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Vol. 74, No. 3, 2010
Issue release date: August 2010
Section title: Original Paper
Horm Res Paediatr 2010;74:212–217
(DOI:10.1159/000295924)

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

Toumba M. · Albanese A. · Azcona C. · Stanhope R.
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

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Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: 2/10/2009
Accepted: 4/3/2010
Published online: 4/29/2010

Number of Print Pages: 6
Number of Figures: 3
Number of Tables: 2

ISSN: 1663-2818 (Print)
eISSN: 1663-2826 (Online)

For additional information: http://www.karger.com/HRP

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.


  

Author Contacts

Dr. Meropi Toumba
Pediatric Endocrinology Department, Makarios Hospital
10, Imbrahim Kiazim, CY–8016 Paphos (Cyprus)
Tel. +357 9957 3633, Fax +357 2684 8300
E-Mail meropitoumba@cytanet.com.cy

  

Article Information

Received: February 10, 2009
Accepted: March 4, 2010
Published online: April 29, 2010
Number of Print Pages : 6
Number of Figures : 3, Number of Tables : 2, Number of References : 26

  

Publication Details

Hormone Research in Paediatrics (From Developmental Endocrinology to Clinical Research)

Vol. 74, No. 3, Year 2010 (Cover Date: August 2010)

Journal Editor: Czernichow P. (Paris)
ISSN: 1663-2818 (Print), eISSN: 1663-2826 (Online)

For additional information: http://www.karger.com/HRP


Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: 2/10/2009
Accepted: 4/3/2010
Published online: 4/29/2010

Number of Print Pages: 6
Number of Figures: 3
Number of Tables: 2

ISSN: 1663-2818 (Print)
eISSN: 1663-2826 (Online)

For additional information: http://www.karger.com/HRP


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