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1.
Br J Haematol ; 203(2): 319-326, 2023 10.
Artículo en Inglés | MEDLINE | ID: mdl-37583261

RESUMEN

Sickle cell anaemia (SCA) is a monogenic disease with a highly variable clinical course. We aimed to investigate associations between microvascular function, haemolysis markers, blood viscosity and various types of SCA-related organ damage in a multicentric sub-Saharan African cohort of patients with SCA. In a cross-sectional study, we selected seven groups of adult patients with SS phenotype in Dakar and Bamako based on the following complications: leg ulcer, priapism, osteonecrosis, retinopathy, high tricuspid regurgitant jet velocity (TRV), macro-albuminuria or none. Clinical assessment, echocardiography, peripheral arterial tonometry, laboratory tests and blood viscosity measurement were performed. We explored statistical associations between the biological parameters and the six studied complications. Among 235 patients, 58 had high TRV, 46 osteonecrosis, 43 priapism, 33 leg ulcers, 31 retinopathy and 22 macroalbuminuria, whereas 36 had none of these complications. Multiple correspondence analysis revealed no cluster of complications. Lactate dehydrogenase levels were associated with high TRV, and blood viscosity was associated with retinopathy and the absence of macroalbuminuria. Despite extensive phenotyping of patients, no specific pattern of SCA-related complications was identified. New biomarkers are needed to predict SCA clinical expression to adapt patient management, especially in Africa, where healthcare resources are scarce.


Asunto(s)
Anemia de Células Falciformes , Úlcera de la Pierna , Osteonecrosis , Priapismo , Enfermedades de la Retina , Masculino , Adulto , Humanos , Hemólisis , Viscosidad Sanguínea , Estudios Transversales , Microcirculación , Senegal , Úlcera de la Pierna/etiología , Enfermedades de la Retina/etiología
2.
Artículo en Inglés | MEDLINE | ID: mdl-38846172

RESUMEN

The Coronavirus disease 2019 pandemic is a real crisis that has exposed the unpreparedness of many healthcare systems worldwide. Several underlying health conditions have been identified as risk factors, including sickle cell disease, a chronic illness with various complications that can increase the risk of severe COVID-19 infection. Our study aimed to investigate the profile of sickle cell patients diagnosed with COVID-19 and explore any potential relationship between these two conditions. We analyzed data from 11 sickle cell patients who contracted COVID-19 between June and December 2020 and were treated at the CRLD (Center for Sickle Cell Disease and Research). The patients' COVID-19 diagnosis was confirmed using the (Real-Time Reverse Transcriptase-Polymerase Chain Reaction) RT-PCR technique on nasopharyngeal swab samples and/or based on clinical and radiological findings, including CT scans. The patients consisted of 7 males and 4 females, with a mean age of 40 ± 12 years. The sickle cell phenotypes observed were SC (45.4%), SS (36.37%), and Sß± thalassemia (18.2%). During the COVID-19 infection, we observed a slight increase in white blood cell and platelet counts, but a decrease in mean hemoglobin levels and red blood cells. Only 3 out of 11 patients (28%) had a fever at the time of diagnosis. Three patients required red blood cell transfusions due to severe anemia, and 7 out of 11 patients (63.6%) were hospitalized, with one patient admitted to the intensive care unit due to pulmonary embolism. All patients recovered from COVID-19.

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