TY - JOUR
T1 - Laron syndrome
T2 - Typical and atypical forms
AU - Woods, K. A.
AU - Savage, M. O.
PY - 1996
Y1 - 1996
N2 - Since the original description of LS by Laron in 1966, this rare condition has continued to assume an importance far beyond the number of affected cases as a unique model of GHR dysfunction. Recently, the potential of therapy with recombinant IGF-I has led to the recruitment and detailed study of patients from a heterogeneous genetic and geographical background and the realization that hereditary GH resistance is itself a heterogeneous condition, with considerable variation in clinical, biochemical and molecular features between patients. For the scientist, further study of the 'atypical' LS patient may lead to important insights into the signalling pathway between GH and IGF-I. For the clinician, the challenge lies in establishing the contribution of inherited forms of GH resistance to the group of children with idiopathic short stature, with the potential of offering therapy to these patients based on an understanding of the aetiological basis of their disorder.
AB - Since the original description of LS by Laron in 1966, this rare condition has continued to assume an importance far beyond the number of affected cases as a unique model of GHR dysfunction. Recently, the potential of therapy with recombinant IGF-I has led to the recruitment and detailed study of patients from a heterogeneous genetic and geographical background and the realization that hereditary GH resistance is itself a heterogeneous condition, with considerable variation in clinical, biochemical and molecular features between patients. For the scientist, further study of the 'atypical' LS patient may lead to important insights into the signalling pathway between GH and IGF-I. For the clinician, the challenge lies in establishing the contribution of inherited forms of GH resistance to the group of children with idiopathic short stature, with the potential of offering therapy to these patients based on an understanding of the aetiological basis of their disorder.
UR - http://www.scopus.com/inward/record.url?scp=0029775038&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0029775038&partnerID=8YFLogxK
U2 - 10.1016/S0950-351X(96)80500-3
DO - 10.1016/S0950-351X(96)80500-3
M3 - Article
C2 - 8853445
AN - SCOPUS:0029775038
SN - 0950-351X
VL - 10
SP - 371
EP - 387
JO - Bailliere's Clinical Endocrinology and Metabolism
JF - Bailliere's Clinical Endocrinology and Metabolism
IS - 3
ER -