Defective growth hormone secretion in children with pycnodysostosis and improved linear growth after growth hormone treatment.
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.
Soliman
A T
AT
Department of Paediatrics, Royal Hospital, Muscat, Oman.
Rajab
A
A
AlSalmi
I
I
Darwish
A
A
Asfour
M
M
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
D007334:Insulin-Like Growth Factor I; D013006:Growth Hormone