Renal involvement in sickle cell disease
Number:27ESURABS0034
Type:Educational Electronic Presentation
Authors: Sara Peixoto, Pedro Lameira, Luísa Lobo
Keywords:“Sickle cell disease”, Kidney
SECTIONS
Educational objective

Understand the pathology and imaging findings of renal involvement in sickle cell disease.


Topic Review

Sickle cell disease is a hereditary hemoglobinopathy characterized by the rigid deformation of red blood cells. The sickling of these cells may impair the vascular supply of multiple organs. The kidneys are frequently involved because of their rich blood flow and complex capillary network.

In the early stages of the disease, the renal blood flow increases as a compensatory response to hypoxia which leads to glomerular hypertrophy and renal enlargement (fig.1). Overtime, the kidneys may become atrophic with renal failure development.

The impairment of the microvascular circulation and subsequent ischemic necrosis of the distal segments of the renal pyramids results in medullary necrosis and fibrosis. At imaging, additionally to renal enlargement we may see medullary or diffusely increased renal echogenicity on US (fig.1) or hyperattenuating renal pyramids on CT with or...

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11-year-old boy with sickle cell disease. Enlarged right kidney with hyperechoic pyramids.
Lumbar CT of a patient with sickle cell disease. Incidental finding of papillary calcifications in the left kidney.


Conclusions

Because of the rich blood flow, kidneys are commonly affected in sickle cell disease. Radiologists should be aware of the pathology and imaging findings of renal involvement in this disorder to provide accurate differential diagnosis.