Endocrine aspects of growth deficiency in OI.
Growth deficiency is the most common secondary feature of osteogenesis imperfecta. It is unrelated to fracture history and appears to be due to the growth failure of the defective bony matrix. There are characteristic growth curves for different types of OI. We have been investigating the endocrine features of this disorder, in which the skeletal target tissue synthesizes defective matrix. We review the results of our evaluation of the growth hormone axis in 28 children with short stature and OI and of our pilot study to stimulate OI bone to increased growth rates. Our current focus is on the effect of growth hormone treatment on linear growth, bony mineral and bony matrix in OI.
Marini
J C
JC
Human Genetics Branch, NICHD, NIH, Bethesda, MD 20892, USA.
Bordenick
S
S
Chrousos
G P
GP
D000293:Adolescent; D001849:Bone Diseases, Endocrine / Q000188:drug therapy / Q000503:physiopathology*; D001857:Bone Matrix / Q000187:drug effects / Q000378:metabolism / Q000503:physiopathology; D002113:Calcification, Physiologic / Q000187:drug effects / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D003000:Clonidine / Q000494:pharmacology / Q000627:therapeutic use; D005260:Female; D006130:Growth Disorders / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology*; D013006:Growth Hormone / Q000097:blood / Q000172:deficiency / Q000627:therapeutic use*; D013007:Growth Hormone-Releasing Hormone / Q000097:blood; D019382:Human Growth Hormone / Q000031:analogs & derivatives* / Q000494:pharmacology / Q000627:therapeutic use; D006801:Humans; D007030:Hypothalamo-Hypophyseal System / Q000503:physiopathology; D007334:Insulin-Like Growth Factor I / Q000378:metabolism; D008297:Male; D010013:Osteogenesis Imperfecta / Q000188:drug therapy / Q000378:metabolism / Q000503:physiopathology*; D010865:Pilot Projects; D016896:Treatment Outcome; D015854:Up-Regulation / Q000187:drug effects / Q000502:physiology
D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I; C447442:somatrem; D013006:Growth Hormone; D013007:Growth Hormone-Releasing Hormone; D003000:Clonidine