As anemias falciformes são doenças hemolíticas crônicas que podem induzir três tipos de acidentes agudos: anemia grave, infecções bacterianas graves e acidentes vasooclusivos isquêmicos (AVO) causados por hemácias em forma de foice que obstruem pequenos vasos sanguíneos e capilares. Muitas complicações diversas podem ocorrer.
Introdução
O que você precisa saber de cara
As anemias falciformes são doenças hemolíticas crônicas que podem induzir três tipos de acidentes agudos: anemia grave, infecções bacterianas graves e acidentes vasooclusivos isquêmicos (AVO) causados por hemácias em forma de foice que obstruem pequenos vasos sanguíneos e capilares. Muitas complicações diversas podem ocorrer.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 23 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 49 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
Triagem neonatal (Teste do Pezinho)
A triagem neonatal permite diagnóstico precoce e início imediato do tratamento.
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
1 gene identificado com associação a esta condição. Padrão de herança: Autosomal recessive.
Involved in oxygen transport from the lung to the various peripheral tissues LVV-hemorphin-7 potentiates the activity of bradykinin, causing a decrease in blood pressure Functions as an endogenous inhibitor of enkephalin-degrading enzymes such as DPP3, and as a selective antagonist of the P2RX3 receptor which is involved in pain signaling, these properties implicate it as a regulator of pain and inflammation
Heinz body anemias
Form of non-spherocytic hemolytic anemia of Dacie type 1. After splenectomy, which has little benefit, basophilic inclusions called Heinz bodies are demonstrable in the erythrocytes. Before splenectomy, diffuse or punctate basophilia may be evident. Most of these cases are probably instances of hemoglobinopathy. The hemoglobin demonstrates heat lability. Heinz bodies are observed also with the Ivemark syndrome (asplenia with cardiovascular anomalies) and with glutathione peroxidase deficiency.
Medicamentos e terapias
Mecanismo: Hemoglobin HbA positive modulator
Mecanismo: P-selectin blocker
Mecanismo: Ribonucleoside-diphosphate reductase RR1 inhibitor
Mecanismo: CAMPATH-1 antigen inhibitor
Mecanismo: Purinergic receptor P2Y12 antagonist
Mecanismo: Coagulation factor X inhibitor
Mecanismo: Selectin E antagonist
Mecanismo: Glucocorticoid receptor agonist
Mecanismo: Purinergic receptor P2Y12 negative allosteric modulator
Mecanismo: Mu opioid receptor agonist
Variantes genéticas (ClinVar)
615 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
10 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Anemia de células falciformes
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Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 2.433
Genetic and Functional Insights into F-Cell Distribution in Sickle Cell Anemia: A Genome-Wide Association Study Approach.
Sickle cell anemia (SCA) is a severe hereditary disorder characterized by hemoglobin polymerization, vaso-occlusion, and chronic hemolysis. The distribution of F-cells, which retain fetal hemoglobin (HbF), plays a crucial role in mitigating disease severity. Genome-wide association studies (GWAS) have identified genetic determinants influencing F-cell persistence, yet the underlying mechanisms require further elucidation. This study aims to analyse publicly available GWAS data to identify key genetic loci associated with F-cell distribution in SCA, assess their biological relevance, and explore potential therapeutic implications. GWAS data were extracted from publicly available repositories and analysed for significant single nucleotide polymorphisms (SNPs) associated with F-cell levels. Functional enrichment analysis was performed using Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analysis. Transcription factor (TF) interactions, microRNA (miRNA) regulation, and protein-protein interactions (PPI) were explored to understand the molecular mechanisms governing HbF expression. The study identified BCL11A, HBS1L-MYB, and β-globin cluster polymorphisms as primary regulators of F-cell distribution. Functional analysis highlighted pathways related to erythropoiesis, chromatin remodelling, and transcriptional regulation. miRNA-target interactions and epigenetic modifiers emerged as critical determinants of HbF persistence. These findings provide insights into the genetic regulation of F-cell distribution, reinforcing the therapeutic potential of HbF modulation. Future studies should focus on functional validation and targeted interventions to enhance HbF expression in SCA. The online version contains supplementary material available at 10.1007/s12288-025-02127-3.
Impact of HBS1L-MYB Gene Single Nucleotide Polymorphisms on Fetal Hemoglobin Expression in Moroccan Sickle Cell Anemia Children: Preliminary Results.
Fetal hemoglobin (HbF) expression is a key modifier of sickle cell disease (SCD) severity, and the HBS1L-MYB intergenic region is a critical regulator of HbF levels. However, the impact of HBS1L-MYB polymorphisms and HbF levels in Moroccan populations remains underexplored. This work aimed to investigate the population-specific distribution of three HBS1L-MYB polymorphisms (rs28384513, rs4895441, and rs9402686) in 160 Moroccan children, including 80 with SCD (1-15 years) and 80 healthy controls. The SCD group was further divided into two groups based on HbF levels (< 15% and ≥ 15%). DNA genotyping was performed using PCR-RFLP analysis. Retrospective demographic and hematological data were collected to assess the association between these SNPs and HbF levels. Significant associations were found between rs28384513 and red blood cell count (p = 0.009), rs4895441 and mean corpuscular haemoglobin (p = 0.02), and rs9402686 with both hemoglobin (p = 0.001) and HbF levels (p = 0.003), confirming the significant influence of this SNP on HbF expression. These findings highlight the beneficial effect of rs9402686 on HbF expression. Investigating the large and genetically diverse cohort of Moroccan SCD will be essential for a deeper understanding of the molecular mechanisms underlying these polymorphisms and for identifying new disease modifier genes.
Gene Therapy for Sickle Cell Anemia in India - Current Status and Challenges.
Sickle cell disease (SCD) is a critical monogenic disorder impacting millions of people in India and severely compromising the quality of life for the patients. At present, the only available curative treatment for sickle cell disease (SCD) is allogenic hematopoietic stem cell transplantation. However, recent advancements in gene therapy offer promising prospects for curing the disease, either through the correction of the pathogenic mutation or the induction of Fetal hemoglobin production. This review focuses on the gene addition and gene editing technologies being currently employed in the treatment of SCD, with a particular emphasis on therapeutic interventions that are undergoing clinical trials globally. Furthermore, this review provides an insight into the extensive research efforts being undertaken in India to develop curative therapies for SCD, while also addressing the significant challenges faced by the healthcare system and patients, including ethical, socio-economic, and regulatory barriers unique to the Indian context.
Narratives unveil knowledge and awareness-related issue, reinforcing patients' self-identity in sickle cell disease.
Sickle cell disease (SCD) increasingly requires a holistic approach. Narratives have been used to complement clinical and observational findings related to quality of life (QoL). The "Sickle Cell Anemia Narrations" project aimed to help frame the illness burden and QoL of the multi-ethnic population with SCD in Italy via a narrative approach. Twenty-one adult patients with SCD and 10 informal caregivers from seven SCD centers and two Patient Associations volunteered for this project. Researchers collected anonymous narratives and independently analyzed them through content analysis. Lack of SCD knowledge on multiple levels was found to strongly impact the illness experience independent of ethnicity. Fear of stigma at school, the challenging management of vaso-occlusive crises, and a lack of SCD knowledge at the workplace were reported. Fifty-five percent of participants reported a lack of SCD knowledge among healthcare professionals working outside the expert centers and 33% reported misdiagnosis. Caregivers highlighted the lack of coordination among territorial healthcare facilities. Our findings confirm that SCD represents a critical burden for patients and caregivers and indicate that lack of SCD knowledge may bolster the barriers to care, thus revealing the urgent need to enhance awareness to foster inclusion and quality of care.
Magnitude of Involvement of Peripheral Nervous System in Sickle Cell Anemia Patients in Vaso-Occlusive Crisis.
Sickle cell disease is an inborn genetic blood disorder that is usually found among residents of Africa, Middle-East Gulf countries, and India. There has been increased knowledge and awareness of the range of neurological complications of Sickle cell disease (SCD) over the past 20 years. Silent neurologic involvement may occur in SCD and there's an opportunity of subclinical involvement in these patients presenting with vasoocclusive crisis. The case-control study was carried out after getting the institutional ethical clearance and obtaining consent from the patients and the controls. They were subjected to nerve conduction studies. Nerve conduction study was performed on median and tibial nerves for motor nerves, and median and sural for sensory nerves unilaterally. The nerves of the upper limb showed changes in neuropathy. The lower limb nerves were completely normal. In our study, we found out that Distal motor latency (DML) is prolonged in the Median motor nerve in cases as compared to controls suggesting motor neuropathy, and Sensory nerve action potential (SNAP) is reduced in the median sensory nerve suggesting sensory neuropathy. The nerve conduction studies in the tibial nerve were normal, suggesting subclinical neuropathy could be present in the patients with sickle cell presenting as mononeuropathy or mononeuritis multiplex. Our study also found a statistically significant correlation of neuropathy with the increasing number of hospital admissions for the vaso-occlusive crisis. Subclinical neuropathy is another manifestation of nervous system in patients with SCD and one should give adjuvant therapy of membrane stabilizing agents for the relief of pain. Detailed studies of this complication must be required further to evaluate its significance in clinical practice. We also conclude the aches and the pain the patient of sickle cell disease complains could not be always due to vaso-occlusive crisis, it could be the neuropathic pain which might mimic the ischemic pain.
Publicações recentes
Immune Imbalance in Sickle Cell Anemia: Flow Cytometric Insights Into Regulatory T Cells and Neutrophil Dynamics.
Reliability of diffuse optical spectroscopies for evaluation of cerebral hemometabolic stress in children with sickle cell anemia.
Molecular mastery: Harnessing DNA technology for disease prevention.
Growth and puberty in African children with sickle cell anemia treated with hydroxyurea.
Systematic Review and Meta-analysis on the Prevalence of Common Hemoglobinopathies in India.
📚 EuropePMC4.225 artigos no totalmostrando 194
Design, Synthesis and Anti-Sickling Activity of some Novel Analogs of Isoindoline-1,3-dione.
Biotechnology and applied biochemistryThe Longitudinal Effect of APOL1 Risk Alleles on Sickle Cell Anemia-Associated Kidney Function.
American journal of hematologyAngiotensin signaling is essential for stress erythropoiesis but causes retention of dysfunctional mitochondria in RBCs.
JCI insightPrevention and Management of Acute Chest Syndrome Without Automated Red Cell Exchange Among Patients With Sickle Cell Disease Not Requiring ICU Care: A Systematic Review and Network Meta-Analysis.
European journal of haematologyIFN-I-Mediated Transcriptional Reprogramming Drives Myeloid-Skewed Hematopoiesis in Sickle Cell Anemia.
American journal of hematologyPhase I study of the safety and pharmacokinetics of CSL889 (hemopexin) in adults with sickle cell anemia.
HaematologicaVariations in mitochondrial genome as potential prognostic markers in sickle cell disease.
HaematologicaExploring plasma microRNA profiling and signaling pathways in comorbidity-free sickle cell anemia: a pilot case-control study.
BMC research notesMagnetic resonance imaging evaluation of myocardial and hepatic tissue remodeling and cardiac function in sickle cell anemia.
Diagnostic and interventional radiology (Ankara, Turkey)Predictors of Acute Chest Syndrome in Patients With Sickle Cell Disease: A Cross-Sectional Observational Study.
CureusStroke risk assessment in children with sickle cell disease using transcranial Doppler ultrasound in Cameroon.
The Pan African medical journalGenetics and Genomics in Sickle Cell Disease in Africa.
American journal of hematologyFrom Iron Deficiency to Overload: A Missing Link in the Mechanisms of Cardiac Autonomic Nervous System Dysfunction.
Journal of clinical medicineMechanism-informed transition from pediatric to adult care in sickle cell disease: a case study.
BMC health services researchOxidative Stress, Antioxidant Capacity, Dyslipidemia and Cardiovascular Risk in Sickle Cell Disease: A Systematic Review and Meta-Analysis.
TheScientificWorldJournalThe Impact of Aging on Organ Systems in Sickle Cell Disease: a Comparative Review of Physiological Adaptation and Dysfunction.
Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacologyLiver Cirrhosis and In-Hospital Outcomes in Patients With Sickle Cell Crises.
EJHaemUtility of First-Line Diagnostic Tests and Molecular Methods for Accurate Characterization of Sickle Cell Disorders.
International journal of laboratory hematologyExpanding the Blood Donor Pool: An Update to Initiatives to Increase New Donor Participation.
Clinics in laboratory medicineRadiomic Features Extracted from Magnetic Resonance Imaging to Identify Arteriopathy in a Humanized Mouse Model of Sickle Cell Anemia and Sickle Cell Trait.
Annals of biomedical engineeringNon-aneurysmal Subarachnoid Hemorrhage in an Adult With Sickle Cell Anemia: A Case Report and Review of the Literature.
CureusGenetic and Functional Insights into F-Cell Distribution in Sickle Cell Anemia: A Genome-Wide Association Study Approach.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionImpact of HBS1L-MYB Gene Single Nucleotide Polymorphisms on Fetal Hemoglobin Expression in Moroccan Sickle Cell Anemia Children: Preliminary Results.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionGene Therapy for Sickle Cell Anemia in India - Current Status and Challenges.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionArtificial Intelligence-Based Analysis of Central Nervous System Vasculopathy in Pediatric Sickle Cell Anemia.
American journal of hematologyBetween crises and care: Childhood realities with sickle cell Anemia: A qualitative study.
Journal of pediatric nursingNewborn Screening for Hemoglobinopathies and Thalassemias: Brief History, Recent Activities, and Global Status-2026.
International journal of neonatal screeningNarratives unveil knowledge and awareness-related issue, reinforcing patients' self-identity in sickle cell disease.
Orphanet journal of rare diseasesFulminant hepatitis secondary to dengue in pediatric patients: a series of four cases.
Revista do Instituto de Medicina Tropical de Sao PauloA Digital Patient Engagement and Monitoring System to Improve Quality, Safety, and Continuity of Care for Pediatric Sickle Cell Anemia: A Cross-Sectional Study of Caregiver Perceptions.
CureusAccelerated brain atrophy in sickle cell anemia despite chronic exchange transfusion therapy-A case report.
Radiology case reportsThe GLOBE Trial: Efficacy and Safety of L-Glutamine Plus Hydroxyurea Versus Hydroxyurea Alone in Sickle Cell Anemia - A Double-Blind, Randomized Study.
Turkish journal of haematology : official journal of Turkish Society of HaematologyDose-dependent effects of diamide and glutaraldehyde on red blood cell deformability, viscosity, and morphology.
Microvascular researchIncreasing daily step counts improves physical fitness, reduces pain and arterial stiffness in sickle cell patients.
HaematologicaWeeping Wound, Disgruntled Gut and Fading Hunger: Acrodermatitis Enteropathica in an Infant.
CureusAI-assisted diagnosis of anemia through peripheral smear image analysis: A cross-validation study.
BioinformationPatterns of hormonal contraception use in women with sickle cell disease and employer-sponsored insurance.
ContraceptionIncreased Endothelin-1 and High-Risk APOL1 Variants Contribute to Albuminuria in Pediatric and Young Adults with Sickle Cell Anemia.
Clinical journal of the American Society of Nephrology : CJASNAdaptation of lentiviral vectors for viral gene therapy and their impact on host cell biology.
Journal of translational medicineBiomarkers for the diagnosis and phenotyping of acute chest syndrome in patients with sickle cell disease.
Expert review of respiratory medicineSickle Cell Disease at a Tertiary Care Center in the Vidarbha Region of India: Protocol for a Clinical and Observational Study.
JMIR research protocolsLiquid Hydroxyurea (Xromi) for Children With Sickle Cell Anemia: A New Solution Compounding Existing Problems.
Pediatric blood & cancerSelf-resolution of spontaneous epidural hematoma in sickle cell anemia: A case report.
Radiology case reportsCost-Effectiveness of Hydroxyurea for Treatment of Children With Sickle Cell Anemia in Ghana.
Value in health regional issuesIdentifying strokes in Nigerian children with sickle cell disease as part of clinical trials: training curriculum for healthcare professionals in low-income settings.
Frontiers in strokeGenotype Differences and Hydroxyurea Utilization Among Adults With Moderate to Severe Sickle Cell Disease.
PharmacotherapyMagnitude of Involvement of Peripheral Nervous System in Sickle Cell Anemia Patients in Vaso-Occlusive Crisis.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionHealth-related quality of life and adherence to hydroxyurea in patients with sickle cell anemia in Saudi Arabia.
MedicineSickle Cell Disease in Eastern Uttar Pradesh, India: Sociodemographic and Clinical Profile from a Previously Undocumented Area.
Journal of racial and ethnic health disparitiesAssessment of Urine Kidney Injury Molecule-1 as an Early Biomarker for Nephropathy in Sickle Cell Anaemia Patients.
Acta medica (Hradec Kralove)Assessing educational needs of sickle cell anemia healthcare providers in sub-Saharan Africa and the Caribbean.
Frontiers in public healthHydroxyurea utilization among individuals with sickle cell disease in Tennessee: a pooled analysis of claims data.
Frontiers in pharmacologySustained expression of hemopexin in an animal model of sickle cell anemia.
Blood vessels, thrombosis & hemostasisMental Resilience, Satisfaction from Life, and Quality of Life of People with Hemoglobinopathies : Quality of Life of People with Haemoglobinopathies.
Advances in experimental medicine and biologyTherapeutic applications of CRISPR-Cas9 gene editing.
Frontiers in genome editingHydroxycarbamide and Sickle Cell Anemia: Paradoxical Effects Related to Redox Mechanisms of Cellular Adaptation.
ACS omegaEfficacy of an Ayurvedic Intervention as an Adjunct to Standard Care in Preventing Acute Pain Crises in Sickle Cell Anemia: Protocol for a Randomized Controlled Trial.
JMIR research protocolsRole of Automated Red Cell Exchange in Sickle Cell Anemia and Poisoning.
Annals of African medicineIron, Oxidative Stress, and Haptoglobin Gene Polymorphism in Sickle Cell Disease Patients With Inflammation in Cameroon: An Analytical Cross-Sectional Study.
Biochemistry research internationalLow Use of FDA-Approved Medications for Sickle Cell Disease in Adults.
European journal of haematologyElectrocardiographic abnormalities among children with sickle cell anaemia at steady state and crises attending federal teaching Hospital, Owerri, South East Nigeria.
BMC pediatricsCase report: Sea Fan Retinopathy, an ocular anomaly of interest.
Archivos de la Sociedad Espanola de OftalmologiaFulminant Pneumococcal Meningitis With Neurovascular Complications in an Adult With Sickle Cell Anemia: A Case Report.
CureusTargeting LncRNAs with CRISPR/Cas9 for Kidney Therapeutics: A Review.
International journal of biological macromoleculesFeasibility and acceptability of a new adapted physical activity program for children with chronic disease: a pilot study.
Archives de pediatrie : organe officiel de la Societe francaise de pediatrieRelation of Adverse Childhood Experiences to Clinical and Patient-Reported Outcomes for Adults With Sickle Cell Disease: A Registry Study.
Pediatric blood & cancerVitamin D receptor gene polymorphisms and 25-hydroxyvitamin D levels: association with cerebrovascular disease in sickle cell anemia.
Pediatric hematology and oncologyHydroxyurea to decrease stroke risk in children with sickle cell anemia: a systematic review and meta-analysis.
Blood global hematologyProlonged, Unintentional Overdose of Hydroxyurea in a Child With Sickle Cell Anemia.
Pediatric blood & cancerClinical presentation and outcomes of children hospitalized with sickle cell anemia at Gulu regional referral hospital, Uganda: a retrospective cross-sectional study.
Scientific reportsCase Report: Complete Coverage Of Chronic Sickle Cell Leg Ulcer Using Hyperbaric Oxygen Therapy Combined With Skin Grafting.
Undersea & hyperbaric medicine : journal of the Undersea and Hyperbaric Medical Society, IncEarly Intervention as a Way of Reducing Neurocognitive Delay in Pediatric Sickle Cell Patients.
CureusMonocyte-red blood cell crosstalk supports clearance and heme metabolism in sickle cell anemia.
Frontiers in immunologyRare Coexistence of Hemoglobin D-Iran and Hemoglobin S in a Case from Central India.
HemoglobinAcute Exercise Challenge and Airway Dynamics in Youth With Sickle Cell Anemia: A Multicenter Study.
American journal of hematologyChronic Hydroxyurea Therapy in Children with Sickle Cell Anemia: Mechanisms of Action, Systemic Effects, and Long-Term Safety.
Journal of clinical medicineHydroxyurea Therapy in Sickle Cell Disease: Evaluating Healthcare Costs and Utilization in a Brazilian Tertiary Setting.
Blood global hematologyReversal of Echocardiographic Alterations Following Hematopoietic Stem Cell Transplantation in Children With Sickle-Cell Anemia.
American journal of hematologyAlbuminuria Predicts a Rapid Decline in Kidney Function in 2 International, Longitudinal Cohorts of Adults With Sickle Cell Anemia.
American journal of hematologyManaging acute myeloid leukemia in the context of sickle cell anemia and suspected Fanconi anemia in Tanzania: a case report.
Journal of medical case reportsRecent advancements in aptamer-mediated theranostics in the management of hematological disorders.
International journal of pharmaceuticsRegional brain age is decreased in children with sickle cell anemia.
Blood advancesApplication of Artificial Intelligence in Sickle Cell Identification From Blood Smears: A Potential Game Changer for Developing Nations.
CureusLongitudinal changes and predictors of cardiac extracellular volume fraction in sickle cell anemia.
Blood. Red cells & ironSuccessful implementation of stroke risk screening for sickle cell anemia in the DISPLACE study: results of a cluster randomized trial.
Implementation science : IS[Priapism: current status and new insights].
Urologie (Heidelberg, Germany)Burden of sickle cell anemia in Africa: A systematic review and meta-analysis.
PloS oneEfficacy of Filial Therapy on Pain and Health-Related Quality of Life of Children With Sickle Cell Disease in a Middle Eastern Country.
Pain management nursing : official journal of the American Society of Pain Management NursesImpact of Hydroxyurea on Liver, Kidney, and Hematological Parameters in Sickle Cell Anemia Patients Above 15 Years Old in Sana'a City.
International journal of general medicineAssociation of hypertension and genetic variants in MYH9 and BMPR1B with increased proteinuria in sickle cell disease.
Clinical biochemistryIncluding malnourished siblings in treatment improves nutritional outcomes for children with sickle cell anemia in Northern Nigeria: Results from a feasibility trial.
Nutrition research (New York, N.Y.)Digestive autoimmune diseases mimicking gastrointestinal manifestations in children with sickle cell anemia: A report of three cases.
JPGN reportsMultimodality, multidisciplinary approach to overcome technical challenges in a pediatric stem cell harvest for sickle cell disease with preformed donor-specific antibodies.
Asian journal of transfusion scienceTherapeutic red blood cell exchange in a sickle cell disease patient with acute bone pain crisis: Our experience in a tertiary care center.
Asian journal of transfusion scienceClinical and laboratory manifestations and outcomes of human parvovirus B19 infections in sickle cell anemia with emphasis on the impact of hydroxyurea.
International journal of infectious diseases : IJID : official publication of the International Society for Infectious DiseasesEarly risk factors for acute chest syndrome in sickle cell anemia: A pediatric study.
PloS onePleiotropic Effects of Polymorphisms in the BCL11A Gene on Laboratory Parameters in Sickle Cell Anemia.
International journal of molecular sciencesPublisher Correction: Performance of creatinine and cystatin C-based equations to estimate glomerular filtration rate in African children with sickle cell anemia.
Pediatric nephrology (Berlin, Germany)The Pediatric Clinician's Approach for Anxiety, Depression, and Suicide Ideation in Children with Sickle Cell Disease.
Pediatric clinics of North AmericaHydroxyurea for Children with Sickle Cell Disease: A Practical Guide for Pediatric Clinicians.
Pediatric clinics of North AmericaSickle cell anemia and social justice: a pathway to equitable healthcare in Uganda.
Annals of medicine and surgery (2012)3D printed hydroxyurea for pediatric use: toward personalized formulations and reduced exposure risk.
International journal of pharmaceuticsTherapeutic drug monitoring of busulfan in pediatric patients: a 5-year observational study.
BMC pharmacology & toxicologyClinical and Laboratory Correlates of Cerebral Blood Flow Velocities and Risks for Stroke Among Steady-State Sickle Cell Anemia Children: A Cross-Sectional Study.
CureusFirst detection of echovirus 18 associated with aseptic meningitis in a child in Niger Republic, 2024: a case report.
BMC infectious diseasesThirty Years of Hydroxyurea for Sickle Cell Anemia - Scientific Progress, Global Health Gaps.
The New England journal of medicineUnexpected absence of fetal hemoglobin induction by lenalidomide in a patient with sickle cell anemia with concurrent multiple myeloma.
Orphanet journal of rare diseasesImproved Preprocedure Hematocrit in Chronic RBC Exchange Patients Following Splenectomy: A Case Report.
Case reports in hematologyPerformance of creatinine and cystatin C-based equations to estimate glomerular filtration rate in African children with sickle cell anemia.
Pediatric nephrology (Berlin, Germany)Cerebral Blood Flow Decline in Adults With Sickle Cell Anemia: What Does It Mean and Is Treatment Required?
NeurologyCerebral Blood Flow in Children and Adults With Sickle Cell Anemia.
NeurologyAn extramedullary hematopoietic lesion causing acute lumbar stenosis in the setting of sickle cell anemia: a case report and review of the literature.
Journal of spine surgery (Hong Kong)Advances in nucleic acid probe-based detection of gene point mutations: a review.
Frontiers in chemistryTen-Year Longitudinal Study Clarifies Impact of Hemolysis and Pulmonary Hypertension on Patients with Sickle Cell Anemia.
American journal of respiratory and critical care medicinePrimary and secondary stroke prophylaxis in children with sickle cell anemia: a meta-analysis.
Blood advancesGender effect on Psychoacoustical abilities and Speech perception in noise in individuals with Sickle cell anemia.
Journal of otologyAnticancer role of Gramicidin A in myeloid leukemia.
Discover oncologyRadiologic Evaluation of Paranasal Sinuses in Sickle Cell Anemia and Thalassemia: Case-Control Study.
Laryngoscope investigative otolaryngologyMicrosensor Array for the Electrochemical Analysis of Hydroxyurea in Blood Samples of Children Affected by Sickle Cell Anemia.
Analytical chemistryHaplo-Identical Stem Cell Transplant Resolves Ventricular Arrhythmias and Dysfunction in a Sickle Cell Anemia Patient: Case Report and Discussion.
EJHaemUse of Incentive Spirometry to Prevent Acute Chest Syndrome (ACS) in Patients With Sickle Cell Disease (SCD): A Systematic Review.
CureusLived Experiences of Clinical Depression Among Adolescents With Sickle Cell Anemia in Dar-es-salaam, Tanzania: A Qualitative Study.
Research squareHydroxyurea pharmacokinetics in children with sickle cell anemia across different global populations.
Blood advancesRare Comorbidity of Sickle Cell Disease and Wilms' Tumor: A Case Report.
Case reports in medicineSeverity patterns and predictors of sickle cell anaemia among Gambian children: A cross-sectional analysis.
Annals of hematology[Sickle cell disease, 2025].
Orvosi hetilapHemoglobin SC Sickle Cell Disease Presenting as Acute Ischemic Stroke With Moyamoya Syndrome in an Adult: A Case Report.
CureusPhysiologically based pharmacokinetic modeling of hydroxyurea: Implications for dose adjustment in patients with renal insufficiency.
Journal of pharmaceutical sciencesNon-invasive depiction of hepatic and myocardial iron overload using MRI.
La Clinica terapeuticaEarly Detection of Diastolic Dysfunction in Sickle Cell Anemia: Can It Help the Patient and Let Us See the Elephant in the Room?
American journal of hematologyLessons Learned From Provider Minder: A Provider Tracking Application for Improving Stroke Risk Screening in Sickle Cell Anemia.
Quality management in health careMorbidity and mortality of sickle cell disease in Côte d'Ivoire.
Annals of hematologyA thyroid storm causing strokes and unmasking moyamoya.
Radiology case reportsMetabolomics in sickle cell disease: Current knowledge and gaps - A scoping review.
Blood reviewsThe Economic Impact of Premarital Screening (PMS) of Sickle Cell Anemia on the Saudi Health System: A Cost Analysis Study.
Healthcare (Basel, Switzerland)Optimized protocol for intracellular labeling of red blood cells with anti-hemoglobin F for confocal microscopy analysis.
Micron (Oxford, England : 1993)Impact of TMPRSS6 gene polymorphism on iron overload among children with sickle cell disease.
Hematology/oncology and stem cell therapyCohort Tracking of Sickle Cell Trait-Positive Births Identified by Newborn Screening, California, 1991-2013: Public Health Surveillance for Sickle Cell Trait.
Public health reports (Washington, D.C. : 1974)Screening for Hemoglobinopathies: An Inceptive Experience of Centre of Excellence for Sickle Cell Disease.
Indian pediatricsUse of Fructosamine for Glycemic Monitoring in Patients With Sickle Cell Disease and Diabetes: A Systematic Review.
CureusSevere Hemolytic Phenotype in Children With Sickle Cell Anemia and Co-Inherited Red Blood Cell Variants.
Pediatric blood & cancerCost-Effectiveness Analysis of Crizanlizumab in Sickle Cell Disease in Iran.
Iranian journal of medical sciencesHarnessing Drosophila melanogaster for biomedical innovation in Africa: Trends, challenges, and opportunities.
Journal of the National Medical AssociationAge Less Than 24 Months is Not an Independent Risk Factor for Serious Bacterial Illness in Febrile Children with Sickle Cell Disease.
The Journal of emergency medicineSalvage Structures, Known as Iron Chelating Agents, Acquired from the Nature and Matured in the Labs.
Current medicinal chemistryDe novo rates of a Trypanosoma-resistant mutation in two human populations.
Proceedings of the National Academy of Sciences of the United States of AmericaSeptic Arthritis due to Salmonella Paratyphi A in a Splenectomized Thalassemia Patient.
The Journal of the Association of Physicians of IndiaGene therapy for HbSC disease and other compound heterozygous sickle hemoglobinopathies: a time for inclusion.
BloodClinical Manifestations in the Maxillofacial Region of Acute Crisis of Sickle Cell Anemia: A Case Report.
Journal of clinical and experimental dentistryEarly pregnancy maternal hemoglobin and the risk of neonatal congenital heart disease: insights from a case-control study, Guangdong, China.
Postgraduate medical journalMacular microvascular alterations in adults with sickle cell anemia detected by optical coherence tomography angiography.
Photodiagnosis and photodynamic therapyCB2 and TRPV1 receptors in inflammatory state of macrophages from sickle cell anemia pediatric/young adults.
Scientific reportsA Case of Sickle Cell Beta Thalassemia and Recurrent Septicemia.
CureusAcute soft head syndrome and orbital compression syndrome in a child with sickle cell disease: a case report.
Journal of medical case reportsHyperhemolysis in a sickle cell disease patient in pregnancy.
Annals of hematologyAdvances in Gene Therapy with Oncolytic Viruses and CAR-T Cells and Therapy-Related Groups.
Current issues in molecular biologySickle Cell Diastolic Cardiomyopathy and Mortality Risk: A Novel Echocardiographic Framework for Prognostic Stratification.
American journal of hematologyPolymorphic Distribution of Human Homeostatic Iron Regulator Gene H63D rs1799945 and Clinico-Hematological Parameters of Sickle Cell Anemia Patients: A Case-Control Study in Northern Ghana.
Health science reportsTransiently acquired hemoglobin variants in patients with sickle cell disease: A case series and systematic review of the literature.
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for HaemapheresisEmpowering Nurses to Enhance Disaster Preparedness for Individuals With Sickle Cell Disease: A Cross-Sectional Study.
International nursing reviewA Comprehensive Guide to Typhoidal Anemia.
Prague medical reportOral manifestations of sickle cell disease and its effects on dental and periodontal health: A systematic review.
Journal of advanced periodontology & implant dentistryReversal of Glomerular Hyperfiltration Following Hematopoietic Stem Cell Transplantation in Children With Sickle-Cell Anemia.
American journal of hematologyHemoglobin Sickle-Beta-Thalassemia With an Acute Crisis.
CureusPrevalence, Etiological Patterns and Factors Associated with Bacteraemia Among Febrile Children with Sickle Cell Anaemia in Jinja Regional Referral Hospital, Uganda.
Infection and drug resistanceThe Association of Serum Level of TGF-β1 and Clinical Manifestations in Sickle Cell Anemia: A Case-Control Study.
Health science reportsThrough a Glass Darkly: Perceptions of Ethnoracial Identity in Artificial Intelligence Generated Medical Vignettes and Images.
Medical science educatorFox-Fordyce Disease in Women: A Case Report Highlighting Laser-Based Interventions.
CureusDifferences in oxygen-gradient ektacytometry parameters and blood viscosity between patients with sickle cell anemia and patients with sickle cell-hemoglobin C disorder.
Clinical hemorheology and microcirculationAssociation of sleep study outcomes and health care utilization in children with sickle cell disease: a multicenter study.
Blood advancesEarly detection and management of extracranial arteriopathy reduces the incidence of silent cerebral infarcts in sickle cell anemia: a long-term prospective cohort study.
HaematologicaRed Blood Cells are Critical for Hemostasis and Thrombosis.
Seminars in thrombosis and hemostasisIdentifying and addressing underuse in hematologic care through systems-based hematology.
Research and practice in thrombosis and haemostasisImpact of elevated red blood cell membrane cholesterol in sickle cell anemia patients: Effects of BRN-002, a 2-hydroxypropyl-β-cyclodextrin derivate, on red blood cell lipids, deformability, sickling and hemolysis.
Blood cells, molecules & diseasesGenome-Wide CRISPR Screen for Unveiling Radiosensitive and Radioresistant Genes.
Journal of visualized experiments : JoVEPlasma Zinc and Magnesium Levels in Sickle Cell Disease Patients in Latakia, Syria.
CureusAssessing the safety of gene therapy vectors expressing an enhanced gamma-globin gene for the cure of sickle cell anemia.
Molecular therapy. Methods & clinical developmentClinical Implications of HbD-Punjab and HbS co-Inheritance - A Rare Case in South India.
HemoglobinPrimary Stroke Screening and Hydroxyurea Treatment for Sickle Cell Anemia in Pediatric Healthcare Settings in East and Central Africa: A Narrative Review of Capacity Gaps and Opportunities.
Public health reviewsThe Prevalence and Predictors of Sickle Cell Anemia in the Saudi Arabia General Population: Findings from a Cross-Sectional Study.
Healthcare (Basel, Switzerland)Emergency Presentations of Pediatric Sickle Cell Disease in French Guiana.
Diseases (Basel, Switzerland)Sequential gastric and ileal perforations, a rare presentation in a man with sickle cell anemia: A case report.
The Journal of international medical researchRacial Inequities Influencing Admission, Disposition and Hospital Outcomes for Sickle Cell Anemia Patients: Insights from the National Inpatient Sample Database.
Hematology reportsAltered Mental Status Due to Hyperammonemia Syndrome and PRES in a 31-Year-old Bone Marrow Transplant Recipient.
Journal of investigative medicine high impact case reportsSelective removal of 7-ketocholesterol by a novel atherosclerosis therapeutic candidate reverts foam cells to a macrophage-like phenotype.
AtherosclerosisComparing Absolute Neutrophil Counts of Children With Sickle Cell Disease With and Without the Duffy Null Phenotype.
Pediatric blood & cancerMulticenter Experience of Catheter Ablation for Atrial Fibrillation in Sickle Cell Anemia.
Journal of cardiovascular electrophysiologyRepair of type A aortic dissection in a patient with sickle cell disease.
Journal of surgical case reportsPrevalence of Antiplatelet and Anticoagulation Therapy in Children with Sickle Cell Anemia and Stroke.
Pediatric strokeImpact of Switching From Race-Based to Race-Neutral Spirometry Reference Equations in Children With Sickle Cell Anemia.
American journal of hematologyHealth-Related Quality of Life in Children With Sickle Cell Anemia in Malaria-Endemic Regions: The Impact of Disease States and Malaria Prevention Strategies.
Value in health regional issuesNoise Propagation and MP-PCA Image Denoising for High-Resolution Quantitative $R_2^{\rm{*}}$, $T_2^{\rm{*}}$, and Magnetic Susceptibility Mapping (QSM).
IEEE transactions on bio-medical engineeringCaffeic Acid-Biogenic Amine Complexes Outperform Standard Drugs in Reducing Toxicity: Insights from In Vivo Iron Chelation Studies.
Molecular pharmaceuticsInflammation and Iron Profile in Children With Sickle Cell Disease in Cameroon: Frequency and Associated Factors, an Analytical Cross-Sectional Study.
Health science reportsAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Genetic and Functional Insights into F-Cell Distribution in Sickle Cell Anemia: A Genome-Wide Association Study Approach.Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion· 2026· PMID 41728188mais citado
- Impact of HBS1L-MYB Gene Single Nucleotide Polymorphisms on Fetal Hemoglobin Expression in Moroccan Sickle Cell Anemia Children: Preliminary Results.Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion· 2026· PMID 41728183mais citado
- Gene Therapy for Sickle Cell Anemia in India - Current Status and Challenges.Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion· 2026· PMID 41728147mais citado
- Narratives unveil knowledge and awareness-related issue, reinforcing patients' self-identity in sickle cell disease.
- Magnitude of Involvement of Peripheral Nervous System in Sickle Cell Anemia Patients in Vaso-Occlusive Crisis.Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion· 2026· PMID 41522549mais citado
- Immune Imbalance in Sickle Cell Anemia: Flow Cytometric Insights Into Regulatory T Cells and Neutrophil Dynamics.
- Reliability of diffuse optical spectroscopies for evaluation of cerebral hemometabolic stress in children with sickle cell anemia.
- Molecular mastery: Harnessing DNA technology for disease prevention.
- Growth and puberty in African children with sickle cell anemia treated with hydroxyurea.
- Systematic Review and Meta-analysis on the Prevalence of Common Hemoglobinopathies in India.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:232(Orphanet)
- OMIM OMIM:603903(OMIM)
- MONDO:0011382(MONDO)
- GARD:8614(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
