The essential role of IGF-I: lessons from the long-term study and treatment of children and adults with Laron syndrome
Abstract
Fifty patients with primary GH resistance (Laron syndrome) due to molecular defects of the GH receptor or post-receptor pathways were followed from infancy through adulthood. This condition leading to long-term insulin-like growth factor-I (IGF-I) deprivation caused marked growth retardation (-4 to 8 height SD), acromicia, organomicria, retarded development of the skeletal and muscular systems, a small cranium, slow motor development, and impairment of intellectual development in some of the patients. In addition, there was progressive obesity, insulin resistance, a tendency for hypoglycemia, followed later in life by hypercholesterolemia and by glucose intolerance and even diabetes. IGF-I treatment of children with Laron syndrome, by our and other groups (150-240 microg/day sc), stimulated growth (8 cm in the first year and 4-5 cm in the following years) and normalized the biochemical abnormalities. Overdosage led to adverse effects such as hypoglycemia, edema, swelling of soft tissues, and hyperandrogenism. It is concluded that primary IGF-I deprivation induces severe auxological, biochemical, and hormonal changes, the only treatment being biosynthetic IGF-I administration.
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Administration of IGF1 in humans results in decreased blood glucose levels and improved insulin sensitivity in those with and without type 2 diabetes.
“Administration of IGF1 in humans results in decreased blood glucose levels and improved insulin sensitivity in those with and without type 2 diabetes [[234], [235], [236]].”
From Hepatokines and metabolism: Deciphering communication from the liver (2021) · cites this paper as [235]
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- Authors
- Laron, Z.
- Affiliations
- Tel Aviv University (IL)
- Corresponding author
- Laron, Zvi
- ORCID
- Laron, Zvi
- Venue
- The Journal of Clinical Endocrinology & Metabolism
- Publisher
- The Endocrine Society
- Published
- 1999-12
- Volume
- 84
- Issue
- 12
- Pages
- 4397-4404
- ISSN
- 0021-972X (ISSN-L)
- 1945-7197
- OpenAlex
- W2082691596
- Cited by
- 96
- References
- 72
- Citations by year
- 2026: 1 · 2024: 2 · 2022: 3 · 2021: 4 · 2020: 4 · 2019: 1 · 2017: 2 · 2016: 5 · 2015: 1 · 2014: 3 · 2013: 1 · 2012: 5
- FWCI
- 2.30
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Journal of Clinical Endocrinology & Metabolism (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Growth Hormone and Insulin-like Growth Factors
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic Syndromes and Imprinting
- Keywords
- Laron syndrome, insulin-like growth factor I, growth retardation, organomegaly, insulin resistance, hypoglycemia, hypercholesterolemia, impaired glucose tolerance, diabetes mellitus, hyperandrogenism, long-term follow-up
- MeSH terms
- Adolescent, Adult, Child, Child, Preschool, Drug Resistance, Female, Humans, Insulin-Like Growth Factor I, Male, Mutation, Receptors, Somatotropin, Recombinant Proteins, Human Growth Hormone
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