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Updated: Oct 15 2018

Sickle Cell Nephropathy

  • Snapshot
    • An 18-year-old African American man with a history of sickle cell trait presents with 1 week of painless gross hematuria. On review of systems, he endorses a 10 pound weight loss within the past 2 months. Physical exam reveals a left-sided abdominal mass. His serum creatinine is found to be elevated. He is sent to the emergency room for urgent CT imaging. (Renal medullary carcinoma secondary to sickle cell trait).
  • Introduction
    • Clinical definition
      • nephropathy associated with sickle cell disease or trait
        • vaso-occlusion
        • renal medullary carcinoma
    • Epidemiology
      • incidence
        • proteinuria
          • 20-25% in sickle cell disease
        • renal insufficiency
          • 5-30% in sickle cell disease
      • risk factors
        • vaso-occlusion
          • sickle cell disease > trait
        • renal medullary carcinoma
          • sickle cell trait > disease
          • patients of African ancestry
    • Pathogenesis
      • normal ↓ O2 tension in the renal medulla is low enough to induce RBC sickling
      • sickling of red blood cells in medulla capillaries cause increased viscosity
      • increased viscosity causes ischemia and infarction in renal medulla
      • this can lead to renal infarcts and papillary necrosis
    • Prognosis
      • prognostic variable
        • negative
          • renal failure
      • increased mortality risk for those with renal failure, regardless of treatment
      • prognosis is < 1 year for renal medullary carcinoma
        • commonly very aggressive and metastasizes early
  • Presentation
    • Symptoms
      • vaso-occlusion
        • nausea
        • vomiting
        • nocturia
        • polyuria
        • painless hematuria
        • history of urinary tract infections
      • renal medullary carcinoma
        • gross painless hematuria
        • weight loss
    • Physical exam
      • vaso-occlusion
        • flank or abdominal pain
        • hypertension
          • renin-mediated
      • renal medullary carcinoma
        • abdominal mass
  • Imaging
    • Ultrasound
      • indications
        • to exclude other abnormalities such as nephrolithiasis
      • recommend views
        • renal
      • findings
        • can detect renal papillary necrosis
    • CT
      • indications
        • if renal medullary carcinoma is suspected (young patient with sickle cell trait and gross hematuria)
      • findings
        • mass in kidney
  • Studies
    • Labs
      • electrolyte abnormalities
        • impaired distal H+ and K+ secretion can cause a renal tubular acidosis
          • hyperkalemia
          • metabolic acidosis
      • ↑ creatinine
    • Urinalysis
      • proteinuria
      • low specific gravity and osmolarity
        • indicates loss of urine concentrating ability
    • Hemoglobin electrophoresis
    • Diagnostic criteria
  • Differential
    • Nephrolithiasis
      • seen on imaging
  • Treatment
    • Conservative
      • hydration and blood transfusions as needed
        • indications
          • isolated hematuria
          • hydration can dislodge blood clots
    • Medical
      • dialysis
        • indications
          • end-stage renal disease
    • Operative
      • renal transplant
        • indications
          • end-stage renal disease not responsive to dialysis
  • Complications
    • End-stage renal disease
    • Renal medullary carcinoma with metastases
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