Laron syndrome - A historical perspective.
Cancer protection
Diabetes
Dwarfism
Glucose intolerance
Growth hormone insensitivity
Hyperandrogenism
IGF-I
Laron syndrome
Obesity
Journal
Reviews in endocrine & metabolic disorders
ISSN: 1573-2606
Titre abrégé: Rev Endocr Metab Disord
Pays: Germany
ID NLM: 100940588
Informations de publication
Date de publication:
03 2021
03 2021
Historique:
accepted:
11
09
2020
pubmed:
24
9
2020
medline:
15
12
2021
entrez:
23
9
2020
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 32964395
doi: 10.1007/s11154-020-09595-0
pii: 10.1007/s11154-020-09595-0
doi:
Substances chimiques
Receptors, Somatotropin
0
Insulin-Like Growth Factor I
67763-96-6
Growth Hormone
9002-72-6
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
31-41Références
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