engleski

Osteogenesis imperfecta patient group - longitudinal follow up by bone markers

Bone turnover affects skeletal integrity and can be assessed by measurement of bone markers. Experience with osteogenesis imperfecta (OI) patients indicates normal, decreased or increased concentrations of bone markers, but also decrement as a result of bisphosphonate treatment. We have performed a longitudinal study of bone markers in 24 OI patients (11 boys, 13 girls) aged 1-19 years. Blood sampling and urine samples were collected on occasions of regular monitoring. The following bone markers were measured in serum by standard methods: osteocalcin, procollagen 1 propeptide (P1CP) and beta crosslaps (also measured in urine), and results compared to reference range for premenopausal women. Osteocalcin was mostly increased, in particular in patients 10-15 years old, which might indicate correspondence with pubertal growth spurt. In older patients, osteocalcin concentrations decreased towards adult values. For bone resorption marker crosslaps 2-3x higher concentrations in serum were measured in patients 8-15 years, and lower and normal in younger and older patients. Bone resorption markers in urine were approximately twice normal value compared to premenopausal women, with reduction in patients 14 years and older. P1CP was mostly reduced indicating impaired collagen I synthesis, with the exception of 2 very young patients (<2 years) and 2 prepubertal patients. Bone metabolism in OI patients as determined by bone markers shows increased concentrations in children, possible effect of pubertal growth acceleration and levelling off towards adulthood. Collagen type 1 derived bone marker demonstrated reduced synthesis in most patients. These results also indicate variations in bone turnover in young OI patients.

osteogenesis imperfecta

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