title: Defective growth hormone secretion in children with pycnodysostosis and improved linear growth after growth hormone treatment.
abstract: Short stature is a characteristic feature of pycnodysostosis. We report defective growth hormone secretion in response to provocation and low insulin-like growth factor-I (IGF-I) concentration in five out of six patients with pycnodysostosis. Physiological replacement with growth hormone increased IGF-I concentration and improved linear growth in these children.
authors.affiliation: Department of Paediatrics, Royal Hospital, Muscat, Oman.
authors.forename: A T
authors.initials: AT
authors.lastname: Soliman
authors.forename: A
authors.initials: A
authors.lastname: Rajab
authors.forename: I
authors.initials: I
authors.lastname: AlSalmi
authors.forename: A
authors.initials: A
authors.lastname: Darwish
authors.forename: M
authors.initials: M
authors.lastname: Asfour
mesh_terms: D000328:Adult; D001848:Bone Diseases, Developmental / Q000378:metabolism* / Q000503:physiopathology / Q000628:therapy; D002675:Child, Preschool; D005260:Female; D006128:Growth / Q000502:physiology; D006130:Growth Disorders / Q000378:metabolism* / Q000503:physiopathology / Q000628:therapy; D013006:Growth Hormone / Q000097:blood / Q000378:metabolism* / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007334:Insulin-Like Growth Factor I / Q000378:metabolism; D008297:Male
chemical_list: D007334:Insulin-Like Growth Factor I; D013006:Growth Hormone