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References from EHA Guidelines on the management of children and adults with hemoglobin SC disease. Local targets link to admitted publications; unresolved targets remain external evidence.
Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000−2021: a systematic analysis from the Global Burden of Disease Study 2021
10.1016/s2352-3026(23)00118-7 · 2023 · External reference
Defining global strategies to improve outcomes in sickle cell disease: a Lancet Haematology Commission
10.1016/s2352-3026(23)00096-0 · 2023 · External reference
Global burden of sickle cell anaemia in children under five, 2010‐2050: modelling based on demographics, excess mortality, and interventions
10.1371/journal.pmed.1001484 · 2013 · External reference
Sickle cell disease
10.1016/s0140-6736(17)30193-9 · 2017 · External reference
The clinical spectrum of HbSC sickle cell disease‐not a benign condition
10.1111/bjh.19523 · 2024 · External reference
The paradox of hemoglobin SC disease
10.1016/s0268-960x(03)00003-1 · 2003 · External reference
The clinical significance of K‐Cl cotransport activity in red cells of patients with HbSC disease
10.3324/haematol.2014.120402 · 2015 · External reference
Males with sickle cell disease have higher risks of cerebrovascular disease, increased inflammation, and a reduced response to hydroxyurea
10.1002/ajh.27074 · 2023 · External reference
Decreased median survival of adults with sickle cell disease after adjusting for left truncation bias: a pooled analysis
10.1182/blood-2018-10-880575 · 2019 · External reference
AGREE II: advancing guideline development, reporting and evaluation in health care
10.1503/cmaj.090449 · 2010 · External reference
Significant haemoglobinopathies: a guideline for screening and diagnosis: a British Society for Haematology Guideline
10.1111/bjh.18794 · 2023 · External reference
The Danish national haemoglobinopathy screening programme: report from 16 years of screening in a low‐prevalence, non‐endemic region
10.1111/bjh.19103 · 2024 · External reference
Sickle cell anemia in Cuba: prevention and management, 1982‐2018
2019 · External reference
Parents' experiences of universal screening for haemoglobin disorders: implications for practice in a new genetics era
10.3399/bjgp08x277276 · 2008 · External reference
Genetic carrier screening for cystic fibrosis, Fragile X Syndrome, hemoglobinopathies, and spinal muscular atrophy
2021 · External reference
EHA recommendations for preconceptual and antenatal screening and prenatal diagnosis for hemoglobinopathies
10.1002/hem3.70381 · 2026 · External reference
Recommendations for preconceptual and antenatal screening and prenatal diagnosis for hemoglobinopathies
10.1002/hem3.70381 · 2026 · External reference
Prenatal genetic counseling in cross‐cultural medicine: a framework for family physicians
2010 · External reference
Who counsels parents of newborns who are carriers of sickle cell anemia or cystic fibrosis?
10.1007/s10897-012-9537-3 · 2013 · External reference
Point‐of‐care diagnostic test accuracy in children and adolescents with sickle cell disease: a systematic review and meta‐analysis
10.1016/j.blre.2024.101243 · 2025 · External reference
Risk of miscarriage following amniocentesis or chorionic villus sampling: systematic review of literature and updated meta‐analysis
10.1002/uog.20353 · 2019 · External reference
Non‐invasive prenatal testing for the diagnosis of sickle cell disease in high‐risk pregnancies: a systematic review and statistical summary of the current literature
10.1016/j.ejogrb.2025.114799 · 2026 · External reference
Knowledge insufficient: the management of haemoglobin SC disease
10.1111/bjh.14444 · 2017 · External reference
Seeing haemoglobin SC: challenging the misperceptions
10.1111/bjh.19580 · 2024 · External reference
Perceptions of patients and stakeholders on a prenatal sickle cell disease screening and its results among tribal populations of Gujarat: a participatory mixed‐method research
10.1007/s12687-025-00768-5 · 2025 · External reference
Sickle cell retinopathy: improving care with a multidisciplinary approach
10.2147/jmdh.s90630 · 2017 · External reference
Natural history and rate of progression of retinopathy in adult patients with sickle cell disease: an 11‐year follow‐up study
10.1182/bloodadvances.2022009147 · 2023 · External reference
Rétinopathie drépanocytaire: analyse rétrospective portant sur 730 patients suivis dans un centre de référence
External reference
Impaired blood rheology plays a role in the chronic disorders associated with sickle cell‐hemoglobin C disease
10.3324/haematol.2014.104745 · 2014 · External reference
Clinical and laboratory risk factors for sickle cell retinopathy and maculopathy: a scoping review of the current evidence
10.3324/haematol.2024.286420 · 2025 · External reference
Anti‐vascular endothelial growth factor therapy for stages 3 and 4 proliferative sickle cell retinopathy results in improved anatomical and visual outcomes
10.1097/iae.0000000000004658 · 2026 · External reference
Central nervous system complications and management in sickle cell disease
10.1182/blood-2015-09-618579 · 2016 · External reference
Neuropsychologic performance in school‐aged children with sickle cell disease: a report from the Cooperative Study of Sickle Cell Disease
10.1067/mpd.2001.116935 · 2001 · External reference
Higher‐than‐expected prevalence of silent cerebral infarcts in children with hemoglobin SC disease
10.1182/blood-2014-10-605964 · 2015 · External reference
Unusually high prevalence of stroke and cerebral vasculopathy in hemoglobin SC disease: a retrospective single institution study
10.1159/000519360 · 2022 · External reference
Genome wide association study of silent cerebral infarction in sickle cell disease (HbSS and HbSC)
10.3324/haematol.2020.265827 · 2021 · External reference
Cerebrovascular accidents in sickle cell disease: rates and risk factors
1998 · External reference
The excess burden of stroke in hospitalized adults with sickle cell disease
10.1002/ajh.21476 · 2009 · External reference
Transcranial Doppler scanning and the assessment of stroke risk in children with haemoglobin sickle cell disease
10.1136/adc.2007.125799 · 2008 · External reference
Transcranial Doppler in hemoglobin SC disease
10.1002/pbc.26342 · 2017 · External reference
American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults
10.1182/bloodadvances.2019001142 · 2020 · External reference
Do statins improve outcomes after acute ischemic stroke?
10.1038/nrneurol.2011.92 · 2011 · External reference
Guidelines for the prevention of stroke in patients with stroke or transient ischemic attack: a guideline for healthcare professionals from the American Heart Association/American Stroke Association
10.1161/str.0b013e3181f7d043 · 2011 · External reference
Hemoglobin sickle cell disease complications: a clinical study of 179 cases
10.3324/haematol.2011.055202 · 2012 · External reference
Treatment of sickle cell disease's hip necrosis by core decompression: a prospective case‐control study
10.1016/j.otsr.2009.07.009 · 2009 · External reference
Sickle cell disease as a cause of osteonecrosis of the femoral head
10.1056/nejm199111213252104 · 1991 · External reference
Does increased red blood cell deformability raise the risk for osteonecrosis in sickle cell anemia?
10.1182/blood-2013-01-480277 · 2013 · External reference
Sickle cell bone disease and response to intravenous bisphosphonates in children
10.1007/s00198-022-06455-2 · 2022 · External reference
Stem cell therapy for the treatment of hip osteonecrosis: a 30‐year review of progress
10.4055/cios.2016.8.1.1 · 2016 · External reference
Total hip arthroplasty in sickle cell disease: a systematic review and meta‐analysis of functional outcomes and complications
10.1016/j.jcot.2025.103060 · 2025 · External reference
Natural history of blood pressure in sickle cell disease: risks for stroke and death associated with relative hypertension in sickle cell anemia
10.1016/s0002-9343(96)00407-x · 1997 · External reference
Weight status of children with sickle cell disease
10.1542/peds.2012-2225 · 2013 · External reference
High body mass index in children with sickle cell disease: a retrospective single‐centre audit
10.1136/bmjpo-2018-000302 · 2018 · External reference
Pulmonary hypertension as a risk factor for death in patients with sickle cell disease
10.1056/nejmoa035477 · 2004 · External reference
Cardiac manifestations in sickle cell disease varies with patient genotype
10.1111/bjh.15238 · 2018 · External reference
Elevated hypercoagulability markers in hemoglobin SC disease
10.3324/haematol.2014.114587 · 2015 · External reference
Venous thromboembolism in adults with sickle cell disease: a serious and under‐recognized complication
10.1016/j.amjmed.2012.12.016 · 2013 · External reference
Causes of death in sickle cell disease: an autopsy study
10.1046/j.1365-2141.2003.04594.x · 2003 · External reference
Risk factors for venous thromboembolism in adults with hemoglobin SC or Sβ+ thalassemia genotypes
10.1016/j.thromres.2016.03.003 · 2016 · External reference
Coronavirus disease among persons with sickle cell disease, United States, March 20‐May 21, 2020
10.3201/eid2610.202792 · 2020 · External reference
Glomerular hyperfiltration and albuminuria in children with sickle cell anemia
10.1007/s00467-011-1857-2 · 2011 · External reference
Kidney disease among patients with sickle cell disease, hemoglobin SS and SC
10.2215/cjn.03940415 · 2016 · External reference
Chronic renal failure in sickle cell disease: risk factors, clinical course, and mortality
10.7326/0003-4819-115-8-614 · 1991 · External reference
Maximum urine concentrating ability in children with Hb SC disease: effects of hydroxyurea
10.1002/(sici)1096-8652(200005)64:1<47::aid-ajh8>3.0.co;2-1 · 2000 · External reference
Gallstones in sickle cell disease: observations from The Jamaican Cohort study
10.1016/s0022-3476(00)90054-4 · 2000 · External reference
Outcome in hemoglobin SC disease: a four‐decade observational study of clinical, hematologic, and genetic factors
10.1002/ajh.10140 · 2002 · External reference
Functional asplenia in hemoglobin SC disease
10.1182/blood.v85.8.2238.bloodjournal8582238 · 1995 · External reference
Relevance of Howell‐Jolly body counts for measuring spleen function in sickle cell disease
10.1002/ajh.26879 · 2023 · External reference
Palpable splenomegaly in children with haemoglobin SC disease: haematological and clinical manifestations
10.1046/j.1365-2257.2000.00304.x · 2000 · External reference
Acute splenic complications in children with sickle cell‐hemoglobin C disease
10.1016/s0022-3476(97)70284-1 · 1997 · External reference
Priapism in sickle‐cell disease; incidence, risk factors and complications—an international multicentre study
10.1046/j.1464-410x.2002.03022.x · 2002 · External reference
How I treat priapism
10.1182/blood-2014-09-551887 · 2015 · External reference
Epidemiology and treatment of priapism in sickle cell disease
10.1182/hematology.2022000380 · 2022 · External reference
European Association of Urology guidelines on priapism
10.1016/j.eururo.2013.11.008 · 2014 · External reference
American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support
10.1182/bloodadvances.2019001143 · 2020 · External reference
Risk factors for alloimmunization by patients with sickle cell disease
10.1590/s0100-879x2005000500004 · 2005 · External reference
Proinflammatory state promotes red blood cell alloimmunization in pediatric patients with sickle cell disease
10.1182/bloodadvances.2022008647 · 2023 · External reference
How I safely transfuse patients with sickle‐cell disease and manage delayed hemolytic transfusion reactions
10.1182/blood-2018-02-785964 · 2018 · External reference
Cellular effects of hydroxyurea in Hb SC disease
10.1046/j.1365-2141.1997.3173132.x · 1997 · External reference
A novel mouse model of hemoglobin SC disease reveals mechanisms underlying beneficial effects of hydroxyurea
10.1182/blood.2024028136 · 2025 · External reference
Management of sickle cell disease: summary of the 2014 evidence‐based report by expert panel members
10.1001/jama.2014.10517 · 2014 · External reference
Hydroxyurea treatment of children with hemoglobin SC disease
10.1002/pbc.24283 · 2013 · External reference
Hydroxyurea therapy for pediatric patients with hemoglobin SC disease
10.1097/00043426-200106000-00014 · 2001 · External reference
Effects of hydroxyurea treatment for patients with hemoglobin SC disease
10.1002/ajh.24255 · 2016 · External reference
Hydroxyurea for children and adults with hemoglobin SC disease
2025 · External reference
Baseline characteristics of Ghanaian children and adults enrolled in PIVOT, a randomised clinical trial of hydroxyurea in HbSC disease in sub‐Saharan Africa
10.1111/bjh.19832 · 2024 · External reference
Iron deficiency in HbSC disease treated with repetitive phlebotomy is associated with fewer sickle cell disease‐related complications
10.1002/ajh.70045 · 2025 · External reference
Iron deficiency is associated with reduced levels of inflammation and haemolysis in patients with HbSS and HbSC and reduced clinical admissions in those with HbSC
10.1111/bjh.70262 · 2026 · External reference
Iron restriction is an important treatment of hemoglobin SC disease
10.1002/ajh.24380 · 2016 · External reference
Original research: use of hydroxyurea and phlebotomy in pediatric patients with hemoglobin SC disease
10.1177/1535370216639737 · 2016 · External reference
Recommendations for diagnosis, treatment, and prevention of iron deficiency and iron deficiency anemia
10.1002/hem3.108 · 2024 · External reference
Iron deficiency is associated with reduced levels of inflammation and haemolysis in patients with HbSS and HbSC and reduced clinical admissions in those with HbSC
10.1111/bjh.70262 · 2026 · External reference
Crizanlizumab for the prevention of pain crises in sickle cell disease
10.1056/nejmoa1611770 · 2017 · External reference
A phase 3 trial of l‐glutamine in sickle cell disease
10.1056/nejmoa1715971 · 2018 · External reference
Emerging therapies in sickle cell disease
10.1111/bjh.16504 · 2020 · External reference
The oral ferroportin inhibitor vamifeport improves hemodynamics in a mouse model of sickle cell disease
10.1182/blood.2021014716 · 2022 · External reference
Safety and efficacy of mitapivat in sickle cell disease (RISE UP): results from the phase 2 portion of a global, double‐blind, randomised, placebo‐controlled trial
10.1016/s2352-3026(24)00319-3 · 2025 · External reference
Iron restricted erythropoiesis under hepcidin mimetic treatment (PN23114) improved disease parameters in a Mouse Model for sickle cell disease
10.1182/blood-2023-182472 · 2023 · External reference
Guidelines for the management of the acute painful crisis in sickle cell disease
10.1046/j.1365-2141.2003.04193.x · 2003 · External reference
Beyond IV push: alternative methods for management of acute pain in SCD
10.1182/hematology.2024000585 · 2024 · External reference
Acute chest syndrome is associated with history of asthma in hemoglobin SC disease
10.1002/pbc.22900 · 2011 · External reference
Causes and outcomes of the acute chest syndrome in sickle cell disease
10.1056/nejm200006223422502 · 2000 · External reference
Splenic complications in pediatric sickle cell disease: a retrospective cohort review
10.1002/pbc.31219 · 2024 · External reference
The spleen and sickle cell disease: the sick(led) spleen
10.1111/bjh.12950 · 2014 · External reference
Dengue in hospitalized children with sickle cell disease: a retrospective cohort study in the French departments of America
10.1016/j.jiph.2019.07.015 · 2020 · External reference
A retrospective analysis of the significance of haemoglobin SS and SC in disease outcome in patients with sickle cell disease and dengue fever
10.1016/j.ebiom.2015.07.002 · 2015 · External reference
Severe parvovirus B19 infection in patients with sickle cell disease hospitalized in intensive care units
10.1182/bloodadvances.2025015947 · 2025 · External reference
Bone marrow necrosis and fat embolism syndrome in sickle cell disease: increased susceptibility of patients with non‐SS genotypes and a possible association with human parvovirus B19 infection
10.1016/j.blre.2013.12.002 · 2014 · External reference
Low incidence of COVID‐19 severe complications in a large cohort of children with sickle cell disease: a protective role for basal interferon‐1 activation?
10.3324/haematol.2021.278573 · 2021 · External reference
An analysis of risk factors for sensorineural hearing loss in children with sickle cell disease
10.1097/mao.0000000000004674 · 2026 · External reference
Proactive management to improve outcomes of high‐risk pregnancy in people with sickle cell disease
10.1182/hematology.2025000744 · 2025 · External reference
Current obstetric outcomes in Jamaican women with sickle hemoglobinopathy—a balance of risks for aspirin?
10.1515/jpm-2023-0378 · 2024 · External reference
Management of sickle cell disease in pregnancy. A British Society for Haematology Guideline
10.1111/bjh.17671 · 2021 · External reference
Hematopoietic stem cell transplantation in thalassemia and sickle cell disease: report from the European Society for Blood and Bone Marrow Transplantation Hemoglobinopathy Registry: 2000−2017
10.1182/blood-2018-168 · 2018 · External reference
Allogeneic transplantation for sickle cell disease offers high rates of cure with low incidence of severe chronic GVHD in both children and adults: real world data from 2010‐2021. An analysis of the European Society for Blood and Bone Marrow Transplantation Haemoglobinopathy Registry
10.1182/blood-2024-207944 · 2024 · External reference
Sickle cell disease: an international survey of results of HLA‐identical sibling hematopoietic stem cell transplantation
10.1182/blood-2016-10-745711 · 2017 · External reference
Hematopoietic stem cell transplantation in sickle cell disease: a multidimentional review
10.1177/09636897241246351 · 2024 · External reference
Allogeneic hematopoietic stem‐cell transplantation for sickle cell disease
10.1056/nejmoa0904971 · 2009 · External reference
Selecting patients with sickle cell disease for gene addition or gene editing‐based therapeutic approaches: Report on behalf of a joint EHA Specialized Working Group and EBMT Hemoglobinopathies Working Party consensus conference
10.1002/hem3.70089 · 2025 · External reference
Gene therapy for HbSC disease and other compound heterozygous sickle hemoglobinopathies: a time for inclusion
10.1182/blood.2025029964 · 2025 · External reference
A mouse model for hemoglobin SC disease recapitulates characteristic human pathologies
10.1182/bloodadvances.2025016793 · 2025 · External reference