SynthesisAnnals of medicine2021
Pathophysiology of respiratory failure in patients with osteogenesis imperfecta: a systematic review.
Synthesis in Annals of medicine, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers, 2 of them syntheses 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.
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Who cites it
24 citing papers in PubMed, 2 syntheses or guidelines pooled it, 37 citations in OpenAlex.
- Adapting to Adulthood: A Review of Transition Strategies for Osteogenesis Imperfecta.Calcified tissue international · 2024Pooled it
- Systematic review of health related-quality of life in adults with osteogenesis imperfecta.Orphanet journal of rare diseases · 2023Pooled it
- Dose matters: haploinsufficiency in osteogenesis imperfecta.Nature reviews. Endocrinology · 2026Review
- Transcriptional profiling of otic capsule and femur in the oim mouse model of osteogenesis imperfecta.JBMR plus · 2026Article
- Article
- Lung scRNA-seq reveals chronic inflammation and emphysemous phenotype in mice with osteogenesis imperfecta.Frontiers in genetics · 2026Article
- Revision of Cemented Reverse Total Shoulder Arthroplasty with Bone Graft in Osteogenesis Imperfecta: A Case Report.Journal of orthopaedic case reports · 2025Article
- Pediatric Scoliosis in Osteogenesis Imperfecta: From Genetic Mechanisms to Therapeutic Strategies.Orthopaedic surgery · 2025Review
- The impact of obesity on sleep, pulmonary and chest wall restriction in Osteogenesis Imperfecta: a pilot study.Orphanet journal of rare diseases · 2024Article
- Lung function in adult patients with osteogenesis imperfecta: a cohort study.Orphanet journal of rare diseases · 2024Article
- Respiratory insufficiency in an infant with osteogenesis imperfecta.Respiratory medicine case reports · 2024Article
- Monochorionic twin pregnancy in a patient with type III osteogenesis imperfecta: a multidisciplinary challenge.BMJ case reports · 2023Article
- A Sporadic Case ofGenes · 2023Review
- Pulmonary function and structure abnormalities in children and young adults with osteogenesis imperfecta point to intrinsic and extrinsic lung abnormalities.Journal of medical genetics · 2023Article
- From Genetics to Clinical Implications: A Study of 675 Dutch Osteogenesis Imperfecta Patients.Biomolecules · 2023Article
- Bronchial obstruction in osteogenesis imperfecta can be detected by forced oscillation technique.Frontiers in medicine · 2023Article
- Respiratory function of children and adolescents with osteogenesis imperfecta: respiratory muscle strength, forced vital capacity, and peak expiratory flow.Revista paulista de pediatria : orgao oficial da Sociedade de Pediatria de Sao Paulo · 2023Article
- Distinct type I collagen alterations cause intrinsic lung and respiratory defects of variable severity in mouse models of osteogenesis imperfecta.The Journal of physiology · 2023Article
- Lung and chest wall volume during vital capacity manoeuvre in Osteogenesis Imperfecta.Orphanet journal of rare diseases · 2022Article
- Prevalence of scoliosis and impaired pulmonary function in patients with type III osteogenesis imperfecta.European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society · 2022Article
Corrections and comments
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Authors and funding
7 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionRespiratory failure is a major cause of death in patients with Osteogenesis Imperfecta. Moreover, respiratory symptoms seem to have a dramatic impact on their quality of life. It has long been thought that lung function disorders in OI are mainly due to changes in the thoracic wall, caused by bone deformities. However, recent studies indicate that alterations in the lung itself can also undermine respiratory health.
objectivesIs there any intrapulmonary alteration in Osteogenesis Imperfecta that can explain decreased pulmonary function? The aim of this systematic literature review is to investigate to what extent intrapulmonary or extrapulmonary thoracic changes contribute to respiratory dysfunction in Osteogenesis Imperfecta.
methodsA literature search (in PubMed, Embase, Web of Science, and Cochrane), which included articles from inception to December 2020, was performed in accordance with the PRISMA guidelines.
resultsPulmonary function disorders have been described in many studies as secondary to scoliosis or to thoracic skeletal deformities. The findings of this systematic review suggest that reduced pulmonary function can also be caused by a primary pulmonary problem due to intrinsic collagen alterations.
conclusionsBased on the most recent studies, the review indicates that pulmonary defects may be a consequence of abnormal collagen type I distorting the intrapulmonary structure of the lung. Lung function deteriorates further when intrapulmonary defects are combined with severe thoracic abnormalities. This systematic review reveals novel findings of the underlying pathological mechanism which have clinical and diagnostic implications for the assessment and treatment of pulmonary function disorders in Osteogenesis Imperfecta.KEY MESSAGESDecreased pulmonary function in Osteogenesis Imperfecta can be attributed to primary pulmonary defects due to intrapulmonary collagen alterations and not solely to secondary problems arising from thoracic skeletal dysplasia.Type I collagen defects play a crucial role in the development of the lung parenchyma and defects, therefore, affect pulmonary function. More awareness is needed among physicians about pulmonary complications in Osteogenesis Imperfecta to develop novel concepts on clinical and diagnostic assessment of pulmonary functional disorders.
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Registered trials
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.