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Serum Osteocalcin in Pediatric Osteogenesis Imperfecta: Impact of Disease Type and Bisphosphonate Therapy
Jakub Krzysztof Nowicki1, Elżbieta Jakubowska-Pietkiewicz1
1Department of Pediatrics, Neonatal Pathology and Metabolic Bone Diseases, Medical University of Lodz, Sporna 36/50, 91-738 Lodz, Poland.
Insights
Serum osteocalcin levels are elevated in children with osteogenesis imperfecta (OI). Type 3 OI and pamidronate treatment reduce osteocalcin, an important bone formation marker.
Area of Science:
- Pediatric Endocrinology
- Bone Metabolism
- Genetic Disorders
Background:
- Osteogenesis imperfecta (OI) is a group of genetic disorders characterized by fragile bones.
- Serum osteocalcin is a marker of bone formation, but its behavior in pediatric OI patients undergoing bisphosphonate treatment is not fully understood.
Purpose of the Study:
- To investigate factors influencing serum osteocalcin levels in children with OI treated with intravenous sodium pamidronate.
- To determine the role of osteocalcin assessment in managing pediatric OI.
Main Methods:
- Retrospective analysis of 61 pediatric patients (ages 2-18) with OI types 1 or 3.
- Collected data included demographics, fracture history, serum osteocalcin, alkaline phosphatase, 25(OH)D3, creatinine, and DXA BMD z-scores.
- Analyzed the relationship between osteocalcin levels and OI type, pamidronate treatment, and other clinical factors.
Main Results:
- Osteocalcin levels were higher in OI patients compared to age- and sex-matched references.
- Patients with OI type 3 had significantly lower osteocalcin than type 1.
- Increased pamidronate treatment frequency correlated with reduced osteocalcin levels.
- OI type was the strongest predictor of osteocalcin concentration; BMD and fracture frequency were not related.
Conclusions:
- Osteocalcin is elevated in pediatric OI but decreases with pamidronate therapy.
- OI type is a key determinant of osteocalcin levels.
- Osteocalcin measurement is valuable at the start of bisphosphonate treatment for pediatric OI.
Abstract:
The aim of this study was to analyze the factors that may influence serum osteocalcin levels in children with osteogenesis imperfecta treated with intravenous sodium pamidronate and to define the role of osteocalcin assessment. The study included 61 patients diagnosed with osteogenesis imperfecta type 1 or 3, aged 2 to 18, hospitalized for intravenous sodium pamidronate administration. A retrospective analysis of medical records was conducted, collecting information on age, sex, body weight, height, the number of long bone fractures throughout life, serum levels of osteocalcin, creatinine, alkaline phosphatase, 25(OH)D3, and DXA BMD z-scores for the L1-L4 spine segment. The concentration of osteocalcin is higher in patients with osteogenesis imperfecta than the reference ranges for sex and age. Patients diagnosed with type 3 have significantly lower osteocalcin levels compared to patients with type 1. Also, increasing the age-standardized pamidronate cycle rate significantly reduced osteocalcin concentration. The strongest predictor of osteocalcin concentration among the factors studied is the type of osteogenesis imperfecta. L1-L4 BMD value and fracture frequency were unrelated to osteocalcin concentration. Osteocalcin is an important marker of bone formation that should be measured at the beginning of treatment, as its concentration decreases after successive doses of bisphosphonates.
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