ReviewInternational journal of environmental research and public health2021
Mechanisms of Bone Impairment in Sickle Bone Disease.
Review in International journal of environmental research and public health, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 33 citations in OpenAlex.
- Article
- Article
- Diagnostic relevance of osteocalcin and bone alkaline phosphatase as markers of bone turnover in Sickle Cell Anaemia: A Zaria study.African health sciences · 2026Article
- Musculoskeletal manifestations of sickle cell disease: an imaging perspective.Skeletal radiology · 2025Review
- Selective AMPKβ1 activation induces fetal hemoglobin in human erythroid cells and sickle cell mice via the noncanonical NRF2 pathway.Science advances · 2025Article
- Magnetic Resonance Imaging of Musculoskeletal Manifestations in Sickle Cell Disease.Journal of clinical medicine · 2025Article
- Molecular Mechanisms of Iron Metabolism and Overload.Biomedicines · 2025Review
- Bone Turnover Markers Levels in a Cohort of Egyptian Children with Sickle Cell Disease.Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion · 2025Article
- Effects of GBT1118, a voxelotor analog, on bone disease in sickle cell disease mice.Scientific reports · 2024Article
- Article
- Bone Health Impairment in Patients with Hemoglobinopathies: From Biological Bases to New Possible Therapeutic Strategies.International journal of molecular sciences · 2024Review
- Low Bone Mass and Recurrent Fractures in Neurofibromatosis With Concomitant Hemoglobin SC Disease.Cureus · 2023Article
- Management of Older Adults with Sickle Cell Disease: Considerations for Current and Emerging Therapies.Drugs & aging · 2023Review
- Sickle cell bone disease and response to intravenous bisphosphonates in children.Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA · 2022Article
- Revisiting anemia in sickle cell disease and finding the balance with therapeutic approaches.Blood · 2022Article
- Complete resolution of stage II avascular necrosis affecting three joints by hyperbaric oxygen in a patient with sickle cell disease: A case report.Frontiers in medicine · 2022Article
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Sickle bone disease (SBD) is a chronic and invalidating complication of Sickle cell disease (SCD), a multisystem autosomal recessive genetic disorder affecting millions of people worldwide. Mechanisms involved in SBD are not completely known, especially in pediatric age. Among the hypothesized pathogenetic mechanisms underlying SBD are bone marrow compensatory hyperplasia and bone ischemic damage, both secondary to vaso-occlusive crisis (VOC), which leads to cell sickling, thus worsening local hypoxia with a negative impact on osteoblast recruitment. Furthermore, the hypoxia is a strong activator of erythropoietin, which in turn stimulates osteoclast precursors and induces bone loss. Hemolysis and iron overload due to a chronic transfusion regimen could also contribute to the onset of bone complications. Vitamin D deficiency, which is frequently seen in SCD subjects, may worsen SBD by increasing the resorptive state that is responsible for low bone mineral density, acute/chronic bone pain, and high fracture risk. An imbalance between osteoblasts and osteoclasts, with a relative decrease of osteoblast recruitment and activity, is a further possible mechanism responsible for the impairment of bone health in SCD. Moreover, delayed pubertal growth spurt and low peak bone mass may explain the high incidence of fracture in SCD adolescents. The aim of this review was to focus on the pathogenesis of SBD, updating the studies on biochemical, instrumental, and biological markers of bone metabolism. We also evaluated the growth development and endocrine complications in subjects affected with SCD.
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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.