EAP 2019 Congress and MasterCourse

Impact of Growth Hormone Supplementation on Adult Height in Turner Syndrome

Liliana Sa 1 Sofia Simões Ferreira 2 Maria Adriana Rangel 2 Ana Luísa Leite 2 Rosa Arménia Campos 2
1Pediatrics and Neonatology, Centro Hospitalar de Entre Douro e Vouga, Portugal
2Pediatrics and Neonatology, Centro Hospitalar Vila Nova Gaia / Espinho, Portugal

Background: Short stature is the most common phenotypic feature of Turner Syndrome (TS). Treatment with Growth Hormone (GH) is recommended to increase growth velocity (GV) and final adult height.

Objective: To describe patients with TS who underwent treatment with GH in a level II hospital in the north region of Portugal.

Methods: Retrospective observational study.

Results: Eight patients with TS underwent treatment with GH with a mean age at diagnosis of 4.5 ±3.9 years. Chromosome analysis demonstrated 3 cases of X-monosomy and 5 cases of mosaicism. None had cardiac pathology and 4 had hypothyroidism. Baseline mean height was 106.1 ±16.5 cm (SDS -2.0 ±0.69), with annualized GV of 4.7 ±1.7 cm/year, and adjusted mid-parental height (MPH) of 158.7 ±4,7 cm (SDS -0.56 ±0.78). Treatment with GH was started on average at 6.7 ±3.3 years of age (subcutaneous daily injection, mean dose of 48 mg/Kg/day). Currently, the eight patients, with a mean age of 15.0 ±4.3 years, present a mean height of 145.5 ±10.7 cm (SDS -1.85 ±0.65), with a gain of about 49 ±10.7 cm. Throughout the treatment the difference between the SDS of the MPH and the SDS of the stature decreased (1.52 ±0.70 to 1.29 ±1.22), and five patients reached the expected MPH-SDS. The growth was about 5.7 ±1.3 cm per year of treatment, with the greatest increase during the first year (mean of 9.0 ±1.8 cm). Five patients had already completed treatment at a mean age of 14.6 ±0.9 years. After an average of 9.4 years of treatment they reached a final height of 149.0 ±3.9 cm (SDS -1.85 ±0.66).

Conclusion: Treatment with GH should be instituted as soon as possible in order to optimize adult height.









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