ReviewJournal of children's orthopaedics2019
Current concepts in osteogenesis imperfecta: bone structure, biomechanics and medical management.
Review in Journal of children's orthopaedics, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 48 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
48 citing papers in PubMed, 1 synthesis or guideline pooled it, 82 citations in OpenAlex.
- Ocular characteristics and complications in patients with osteogenesis imperfecta: a systematic review.Acta ophthalmologica · 2022Pooled it
- Setrusumab for the treatment of osteogenesis imperfecta: 12-month results from the phase 2b asteroid study.Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research · 2024Trial
- Efficacy and Safety of Denosumab vs Zoledronic Acid in OI Adults: A Prospective, Open-Label, Randomized Study.The Journal of clinical endocrinology and metabolism · 2024Trial
- Skin symptoms and their impact on quality-of-life in adults with osteogenesis imperfecta.Journal of the Endocrine Society · 2026Article
- Collagen secretion and maturation in osteogenesis imperfecta: Systematic review and meta-analysis.Bone reports · 2026Article
- Histological and molecular characterization of bone integrity in osteogenesis imperfecta: a case series across genetic subtypes.JBMR plus · 2026Article
- Protrusio Acetabuli in Osteogenesis Imperfecta: A Two-Center Study on Incidence and Associated Factors.Calcified tissue international · 2026Article
- Post-translational modifications of collagen type I in osteogenesis imperfecta: Systematic review and meta-analysis.Bone reports · 2026Article
- Mechanical characterization ofCell biomaterials · 2026Article
- Molecular drivers of osteogenesis imperfecta: a cellular and extracellular collagen disease.Clinical science (London, England : 1979) · 2025Review
- Assessment of Collagen and Fibroblast Properties via Label-Free Higher Harmonic Generation Microscopy in Three-Dimensional Models of Osteogenesis Imperfecta and Ehlers-Danlos Syndrome.International journal of molecular sciences · 2025Article
- Improvement of bone properties in children with osteogenesis imperfecta after pamidronate: a bone biopsy study.JBMR plus · 2025Article
- Bone Quality and Mineralization and Effects of Treatment in Osteogenesis Imperfecta.Calcified tissue international · 2024Review
- Skeletal pathology in mouse models of Gould syndrome is partially alleviated by genetically reducing TGFβ signaling.Matrix biology : journal of the International Society for Matrix Biology · 2024Article
- Article
- Matrix first, minerals later: fine-tuned dietary phosphate increases bone formation in zebrafish.JBMR plus · 2024Article
- Birefringence-induced phase delay enables Brillouin mechanical imaging in turbid media.Nature communications · 2024Article
- A review of the current state of natural biomaterials in wound healing applications.Frontiers in bioengineering and biotechnology · 2024Review
- Radiofrequency Echographic Multispectrometry (REMS): A New Option in the Assessment Bone Status in Adults with Osteogenesis Imperfecta.Journal of imaging · 2023Article
- The role of vesicle trafficking genes in osteoblast differentiation and function.Scientific reports · 2023Article
Corrections and comments
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Authors and funding
7 authors at 5 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The majority of patients with osteogenesis imperfecta (OI) have mutations in the COL1A1 or COL1A2 gene, which has consequences for the composition of the bone matrix and bone architecture. The mutations result in overmodified collagen molecules, thinner collagen fibres and hypermineralization of bone tissue at a bone matrix level. Trabecular bone in OI is characterized by a lower trabecular number and connectivity as well as a lower trabecular thickness and volumetric bone mass. Cortical bone shows a decreased cortical thickness with less mechanical anisotropy and an increased pore percentage as a result of increased osteocyte lacunae and vascular porosity. Most OI patients have mutations at different locations in the COL1 gene. Disease severity in OI is probably partly determined by the nature of the primary collagen defect and its location with respect to the C-terminus of the collagen protein. The overall bone biomechanics result in a relatively weak and brittle structure. Since this is a result of all of the above-mentioned factors as well as their interactions, there is considerable variation between patients, and accurate prediction on bone strength in the individual patient with OI is difficult. Current treatment of OI focuses on adequate vitamin-D levels and interventions in the bone turnover cycle with bisphosphonates. Bisphosphonates increase bone mineral density, but the evidence on improvement of clinical status remains limited. Effects of newer drugs such as antibodies against RANKL and sclerostin are currently under investigation. This paper was written under the guidance of the Study Group Genetics and Metabolic Diseases of the European Paediatric Orthopaedic Society.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.