Reshaping of Vertebrae during Treatment with Neridronate or Pamidronate in Children with Osteogenesis Imperfecta

Abstract

Background/Aims: Osteogenesis imperfecta (OI) is a hereditary disease causing increased bone fragility. Pamidronate (PAM), which has to be administered as a 3-day course according to the original protocol by Glorieux, is the most frequently used therapy. Other bisphosphonates like neridronate (NER), which can be infused during an outpatient visit, have also proven to be effective. This is the first analysis comparing the effect of PAM and NER using vertebral morphometry. Methods: 28 patients with OI type III and IV were retrospectively analyzed by matched pairs. Results: No differences were detected between patients treated with PAM or NER at the start of therapy: mean age 4.4 years (p = 0.730), mean height 86.8 cm/85.3 cm (p = 0.854), lumbar vertebral area 208.9 mm²/206.0 mm² (p = 0.555), and in all vertebral indices. After 1 year of treatment (mean 1.16 years; p = 0.854) both groups showed a significant increase in the vertebral area and improved vertebral indices. Again there were no differences between the groups in the vertebral area (p = 0.590). Conclusion: In this study there was no difference between patients treated with PAM or NER regarding vertebral morphometry during the first year of therapy. Because of the possibility of an outpatient setting, NER is convenient for these children.

ENDOCRINOLOGY AND ADOLESCENCE: Osteoporosis in children: diagnosis and management

“…Children are different from adults as growth and puberty are continuously elongating, widening and strengthening their bones. Specific for children is the ability for BP-associated reshaping of fractured vertebrae (87,88,89,90), a phenomenon explained by continuous bone formation during halted resorption. However, spontaneous reshaping of fractured vertebrae can also occur in secondary osteoporotic conditions during remission (91, 92) Therefore, it is important to better understand the factors associated with spontaneous healing to avoid unnecessary treatment (72,93).…”

“…Intravenous infusions of zoledronate (0.025-0.05 mg/kg per day, commonly given over 30 min as a single dose, every 6 months) are associated with improvement in bone mass and subsequent reduction in fracture risk (99,100,101,102). Similarly, intravenous neridronate (2mg/kg per day over 30 min every 6 months) improves BMD and reduces fracture rates (89,103).…”

Efficacy of Denosumab for Osteoporosis in Three Female Patients with Osteogenesis Imperfecta

“…The most commonly prescribed medications for OI are intravenous bisphosphonates (BPs) (Glorieux et al 1998;Semler et al 2011), although their effects have been limited to only modest reductions in the incidence of fractures (Land et al 2007). In a clinical trial of the BP risedronate for OI, the mean increase in lumbar bone mineral density after 1 year was 16.3%, but clinical fractures had occurred in 29 (31%) of 94 patients.…”

Cranial base pathology in pediatric osteogenesis imperfecta patients treated with bisphosphonates

“…Reported benefits include increased bone mineral density and cortical bone thickness; reduction in fracture rate, deformity of long bones, and bone pain; normalization of vertebral morphology; and increased mobility. 5,7,32,40,41 The aim of the present study was to analyze whether bisphosphonates also affect the morphology of the craniocervical junction and cranial base. This possible effect has previously been largely unexplored.…”