22-31: Sickle Cell Disease

Kidney dysfunction associated with sickle cell disease is most commonly due to sickling of red blood cells in the renal medulla because of low oxygen tension and hypertonicity. Congestion and stasis lead to hemorrhage, interstitial inflammation, and papillary infarcts with resultant necrosis. Clinically, hematuria is common, and proteinuria can be present as well, portending a poorer prognosis. Damage to renal capillaries also leads to diminished concentrating ability. Isosthenuria (urine osmolality equal to that of serum) is routine, and patients can easily become dehydrated. These abnormalities are also encountered in patients with sickle cell trait. Sickle cell glomerulopathy is less common but inexorably progresses to ESKD. Its primary clinical manifestation is proteinuria. Optimal treatment requires adequate hydration and control of the sickle cell disease.