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Differences in Bone Metabolism between Children with Prader-Willi Syndrome during Growth Hormone Treatment and
Joanna Gajewska1, Magdalena Chełchowska1, Katarzyna Szamotulska2
1Department of Screening Tests and Metabolic Diagnostics, Institute of Mother and Child, Kasprzaka 17a, 01-211 Warsaw, Poland.
Insights
Children with Prader-Willi syndrome (PWS) on growth hormone (GH) therapy show altered bone turnover markers, including vitamin K-dependent proteins (VKDPs). These changes suggest impaired bone formation and potentially altered VKDP carboxylation in PWS patients.
Area of Science:
- Pediatric Endocrinology
- Bone Metabolism
- Genetics and Rare Diseases
Background:
- Children with Prader-Willi syndrome (PWS) often experience low bone mineral density and orthopedic issues despite growth hormone (GH) therapy.
- Understanding bone turnover markers, particularly vitamin K-dependent proteins (VKDPs), is crucial for managing bone health in PWS.
- The interplay between GH therapy, dietary interventions, and bone metabolism in PWS requires further investigation.
Purpose of the Study:
- To analyze bone markers, focusing on VKDPs, in normal-weight children with PWS receiving GH therapy and dietary intervention.
- To compare bone marker profiles between children with PWS and healthy controls.
- To explore the relationship between insulin-like growth factor-I (IGF-I) and bone metabolism markers in PWS.
Main Methods:
- Included 24 children with PWS and 30 healthy controls.
- Measured serum concentrations of bone alkaline phosphatase (BALP), osteocalcin (OC), carboxylated-OC (Gla-OC), undercarboxylated-OC (Glu-OC), periostin, osteopontin, osteoprotegerin (OPG), sclerostin, C-terminal telopeptide of type I collagen (CTX-I), and IGF-I.
- Utilized immunoenzymatic methods for serum marker determination.
Main Results:
- Children with PWS had lower osteocalcin (OC) levels and OC/CTX-I ratios compared to controls (p=0.011, p=0.006).
- Lower Glu-OC (p=0.002) but higher Gla-OC and periostin concentrations (p=0.005, p<0.001) were observed in PWS patients.
- Significant relationships were found between IGF-I and OC, Gla-OC, and OC/CTX-I ratio in PWS children after age adjustment (p=0.013, p=0.042, p=0.017).
Conclusions:
- Impaired bone formation in PWS may stem from reduced OC concentrations and OC/CTX-I ratios.
- Altered OC forms and elevated periostin suggest intensified VKDP carboxylation processes in PWS.
- Further research is needed to elucidate the complex interactions between the GH/IGF-I axis and bone metabolism markers, especially VKDPs, in PWS.
Abstract:
Despite therapy with growth hormone (GH) in children with Prader-Willi syndrome (PWS), low bone mineral density and various orthopedic deformities have been observed often. Therefore, this study aimed to analyze bone markers, with an emphasis on vitamin K-dependent proteins (VKDPs), in normal-weight children with PWS undergoing GH therapy and a low-energy dietary intervention. Twenty-four children with PWS and 30 healthy children of the same age were included. Serum concentrations of bone alkaline phosphatase (BALP), osteocalcin (OC), carboxylated-OC (Gla-OC), undercarboxylated-OC (Glu-OC), periostin, osteopontin, osteoprotegerin (OPG), sclerostin, C-terminal telopeptide of type I collagen (CTX-I), and insulin-like growth factor-I (IGF-I) were determined using immunoenzymatic methods. OC levels and the OC/CTX-I ratios were lower in children with PWS than in healthy children (p = 0.011, p = 0.006, respectively). Glu-OC concentrations were lower (p = 0.002), but Gla-OC and periostin concentrations were higher in patients with PWS compared with the controls (p = 0.005, p < 0.001, respectively). The relationships between IGF-I and OC (p = 0.013), Gla-OC (p = 0.042), and the OC/CTX-I ratio (p = 0.017) were significant after adjusting for age in children with PWS. Bone turnover disorders in children with PWS may result from impaired bone formation due to the lower concentrations of OC and the OC/CTX-I ratio. The altered profile of OC forms with elevated periostin concentrations may indicate more intensive carboxylation processes of VKDPs in these patients. The detailed relationships between the GH/IGF-I axis and bone metabolism markers, particularly VKDPs, in children with PWS requires further research.
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