Increased IGF Binding-protein (IGFBP) Proteolysis, IGF-I Bioavailability, and Pappalysin Levels in Children With Prader-Willi Syndrome
Authors' conclusion
The increase in PAPP-A could be involved in increased IGFBP proteolysis, promoting IGF-I bioavailability in children with PWS. Further studies are needed to establish the relationship between growth, GH resistance, and changes in the IGF axis during development and after GH treatment in these patients.
Study context
- Population
- Prepubertal and pubertal children and adolescents with prader-Willi syndrome (PWS) and sex- and age-matched controls
- Participants
- 40 vs 120
- Comparison
- Children with PWS vs controls; Growth hormone (GH) treatment effect
- PWSn = 40
- Controlsn = 120reference
- Outcome
- Serum levels of PAPP-As and STCs in association with IGF axis components
Methodological detailssex- and age-matched controls
- Sex- and age-matched controls
Main finding
As reported in the abstract
Serum IGF axis components, PAPP-As and STCs
- PWS had lower total IGF-I, total and intact IGFBP-3, acid-labile subunit, intact IGFBP-4 and stanniocalcins (STC-1) vs controls
- PWS had higher free IGF-I, IGFBP-5 and PAPP-A vs controls
Observational data: associations, not effects.
Further findings
PWS after pubertal onset
- Decreased total IGF-I, total and intact IGFBP-3 and intact IGFBP-4; increased total IGFBP-4 and STCs
Effect of GH treatment (n=11) · 6 months
- GH increased total IGF-I, total and intact IGFBP-3 and intact IGFBP-4; no changes in PAPP-As, STCs and free IGF-I
Correlates of standardized height
- Height correlated directly with intact IGFBP-3 and inversely with PAPP-As and free/total IGF-I ratio
Original publication
Increased IGFBP Proteolysis, IGF-I Bioavailability, and Pappalysin Levels in Children With Prader-Willi Syndrome. J Clin Endocrinol Metab. Published 23 December 2023. doi:10.
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