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High bone mass in XLH not caused by primary sclerostin dysregulation
Elevated sclerostin serum levels in patients with X-linked hypophosphatemic rickets and the normal concentrations observed in osteogenesis imperfecta suggest that the bone mass abnormalities in these disorders are not caused by primary sclerostin dysregulation, according to data published in the Journal of Clinical Endocrinology & Metabolism.
“X-linked hypophosphatemic rickets (XLH) and osteogenesis imperfecta are two of the most common heritable metabolic bone disorders in children and adolescents,” Telma Palomo, MD, of Shriners Hospital for Children and the department of pediatrics at McGill University in Montreal, and colleagues wrote.
Their study included 128 patients, including 30 with XLH, 76 with osteogenesis imperfecta types I, III and IV, and 22 healthy individuals.
Data indicated that patients with XLH demonstrated greater circulating sclerostin concentrations (mean, 30.2 pmol/L) vs. healthy controls (mean, 21.4 ng/mL; P=.02).
The researchers also reported relatively high lumbar spine areal bone mineral density z scores (1.1). Those in the XLH cohort displayed serum sclerostin levels that were positively associated with lumbar spine areal BMD z scores (r=0.56; P<.002) and with alkaline phosphatase (r=0.45; P=.01).
Patients with osteogenesis imperfecta also demonstrated similar sclerostin serum levels as healthy controls despite low lumbar spine areal BMD z scores, according to researchers.
“These results indicate that high bone mass in XLH patients is not a result of primary sclerostin dysregulation in these disorders,” they wrote.
Disclosure: The researchers report no relevant financial disclosures.