In children with Prader−Willi syndrome (PWS), the standard growth hormone (GH) dose often results in high immunoreactive IGF-I levels. These high immunoreactive IGF-I levels lead to concern because their long-term effects are unknown. As a result, clinicians have to lower the GH dose, which worsens body composition and quality of life. As clinical features do not seem to correspond to immunoreactive IGF-I values, it is questionable whether immunoreactive IGF-I is a suitable marker for GH dosing, or whether another parameter better reflects IGF-I bioavailability and bioactivity. We, therefore, investigate serum immunoreactive IGF-I, free IGF-I and IGFBP-3 levels in 70 GH-treated children with PWS. Our study showed that, although immunoreactive IGF-I levels were high (>2 SDS) in the vast majority of prepubertal and pubertal children, free IGF-I SDS levels were <0 SDS in most and <1 SDS in all. Free IGF-I correlated with the immunoreactive IGF-I, IGFBP-3 and IGF-I/IGFBP-3 ratio. We conclude that there is a major discrepancy between immunoreactive and free IGF-I levels. While in the majority of GH-treated children with PWS, immunoreactive IGF-I levels were high, free IGF-I levels were <0 SDS in most. Our data appear to be very reassuring and suggest that free IGF-I levels should also be taken into consideration when the immunoreactive IGF-I levels are >2 SDS in GH-treated children with PWS.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8911349PMC
http://dx.doi.org/10.3390/jcm11051280DOI Listing

Publication Analysis

Top Keywords

immunoreactive igf-i
24
igf-i levels
12
igf-i
10
children pws
8
high immunoreactive
8
levels high
8
free igf-i
8
immunoreactive
6
levels
5
free insulin-like
4

Similar Publications

Pituitary gigantism due to a novel germline splice-site variant.

Endocr Oncol

January 2024

Neuroendocrinology Research Center, Endocrinology Section, Medical School and Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Brazil.

Article Synopsis
  • Pituitary gigantism is a rare condition in kids caused by excessive growth hormone, often linked to genetic factors, particularly mutations in the aryl hydrocarbon receptor-interacting protein gene.
  • A case study of an 11-year-old boy highlighted symptoms like progressive vision loss, rapid growth, and weight gain, with tests confirming high levels of growth hormone and indicating a tumor causing these issues.
  • Despite two surgeries and medications, including Pasireotide and cabergoline, his condition showed only partial improvement, and researchers identified a new likely pathogenic genetic variant contributing to his gigantism.
View Article and Find Full Text PDF

Background: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by a progressive loss of motor neurons. The limited efficacy of recent therapies in clinical development may be linked to lack of drug penetration to the affected motor neurons due to the blood-brain barrier (BBB) and blood-spinal cord barrier (BSCB).

Methods: In this work, the safety and efficacy of repeated short transient opening of the BSCB by low intensity pulsed ultrasound (US, sonication) was studied in females of an ALS mouse model (B6.

View Article and Find Full Text PDF

Prematurity has physical consequences, such as lower birth weight, decreased muscle mass and increased risk of adult-onset metabolic disease. Insulin-like growth factor 1 (IGF-1) has therapeutic potential to improve the growth and quality of muscle and tendon in premature births, and thus attenuate some of these sequalae. We investigated the effect of IGF-1 on extensor carpi radialis muscle and biceps brachii tendon of preterm piglets.

View Article and Find Full Text PDF

Bidirectional modulation of synaptic transmission by insulin-like growth factor-I.

Front Cell Neurosci

June 2024

Departamento de Anatomía, Histología y Neurociencia, Facultad de Medicina, Universidad Autónoma de Madrid, Madrid, Spain.

Insulin-like growth factor-I (IGF-I) plays a key role in the modulation of synaptic plasticity and is an essential factor in learning and memory processes. However, during aging, IGF-I levels are decreased, and the effect of this decrease in the induction of synaptic plasticity remains unknown. Here we show that the induction of N-methyl-D-aspartate receptor (NMDAR)-dependent long-term potentiation (LTP) at layer 2/3 pyramidal neurons (PNs) of the mouse barrel cortex is favored or prevented by IGF-I (10 nM) or IGF-I (7 nM), respectively, when IGF-I is applied 1 h before the induction of Hebbian LTP.

View Article and Find Full Text PDF

Short Stature due to Bioinactive Growth Hormone (Kowarski Syndrome).

Endocr Pract

November 2023

Department of Pediatric Endocrinology, Faculty of Medicine, Gaziantep University, Gaziantep, Turkey. Electronic address:

Objective: Bioinactive growth hormone (BGH) is a structurally abnormal, biologically inactive, but immunoreactive form of growth hormone encoded by pathogenic growth hormone 1 gene variants. The underlying cause of the defective physiology is decreased BGH binding affinity to both growth hormone binding proteins and growth hormone receptors (GHRs). GHR cannot dimerize when it is in a quiescent state because BGH cannot activate it.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!