What should be done if an 8-year-old child with sickle cell (SS) disease has hematuria (blood in the urine)?

Immediate Evaluation and Management of Hematuria in an 8-Year-Old with Sickle Cell Disease

Your grandson needs urgent medical evaluation to determine if this is benign sickling-related hematuria versus a more serious complication like renal papillary necrosis, infection, or glomerular disease—start with urinalysis with microscopy, urine culture, and assessment of hydration status. 1

Initial Clinical Assessment

Obtain a focused history looking for:

• Presence of pain (flank, abdominal, or dysuria) versus painless hematuria 1
• Recent fever, upper respiratory infection, or streptococcal throat infection 1
• Recent trauma or strenuous physical activity 1
• Associated symptoms: frequency, dysuria, joint pains, rash, or bloody diarrhea 1
• Color of urine: bright red (suggests lower tract bleeding) versus tea-colored (suggests glomerular source) 1

Physical examination must include:

• Blood pressure measurement (hypertension may indicate glomerular involvement) 1
• Temperature (fever suggests infection) 1
• Abdominal examination for masses, nephromegaly, or costovertebral angle tenderness 1
• Assessment for edema, rash, or arthritis 1

Essential Laboratory Workup

Perform urinalysis with microscopy immediately to differentiate the source: 1

• Glomerular hematuria shows: tea-colored urine, proteinuria (>2+ on dipstick), red blood cell casts, and dysmorphic red blood cells on phase contrast microscopy 1
• Non-glomerular hematuria shows: bright red blood, absence of casts, normal-appearing red blood cells 1

Additional initial labs: 1

• Urine culture to rule out urinary tract infection 1
• Complete blood count with platelets (assess baseline hemoglobin and rule out thrombocytopenia) 1
• Blood urea nitrogen and serum creatinine (evaluate renal function) 1
• Spot urine calcium-to-creatinine ratio (evaluate for hypercalciuria) 1

Sickle Cell-Specific Considerations

Painless hematuria in sickle cell disease is common and usually benign, caused by sickling in the renal medulla: 2

• The renal medullary countercurrent mechanism creates a hypoxic, acidotic, and hypertonic environment that promotes sickling 2, 3
• This typically affects the left kidney more than the right 2
• Hematuria can be massive but is usually self-limited 4, 2

However, you must rule out renal papillary necrosis, which is a serious complication: 5

• Presents with painless gross hematuria in children with sickle cell disease 5
• Can lead to severe consequences including obstruction and infection if not diagnosed 5
• Requires imaging if suspected 5

Imaging Decisions

For isolated microscopic hematuria without proteinuria or dysmorphic red blood cells, no imaging is indicated initially. 1

Ultrasound is indicated if: 1, 5

• Macroscopic (gross) hematuria is present 1, 5
• Proteinuria is present (>2+ on dipstick) 1
• There are signs suggesting structural abnormality or renal papillary necrosis 1, 5
• Renal biopsy is being considered 1

Renal ultrasound will assess: 1

• Kidney size and anatomy 1
• Presence of masses, stones, or hydronephrosis 1, 5
• Signs of chronic kidney disease 1

Treatment Approach

For benign sickling-related hematuria (painless, no infection, no proteinuria): 2, 3

• Aggressive hydration is the primary treatment—this dilutes the urine and reduces sickling 2, 3
• Encourage oral fluids if the child can tolerate them 2
• Consider IV hydration if oral intake is inadequate 2
• Bed rest until hematuria resolves 2
• Avoid NSAIDs due to potential renal toxicity 2

If hematuria persists beyond 3-5 days despite hydration, consider: 3

• Oral urea therapy (though evidence is limited to case reports) 3
• Urologic consultation if bleeding is massive or prolonged 4

If urinary tract infection is confirmed:

• Treat with appropriate antibiotics based on culture results 1

If glomerular disease is suspected (proteinuria, red cell casts, dysmorphic RBCs):

• Refer to pediatric nephrology urgently 1
• Consider ACE inhibitor or ARB therapy if proteinuria is confirmed on repeat testing 1
• May require renal biopsy for definitive diagnosis 1

Long-Term Monitoring

All children with sickle cell disease should have annual screening for proteinuria starting at age 10 years. 1

However, given this acute episode at age 8, establish baseline renal function now: 1, 6

• Repeat urinalysis in 1-2 weeks after hematuria resolves 1, 6
• If proteinuria persists (>1+ for at least 6 months), this indicates early sickle nephropathy 6
• Proteinuria in children with sickle cell disease is associated with disease severity and can progress to chronic kidney disease 6

Critical Pitfalls to Avoid

Do not assume all hematuria in sickle cell disease is benign: 5

• Renal papillary necrosis can present identically to benign sickling hematuria but requires different management 5
• Renal medullary carcinoma, though rare, is a highly aggressive tumor that occurs almost exclusively in patients with sickle cell trait or disease 2

Do not overlook infection: 1

• Children with sickle cell disease have functional asplenia and are at increased risk for serious bacterial infections 1
• Always obtain urine culture even if urinalysis is not clearly suggestive of infection 1

Do not delay nephrology referral if proteinuria is present: 1

• Early intervention with ACE inhibitors or ARBs may slow progression of sickle nephropathy 1
• Proteinuria beginning in childhood predicts worse long-term renal outcomes 6