ABSTRACT
An outline of the pathogenesis of renal disease in SCD, primarily based on the principles of microvascular obstruction and tissue damage, is shown in Figure 27.2. However, the clinical spectrum of SCN cannot be fully explained by vasoocclusive pathophysiologic processes alone. Genetic factors undoubtedly inuence renal and other complications in SCD. Platt et al.15 and Lebensburger et al.16 identied low fetal hemoglobin levels as a risk factor in adults with early death. It has long been noted that the presence of β globin Central African Republic haplotype is associated with renal involvement.17 Some studies suggest that microdeletions of α globin genes in α thalassemia are protective and are associated with lower prevalence of proteinuria.18 Genetic polymorphisms may explain the variation for risk of developing CKD.
