Adult patients with Laron syndrome tend to develop the metabolic syndrome.

Background: The metabolic Syndrome is the name of a cluster of abnormal clinical and metabolic states, which constitute a risk factor for diabetes and cardiovascular disease.

Aim: To determine whether adult patients with Laron Syndrome with excessive obesity develop the characteristics of the Metabolic Syndrome.

Subjects: Out of a cohort of adult patients with Laron Syndrome followed in our clinic, records of 23 patients (12 females, 11 males) were found to have sufficient data for analysis.

The COVID-19 Pandemic Increased the Incidence of New-Onset Type One Diabetes in Children.

: The impact of the COVID-19 pandemic on the incidence rate of childhood type 1 diabetes (T1D) is controversial. Our aim was to analyze the incidence of new-onset T1D among children aged 0-17 before and during the COVID-19 pandemic in Israel. : Data obtained from the national T1D registry for children aged 0-17 were analyzed for the pre-pandemic (1997-2019) and pandemic (2020-2022) periods.

Challenges in Pediatric Endocrinology.

As the Section Editor-in-Chief, it is my pleasure to introduce the new section of devoted to pediatric endocrinology [...

Insulin-like growth factors and aging: lessons from Laron syndrome.

The growth hormone (GH)-insulin-like growth factor-1 (IGF1) signaling pathway emerged in recent years as a key determinant of aging and longevity. Disruption of this network in different animal species, including flies, nematodes and mouse, was consistently associated with an extended lifespan. Epidemiological analyses have shown that patients with Laron syndrome (LS), the best-characterized disease under the umbrella of the congenital IGF1 deficiencies, seem to be protected from cancer.

Growth hormone insensitivity and adipose tissue: tissue morphology and transcriptome analyses in pigs and humans.

Purpose: Growth hormone receptor knockout (GHR-KO) pigs have recently been developed, which serve as a large animal model of Laron syndrome (LS). GHR-KO pigs, like individuals with LS, are obese but lack some comorbidities of obesity. The purpose of this study was to examine the histological and transcriptomic phenotype of adipose tissue (AT) in GHR-KO pigs and humans with LS.

Administration of insulin like growth factor I (IGFI) lowers serum lipoprotein(a)-impact on atherosclerotic cardiovascular disease.

Insulin like growth factor I (IGFI) secreted by the liver upon stimulation by pituitary growth hormone (GH) acts as the most important growth stimulating hormone in children. The present review presents evidence that among its additional metabolic effects, IGF-I suppresses the synthesis of lipoprotein(a) [Lp(a)], an independent risk factor for atherosclerotic cardiovascular disease. In view of this property, it is suggested that the addition of IGF-I to the armamentarium of hyperlipoproteinemia treatment should be considered.

Congenital IGF-1 deficiency protects from cancer: lessons from Laron syndrome.

Many clinical and experimental studies have implicated the growth hormone (GH)-insulin-like growth factor (IGF-1) axis with the progression of cancer. The epidemiological finding that patients with Laron syndrome (LS), the best-characterized disease under the spectrum of congenital IGF-1 deficiencies, do not develop cancer is of major scientific and translational relevance. The evasion of LS patients from cancer emphasizes the central role of the GH-IGF-1 system in cancer biology.

Hypothesis: Viral infections of pregnant women may be early triggers of childhood type 1 diabetes and other autoimmune disease.

Children and adolescents with early onset autoimmune diseases have a different seasonality of month of birth than the general population. This pattern is consistent with an infection during pregnancy affecting the fetus or an infection immediately after birth that act as early triggers of the autoimmune diseases. We present data supporting the use of Rotavirus vaccinations in the reduction of incidence of childhood T1D and propose further investigations into whether other anti-virus vaccinations may reduce the burden of other autoimmune diseases such as multiple sclerosis, atopic dermatitis, psoriasis and subtypes of rheumatoid arthritis, Hashimoto thyroiditis.

Long-Term IGF1 Stimulation Leads to Cellular Senescence via Functional Interaction with the Thioredoxin-Interacting Protein, TXNIP.

The growth hormone (GH)-insulin-like growth factor-1 (IGF1) signaling pathway plays a major role in orchestrating cellular interactions, metabolism, growth and aging. Studies from worms to mice showed that downregulated activity of the GH/IGF1 pathway could be beneficial for the extension of lifespan. Laron syndrome (LS) is an inherited autosomal recessive disorder caused by molecular defects of the GH receptor (GHR) gene, leading to congenital IGF1 deficiency.

Increase of serum lipoprotein (a), an adverse effect of growth hormone treatment.

A number of reports show that high endogenous, or therapeutic administration of human growth hormone (hGH) cause an increase of serum lipoprotein a, Lp(a). Being thrombogenic Lp(a) is an independent risk factor of atherosclerotic cardiovascular disease (ASCVD). Hence, it is hypothesized that the recently reported association between childhood hGH treatment and cardiovascular morbidity is probably due to the GH effect on Lp(a) synthesis.

Comparison of Commonly Used Methods to Predict the Final Height in Constitutional Tall Stature.

Objective: To determine the accuracy of adult height prediction in children with constitutional tall stature.

Methods: The medical records of 138 non-syndromatic prepubertal and early pubertal children (52 male, 86 female) with a height of ≥90 percentile born between the years 1975 and 1988 were included in this study. Using the Bayley-Pinneau (BP) and Tanner-Whitehouse I (TWI) prediction methods, their height standard deviation score (SDS) at referral was compared with their height SDS at age 17 years when measured at the IDF conscription center.

Prolactin response to growth hormone stimulation tests.

Background: Despite many similarities between the structure, receptors, proliferative and growth promoting actions, the relationship between Prolactin (PRL) and Growth Hormone (GH) in clinical conditions has received little attention.

Objective: To determine the PRL response to GH stimulation tests.

Subjects: Prepubertal and early pubertal boys (n = 581) and girls (n = 502) with idiopathic, non-syndromatic short stature.

Identification of UDP-Glucuronosyltransferase 2B15 (UGT2B15) as a Target for IGF1 and Insulin Action.

Normal growth and development in mammals are tightly controlled by numerous genetic factors and metabolic conditions. The growth hormone (GH)-insulin-like growth factor-1 (IGF1) hormonal axis is a key player in the regulation of these processes. Dysregulation of the GH-IGF1 endocrine system is linked to a number of pathologies, ranging from growth deficits to cancer.

Focal Epilepsy in Individuals with Laron Syndrome.

Objective: The aim of the study was to describe focal epilepsy in patients with Laron syndrome (LS).

Methods: Data were retrieved from medical records of a single-center cohort of 75 patients with LS.

Results: We describe for the first time 4 patients with concomitant focal epilepsy and LS.

MicroRNA 132-3p Is Upregulated in Laron Syndrome Patients and Controls Longevity Gene Expression.

The growth hormone (GH)-insulin-like growth factor-1 (IGF1) endocrine axis is a central player in normal growth and metabolism as well as in a number of pathologies, including cancer. The GH-IGF1 hormonal system, in addition, has emerged as a major determinant of lifespan and healthspan. Laron syndrome (LS), the best characterized entity under the spectrum of the congenital IGF1 deficiencies, results from mutation of the GH receptor (GHR) gene, leading to dwarfism, obesity and other defects.

ZYG11A Is Expressed in Epithelial Ovarian Cancer and Correlates With Low Grade Disease.

The insulin-like growth factors (IGF) are important players in the development of gynecological malignancies, including epithelial ovarian cancer (EOC). The identification of biomarkers that can help in the diagnosis and scoring of EOC patients is of fundamental importance in clinical oncology. We have recently identified the gene as a new candidate target of IGF1 action.

The Olfactory Receptor Gene Product, OR5H2, Modulates Endometrial Cancer Cells Proliferation via Interaction with the IGF1 Signaling Pathway.

Endometrial cancer is the most common gynecologic malignancy in Western countries. The insulin-like growth factor-1 (IGF1) axis has an important role in endometrial cancer biology and emerged as a promising therapeutic target in oncology. However, there is an urgent need to identify biomarkers that may help in patient stratification and prognosis.

The Role of Nuclear Insulin and IGF1 Receptors in Metabolism and Cancer.

Insulin (InsR) and insulin-like growth factor-1 (IGF1R) receptors mediate the metabolic and growth-promoting actions of insulin and IGF1/IGF2, respectively. Evidence accumulated in recent years indicates that, in addition to their typical cell-surface localization pattern and ligand-activated mechanism of action, InsR and IGF1R are present in the cell nucleus of both normal and transformed cells. Nuclear translocation seems to involve interaction with a small, ubiquitin-like modifier protein (SUMO-1), although this modification is not always a prerequisite.

Laron Syndrome Research Paves the Way for New Insights in Oncological Investigation.

Laron syndrome (LS) is a rare genetic endocrinopathy that results from mutation of the growth hormone receptor () gene and is typically associated with dwarfism and obesity. LS is the best characterized entity under the spectrum of the congenital insulin-like growth factor-1 (IGF1) deficiencies. Epidemiological analyses have shown that LS patients do not develop cancer, whereas heterozygous family members have a cancer prevalence similar to the general population.

Identification of nephronectin as a new target for IGF1 action.

Introduction: The growth hormone (GH)-insulin-like growth factor-1 (IGF1) endocrine axis has a key role in normal growth and development. Laron syndrome (LS) is a type of dwarfism that results from mutation of the GH receptor, leading to congenital IGF1 deficiency. Epidemiological studies have shown that LS patients are protected from cancer.

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 gene, causing high serum GH and low IGF-I serum levels.

Role of the GH-IGF1 system in progression of cancer.

Emerging evidence links the growth hormone (GH)-insulin-like growth factor-1 (IGF1) endocrine axis to cancer development. While this putative correlation is of major translational relevance, most clinical and epidemiological reports to date found no causal linkage between GH therapy and enhanced cancer risk. Thus, it is generally agreed that GH therapy constitutes a safe pharmacological intervention.