ReviewOsteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA2016
Bone disease in cystic fibrosis: new pathogenic insights opening novel therapies.
Review in Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 1 of them a synthesis that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
20 citing papers in PubMed, 1 synthesis or guideline pooled it, 49 citations in OpenAlex.
- Leptin Levels and Bone Mineral Density: A Friend or a Foe for Bone Loss? A Systematic Review of the Association Between Leptin Levels and Low Bone Mineral Density.International journal of molecular sciences · 2025Pooled it
- Trial
- Bone Structural Parameters in Adults with Cystic Fibrosis: Contribution of Adherence to the Mediterranean Diet.Journal of clinical medicine · 2026Article
- Treatment with CFTR Modulators for Cystic Fibrosis: What a Pediatric Gastroenterologist Needs to Know.Children (Basel, Switzerland) · 2025Review
- Cystic fibrosis-related bone disease: an update on screening, diagnosis, and treatment.Therapeutic advances in endocrinology and metabolism · 2025Review
- Opportunistic Screening for Low Bone Mineral Density in Adults with Cystic Fibrosis Using Low-Dose Computed Tomography of the Chest with Artificial Intelligence.Journal of clinical medicine · 2024Article
- Future Comorbidities in an Aging Cystic Fibrosis Population.Life (Basel, Switzerland) · 2023Review
- Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic Inflammation.Biomolecules · 2023Review
- Auxological and Endocrinological Features in Children and Adolescents with Cystic Fibrosis.Journal of clinical medicine · 2022Review
- Magnesium implantation or supplementation ameliorates bone disorder in CFTR-mutant mice through an ATF4-dependent Wnt/β-catenin signaling.Bioactive materials · 2022Article
- IL-8 correlates with reduced baseline femoral neck bone mineral density in adults with cystic fibrosis: a single center retrospective study.Scientific reports · 2021Article
- Automatic referrals within a cystic fibrosis multidisciplinary clinic improve patient evaluation and management.Journal of clinical & translational endocrinology · 2021Article
- Bone fragility in patients affected by congenital diseases non skeletal in origin.Orphanet journal of rare diseases · 2021Review
- Bone Cells Differentiation: How CFTR Mutations May Rule the Game of Stem Cells Commitment?Frontiers in cell and developmental biology · 2021Review
- Evaluation of bone disease in patients with cystic fibrosis and end-stage lung disease.Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia · 2019Article
- An Optimized Method to Generate Human Active Osteoclasts From Peripheral Blood Monocytes.Frontiers in immunology · 2018Article
- Celiac Disease in Patients with Cystic Fibrosis-Related Bone Disease.Case reports in endocrinology · 2017Article
- Reduced bone volumetric density and weak correlation between infection and bone markers in cystic fibrosis adult patients.Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA · 2016Article
- Endocrine Disorders in Cystic Fibrosis.Pediatric clinics of North America · 2016Review
- Young adults with cystic fibrosis have altered trabecular microstructure by ITS-based morphological analysis.Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA · 2016Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
5 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Mutations within the gene encoding for the chloride ion channel cystic fibrosis transmembrane conductance regulator (CFTR) results in cystic fibrosis (CF), the most common lethal autosomal recessive genetic disease that causes a number of long-term health problems, as the bone disease. Osteoporosis and increased vertebral fracture risk associated with CF disease are becoming more important as the life expectancy of patients continues to improve. The etiology of low bone density is multifactorial, most probably a combination of inadequate peak bone mass during puberty and increased bone losses in adults. Body mass index, male sex, advanced pulmonary disease, malnutrition and chronic therapies are established additional risk factors for CF-related bone disease (CFBD). Consistently, recent evidence has confirmed that CFTR plays a major role in the osteoprotegerin (OPG) and COX-2 metabolite prostaglandin E2 (PGE2) production, two key regulators in the bone formation and regeneration. Several others mechanisms were also recognized from animal and cell models contributing to malfunctions of osteoblast (cell that form bone) and indirectly of bone-resorpting osteoclasts. Understanding such mechanisms is crucial for the development of therapies in CFBD. Innovative therapeutic approaches using CFTR modulators such as C18 have recently shown in vitro capacity to enhance PGE2 production and normalized the RANKL-to-OPG ratio in human osteoblasts bearing the mutation F508del-CFTR and therefore potential clinical utility in CFBD. This review focuses on the recently identified pathogenic mechanisms leading to CFBD and potential future therapies for treating CFBD.
Indexed as
Identifiers
What OpenQuestion holds
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.