Differences in Bone Metabolism between Children with Prader-Willi Syndrome during Growth Hormone Treatment and Healthy Subjects: A Pilot Study.

Publisher: MDPI Country of Publication: Switzerland NLM ID: 101092791 Publication Model: Electronic Cited Medium: Internet ISSN: 1422-0067 (Electronic) Linking ISSN: 14220067 NLM ISO Abbreviation: Int J Mol Sci Subsets: MEDLINE

Original Publication: Basel, Switzerland : MDPI, [2000-

Prader-Willi Syndrome*/metabolism ; Prader-Willi Syndrome*/drug therapy ; Prader-Willi Syndrome*/blood ; Bone and Bones*/metabolism ; Bone and Bones*/drug effects ; Biomarkers*/blood; Humans ; Child ; Male ; Female ; Pilot Projects ; Human Growth Hormone/blood ; Child, Preschool ; Osteocalcin/blood ; Osteocalcin/metabolism ; Adolescent ; Insulin-Like Growth Factor I/metabolism ; Bone Density/drug effects ; Alkaline Phosphatase/blood ; Case-Control Studies

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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Keywords: Gla-osteocalcin; Glu-osteocalcin; Prader–Willi syndrome; insulin-like growth factor-I; periostin; total-IGF-binding protein-3

0 (Biomarkers)
12629-01-5 (Human Growth Hormone)
104982-03-8 (Osteocalcin)
67763-96-6 (Insulin-Like Growth Factor I)
EC 3.1.3.1 (Alkaline Phosphatase)

Date Created: 20240914 Date Completed: 20240914 Latest Revision: 20240916

20240916

PMC11394978

10.3390/ijms25179159

39273107