This Article Full Text Full Text (PDF) Submit a related Letter to the Editor Purchase Article View Shopping Cart Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Copyright Permission Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Laron, Z. Search for Related Content PubMed PubMed Citation Articles by Laron, Z.

Laron Syndrome (Primary Growth Hormone Resistance or Insensitivity): The Personal Experience 1958–2003

Endocrinology and Diabetes Research Unit, Schneider Children’s Medical Center, World Health Organization Collaborating Center for the Study of Diabetes in Youth, Tel Aviv University, Tel Aviv 49202, Israel

Address all correspondence and requests for reprints to: Professor Zvi Laron, Endocrinology and Diabetes Research Unit, Schneider Children’s Medical Center, 14 Kaplan Street, Petah Tikva 49202, Israel. E-mail: laronz{at}clalit.org.il.

Clinical and laboratory investigations starting in 1958 of a group of dwarfed children resembling isolated GH deficiency but who had very high serum levels of GH led to the description of the syndrome of primary GH resistance or insensitivity (Laron syndrome) and subsequently to the discovery of its molecular defects residing in the GH receptor and leading to an inability of IGF-I generation. With the biosynthesis of IGF-I in 1986, therapeutic trials started. Continuously more and more patients are being diagnosed in many parts of the world with a variety of molecular defects. This syndrome proved to be a unique model that enables the study of the consequences of GH receptor defects, the physiopathology of GH-IGF-I disruption, and comparison of the GH-independent IGF-I effects. This review presents the personal experience gained from the study follow-up and treatment of the 60 patients followed up for many years in the Israeli cohort.

Abbreviations: CT, Computed tomography; DEXA, dual-energy x-ray absorptiometry; GHBP, GH binding protein; GH-R, GH receptor; hGH, human GH; IGFBP, IGF binding protein; LS, Laron syndrome; MRI, magnetic resonance imaging.

This article has been cited by other articles:

				P. Fang, R. Girgis, B. M. Little, K. L. Pratt, J. Guevara-Aguirre, V. Hwa, and R. G. Rosenfeld Growth Hormone (GH) Insensitivity and Insulin-Like Growth Factor-I Deficiency in Inuit Subjects and an Ecuadorian Cohort: Functional Studies of Two Codon 180 GH Receptor Gene Mutations J. Clin. Endocrinol. Metab., March 1, 2008; 93(3): 1030 - 1037. [Abstract] [Full Text] [PDF]

				P. Fang, S. Riedl, S. Amselem, K. L. Pratt, B. M. Little, G. Haeusler, V. Hwa, H. Frisch, and R. G. Rosenfeld Primary Growth Hormone (GH) Insensitivity and Insulin-Like Growth Factor Deficiency Caused by Novel Compound Heterozygous Mutations of the GH Receptor Gene: Genetic and Functional Studies of Simple and Compound Heterozygous States J. Clin. Endocrinol. Metab., June 1, 2007; 92(6): 2223 - 2231. [Abstract] [Full Text] [PDF]

				L. L. Levitsky Defining the Role of IGF-I Therapy for Short Children J. Clin. Endocrinol. Metab., March 1, 2007; 92(3): 813 - 814. [Full Text] [PDF]

				A. David, C. Camacho-Hubner, A. Bhangoo, S. J. Rose, F. Miraki-Moud, S. A. Akker, G. E. Butler, S. Ten, P. E. Clayton, A. J. L. Clark, et al. An Intronic Growth Hormone Receptor Mutation Causing Activation of a Pseudoexon Is Associated with a Broad Spectrum of Growth Hormone Insensitivity Phenotypes J. Clin. Endocrinol. Metab., February 1, 2007; 92(2): 655 - 659. [Abstract] [Full Text] [PDF]

				D. J. Waxman and C. O'Connor Growth Hormone Regulation of Sex-Dependent Liver Gene Expression Mol. Endocrinol., November 1, 2006; 20(11): 2613 - 2629. [Abstract] [Full Text] [PDF]

				M. J. E. Walenkamp, H. J. van der Kamp, A. M. Pereira, S. G. Kant, H. A. van Duyvenvoorde, M. F. Kruithof, M. H. Breuning, J. A. Romijn, M. Karperien, and J. M. Wit A Variable Degree of Intrauterine and Postnatal Growth Retardation in a Family with a Missense Mutation in the Insulin-Like Growth Factor I Receptor J. Clin. Endocrinol. Metab., August 1, 2006; 91(8): 3062 - 3070. [Abstract] [Full Text] [PDF]

				J. D. Veldhuis, J. N. Roemmich, E. J. Richmond, and C. Y. Bowers Somatotropic and Gonadotropic Axes Linkages in Infancy, Childhood, and the Puberty-Adult Transition Endocr. Rev., April 1, 2006; 27(2): 101 - 140. [Abstract] [Full Text] [PDF]

				K Iida, C J Rosen, C Ackert-Bicknell, and M O Thorner Genetic differences in the IGF-I gene among inbred strains of mice with different serum IGF-I levels J. Endocrinol., September 1, 2005; 186(3): 481 - 489. [Abstract] [Full Text] [PDF]

				L. V. Ferreira, S. A. L. Souza, I. J. P. Arnhold, B. B. Mendonca, and A. A. L. Jorge PTPN11 (Protein Tyrosine Phosphatase, Nonreceptor Type 11) Mutations and Response to Growth Hormone Therapy in Children with Noonan Syndrome J. Clin. Endocrinol. Metab., September 1, 2005; 90(9): 5156 - 5160. [Abstract] [Full Text] [PDF]