Publications by authors named "Elizabete Ribeiro Barros"
Pamidronate (PAM) infusion is the standard treatment in children with osteogenesis imperfecta (OI). Zoledronic acid (ZOL) is a bisphosphonate with higher potency and faster intravenous infusion, but its efficacy and safety has not been established for OI patients. We report an open-label, prospective, and randomized clinical analysis to study the safety and efficacy of ZOL compared with PAM in 23 children with OI.
View Article and Find Full Text PDFOsteoporosis Pseudoglioma (OPPG) is characterized by severe juvenile-onset osteoporosis and ocular abnormalities. It is caused by one of several inactivating mutations in LRP5, a gene importantly involved in bone formation. The objective of this study was to evaluate the efficacy of teriparatide in a young man with OPPG.
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- Lrp5 regulates bone formation primarily through its role in the gut, influencing the gut serotonin-bone endocrine axis, but its impact on osteoblast progenitor cells has not been previously examined.
- Mice with Lrp5 disrupted specifically in osteoblast progenitor cells showed no differences in skeletal development or bone characteristics compared to normal mice, indicating that Lrp5 doesn't directly influence these cells.
- Additionally, elevated serum serotonin levels were found in osteoporosis pseudoglioma patients, reinforcing the idea that serotonin dysregulation may be linked to bone mass issues rather than direct Lrp5 function in osteoblasts.
Unlabelled: Osteoporosis-pseudoglioma (OPPG) is a rare syndrome characterized by severe osteoporosis and ocular defects caused by homozygotic inactivation mutations in the LRP5 gene. Bisphosphonate has been demonstrated to improve bone mineral density (BMD) in children with OPPG. We present here a 3 years follow-up of two brothers with OPPG carrying a novel mutation in the LRP5 gene, who were treated with intravenous pamidronate.
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