The clinical significance of K-Cl cotransport activity in red cells of patients with HbSC disease
| Autor: | A Osei, Swee Lay Thein, Emma Drasar, Anke Hannemann, David C. Rees, John S. Gibson, Sanjay Tewari |
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| Přispěvatelé: | Hannemann, Anke [0000-0002-2925-1124], Gibson, John [0000-0001-6145-9139], Apollo - University of Cambridge Repository |
| Rok vydání: | 2015 |
| Předmět: |
Erythrocytes
Abnormal/metabolism inorganic chemicals Adult Male Pathology medicine.medical_specialty Cellular pathology Erythrocytes Adolescent Erythrocytes Abnormal Disease Anemia Sickle Cell Hemoglobin SC Disease/diagnosis digestive system environment and public health Young Adult Internal medicine medicine Humans Clinical significance Young adult Anemia Sickle Cell/diagnosis Child Hemoglobin SC Disease Symporters business.industry urogenital system Symporters/metabolism Hematology Articles medicine.disease Pathophysiology Sickle cell anemia Hospitalization Endocrinology Erythrocytes/metabolism Child Preschool Biomarker (medicine) Female business hormones hormone substitutes and hormone antagonists |
| Zdroj: | Rees, D C, Thein, S L, Osei, A, Drasar, E, Tewari, S, Hannemann, A & Gibson, J S 2015, ' The clinical significance of K-Cl cotransport activity in red cells of patients with HbSC disease ', Haematologica, vol. 100, no. 5, pp. 595-600 . https://doi.org/10.3324/haematol.2014.120402 |
| DOI: | 10.3324/haematol.2014.120402 |
| Popis: | HbSC disease is the second commonest form of sickle cell disease, with poorly understood pathophysiology and few treatments. We studied the role of K-Cl cotransport activity in determining clinical and laboratory features, and investigated its potential role as a biomarker. Samples were collected from 110 patients with HbSC disease and 41 with sickle cell anemia (HbSS). K-Cl cotransport activity was measured in the oxygenated (K-Cl cotransport(100)) and deoxygenated (K-Cl cotransport(0)) states, using radioactive tracer studies. K-Cl cotransport activity was high in HbSC and decreased significantly on deoxygenation. K-Cl cotransport activity correlated significantly and positively with the formation of sickle cells. On multiple regression analysis, K-Cl cotransport increased significantly and independently with increasing reticulocyte count and age. K-Cl cotransport activity was increased in patients who attended hospital with acute pain in 2011 compared to those who did not (K-Cl cotransport(100): mean 3.87 versus 3.20, P=0.009, independent samples T-test; K-Cl cotransport(0): mean 0.96 versus 0.68, P=0.037). On logistic regression only K-Cl cotransport was associated with hospital attendance. Increased K-Cl cotransport activity was associated with the presence of retinopathy, but this effect was confounded by age. This study links variability in a fundamental aspect of cellular pathology with a clinical outcome, suggesting that K-Cl cotransport is central to the pathology of HbSC disease. Increased K-Cl cotransport activity is associated with increasing age, which may be of pathophysiological significance. Effective inhibition of K-Cl cotransport activity is likely to be of therapeutic benefit. |
| Databáze: | OpenAIRE |
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