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Effect of Long-Term Growth Hormone Treatment on Final Height of Children with Russell-Silver Syndrome

Toumba M.a · Albanese A.b · Azcona C.c · Stanhope R.a
aDepartment of Endocrinology, Great Ormond Street Hospital for Children and The University College London Hospitals, bPaediatric Endocrinology Unit, St. George’s Hospital, London, UK; cMetabolic Research Laboratory, Clínica Universitaria de Navarra, University of Navarra, Pamplona, Spain Horm Res Paediatr 2010;74:212–217 (DOI:10.1159/000295924)

Abstract

Background: The aim of this study was to determine the beneficial effects of long-term growth hormone (GH) treatment on final height (FH) in 26 children with Russell-Silver syndrome (RSS). Methods: Twenty-six patients (16 males) were diagnosed with RSS at a median age of 2.9 years according to clinical criteria. All patients were prepubertal at the commencement of treatment. They received treatment with biosynthetic human GH for 9.8 years (median) and all attained FH. Results: The median height at the commencement of treatment was –2.7 SDS and increased to –1.3 SDS (p = 0.001). However, FH did not reach target height (–0.90 SDS, p = 0.003). Predictors of FH outcome were: the height at the start of treatment (r2 = 0.419, p < 0.001) (inversely related) and the height gain at onset of puberty (r2 = 0.257, p < 0.001) (positively related). The overall prediction model accounted for 67.6% of height gain. Sitting height improved gradually during GH treatment (–3.3 to –1.0 SDS, p = 0.012), as did weight (–3.3 to –1.3 SDS, p < 0.001) and BMI (–1.5 to –0.2 SDS, p < 0.001). Conclusions: A significant improvement of growth in RSS children has been shown after 10 years of GH treatment with a FH of –1.3 SDS. The shorter the patient at the start of treatment is, the greater the increment in FH. A significant response is also shown at the onset of puberty. GH treatment may also have a beneficial effect on the spinal length of RSS children.

 

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