Follow-up of six patients with neurofibromatosis 1-related osteoporosis treated with alendronate for 23 months.

摘要: This is the first prospective follow-up study to describe effects of oral alendronate medication on neurofibromatosis 1 (NF1)-related osteoporosis. NF1 a neurocutaneous skeletal syndrome associated with increased fracture risk and high frequency osteopenia Alendronate bisphosphonate drug which inhibits function bone-resorbing osteoclasts, ultimately leading an increase in bone mineral density (BMD) reduction risk. However, vitro studies have shown that osteoclasts display insensitivity apoptotic signals caused by bisphosphonates. Our aim was monitor patients NF1. Five men one woman, aged 28–76 years, NF1-related osteoporosis were enrolled study. Study participants did not other conditions taking any known affect bone. The included weekly dose 70 mg daily 20 μg vitamin D supplementation. After 23 months follow-up, BMD five out six patients, but statistically significant. Serum levels turnover markers CTX PINP reduced, suggesting slower remodeling, as expected. An unexpected result serum osteoclast activity marker TRAP5b change during follow-up. One new stress tibia documented therapy. Even though group small, findings current (one patient decreased BMD) call for larger assess efficacy bisphosphonates