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Cardiology1351 papers

Sickle cell-thalassemia disease

Last edited: 4/23/2026

Overview

Sickle cell disease (SCD) is a genetic blood disorder characterized by abnormal hemoglobin (HbS) leading to rigid, sickle-shaped red blood cells, causing chronic hemolysis, vaso-occlusive crises, and organ damage 1 10.

Diagnosis

Clinical Presentation: Recurrent painful crises, chronic hemolysis, and complications like acute chest syndrome, stroke, and nephropathy 1 3 10.

Laboratory Tests: Hemoglobin electrophoresis to confirm HbS presence 10.

Imaging: Transcranial Doppler (TCD) for stroke risk assessment in pediatric patients 11 44.

Ophthalmic Evaluation: Regular eye exams for retinopathy using optical coherence tomography angiography 26.

Management

First-Line Treatments:

- Hydroxyurea: Reduces vaso-occlusive crises and mortality; typical dose 15-20 mg/kg/day 4 8 22 25 42. - Crizanlizumab: An anti-P-selectin antibody for reducing vaso-occlusive crises; dosing typically 2.5 mg/kg every 3-4 weeks 24.

Adjunctive Treatments:

- Pain Management: Rapid administration of analgesics in emergency settings; median time to first dose ≤30 minutes 4. - Hydroxyurea Escalation: Higher doses may be considered for better efficacy in some adults 64. - Antioxidants: Consideration for oxidative stress management, though evidence varies 9 29 59.

Special Populations

Pregnancy: Hydroxyurea safety reviewed; consult guidelines for management 22 27.

Pediatrics: Regular TCD screening for stroke risk; early intervention crucial 11 44.

Comorbidities:

- Hypertension: Management strategies include renin-angiotensin system inhibitors; genetic variants like MYH9 and BMPR1B influence proteinuria risk 6 17. - Kidney Disease: Monitor for albuminuria and manage with appropriate renal protective strategies 18.

Key Recommendations

Offer hydroxyurea to all eligible patients to reduce complications and improve survival (Evidence: Strong 4 8 22).

Implement rapid pain management protocols with first analgesic administration within 30 minutes in ED settings (Evidence: Moderate 4).

Use transcranial Doppler screening for stroke risk assessment in children aged 2-16 years with SCD (Evidence: Strong 11 44).

Consider crizanlizumab for patients with frequent vaso-occlusive crises despite hydroxyurea (Evidence: Moderate 24).

Regularly monitor for and manage complications such as hypertension and nephropathy, considering genetic factors (Evidence: Moderate 6 17 18).

References

Showing 100 most recent of 999 indexed papers.

1 Ribeiro EE, Fernandes KS, Pereira ESJ, Marinho AMCL, Tenório JR, Dos Santos MPA. Does Sickle Cell Anaemia Have a Relationship With Avascular Pulp Necrosis? A Systematic Review. Australian endodontic journal : the journal of the Australian Society of Endodontology Inc 2026. link 2 Simo JL, Tuono RM, Njopwouo MS, Tayou CT. Oxidative Stress, Antioxidant Capacity, Dyslipidemia and Cardiovascular Risk in Sickle Cell Disease: A Systematic Review and Meta-Analysis. TheScientificWorldJournal 2026. link 3 Kaweme NM, Buttress SMN, Nguweneza A, Phiri C, Mtisi TJ, Sambo PM et al.. Burden and outcomes of cardiovascular and pulmonary complications in adolescents and adults with sickle cell disease: a systematic review and meta-analysis. Systematic reviews 2026. link 4 Gwarzo I, Dega HC, Tanabe P, Miller R, Brousseau DC. Evaluating the American Society of Hematology quality measure on timeliness of analgesics for sickle cell disease pain crisis. Blood advances 2026. link 5 Ren G, Fraidenburg DR, Setty S, Chen J, Gonzales J, Ruiz MA et al.. Thrombomodulin protects against acute vascular and multiorgan injury in sickle cell disease. JCI insight 2026. link 6 Malick Ndour EH, Mnika K, Ousmane Sene EH, Tall FG, Nembaware V, Ly ID et al.. Association of hypertension and genetic variants in MYH9 and BMPR1B with increased proteinuria in sickle cell disease. Clinical biochemistry 2026. link 7 Moura MJ, Costa É, Canelas MA, Maia R, Ramalheira S. Acute Chest Syndrome and Critical Illness-Associated Cerebral Microbleeds in Sickle Cell Disease: A Case Report. Acta medica portuguesa 2026. link 8 Dain AS, Li Y, Master S, Bailey CL, Vossough A, Ichord RN et al.. Trends in HU utilization and cerebrovascular outcomes before and after publication of the 2014 National Heart, Lung, and Blood Institute sickle cell disease guidelines. Pediatric hematology and oncology 2026. link 9 Liu W, Kassasseya C, Papamanolis L, Nguyen-Peyre KA, Garreau M, Eckert S et al.. Vascular Geometry Drives Stroke Risk in Sickle Cell Disease. American journal of hematology 2026. link 10 Colombatti R, Jastaniah W, Makani J, Andemariam B. Sickle cell disease. Lancet (London, England) 2026. link02278-0) 11 Edwards JG, Humphrey DT, Mueller M, Phillips S, Schlenz A, Oni MO et al.. Socioeconomic Status and Stroke Risk in Pediatric Sickle Cell Disease: A DISPLACE Study Secondary Analysis. Pediatric blood & cancer 2026. link 12 Ramadas N, Lowder K, Dutton J, Kazen RC, Sellers R, DeRousse JT et al.. Targeting PAR1 biased signaling with parmodulin reduces thromboinflammation and acute lung injury in sickle cell disease. Blood advances 2026. link 13 Bhat V, Yoo JJ, Ponna S, Potdar AA, Patel AP, Yu GK et al.. Gene expression profiling identifies potential biomarkers for vaso-occlusive episodes in sickle cell disease. JCI insight 2026. link 14 Patel A, Wright C, Coyne F, Klein RJ, Nguemeni Tiako MJ, Kuo AA et al.. Addressing Primary Care Needs in People Living With Sickle Cell Disease : A Narrative Review. Annals of internal medicine 2026. link 15 Jalil A, Lippera M, Ivanova T. CENTRAL RETINAL ARTERY OCCLUSION AFTER SCLERAL BUCKLING SURGERY WITH GAS INSERTION IN A PATIENT WITH SICKLE CELL DISEASE. Retinal cases & brief reports 2026. link 16 Aljishi AK, Alrabia FA, Al Abbas AM, Alfaraj MM, Alomar FA, Abohelaika S. Therapeutic Plasma Exchange as a Rescue Therapy in Sickle Cell Disease-Associated Fat Embolism Syndrome: Case Series and Literature Review. Hemoglobin 2026. link 17 Obeagu EI. Strategies and innovations in hypertension management for sickle cell patients: A narrative review. Medicine 2026. link 18 Lebensburger J, Zahr R. The Pediatric Clinician's Approach to the Diagnosis and Management of Nocturnal Enuresis, Hypertension, and Albuminuria in Children with Sickle Cell Disease. Pediatric clinics of North America 2026. link 19 St-Onge J, Hristova T, Charles C, Bereznyakova O, Jacquin G, Pouliot O et al.. Stroke burden and functional impacts in adults with sickle cell disease. British journal of haematology 2026. link 20 Wolf J, Blais-Normandin I, Bathla A, Keshavarz H, Chou ST, Al-Riyami AZ et al.. Red cell specifications for blood group matching in patients with haemoglobinopathies: An updated systematic review and clinical practice guideline from the International Collaboration for Transfusion Medicine Guidelines. British journal of haematology 2025. link 21 Bawazir WM. A Meta Analysis of RBC Alloimmunization in Transfused Sickle Cell and Thalassemia Patients in Saudi Arabia. Clinical laboratory 2025. link 22 Al Sulaimani R, Zitoun N, Alothman H, Hutson JR, Garcia-Bournissen F. Safety of Hydroxyurea in Pregnancy: A Systematic Review of the Literature. Journal of obstetrics and gynaecology Canada : JOGC = Journal d'obstetrique et gynecologie du Canada : JOGC 2025. link 23 Idowu M, Otieno L, Dumitriu B, Lobo CLC, Thein SL, Andemariam B et al.. 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. The Lancet. Haematology 2025. link00319-3) 24 Abboud MR, Cançado RD, De Montalembert M, Smith WR, Rimawi H, Voskaridou E et al.. Crizanlizumab with or without hydroxyurea in patients with sickle cell disease (STAND): primary analyses from a placebo-controlled, randomised, double-blind, phase 3 trial. The Lancet. Haematology 2025. link00384-3) 25 Dei-Adomakoh YA, Segbefia CI, Latham TS, Lane AC, Dzefi-Tettey K, Amissah-Arthur K et al.. Hydroxyurea for Children and Adults with Hemoglobin SC Disease. NEJM evidence 2025. link 26 Clarke K, Mannath A, Anastasi M, Nasr M, Pan S, Balaskas K et al.. Optical coherence tomography angiography as a tool for diagnosis and monitoring of sickle cell related eye disease: a systematic review and meta-analysis. Eye (London, England) 2025. link 27 Yue M, Mason K, Rowlands S, Kaplan Z, Kennedy D, Kidson-Gerber G. Position statement on the management of pregnancy in sickle cell disease. The Australian & New Zealand journal of obstetrics & gynaecology 2025. link 28 da Conceicao Souza J, Macedo APA, Dos Santos Goncalves M, de Souza Santos Monteiro A, Barbosa CG, Couto FD et al.. DHA and EPA in Sickle Cell Disease Favor Clinical Improvement and Contribute to Better Quality of Life: A Qualitative Systematic Review. Current drug research reviews 2025. link 29 Issa A, Ibrahim OR, Ibraheem RM, Lawal AF, Abdulbaki M, AbdulKadir MB et al.. Prevalence of sickle cell disease and sickle cell traits among children and adolescents in Nigeria: a systematic review and meta-analysis. Systematic reviews 2025. link 30 Morris CR, Hatabah D, Korman R, Gillespie S, Bakshi N, Brown LA et al.. Arginine Therapy for Pain in Sickle Cell Disease: A Phase-2 Randomized, Placebo-Controlled Trial. American journal of hematology 2025. link 31 Ghazaiean M, Darvishi-Khezri H, Najafi B, Karami H, Kosaryan M. Global prevalence of elevated estimated pulmonary artery systolic pressure in clinically stable children and adults with sickle cell disease: A systematic review and meta-analysis. PloS one 2025. link 32 Prussien KV, Burke MM, Gollomp K, Wright RJ, Thompson AA. Cortisol in sickle cell disease: a systematic review and meta-analysis. Blood advances 2025. link 33 Abd Elglil AM, El-Shanshory MR, Elsharkawy HM, Keshk WA, Hablas NM, Morad LM. The efficacy of oral L-arginine therapy for the treatment of vaso-occlusive pain episodes in children with sickle cell disease. Pediatric hematology and oncology 2025. link 34 Zare P, Ahmadkhani A, Taherifard E, Apelian S, Behjoo H, Taherifard E. Factors Associated With Electrocardiographic Abnormalities in Patients With Sickle Cell Disease: A Systematic Review and Meta-Analysis. Pediatric blood & cancer 2025. link 35 Banda K, Nguweneza A, Mnika K, Nembaware V, Chagaluka G, Wonkam A. Clinical and Genetic Predictors of Sickle Cell Nephropathy: A Global Systematic Review. Omics : a journal of integrative biology 2025. link 36 Ibemere SO, Barnhart H, Myers J, Miller J, Osunkwo I, Bosworth HB et al.. 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 2025. link 37 Chaudhary SR, Sharma K, Khatib MN, Saxena DB. Efficacy and safety of Mitapivat in sickle cell disease: a systematic review. Expert opinion on pharmacotherapy 2025. link 38 Karafin MS, Grier AL, Fasano RM, Ilich A, Wichlan D, Chang A et al.. Blood-storage duration affects hematological and metabolic profiles in patients with sickle cell disease receiving transfusions. The Journal of clinical investigation 2025. link 39 Gourdin A, Oudin Doglioni D, Dannoune M, Astié M, Hamelin F, Monnier S et al.. Measurement of fatigue in sickle cell disease: a systematic review of fatigue measures. Orphanet journal of rare diseases 2025. link 40 Smilow E. Gene Therapy for Sickle Cell Disease: No Donor, No Problem. Clinical journal of oncology nursing 2025. link 41 Quinn CT, Ware RE. The Voxelotor Effect: Decreased Affinity for New Drugs for Sickle Cell Disease?. Pediatric blood & cancer 2025. link 42 Starlard-Davenport A. Complexity within simplicity: Exploring the multifactorial nature of sickle cell disease. American journal of human genetics 2025. link 43 Grazioli A, Rabin J, Rector RP, Wu ZJ, Burke AP, Sharifai N et al.. Venoarterial Extracorporeal Membrane Oxygenation Therapy in Patients with Sickle Cell Disease: Case Series and Review for Intensive Care Physicians. Journal of intensive care medicine 2025. link 44 Ortega RP. Gaps remain in college sports' 15-year-old sickle cell policy. Science (New York, N.Y.) 2025. link 45 Al-Judaibi B, Alzahrani H, Alahmari A, Almohareb F, Albenmousa A, Al-Hamoudi W et al.. Emerging need for a hepato-hematology program for patients with sickle cell disease in Saudi Arabia. Saudi journal of gastroenterology : official journal of the Saudi Gastroenterology Association 2025. link 46 Wijngaarde RO, Gouw SC, Ubbink DT. Improving shared decision‑making between paediatric haematologists, children with sickle cell disease, and their parents: an observational post-intervention study. European journal of pediatrics 2025. link 47 Louie E, Tang A, Patel A, Srinivasan R, Bohn S, Wilson M. Improving Sickle Cell Patient Appointment Compliance in a Hospital-based Pediatric Ophthalmology Clinic. Journal of pediatric hematology/oncology 2025. link 48 Taha OB, Liu T, Mandernach MW. Management of severe acute chest syndrome in a patient with a history of severe delayed haemolytic transfusion reaction. BMJ case reports 2025. link 49 Elenga N. Managing life-threatening complications of dengue fever in two sickle cell disease patients: A case series. International journal of infectious diseases : IJID : official publication of the International Society for Infectious Diseases 2025. link 50 Lin Y, Li H, Dong Y, Fang W, Huang H, He M et al.. A retrospective research of adverse event reporting system events for voxelotor based on the FAERS database. BMC pharmacology & toxicology 2025. link 51 Fomekong SD, Wandji Y, Epotto M, Tambe J, Fokam YDP, Tapouh JRM et al.. Stroke risk assessment in children with sickle cell disease using transcranial Doppler ultrasound in Cameroon. The Pan African medical journal 2025. link 52 Abdallah KE, Cooper KE, Buscetta AJ, Ramirez HC, Neighbors HW, Bonham VL. An Examination of John Henryism in Adults Living with Sickle Cell Disease. Journal of racial and ethnic health disparities 2025. link 53 Amseian G, Ortiz-Fernández M, Doti P, Massuet A, Castro P, Pineda C. Brain microbleeds resulting from presumed extensive fat emboli in a patient with bone marrow necrosis following a sickle cell disease vaso-occlusive crisis. Emergency radiology 2025. link 54 Guy D, Bagnall R, Morgan RL, Babatunde I, Nevière A, Friedrich G et al.. Impact of transcranial Doppler screening on stroke prevention in children and adolescents with sickle cell disease: A systematic review and meta-analysis. Blood reviews 2025. link 55 Patel R, Reisert H, Keymakh M, Drakou E, Briggs J, Cohen J et al.. Management of Spontaneous Epidural Hematoma in the Setting of Vaso-occlusive Crisis Among Pediatric Patients With Sickle Cell Disease: A Case Series and Scoping Literature Review. Journal of pediatric hematology/oncology 2025. link 56 Pallangyo P, Mkojera Z, Mfanga L, Komba M, Kisenge PR. Peripartum Cardiomyopathy as the Initial Manifestation of Undiagnosed Sickle Cell Disease: A Case Report. Journal of investigative medicine high impact case reports 2025. link 57 Walden J, Creary S. Practical guide for disease-modifying medication management of children and adolescents with sickle cell disease. Hematology. American Society of Hematology. Education Program 2024. link 58 Lizarralde-Iragorri MA, Parachalil Gopalan B, Merriweather B, Brooks J, Hill M, Lovins D et al.. Isoquercetin for thromboinflammation in sickle cell disease: a randomized double-blind placebo-controlled trial. Blood advances 2024. link 59 Bolarinwa AB, Oduwole O, Okebe J, Ogbenna AA, Otokiti OE, Olatinwo AT. Antioxidant supplementation for sickle cell disease. The Cochrane database of systematic reviews 2024. link 60 Jonassaint CR, Lalama CM, Carroll CP, Badawy SM, Hamm ME, Stinson JN et al.. Digital cognitive behavioral therapy vs education for pain in adults with sickle cell disease. Blood advances 2024. link 61 Saraf SL, Hagar R, Idowu M, Osunkwo I, Cruz K, Kuypers FA et al.. Multicenter, phase 1 study of etavopivat (FT-4202) treatment for up to 12 weeks in patients with sickle cell disease. Blood advances 2024. link 62 Karkoska KA, Gollamudi J, Sawyer RP, Woo D, Hyacinth HI. Quantifying dilated perivascular spaces in children with sickle cell disease. Pediatric blood & cancer 2024. link 63 Allan JM, Fox BM, Kasztan M, Kelly GC, Molina PA, King MA et al.. Enhanced vasoconstriction in sickle cell disease is dependent on ETA receptor activation. Clinical science (London, England : 1979) 2024. link 64 Ogu UO, Mukhopadhyay A, Patel K, Nelson MN, Strahan KS, Wu L et al.. Hydroxyurea at escalated dose versus fixed low-dose hydroxyurea in adults with sickle cell disease. European journal of haematology 2024. link 65 Kumari A, Chauhan G, Chaudhuri PK, Kumari S, Prasad A. Genetic Variants Associated with the Risk of Stroke in Sickle Cell Anemia: Systematic Review and Meta-Analysis. Hemoglobin 2024. link 66 Awor S, Bongomin F, Kaggwa MM, Pebolo FP, Epila J, Malinga GM et al.. Liver and renal biochemical profiles of people with sickle cell disease in Africa: a systematic review and meta-analysis of case-control studies. Systematic reviews 2024. link 67 Matthie N, Higgins M, Doorenbos A, Maddox T, Jenerette C. Feasibility of In-Home Virtual Reality for Chronic Pain in Sickle Cell Disease. Pain management nursing : official journal of the American Society of Pain Management Nurses 2024. link 68 Dovern E, Aydin M, DeBaun MR, Alizade K, Biemond BJ, Nur E. Effect of allogeneic hematopoietic stem cell transplantation on sickle cell disease-related organ complications: A systematic review and meta-analysis. American journal of hematology 2024. link 69 Eades-Brown NT, Oguntoye AO, Aldossary D, Ezenwa MO, Duckworth L, Dede D et al.. Adherence to a reproductive health intervention for young adults with sickle cell. Journal of the American Association of Nurse Practitioners 2024. link 70 AlMoshary M, Arabdin M. The role of prophylactic transfusion on the maternal and fetal outcomes in pregnant women with sickle cell disease: A systematic review and meta-analysis. Medicine 2024. link 71 Gladwin MT, Gordeuk VR, Desai PC, Minniti C, Novelli EM, Morris CR et al.. 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 2024. link00045-0) 72 Casella JF, Furstenau DK, Adams RJ, Brambilla DJ, Lebensburger JD, Fehr JJ et al.. Hydroxyurea to prevent brain injury in children with sickle cell disease (HU Prevent)-A randomized, placebo-controlled phase II feasibility/pilot study. American journal of hematology 2024. link 73 Laredo J, Torres-Small S, Wu L, Makishima T, Richard C. Vestibular Dysfunction in Patients With Sickle Cell Disease: A Systematic Review. Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 2024. link 74 Silva CS, Passos NM, Ribeiro APMR, Mattos RR, Torquato E, Mirada EP et al.. Functional ovarian reserve in women with Sickle Cell disease: A systematic review. JBRA assisted reproduction 2024. link 75 Dawudi Y, Azoyan L, Bonjour M, Steichen O. COVID-19 outcomes among patients with sickle cell disease or sickle cell trait compared to the general population: a systematic review and meta-analyses. Annals of hematology 2024. link 76 Higgs D, Kassouf M. Developing a pill to treat sickle cell disease. Science (New York, N.Y.) 2024. link 77 . Julie Makani: leveraging innovation to tackle sickle cell disease. Bulletin of the World Health Organization 2024. link 78 Keskin M. Spleen Uptake in Sickle Cell Anemia on Bone Scan. Journal of nuclear medicine technology 2024. link 79 Roush K. First Cell-Based Gene Therapy Approved for Sickle Cell Disease. The American journal of nursing 2024. link 80 Rosenberg K. Lactated Ringer Instead of Normal Saline Solution for Sickle Cell Vaso-Occlusive Episodes. The American journal of nursing 2024. link 81 Bangudi S, Stanek C, Shankar D, Hart L, Nahata L, Cronin RM et al.. Primary care use and depression screening among young adults with sickle cell disease during their final year of pediatric hematology care. Pediatric blood & cancer 2024. link 82 Merz LE, Osei MA, Green A, Story CM, Schuering KM, Achebe MO. Development of a Hematology-General Medicine Hybrid Team to Improve Care of Patients With Sickle Cell Disease. Academic medicine : journal of the Association of American Medical Colleges 2024. link 83 Prince EJ, Feder KJ, Calhoun C, Lee AI, Carroll CP, Restrepo V et al.. Trainees' perspectives on sickle cell education: a qualitative needs assessment. BMC medical education 2024. link 84 Amini A, Taherifard E, Akbari M, Taherifard E, Dehdari Ebrahimi N, Rajabi MR et al.. The medication usage pattern and prevalence of polypharmacy among patients with sickle cell disease: a population-based study in southern Iran. Expert review of hematology 2024. link 85 Palermo TM, Lalloo C, Zhou C, Dampier C, Zempsky W, Badawy SM et al.. A cognitive-behavioral digital health intervention for sickle cell disease pain in adolescents: a randomized, controlled, multicenter trial. Pain 2024. link 86 Kisali EP, Iversen PO, Makani J. Low vitamin B. British journal of haematology 2024. link 87 Hanna D, Kamal DE, Fawzy HM, Abd Elkhalek R. Safety and efficacy of monthly high-dose vitamin D. European journal of pediatrics 2024. link 88 Early ML, Raja M, Luo A, Solow M, Matusiak K, Eke AC et al.. Blood pressure thresholds for the diagnosis of hypertensive disorders of pregnancy in sickle cell disease. British journal of haematology 2024. link 89 García-Morin M, Bardón-Cancho EJ, Beléndez C, Dulín E, Blanco-Soto P, Puertas-López C et al.. Madrid Newborn Sickle Cell Disease Cohort: clinical outcomes, stroke prevention and survival. Annals of hematology 2024. link 90 Okonkwo ON, Hassan AO, Oyekunle I, Akanbi T, Agweye C. Visual outcome of treating proliferative sickle cell retinopathy in 108 eyes. European journal of ophthalmology 2024. link 91 Ilonze C, Echefu GC, Broadnax AL, Johnson A, Etuk A, Ilonze OJ. Cardiovascular complications of sickle cell disease: A primer for the general clinician. Journal of the National Medical Association 2024. link 92 Zhang M, Hillegass WB, Yu X, Majumdar S, Daryl Pollard J, Jackson E et al.. Genetic variants and effect modifiers of QT interval prolongation in patients with sickle cell disease. Gene 2024. link 93 Limerick E, Shmukler J, Sirajuddin A, Nguyen ML, Jeffries N, Sachdev V et al.. Improvement in Cardiac Morphology Demonstrated by Cardiac Magnetic Resonance Imaging and Echocardiography after Haploidentical Hematopoietic Cell Transplantation in Adults with Sickle Cell Disease. Transplantation and cellular therapy 2024. link 94 Aljohani OI, Almustafa RN, Almalki BT, Allehaibi MH. Non-traumatic bilateral epidural hematoma in a child with sickle cell anemia: A case report and a review of the literature. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2024. link 95 Hajeh H, Garcia A, Mishra S, Radicic K. Malposition of the central venous catheter secondary to accessory hemiazygos vein variant. The journal of vascular access 2024. link 96 Bain BJ, Daniel Y, Henthorn J, de la Salle B, Hogan A, Roy NBA et al.. Significant haemoglobinopathies: A guideline for screening and diagnosis: A British Society for Haematology Guideline: A British Society for Haematology Guideline. British journal of haematology 2023. link 97 da Silva BB, Pontes AEF, Lemos CAA, Ortega RM. Association between sickle cell disease and periodontal disease: A systematic review. Journal of periodontal research 2023. link 98 Kirkham JK, Estepp JH, Weiss MJ, Rashkin SR. Genetic Variation and Sickle Cell Disease Severity: A Systematic Review and Meta-Analysis. JAMA network open 2023. link 99 Lapite A, Lavina I, Goel S, Umana J, Ellison AM. A Qualitative Systematic Review of Pediatric Patient and Caregiver Perspectives on Pain Management for Vaso-Occlusive Episodes in the Emergency Department. Pediatric emergency care 2023. link 100 Galacteros F, Ethgen O, Beillat M. Modeling the public health impact of voxelotor in the management of sickle cell disease in France. PloS one 2023. link

Original source

[1]
Does Sickle Cell Anaemia Have a Relationship With Avascular Pulp Necrosis? A Systematic Review. — Ribeiro EE, Fernandes KS, Pereira ESJ, Marinho AMCL, Tenório JR, Dos Santos MPA Australian endodontic journal : the journal of the Australian Society of Endodontology Inc (2026)
[2]
Oxidative Stress, Antioxidant Capacity, Dyslipidemia and Cardiovascular Risk in Sickle Cell Disease: A Systematic Review and Meta-Analysis. — Simo JL, Tuono RM, Njopwouo MS, Tayou CT TheScientificWorldJournal (2026)
[3]
Burden and outcomes of cardiovascular and pulmonary complications in adolescents and adults with sickle cell disease: a systematic review and meta-analysis. — Kaweme NM, Buttress SMN, Nguweneza A, Phiri C, Mtisi TJ, Sambo PM et al. Systematic reviews (2026)
[4]
Evaluating the American Society of Hematology quality measure on timeliness of analgesics for sickle cell disease pain crisis. — Gwarzo I, Dega HC, Tanabe P, Miller R, Brousseau DC Blood advances (2026)
[5]
Thrombomodulin protects against acute vascular and multiorgan injury in sickle cell disease. — Ren G, Fraidenburg DR, Setty S, Chen J, Gonzales J, Ruiz MA et al. JCI insight (2026)
[6]
Association of hypertension and genetic variants in MYH9 and BMPR1B with increased proteinuria in sickle cell disease. — Malick Ndour EH, Mnika K, Ousmane Sene EH, Tall FG, Nembaware V, Ly ID et al. Clinical biochemistry (2026)
[7]
Acute Chest Syndrome and Critical Illness-Associated Cerebral Microbleeds in Sickle Cell Disease: A Case Report. — Moura MJ, Costa É, Canelas MA, Maia R, Ramalheira S Acta medica portuguesa (2026)
[8]
Trends in HU utilization and cerebrovascular outcomes before and after publication of the 2014 National Heart, Lung, and Blood Institute sickle cell disease guidelines. — Dain AS, Li Y, Master S, Bailey CL, Vossough A, Ichord RN et al. Pediatric hematology and oncology (2026)
[9]
Vascular Geometry Drives Stroke Risk in Sickle Cell Disease. — Liu W, Kassasseya C, Papamanolis L, Nguyen-Peyre KA, Garreau M, Eckert S et al. American journal of hematology (2026)
[10]
Sickle cell disease. — Colombatti R, Jastaniah W, Makani J, Andemariam B Lancet (London, England) (2026)
[11]
Socioeconomic Status and Stroke Risk in Pediatric Sickle Cell Disease: A DISPLACE Study Secondary Analysis. — Edwards JG, Humphrey DT, Mueller M, Phillips S, Schlenz A, Oni MO et al. Pediatric blood & cancer (2026)
[12]
Targeting PAR1 biased signaling with parmodulin reduces thromboinflammation and acute lung injury in sickle cell disease. — Ramadas N, Lowder K, Dutton J, Kazen RC, Sellers R, DeRousse JT et al. Blood advances (2026)
[13]
Gene expression profiling identifies potential biomarkers for vaso-occlusive episodes in sickle cell disease. — Bhat V, Yoo JJ, Ponna S, Potdar AA, Patel AP, Yu GK et al. JCI insight (2026)
[14]
Addressing Primary Care Needs in People Living With Sickle Cell Disease : A Narrative Review. — Patel A, Wright C, Coyne F, Klein RJ, Nguemeni Tiako MJ, Kuo AA et al. Annals of internal medicine (2026)
[15]
CENTRAL RETINAL ARTERY OCCLUSION AFTER SCLERAL BUCKLING SURGERY WITH GAS INSERTION IN A PATIENT WITH SICKLE CELL DISEASE. — Jalil A, Lippera M, Ivanova T Retinal cases & brief reports (2026)
[16]
Therapeutic Plasma Exchange as a Rescue Therapy in Sickle Cell Disease-Associated Fat Embolism Syndrome: Case Series and Literature Review. — Aljishi AK, Alrabia FA, Al Abbas AM, Alfaraj MM, Alomar FA, Abohelaika S Hemoglobin (2026)
[17]
Strategies and innovations in hypertension management for sickle cell patients: A narrative review. — Obeagu EI Medicine (2026)
[18]
The Pediatric Clinician's Approach to the Diagnosis and Management of Nocturnal Enuresis, Hypertension, and Albuminuria in Children with Sickle Cell Disease. — Lebensburger J, Zahr R Pediatric clinics of North America (2026)
[19]
Stroke burden and functional impacts in adults with sickle cell disease. — St-Onge J, Hristova T, Charles C, Bereznyakova O, Jacquin G, Pouliot O et al. British journal of haematology (2026)
[20]
Red cell specifications for blood group matching in patients with haemoglobinopathies: An updated systematic review and clinical practice guideline from the International Collaboration for Transfusion Medicine Guidelines. — Wolf J, Blais-Normandin I, Bathla A, Keshavarz H, Chou ST, Al-Riyami AZ et al. British journal of haematology (2025)
[21]
A Meta Analysis of RBC Alloimmunization in Transfused Sickle Cell and Thalassemia Patients in Saudi Arabia. — Bawazir WM Clinical laboratory (2025)
[22]
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