The clinical significance of K-Cl cotransport activity in red cells of patients with HbSC disease

Haematologica
David C ReesJohn S Gibson

Abstract

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 activi...Continue Reading

Citations

Dec 17, 2016·British Journal of Haematology·Lydia H PeckerLori Luchtman-Jones
Oct 21, 2017·Blood·Patrick G Gallagher
Jan 30, 2018·British Journal of Haematology·David C ReesJo Howard
Jan 30, 2020·PloS One·Caroline Conceição da GuardaMarilda Souza Goncalves
Jun 19, 2018·Frontiers in Physiology·Rick HuisjesRichard van Wijk
Apr 5, 2018·Clinical Hemorheology and Microcirculation·Carlo Brugnara
Aug 9, 2020·Frontiers in Physiology·Eszter BecskeháziViktória Venglovecz

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