Lessons from Laron Syndrome (LS) 1966-1992: A Model of GH and IGF-1 Action and Interaction : Ares-Serono Symposium, Lisbon, May 25-26, 1992

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Zvi Laron, John S. Parks
Karger Medical and Scientific Publishers, Jan 1, 1993 - Medical - 367 pages
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Laron Syndrome (LS) is a disease characterized by resistance to growth hormone (GH) and caused by molecular defects of the GH receptor, leading to a deficiency of insulin-like growth factor-1 (IGF-1) and a significant impairment of growth. The syndrome is a unique human model of a peptide hormone receptor defect and GH-IGF-1 interaction. It offers the opportunity to study the consequences of long-term IGF-1 deficiency and the role of IGF-1 in growth and metabolism. With the recent biosynthesis of IGF-1 and the initiation of IGF-1 therapy, a whole new world of basic biological interactions is opening up. This volume brings together leading researchers in genetics, endocrinology, pediatrics, biochemistry, physiology and pharmacology to summarize what is now known about LS, and to discuss the lessons learned from studying and treating this disease since its initial description in 1966. Special features include the demography of LS, and the first data on the long-term IGF-1 treatment of a large group of patients.
 

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Contents

First Direct Evidence
24
Laron Z Tel Aviv Cognitive
53
An Additional Patient
70
Laron Syndrome A Review of 8 Iranian Patients with
93
The First Reported Case
101
Tchelet A Sakal E Vogel T Rehovot Krivi G G Creely D St Louis Mo
114
Defects of the GH Receptor in Laron Syndrome
127
A Stanford Calif GuevaraAguirre J Quito Rosenbloom A L
140
LongTerm IGF1 Treatment of Children
226
IGF1 Treatment of Adult
237
Nishi Y Hiroshima Okada S Tajiri H Nose O Osaka Hasegawa Y
244
Prévot C Rappaport R Paris Effects of Nutrient
254
Serum Prolactin
261
Animal Models of Abnormal GH Receptor
282
M Leuven TixierBoichard M JouyenJosas Vleurick L Hever
288
Hull K L Harvey S Edmonton Marsh J A Ithaca N Y Boone F McCann
294

Insulin Growth Factor Binding Proteins in Laron Syndrome
167
Interest of IGF1 and IGFBPs
181
Ontogeny
192
The Role of IGF1 in Ovarian Physiology Basic
202
Eshet R Klinger B Tel Aviv Werner H Roberts C T Jr LeRoith D
209
TCell Receptor Activated Lymphocytes Secrete IFG
216
M Darras V M Leuven TixierBoichard M JouyenJosas Van
310
Comparison between hCH and IGF1
319
Divergence
346
Conclusions
359
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