Endocrine aspects of growth deficiency in OI

JC Marini, S Bordenick… - Connective Tissue …, 1995 - Taylor & Francis
JC Marini, S Bordenick, GP Chrousos
Connective Tissue Research, 1995Taylor & Francis
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 …
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
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