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Vol. 60, Suppl. 3, 2003
Issue release date: December 2003
Section title: Skeletal Dysplasia
Horm Res 2003;60(suppl 3):65–70
(DOI:10.1159/000074504)

Skeletal Dysplasia, Growth Hormone Treatment and Body Proportion: Comparison with Other Syndromic and Non-Syndromic Short Children

Hagenäs L.a · Hertel T.b
aPaediatric Endocrine Unit, Paediatric Clinic, Karolinska Hospital, Stockholm, Sweden; bDepartment of Paediatrics, Odense University Hospital, Odense, Denmark

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Article / Publication Details

First-Page Preview
Abstract of Skeletal Dysplasia

Published online: 11/17/2004
Issue release date: December 2003

Number of Print Pages: 6
Number of Figures: 3
Number of Tables: 1

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

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

Abstract

Skeletal dysplasias comprise a diverse group of conditions that usually compromise both linear growth and body proportions. It is of theoretical interest to evaluate the effect of GH treatment on linear growth, body proportion and final height in the different skeletal dysplasias. Reported experience of GH treatment in short children with skeletal dysplasia is sparse and often limited to short treatment periods and knowledge of its effects on final height and body proportion is generally lacking. Formal studies are almost all confined to achondroplasia as the most common entity. First-year response is typically a 2–3 cm increase in growth velocity in prepubertal children, or a gain of about 0.5 SDS or less in relative height from a baseline level of –4 to –5 SDS. GH treatment for up to 5 years in achondroplasia can produce a total height gain of about 1 SDS. Apart from achondroplasia, treatment of hypochondroplasia and dyschondrosteosis with GH has been reported in a small number of patients. Long-term data are, however, lacking. Of theoretical interest is that in many syndromic or non-syndromic short-statured children body proportion, i.e. trunk to leg length ratio, does not seem to be dependent on the degree of GH sufficiency and does not seem to be changed by GH treatment. GH treatment, at least in the prepubertal period, does seem to influence degree of disproportion.

© 2003 S. Karger AG, Basel


  

Author Contacts

Dr. Lars Hagenäs
Paediatric Endocrine Unit, Paediatric Clinic
Karolinska Hospital
SE–17176 Stockholm (Sweden)
Tel. +46 8 5177 2367, Fax +46 8 5177 5128 , E-Mail lars.hagenas@kbh.ki.se

  

Article Information

Number of Print Pages : 6
Number of Figures : 3, Number of Tables : 1, Number of References : 23

  

Publication Details

Hormone Research (International Journal of Experimental and Clinical Endocrinology)
Founded 1970 as ‘Hormones’ by M. Marois, Continued 1976 by J. Girard (1976–1995)
Official Organ of the European Society for Paediatric Endocrinology

Vol. 60, No. Suppl. 3, Year 2003 (Cover Date: Released December 2003)

Journal Editor: M.B. Ranke, Tübingen
ISSN: 0301–0163 (print), 1423–0046 (Online)

For additional information: http://www.karger.com/journals/hre


Article / Publication Details

First-Page Preview
Abstract of Skeletal Dysplasia

Published online: 11/17/2004
Issue release date: December 2003

Number of Print Pages: 6
Number of Figures: 3
Number of Tables: 1

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

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


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