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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.

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