Gross or microscopic hematuria may be seen in children with sickle cell disease or sickle trait and tends to resolve spontaneously in the majority of children (Chapter 456.1). With the exception of the associated renal cell carcinoma, clinically apparent renal involvement occurs more commonly in patients with sickle cell disease than those with sickle cell trait.

Etiology

The renal manifestations of sickle cell nephropathy are generally related to microthrombosis secondary to sickling in the relatively hypoxic, acidic, hypertonic renal medulla where vascular stasis is present. Analgesic use, volume depletion with consequent prerenal failure, infectious complications, and iron-related hepatic disease are independent contributing factors.

Pathology

Ischemia, papillary necrosis, and interstitial fibrosis are present in the kidneys of these patients.

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