Zoledronic acid in children with osteogenesis imperfecta and Bruck syndrome: a 2-year prospective observational study

Abstract

Zoledronic acid proved to be safe and effective in the treatment of OI and BS. The biannual infusion protocol was convenient to patients. There was a positive correlation between disease severity and benefits of the treatment. The use of the CSS proved to be of value in the assessment of the degree of severity in OI, and with some modifications, it was a valuable tool for the assessment of response to treatment.

Effect of Bisphosphonates on Function and Mobility Among Children With Osteogenesis Imperfecta: A Systematic Review

“…Two more were excluded because the main intervention was not bisphosphonate administration . Six more were excluded because the outcomes used were not objective . Two more were excluded because they were found to be abstracts presented in proceedings .…”

“…(31,32) Six more were excluded because the outcomes used were not objective. (33)(34)(35)(36)(37)(38) Two more were excluded because they were found to be abstracts presented in proceedings. (39,40) A total of 26 full-text articles (801 male and female children) including four RCTs, (23,(41)(42)(43) 17 NROs, (44,45,47,48,(50)(51)(52)(53)(54)(55)57,58,(60)(61)(62)(63)(64) three NROs with a historic control group, (49,56,59) and two retrospective studies (46,65) met the inclusion criteria and were included.…”

A Rare Case of Bruck Syndrome Type 2 in Siblings With Broad Phenotypic Variability

“…These treatments have been proven to be effective for both disease processes; however, the prognosis for Bruck syndrome is more severe given the complications of joint contractures. 10 When joint contractures are not pronounced, distinguishing between Bruck syndrome and osteogenesis imperfecta without genotyping may be difficult, as the fracture patterns on radiographic imaging and the physical examination may be congruous. Both autosomal recessive and autosomal dominant inheritance patterns have been described for osteogenesis imperfecta, and distinguishing Bruck syndrome from the rare autosomal recessive subtype of osteogenesis imperfecta may be difficult, as multiple loci on the FKBP10 and PLOD2 genes have been implicated in both disease processes.…”

Pathophysiology and therapeutic options in osteogenesis imperfecta: an update

“…However, recent publications indicate the relative short-term safety of zolendronate administered at 6-month intervals in children with OI. 52,53 The advantage of this drug compared to pamidronate is the schedule of infusion, that is, a shorter infusion requires short time and is less frequent. Zoledronate is given for 30 minutes every 6 months and pamidronate is infused for 4 hours every 3-4 months.…”

“…Zoledronate is given for 30 minutes every 6 months and pamidronate is infused for 4 hours every 3-4 months. 53,54 There is no data indicating that zoledronate is more effective in decreasing fracture rate compared to pamidronate. On the basis of this, zoledronate was designed to be administered yearly in adults with osteoporosis; the current practice of zoledronate therapy in children with OI remains to be assessed.…”