Laron syndrome – A historical perspective

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...

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Bibliographic Details
Published in:Reviews in endocrine & metabolic disorders 2021-03, Vol.22 (1), p.31-41
Main Authors: Laron, Zvi, Werner, Haim
Format: Article
Language:English
Subjects:
Adult
Child
Children
Diabetes
Diabetes mellitus
Dwarfism
Endocrinology
Glucose tolerance
Growth Hormone - blood
Heterozygotes
Humans
Hypoglycemia
Insulin-like growth factor I
Insulin-Like Growth Factor I - analysis
Internal Medicine
Intolerance
Laron Syndrome - genetics
Malignancy
Medicine
Medicine & Public Health
Mutation
Neoplasms
Obesity
Phenotypes
Receptors, Somatotropin - genetics
Review Article (invited)
Serum levels
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Summary: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.
ISSN:1389-9155
1573-2606
DOI:10.1007/s11154-020-09595-0