Laron syndrome – A historical perspective

Zvi Laron*, Haim Werner

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

Abstract

Laron Syndrome (LS) [OMIm#262500], or primary GH insensitivity, was first described in 1966 in consanguineous Jewish families from Yemen. LS is characterized by a typical phenotype that includes dwarfism, obesity and hypogenitalism. The disease is caused by deletions or mutations of the GH-receptor gene, causing high serum GH and low IGF-I serum levels. We studied 75 patients from childhood to adult age. After early hypoglycemia due to the progressive obesity, patients tend to develop glucose intolerance and diabetes. The treatment is by recombinant IGF-I, which improves the height and restores some of the metabolic parameters. An unexpected finding was that patients homozygous for GH-R defects are protected from malignancy lifelong, not so heterozygotes or double heterozygote subjects. We estimate that there are at least 500 patients worldwide, unfortunately only few treated.

Original languageEnglish
Pages (from-to)31-41
Number of pages11
JournalReviews in Endocrine and Metabolic Disorders
Volume22
Issue number1
DOIs
StatePublished - Mar 2021

Keywords

  • Cancer protection
  • Diabetes
  • Dwarfism
  • Glucose intolerance
  • Growth hormone insensitivity
  • Hyperandrogenism
  • IGF-I
  • Laron syndrome
  • Obesity

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