Brieflands | en |
| Title | Effect of Growth Hormone Therapy in Patients with Noonan Syndrome: A Retrospective Study | en |
| Type | Research Article | en |
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