Lessons from Laron Syndrome (LS) 1966-1992

Book excerpt: 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.