Which OI types are severe vs mild?

Type I (mildest): non-deforming, blue sclerae; Type II: perinatal lethal; Type III: progressively deforming; Type IV: moderately deforming; Types V-XIII have specific features; BRTL and Marfanoid OI are newer types.

What DXA Z-score change indicates bisphosphonate response?

Lumbar spine Z-score improvement of >0.5 SD over 2-3 years of bisphosphonate therapy indicates favorable response; fracture rate reduction is the clinical outcome measure.

What is the recommended bisphosphonate regimen in severe pediatric OI?

IV pamidronate 0.5-1 mg/kg/day x 3 days every 3-4 months or zoledronic acid 0.025-0.05 mg/kg IV annually; calcium and vitamin D supplementation is essential throughout.

When should bisphosphonates be stopped before surgery in OI?

Bisphosphonate holiday 1-2 years before osteotomy with intramedullary rod insertion may reduce "chalky" bone density that interferes with healing; shorter breaks for minor procedures.

What is the role of sclerostin inhibition in OI?

Romosozumab (anti-sclerostin antibody) trials in OI show significant increases in bone mineral density; it activates bone formation rather than just inhibiting resorption like bisphosphonates.

Osteogenesis Imperfecta Fracture Risk and Bisphosphonate Response

Calculates fracture risk in osteogenesis imperfecta using Sillence classification type, annual fracture rate, DXA Z-score, and pamidronate/zoledronic acid treatment response monitoring.

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Frequently Asked Questions

Which OI types are severe vs mild?

What DXA Z-score change indicates bisphosphonate response?

What is the recommended bisphosphonate regimen in severe pediatric OI?

When should bisphosphonates be stopped before surgery in OI?

What is the role of sclerostin inhibition in OI?