ReviewAnnual review of pathology2019
Pathophysiology of Sickle Cell Disease.
Review in Annual review of pathology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to 3 registered trials, which are not on this map. Cited by 409 papers, 4 of them syntheses 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.
Pilot Study to Investigate the Use of Virtual Reality Devices as an Adjunct to Usual Care for Patients With Sickle Cell Disease Experiencing Vaso-Occlusive Crises
A Phase III, Multi-Center, Double-Blind, Placebo-Controlled, Randomized Study of Intravenous L-Citrulline in Sickle Cell Disease Presenting to Emergency Departments in Acute Vaso-Occlusive Crisis in Children, Adolescents and Young Adults (6 to 21 Years)
Randomized EvaLuation of ImmErsive Virtual Experiences in Sickle Cell Disease (RELIEVE-SCD)
Who cites it
409 citing papers in PubMed, 4 syntheses or guidelines pooled it, 720 citations in OpenAlex.
- Is There a Difference in Occurrence of Complications Between Adults With Hemoglobin SS and Hemoglobin SC Disease: An Extended Systematic Review.European journal of haematology · 2026Pooled it
- Nontrauma-Associated Cerebral Fat Embolism Syndrome in Sickle Cell-Related Hemoglobinopathies: A Case Series and Systematic Review.AJNR. American journal of neuroradiology · 2025Pooled it
- Practical guide for disease-modifying medication management of children and adolescents with sickle cell disease.Hematology. American Society of Hematology. Education Program · 2024Guideline
- Antioxidant supplementation for sickle cell disease.The Cochrane database of systematic reviews · 2024Pooled it
- Clinical outcomes of lentiviral vector gene therapy for sickle cell disease.Blood advances · 2026Trial
- Increasing daily step counts improves physical fitness, and reduces pain and arterial stiffness in sickle cell patients.Haematologica · 2026Trial
- Improvements in health-related quality of life in patients with severe sickle cell disease after exagamglogene autotemcel.Blood advances · 2025Trial
- Impact of Individualized Versus Weight-Based Pain Protocols on Patient Satisfaction for Patients With Sickle Cell Disease Experiencing a Vaso-Occlusive Episode.Journal of emergency nursing · 2025Trial
- Haploidentical Bone Marrow Transplantation for Sickle Cell Disease.NEJM evidence · 2025Trial
- Riociguat in patients with sickle cell disease and hypertension or proteinuria (STERIO-SCD): a randomised, double-blind, placebo controlled, phase 1-2 trial.The Lancet. Haematology · 2024Trial
- Kidney failure risk equation and early renal risk in adults with sickle cell disease: comparison with KDIGO classification using measured GFR.Renal failure · 2026Article
- A global survey of blood transfusion practices for patients with sickle cell disease.Transfusion · 2026Article
- Variations in mitochondrial genome as potential prognostic markers in sickle cell disease.Haematologica · 2026Article
- Brain MRI and cognitive outcomes in adults with sickle cell disease treated with crizanlizumab.Blood red cells & iron · 2026Article
- Inhibition of FGFR signaling attenuates phosphaturia and early kidney injury in sickle cell disease mice.Biochemistry and biophysics reports · 2026Article
- The SickleFit Tele-Exercise Program for older adults with sickle cell disease: feasibility, acceptability, safety study.Blood red cells & iron · 2026Article
- The Gut-Iron-Immune Axis in Severe Anaemia and Bacteraemia.Nutrients · 2026Review
- Haemolytic injury and cardiovascular disease.Nature reviews. Cardiology · 2026Review
- Reactive Oxygen Species in Cardiovascular and Hematological Diseases: Mechanisms and Therapeutic Strategies.Cureus · 2026Review
- Acute therapy with hemopexin (CSL889) resolves LPS-induced lung vaso-occlusion in sickle mice.Blood vessels, thrombosis & hemostasis · 2026Article
349 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
3 authors at 1 institution in 1 country.
Funding
Abstract
Since the discovery of sickle cell disease (SCD) in 1910, enormous strides have been made in the elucidation of the pathogenesis of its protean complications, which has inspired recent advances in targeted molecular therapies. In SCD, a single amino acid substitution in the β-globin chain leads to polymerization of mutant hemoglobin S, impairing erythrocyte rheology and survival. Clinically, erythrocyte abnormalities in SCD manifest in hemolytic anemia and cycles of microvascular vaso-occlusion leading to end-organ ischemia-reperfusion injury and infarction. Vaso-occlusive events and intravascular hemolysis promote inflammation and redox instability that lead to progressive small- and large-vessel vasculopathy. Based on current evidence, the pathobiology of SCD is considered to be a vicious cycle of four major processes, all the subject of active study and novel therapeutic targeting: ( a) hemoglobin S polymerization, ( b) impaired biorheology and increased adhesion-mediated vaso-occlusion, ( c) hemolysis-mediated endothelial dysfunction, and ( d) concerted activation of sterile inflammation (Toll-like receptor 4- and inflammasome-dependent innate immune pathways). These molecular, cellular, and biophysical processes synergize to promote acute and chronic pain and end-organ injury and failure in SCD. This review provides an exhaustive overview of the current understanding of the molecular pathophysiology of SCD, how this pathophysiology contributes to complications of the central nervous and cardiopulmonary systems, and how this knowledge is being harnessed to develop current and potential therapies.
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.