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Sickle Cell Disease

PROJECTS

PROJECT 1

To determine the variation in the levels of five major haemolysis cytoprotective proteins in SCD by studying a cohort of 7,000 patients from six cities in Ghana, Nigeria and Tanzania. To document the prevalence of acute damage to major organs during hospitalization of SCD patients in a cohort of 7,000 SCD patients. To catalogue genome-wide variants associated with the levels of the five haemolysis cytoprotective proteins in SCD patients. To provide evidence in support of or against the idea that SCD patients with higher plasma concentrations of haemolysis cytoprotective proteins have a lower risk than age- and gender-matched counterparts in developing acute damage to major organs during VOC and hyper hemolysis.

PROJECT 2

To determine the prevalence of malaria, severe malaria anaemia, malaria ARDS and cerebral malaria in SCD by longitudinally tracking a cohort of 7,000 patients Catalogue genome-wide variants associated with severe malaria complications among SCD patients.Provide observational evidence to support or refute the idea that polymorphisms associated with levels of key haemolysis cytoprotective proteins influence development of severe malaria among SCD patients. Provide evidence of the inflammatory response (cytokines/chemokines and adhesion molecules) in SCD patients with and without malaria, and determine levels of biomarkers of malaria severity and their relationship with plasma haem levels and genetic variants that influence haemolysis cytoprotective protein levels.

PROJECT 3

To phenotype major echo-cardiovascular biomarkers (cardiac dilation, cardiac index, blood pressure, diastolic dysfunction, tricuspid jet regurgitation velocity (TRJV) and RV dysfunction) in 2,000 adults with SCD patients from West and East Africa. To catalogue genetic variants associated with the aforementioned echo- cardiovascular phenotypes in adult SCD patient. To determine whether polymorphism associated with HCP levels are linked to specific echo-cardiovascular phenotypes.


CORES