Cited by 16Growth Hormone and Insulin-like Growth FactorsHypothalamic control of reproductive hormonesHormonal and reproductive studies

Role of the GH-IGF1 Axis on the Hypothalamus-Pituitary-Testicular Axis Function: Lessons From Laron Syndrome

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Cannarella, Rossella, Crafa, Andrea, La Vignera, Sandro, Condorelli, Rosita A, Calogero, Aldo E·2021·Endocrine Connections

Abstract

BACKGROUND: Animal studies suggest that insulin-like growth factor 1 (IGF1) may influence the function of the hypothalamus-pituitary-testicular axis, especially in childhood, but the evidence in humans is scanty. Laron syndrome, a human model of IGF1 deficiency, may help to solve this issue. PURPOSE: This systematic review aims to analyze puberty onset and progression, testicular volume, gonadotropin, and total testosterone serum levels, sperm parameters and fertility, and penile length in patients with Laron syndrome. METHODS: Specific keywords were used. All data on male patients with Laron syndrome were included. RESULTS: Seventeen articles matched the inclusion criteria and were entered in the analysis, for a total of 125 male patients. Puberty was absent in 8.9% and delayed in 35.6% of untreated patients of pubertal age. After onset, the duration of the pubertal process was prolonged in 76.9% of untreated patients. The growth spurt was absent in 52.6% and delayed in 31.6% of untreated patients. The testicular volume was small in the two patients who did not receive any treatment. Treatment with IGF1 increased gonadotropin and testosterone serum levels in five out of five patients of pubertal age. No effect was found in four out of four patients younger than 5 years. No study reported data on sperm parameters and fertility. Micropenis occurred in 67.2% of patients. CONCLUSION AND FUTURE PERSPECTIVES: Delayed puberty is common in patients with Laron syndrome. The growth hormone-IGF1 axis may influence the time of puberty onset. Serum levels of IGF1 should be investigated in children with delayed puberty, scarce progression of testicular growth, and/or micropenis. IGF1 levels might be measured in children with delayed puberty, poor testicular growth, and/or micropenis.

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  • In GH insensitivity (Laron syndrome) patients, the onset of puberty is delayed to 15.6 ± 2.6 years and the duration of pubertal development is prolonged to 6.2 ± 0.6 years.

    “In GH insensitivity patients, also known as Laron syndrome, the onset of puberty delays to the age of 15.6 ± 2.6 years and the duration of the pubertal development procession elongates to 6.2 ± 0.6 years (19).”

    From Growth Hormone Is Beneficial for Induction of Spermatogenesis in Adult Patients With Congenital Combined Pituitary Hormone Deficiency (2022) · cites this paper as [19]

    The Results section (“Puberty onset and progression”) reports both figures, but they apply to males with Laron syndrome, not patients generally. It gives a puberty-onset age of 11.8 ± 0.2 years and a pubertal-process duration of 4.6 ± 0.5 years for females with the syndrome. (LLM, conf=0.90)

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  1. [16]Pertzelan A Lazar L Klinger B Laron Z. Puberty in fifteen patients with Laron syndrome: a Longitudina Study. In Lessons from Laron syndrome (LS) 1966–1992: Pediatric and Adolescent Endocrinology, Vol. 24, pp. 27– 33. Eds Laron Z Parks JS. Basel: Karger, 1993.

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Authors
Cannarella, R., Crafa, A., La Vignera, S., Condorelli, R. A., & Calogero, A. E.
Affiliations
  • University of Catania (IT)
  • 1Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy
Corresponding author
Calogero, Aldo E
Venue
Endocrine Connections
Published
2021-09-01
Volume
10
Issue
9
Pages
1006-1017
ISSN
2049-3614 (ISSN-L)
OpenAlex
W3184875369
Cited by
16
References
53
Citations by year
2025: 2 · 2024: 2 · 2023: 5 · 2022: 5 · 2021: 2
FWCI
2.61
Type
journal article
Language
en
Open access
Locations
  • Endocrine Connections (publishedVersion, cc-by-nc)
  • PubMed Central (publishedVersion, cc-by-nc)
  • PubMed (publishedVersion)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Topics
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Growth Hormone and Insulin-like Growth Factors
  • Health Sciences › Medicine › Reproductive Medicine › Hypothalamic control of reproductive hormones
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Hormonal and reproductive studies
Keywords
Laron syndrome, delayed puberty, testicular volume, gonadotropins, serum testosterone, micropenis, male hypogonadism

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