title: Skeletal dysplasia, growth hormone treatment and body proportion: comparison with other syndromic and non-syndromic short children.
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.
authors.affiliation: Paediatric Endocrine Unit, Paediatric Clinic, Karolinska Hospital, Stockholm, Sweden. lars.hagenas@kbh.ki.se
authors.forename: Lars
authors.initials: L
authors.lastname: Hagenäs
authors.forename: Thomas
authors.initials: T
authors.lastname: Hertel
mesh_terms: D000130:Achondroplasia / Q000188:drug therapy / Q000473:pathology; D000293:Adolescent; D000328:Adult; D001827:Body Height / Q000187:drug effects; D001848:Bone Diseases, Developmental / Q000188:drug therapy* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D019382:Human Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D008297:Male; D009634:Noonan Syndrome / Q000188:drug therapy; D012189:Retrospective Studies; D014424:Turner Syndrome / Q000188:drug therapy
chemical_list: D019382:Human Growth Hormone