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Rev Endocr Metab Disord. 2021 Mar;22(1):31-41. doi: 10.1007/s11154-020-09595-0. Epub 2020 Sep 22.

Laron syndrome - A historical perspective.

Reviews in endocrine & metabolic disorders

Zvi Laron, Haim Werner

Affiliations

  1. Endocrinology and Diabetes Research Unit, Schneider Children's Medical Center, 14 Kaplan Street, 4920200, Petah Tikva, Israel. [email protected].
  2. Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

PMID: 32964395 DOI: 10.1007/s11154-020-09595-0

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.

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

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