Management of Kidney diseases in Sickle Cell children in Belgium and in the Democratic Republic of Congo
Sickle cell disease is one of the most common heritable genetic diseases in the world, caused by the mutation in the beta globin chain of the adult haemoglobin. Nephropathy is a serious complication of SCD and is associated with early mortality. Previous studies reported that renal complications occur in 5-18% of sickle cell children and adolescents and >9% of deaths in young adults were due to complications related to kidney disease. Sickle cell nephropathy (SCN) begins in childhood and may progress to overt renal failure. Several risk factors have been reported to play an important role in the development and progression of SCN, such as specific variants in the foetal haemoglobin (HbF) promoting loci, pre-hypertensive blood pressure (BP) profiles, increased BP, the HBBs (gene ßs) haplotypes, low hemoglobin levels, hemolysis and hemoglobinuria. However, the relative importance of these risk factors (especially hemolysis) has not been found in all cohorts, reflecting the complexity of SCN physiopathology and the contribution of other factors, particularly genetic and/or epigenetic ones such as some genetic variants in apolipoprotein L-1 (APOL1) and in heam-oxygenase type 1 (HMOX1). Sickle cell anaemia is basically common among sub-Saharan African, India, Saudi Arabia and Mediterranean countries descendants. Migration of these set of people into Europe and America continent, increases the gene in these continents. Considering the large African community living in Western countries, sickle cell disease and its complications might significantly contribute to the common morbidity faced by some European countries. Therefore, the current study aims to describe the extent of subclinical SCN (proteinuria, microalbuminuria, hyperfiltration and decreased renal function) in children living in Belgium and in the Democratic Republic of Congo, and to assess their predictors with special emphasis on APOL1 and HEMOX risk variants. In addition, to gain insight into the basic mechanism of kidney injury induced by these variants using podocytes cell lines generated from urine of patients carrying APOL1 risk variants.