To begin at the beginning: sickle cell disease in Africa

Abstract

Sickle cell disease is one of the most common severe inherited disorders in the world, with numbers projected to increase substantially in the next 40 years. 1 Piel FB Hay SI Gupta S Weatherall DJ Williams TN Global burden of sickle cell anaemia in children under five, 2010–2050: modelling based on demographics, excess mortality, and interventions. PLoS Med. 2013; 10: e1001484 Crossref PubMed Scopus (509) Google Scholar It is characterised by unpredictable episodes of acute illness, and chronic organ damage. Sickle cell disease has been studied in high-income countries for about 100 years, but treatment options are few and life expectancy is still reduced by about 30 years compared with those without the disease. 2 Brousse V Makani J Rees DC Management of sickle cell disease in the community. BMJ. 2014; 348: g1765 Crossref PubMed Scopus (42) Google Scholar Early renal damage in patients with sickle cell disease in sub-Saharan Africa: a multinational, prospective, cross-sectional studyHyperhaemolysis is associated with albuminuria, with an age-dependent effect, in the SS and Sβ0 phenotypes only, suggesting a different pathological mechanism for glomerular disease in the patients with SC and Sβ+ phenotypes. However, both phenotypes are associated with a high prevalence of albuminuria in childhood. Therefore, screening for albuminuria is advised in African children with sickle cell disease to detect early renal damage. Full-Text PDF