Orthopedic Complications Of Sickle Cell Disease: A Retrospective Evaluation Of 469 Patients In Port Harcourt, Nigeria DOI Creative Commons

Friday Enwumelu Aaron,

Linda Anucha Dublin-Green,

Rex Friday Ogoronte Alderton Ijah

et al.

Advances In Clinical And Medical Research, Journal Year: 2024, Volume and Issue: 5(1), P. 1 - 10

Published: Jan. 3, 2024

Background: Sickle cell anemia (SCA) is a hereditary blood disorder characterized by abnormal hemoglobin, leading to the deformation of red cells.There growing impact this condition on musculoskeletal system.This study aimed evaluate Orthopedic complications in patients with SCA Port Harcourt between While standard care known be use analgesia, oxygenation, hydration, transfusions, and prevention triggers sickle crisis, [18] drug -Voxelotor, an inhibitor polymerization Hemoglobin S increasing oxygen affinity, has recently been shown very useful [19][20][21][22].Orthopedic presentations reported Eastern Nigeria, [23][24] Western [25][26] southern [27] Northern Nigeria [28].There July 2013 June 2023.Materials Methods: A retrospective analytical who presented Hematology clinics two teaching Hospitals was conducted.Collected data analyzed Statistical Package for Social Sciences.Results: Among 469 SCA, 198 were males.The mean age population 25.21 years.The assessed included Avascular Necrosis Femoral head, Chronic leg ulcers (CLU), Osteomyelitis, Osteoarthritis.A vascular (AVN) head observed 50 (44.6%)patients.Chronic present 57 (50.9%) participants, while Osteomyelitis (OM) identified 14 (12.5%)patients.Osteoarthritis (OA) least common complication, occurring only 3 individuals.The group above 25years statistically associated development especially CLU OM. Conclusion:Orthopedic are patients, ulcers, Osteoarthritis being most frequently complications.These findings underscore need comprehensive management strategies multidisciplinary minimize these patients' quality life.

Language: Английский

Quercetin: A Potential Polydynamic Drug DOI Creative Commons
Nikitas Georgiou,

Margarita Georgia Kakava,

E. Alexandros Routsi

et al.

Molecules, Journal Year: 2023, Volume and Issue: 28(24), P. 8141 - 8141

Published: Dec. 17, 2023

The study of natural products as potential drug leads has gained tremendous research interest. Quercetin is one those products. It belongs to the family flavonoids and, more specifically, flavonols. This review summarizes beneficial pharmaceutical effects quercetin, such its anti-cancer, anti-inflammatory, and antimicrobial properties, which are some quercetin described in this review. Nevertheless, shows poor bioavailability low solubility. For reason, encapsulation macromolecules increases therefore efficiency. In review, a brief description different forms described, new ones proposed. applying nanotechnology outlined.

Language: Английский

Citations

34

Mathematical Analysis for the Flow of Sickle Red Blood Cells in Microvessels for Bio Medical Application DOI Creative Commons
Purnima Chaturvedi,

Sapna Ratan Shah

The Yale Journal of Biology and Medicine, Journal Year: 2023, Volume and Issue: 96(1), P. 13 - 21

Published: March 31, 2023

Sickle cell disease (SCD) is an inherited monogenic which characterized by distorted red blood cells (RBCs) that cause vaso-occlusion and vasculopathy. In the pathogenesis of SCD, polymerized hemoglobin turn RBCs into fragile, less deformable cells, are subsequently more susceptible to endothelial adhesion after deoxygenation. Presently, electrophoresis genotyping used as routine tests for diagnosis SCD. These techniques expensive require specialized laboratories. Lab-on-a-chip technology a low-cost microfluidics-based diagnostic tool holds significant promise rapid screening RBC deformability. To explore sickle mechanics purposes, we present mathematical model flow single with altered rheological properties slip effect on capillary wall in microcirculation. We consider single-file through axis symmetrical cylindrical duct, applying lubrication theory plasma trapped between successive cells. The parameters from published literature normal corresponding variation has been taken purpose this simulation condition disease. An analytical solution found realistic boundary conditions results simulated using MATLAB. height film increases increase deformability compliance affects forward velocity capillary. Rigid increased shows reduction occurrence events extreme conditions. coupled microfluidics can mimic physiological provides unique insights novel possibilities design base kit towards effective therapeutic intervention

Language: Английский

Citations

27

Sickle Cell Disease Update: New Treatments and Challenging Nutritional Interventions DOI Open Access
Victoria Bell, Theodoros Varzakas, Θεοδώρα Ψαλτοπούλου

et al.

Nutrients, Journal Year: 2024, Volume and Issue: 16(2), P. 258 - 258

Published: Jan. 15, 2024

Sickle cell disease (SCD), a distinctive and often overlooked illness in the 21st century, is congenital blood disorder characterized by considerable phenotypic diversity. It comprises group of disorders, with sickle anemia (SCA) being most prevalent serious genotype. Although there have been some systematic reviews global data, worldwide statistics regarding SCD prevalence, morbidity, mortality remain scarce. In developed countries lower number patients, cutting-edge technologies led to development new treatments. However, developing settings where (SCD) more prevalent, medical management, rather than cure, still relies on use hydroxyurea, transfusions, analgesics. This that affects red cells, consequently affecting organs diverse manners. We discuss its etiology advent technologies, but aim this study understand various types nutrition-related studies involving individuals suffering from SCD, particularly Africa. The interplay environment, food, gut microbiota, along their respective genomes collectively known as microbiome, host metabolism responsible for mediating metabolic phenotypes modulating microbiota. addition, it serves purpose providing essential nutrients. Moreover, engages direct interactions homeostasis immune system, well indirect via metabolites. Nutrition interventions nutritional care are mechanisms addressing increased nutrient expenditures important aspects supportive management patients SCD. Underprivileged areas Sub-Saharan Africa should be accompanied efforts define promote Their importance key maintaining well-being quality life, especially because products limited, while native medicinal plant resources acknowledged.

Language: Английский

Citations

14

Iron restriction in sickle cell disease: When less is more DOI Creative Commons
Oswaldo Castro, Lucia De Franceschi, Tomas Ganz

et al.

American Journal of Hematology, Journal Year: 2024, Volume and Issue: 99(7), P. 1349 - 1359

Published: Feb. 23, 2024

Primum non nocere! Can iron deficiency, an abnormality that causes anemia, benefit people with sickle cell disease (SCD) who already have anemia? The published literature we review appears to answer this question in the affirmative: basic science considerations, animal model experiments, and noncontrolled clinical observations all suggest a therapeutic potential of restriction SCD. This is because SCD's manifestations are ultimately attributable polymerization hemoglobin S (HbS), process strongly influenced by intracellular HbS concentration. Even small decrements concentration greatly reduce polymerization, deficiency lowers erythrocyte Thus, could improve SCD changing its features those more benign anemia (i.e., condition fewer or no vaso-occlusive events). We propose well-designed studies be implemented definitively determine whether safe effective option These investigations particularly timely now pharmacologic agents being developed, which may directly red concentrations without need for phlebotomies deplete total body iron.

Language: Английский

Citations

5

Gene therapy in pediatrics – Clinical studies and approved drugs (as of 2023) DOI Creative Commons
Tahereh Mohammadian, Fatemeh Zahedipour,

Paul Trosien

et al.

Life Sciences, Journal Year: 2024, Volume and Issue: 348, P. 122685 - 122685

Published: May 6, 2024

Gene therapy in pediatrics represents a cutting-edge therapeutic strategy for treating range of genetic disorders that manifest childhood. involves the modification or correction mutated gene introduction functional into patient's cells. In general, it is implemented through two main modalities namely ex vivo and therapy. Currently, noteworthy array products has received valid market authorization, with several others various stages approval process. Additionally, multitude clinical trials are actively underway, underscoring dynamic progress within this field. Pediatric fields hematology, oncology, vision hearing loss, immunodeficiencies, neurological, metabolic areas interventions. This review provides comprehensive overview evolution current therapy-based treatments clinic pediatric patients. It navigates historical milestones therapies, currently approved by U.S. Food Drug Administration (FDA) and/or European Medicines Agency (EMA) children, promising future disorders. By providing thorough compilation drugs published results completed ongoing trials, serves as guide clinicians to get quick situation studies 2023.

Language: Английский

Citations

4

Reference Intervals of Haematological Indices for Young Adults with Sickle Cell Anaemia in Southwestern Nigeria DOI Creative Commons

M. A. Asafa,

Ibrahim O. Ahmed,

Mary Boluwatife. Agim

et al.

Research Square (Research Square), Journal Year: 2025, Volume and Issue: unknown

Published: Jan. 8, 2025

Abstract Background Reference intervals (RIs) are crucial for the interpretation of results and overall patient management. Of note, there no established reference values haematological parameters patients with sickle cell anaemia (SCA) in Nigeria, making it difficult clinicians to interpret diagnose abnormalities appropriately. Objective This study aimed at establishing RIs some indices individuals SCA. Methods descriptive cross-sectional study, included a total six hundred seventy-eight (337 males 341 females) young adults SCA aged 18–35 years. The full blood count (FBC) was done using haematology autoanalyzer. were classified 2.5th 97.5th percentiles delineated as low (< percentile), normal interval (2.5–97.5th percentile) high (> percentile). All analyses SPSS IBM, version 20.0 software Results Generally, Haematocrit is 5.2-9.2g/dl, while WBC ranged between 5.9 12.1 x 109/l platelet 166–465 109/l. For male participants, haematocrit found be 5.2-9.3g/dl, 6.7 166 399 female subjects had 5.5g/dl 8.8 g/dl, 12.96 225 465 Conclusion has

Language: Английский

Citations

0

Associations between disease severity, depression, health-related quality of life, and physical activity in adults with sickle cell disease DOI Creative Commons
Adekola Babatunde Ademoyegun,

Mutiu Ademayowa Adeyemo,

Babatunde Y. Adewuyi

et al.

Bulletin of Faculty of Physical Therapy, Journal Year: 2025, Volume and Issue: 30(1)

Published: Jan. 22, 2025

Abstract Background Individuals with sickle cell disease (SCD) are faced a plethora of challenges that affect their quality life, mood, and physical social participation. Physical activity (PA) is known to reduce inflammatory enhance psychosocial health in individuals chronic illnesses; however, there exist controversies on the role PA severity (DS), depression, health-related life (HRQoL) SCD. Objective This study aimed assess level DS, HRQoL SCD investigate mediation models among HRQoL, adults Methods A total 385 patients (mean age 35.45 ± 12.01 years; 51.70% female) attending hematology clinics two Nigerian tertiary hospitals participated this cross-sectional study. Data were assessed through modified scoring protocol, patient questionnaire-9, 12-item short-form survey, international questionnaire short-form, respectively. Multiple hierarchical regression analyses applied. Results The findings show 53.5% physically inactive, accounts for 27.50%, 18.40%, 38.80%, 8.50% variance observed component summary (PCS-12) mental (MCS-12) There was significant mediating DS relationship between depression (β = − 0.0026; LLCI 0.0031; ULCI 0.0022), PCS-12 0.0019; 0.0013; 0.0024), MCS-12 0.0009; 0.0001; 0.0018). mediated only 0.2975; 0.1825; 0.4066). Similarly, significantly 0.0021; 0.0017). Conclusion high levels have low symptoms reported better HRQoL. Furthermore, especially showed depressive Clinicians policymakers should consider incorporating assessments interventions into care formulation specific guidelines also warranted.

Language: Английский

Citations

0

Novel N-6-X-3-pyridinoyl-N'-4-chlorophenylthioureas (X = Cl, Me): Synthesis, characterization, computational analysis and in silico biological activity studies DOI
Karzan Mahmood Ahmed,

Rahman Azeez Muhammed,

Rebaz Anwar Omar

et al.

Journal of Molecular Structure, Journal Year: 2025, Volume and Issue: unknown, P. 142235 - 142235

Published: March 1, 2025

Language: Английский

Citations

0

Occlusive cutaneous vasculopathies: rare differential diagnoses DOI

Maria Rosa Burg,

Inga Hansen, Leopold Torster

et al.

JDDG Journal der Deutschen Dermatologischen Gesellschaft, Journal Year: 2025, Volume and Issue: unknown

Published: April 4, 2025

In addition to erythematous plaques, nodules, ulcerations, and necrosis, retiform purpura livedo racemosa are indications of occlusive cutaneous vasculopathies. contrast vasculitis, vasculopathies primarily lead vascular occlusion the skin only secondarily signs inflammation. The lower legs typically affected, but especially in presence acral changes, should also be considered. Various stimuli can trigger vasculopathies, including systemic or coagulopathies, emboli, platelet erythrocyte aggregates, cold-dependent gelling agglutination immunoglobulins, or, less commonly, medications.

Language: Английский

Citations

0

Scanning electron microscopy in hemotology DOI
Alexander A. Artamonov, А. М. Иванов

Laboratory Service, Journal Year: 2025, Volume and Issue: 14(1), P. 38 - 38

Published: April 8, 2025

Today, in most cases, the morphology of red blood cells is examined peripheral smears using light microscopy. Computer visualization methods are being implemented everywhere, automated cell counting technologies used, and pathological forms successfully differentiated neural networks trained on microscopy images. However, these have a certain degree subjectivity, due to fact that if does not pronounced defect, operator or network always manage differentiate various forms. This review article discusses main for assessing laboratory practice, describes characteristics cells, criteria their assessment, mechanisms formation forms, considers scanning electron as an additional method morphology.

Language: Английский

Citations

0