Laron syndrome (primary growth hormone resistance or insensitivity): the personal experience 1958-2003 - PubMed (original) (raw)
. 2004 Mar;89(3):1031-44.
doi: 10.1210/jc.2003-031033.
Affiliations
- PMID: 15001582
- DOI: 10.1210/jc.2003-031033
Laron syndrome (primary growth hormone resistance or insensitivity): the personal experience 1958-2003
Zvi Laron. J Clin Endocrinol Metab. 2004 Mar.
Abstract
Clinical and laboratory investigations starting in 1958 of a group of dwarfed children resembling isolated GH deficiency but who had very high serum levels of GH led to the description of the syndrome of primary GH resistance or insensitivity (Laron syndrome) and subsequently to the discovery of its molecular defects residing in the GH receptor and leading to an inability of IGF-I generation. With the biosynthesis of IGF-I in 1986, therapeutic trials started. Continuously more and more patients are being diagnosed in many parts of the world with a variety of molecular defects. This syndrome proved to be a unique model that enables the study of the consequences of GH receptor defects, the physiopathology of GH-IGF-I disruption, and comparison of the GH-independent IGF-I effects. This review presents the personal experience gained from the study follow-up and treatment of the 60 patients followed up for many years in the Israeli cohort.
Similar articles
- Prismatic cases: Laron syndrome (primary growth hormone resistance) from patient to laboratory to patient.
Laron Z. Laron Z. J Clin Endocrinol Metab. 1995 May;80(5):1526-31. doi: 10.1210/jcem.80.5.7744997. J Clin Endocrinol Metab. 1995. PMID: 7744997 Review. - Fifty seven years of follow-up of the Israeli cohort of Laron Syndrome patients-From discovery to treatment.
Laron Z, Kauli R. Laron Z, et al. Growth Horm IGF Res. 2016 Jun;28:53-6. doi: 10.1016/j.ghir.2015.08.004. Epub 2015 Aug 18. Growth Horm IGF Res. 2016. PMID: 26307357 - Insulin-like growth factor-I treatment of children with Laron syndrome (primary growth hormone insensitivity).
Laron Z. Laron Z. Pediatr Endocrinol Rev. 2008 Mar;5(3):766-71. Pediatr Endocrinol Rev. 2008. PMID: 18367997 Review. - Growth hormone insensitivity syndrome (Laron syndrome): main characteristics and effects of IGF1 treatment.
Carel JC, Chaussain JL, Chatelain P, Savage MO. Carel JC, et al. Diabetes Metab. 1996 Jul;22(4):251-6. Diabetes Metab. 1996. PMID: 8767171 Review. - Insulin-like growth factor binding protein-3 generation: an index of growth hormone insensitivity.
Thalange NK, Price DA, Gill MS, Whatmore AJ, Addison GM, Clayton PE. Thalange NK, et al. Pediatr Res. 1996 May;39(5):849-55. doi: 10.1203/00006450-199605000-00018. Pediatr Res. 1996. PMID: 8726240
Cited by
- The somatotroph pituitary gland function in high-aged multimorbid hospitalized patients with IGF-I deficiency.
Tausendfreund O, Bidlingmaier M, Martini S, Reif H, Rippl M, Schilbach K, Schmidmaier R, Drey M. Tausendfreund O, et al. Pituitary. 2024 May 31. doi: 10.1007/s11102-024-01406-y. Online ahead of print. Pituitary. 2024. PMID: 38819617 - Tmem263 deletion disrupts the GH/IGF-1 axis and causes dwarfism and impairs skeletal acquisition.
Sarver DC, Garcia-Diaz J, Saqib M, Riddle RC, Wong GW. Sarver DC, et al. Elife. 2024 Jan 19;12:RP90949. doi: 10.7554/eLife.90949. Elife. 2024. PMID: 38241182 Free PMC article. - Laron Syndrome: A Tale of Two Siblings.
Das N, Tarenia SS, Saha S, Gaikwad PM, Hathi DK, Goswami S, Baidya A, Sengupta N. Das N, et al. J ASEAN Fed Endocr Soc. 2023;38(2):124-127. doi: 10.15605/jafes.038.02.22. Epub 2023 Sep 18. J ASEAN Fed Endocr Soc. 2023. PMID: 38045665 Free PMC article. - Challenges in Pediatric Endocrinology.
Laron Z. Laron Z. Children (Basel). 2023 Oct 30;10(11):1757. doi: 10.3390/children10111757. Children (Basel). 2023. PMID: 38002847 Free PMC article. - Insulin-like growth factors and aging: lessons from Laron syndrome.
Werner H, Laron Z. Werner H, et al. Front Endocrinol (Lausanne). 2023 Oct 24;14:1291812. doi: 10.3389/fendo.2023.1291812. eCollection 2023. Front Endocrinol (Lausanne). 2023. PMID: 37941907 Free PMC article. Review.
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Other Literature Sources
Medical