You will be presented with 12 clinical scenarios of children with hematuria.
For each, interpret the lab data and provide the most likely diagnosis, mechanism, and next step.
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Glomerular: NonglomerularIgA: Alport:
Case 1
A 10-year-old boy presents with cola-colored urine, periorbital edema, and hypertension.
Urinalysis
Gross hematuria, RBC casts, protein 2+
Serum Creatinine
1.2 mg/dL
C3 Complement
Low (30 mg/dL)
C4 Complement
Normal
ASO Titer
Elevated
Blood Pressure
145/92 mm Hg
β Model Answer:
β’ Diagnosis: Acute poststreptococcal glomerulonephritis (APSGN).
β’ Evidence: Latency 1-2 weeks post-strep, gross hematuria with RBC casts, hypertension, edema, low C3 (classic pathway), normal C4, elevated ASO.
β’ Next step: Monitor BP, fluid/salt restriction, furosemide if edema, treat hypertension. C3 normalizes in 8-10 weeks.
Case 2
A 12-year-old girl with recurrent gross hematuria occurring 1-2 days after upper respiratory infections. She is otherwise well.
Urinalysis
Gross hematuria, no RBC casts, mild protein (trace)
Serum Creatinine
0.8 mg/dL
C3 Complement
Normal
C4 Complement
Normal
Serum IgA
Elevated
Blood Pressure
110/70 mm Hg
β Model Answer:
β’ Diagnosis: IgA nephropathy (Berger disease).
β’ Evidence: Synpharyngitic hematuria (1-2 days post-URI), normal C3, elevated serum IgA, normal BP, no renal dysfunction.
β’ Next step: Monitor BP, proteinuria, creatinine. Renal biopsy if persistent proteinuria (>0.5 g/day) or hypertension.
Case 3
A 6-year-old boy with palpable rash on lower extremities, abdominal pain, and hematuria.
Urinalysis
Microscopic hematuria, protein 1+
Serum Creatinine
0.7 mg/dL
C3 Complement
Normal
C4 Complement
Normal
Platelets
300,000/Β΅L
Blood Pressure
105/68 mm Hg
β Model Answer:
β’ Diagnosis: IgA vasculitis (Henoch-SchΓΆnlein purpura) with nephritis.
β’ Evidence: Palpable purpura, arthritis, abdominal pain, hematuria, normal platelets (excludes ITP), normal C3.
β’ Next step: Monitor BP, urinalysis weekly. If proteinuria >0.5 g/day, start ACE inhibitor. Renal biopsy if nephrotic-range proteinuria.
Case 4
A 14-year-old boy with persistent microscopic hematuria and sensorineural hearing loss.
Urinalysis
Microscopic hematuria, no protein
Serum Creatinine
0.9 mg/dL
Audiometry
High-frequency sensorineural hearing loss
Ophthalmology
Anterior lenticonus
Family History
Maternal uncle with ESKD
Blood Pressure
118/72 mm Hg
β Model Answer:
β’ Diagnosis: Alport syndrome (X-linked COL4A5 mutation).
β’ Evidence: Microscopic hematuria, sensorineural hearing loss, anterior lenticonus, X-linked family history (maternal uncle).
β’ Next step: Skin biopsy (absent Ξ±5 chain of type IV collagen) or genetic testing (COL4A5). ACE inhibitor if proteinuria develops.
Case 5
A 7-year-old girl with recurrent gross hematuria, dysuria, and abdominal pain. .
Urinalysis
Gross hematuria, no protein, no casts
Serum Creatinine
0.6 mg/dL
Serum Calcium
9.5 mg/dL
Spot Urine Ca:Cr
0.35
24-hour Urine Calcium
5.2 mg/kg/day
Blood Pressure
108/70 mm Hg
β Model Answer:
β’ Diagnosis: Idiopathic hypercalciuria.
β’ Evidence: Recurrent hematuria, Ca:Cr >0.2 (>2 years), 24-hour urine Ca >4 mg/kg/day, normal serum calcium, normal ultrasound.
β’ Treatment: High fluid intake (2-3 L/day), low sodium diet, normal calcium intake. Hydrochlorothiazide if recurrent symptoms.
Case 6
A neonate with gross hematuria, and left flank mass. Born after a difficult delivery with perinatal asphyxia.
Urinalysis
Gross hematuria
Platelet Count
40,000/Β΅L
Serum Creatinine
1.0 mg/dL
Blood Pressure
65/40 mm Hg
β Model Answer:
β’ Diagnosis: Renal vein thrombosis (neonatal).
β’ Evidence: Perinatal asphyxia, gross hematuria, flank mass, thrombocytopenia, absent venous flow on Doppler.
β’ Treatment: Supportive care. Anticoagulation (heparin) if bilateral or IVC extension. Monitor renal function.
Case 7
A 6-year-old boy with sickle cell disease presents with gross hematuria, left flank pain, and fever.
Urinalysis
Gross hematuria, no casts
Hemoglobin
8.0 g/dL
Serum Creatinine
0.8 mg/dL
CT Urography
Medullary clefts, sloughed papillae
Blood Pressure
110/70 mm Hg
β Model Answer:
β’ Diagnosis: Papillary necrosis (sickle cell nephropathy).
β’ Evidence: Sickle cell disease, gross hematuria, flank pain, CT showing medullary clefts and sloughed papillae.
β’ Treatment: Hydration, pain control, avoid NSAIDs. If severe hematuria, consider Ξ΅-aminocaproic acid or desmopressin.
Case 8
A thin 14-year-old girl with intermittent gross hematuria, and left flank pain . Symptoms worse after exercise.
Urinalysis
Gross hematuria, no casts, trace protein
Serum Creatinine
0.7 mg/dL
Doppler Ultrasound
Left renal vein compression between aorta and SMA
Blood Pressure
108/68 mm Hg
Body Mass Index
17 (underweight)
β Model Answer:
β’ Diagnosis: Nutcracker syndrome (left renal vein compression).
β’ Evidence: Thin adolescent, left flank pain, hematuria, orthostatic proteinuria, Doppler showing LRV compression.
β’ Treatment: Conservative (weight gain, hydration). If severe hematuria/pain, consider surgical intervention (LRV transposition).
Case 9
A 4-year-old girl with gross hematuria, dysuria, and frequency. She has a palpable mass at the urethral meatus.
Urinalysis
Gross hematuria, WBCs +
Serum Creatinine
0.5 mg/dL
Blood Pressure
100/65 mm Hg
Urine Culture
Negative
β Model Answer:
β’ Diagnosis: Urethral prolapse.
β’ Evidence: Prepubertal girl, donut-shaped friable mass at urethral meatus, hematuria, dysuria.
β’ Treatment: Topical estrogen cream, sitz baths. Surgical reduction if severe or urinary retention.
Case 10
A 7-year-old boy with microscopic hematuria found on routine urinalysis. He has normal hearing and vision.
Urinalysis
Microscopic hematuria, no protein, no casts
Serum Creatinine
0.6 mg/dL
Audiometry
Normal
Ophthalmology
Normal
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Blood Pressure
105/68 mm Hg
β Model Answer:
β’ Diagnosis: Thin basement membrane disease (benign familial hematuria).
β’ Evidence: Isolated microscopic hematuria, normal hearing/vision, autosomal dominant family history, normal renal function.
β’ Next step: Reassurance. Electron microscopy shows diffuse GBM thinning. No treatment needed. Monitor annually.
Case 11
A 5-year-old boy with gross hematuria, left flank pain, and a palpable abdominal mass. He has hypertension.
Urinalysis
Gross hematuria, no casts
Hemoglobin
10.5 g/dL
Serum Creatinine
0.8 mg/dL
Abdominal Ultrasound
Large left renal mass
Blood Pressure
135/85 mm Hg
β Model Answer:
β’ Diagnosis: Wilms tumor (nephroblastoma).
β’ Evidence: Abdominal mass, hematuria, hypertension (renin secretion), palpable mass.
β’ Next step: CT chest/abdomen, surgical nephrectomy, chemotherapy based on staging (NWTS/COG protocol).
Case 12
A 6-year-old boy known case of leukemia , now on treatment, presents with gross hematuria, dysuria, and suprapubic pain.
Urinalysis
Gross hematuria, WBCs +, no bacteria
Serum Creatinine
0.6 mg/dL
Urine Culture
Negative
Blood Pressure
110/70 mm Hg
β Model Answer:
β’ Diagnosis: Hemorrhagic cystitis (cyclophosphamide-induced - acrolein metabolite).
β’ Evidence: Cyclophosphamide use, gross hematuria, dysuria, sterile urine.
β’ Treatment: Hydration, bladder irrigation. Prevention: mesna + hydration before and after cyclophosphamide. Long-term risk: bladder cancer.
β‘ Quick FCPSβstyle MCQ
A child with recurrent gross hematuria 1-2 days after URI, normal C3, and mesangial IgA deposits on biopsy. The most likely diagnosis is:
A. IgA nephropathyB. Poststreptococcal glomerulonephritisC. Alport syndromeD. Thin basement membrane disease
β οΈ Key concept:Hematuria β Nelson Ch 558
β’ Glomerular: Tea/cola urine, RBC casts, dysmorphic RBCs (acanthocytes), proteinuria, hypertension, edema.
β’ Nonglomerular: Bright red/pink urine, clots, normal RBC morphology, minimal proteinuria.
β’ IgA nephropathy: Synpharyngitic hematuria (1-2 days post-URI), normal C3, mesangial IgA deposits.
β’ Alport syndrome: Hematuria + sensorineural hearing loss + anterior lenticonus. X-linked COL4A5.
β’ Hypercalciuria: Spot urine Ca:Cr >0.2. Treat with hydration, low sodium, thiazide.
β’ APSGN: Latency 1-2 weeks post-strep, low C3 (returns 8-10 weeks).
β’ Thin basement membrane: Benign familial hematuria, normal prognosis.
π― Examiner Scoring Checklist
β’ Correctly identifies glomerular vs nonglomerular hematuria
β’ Recognizes IgA nephropathy (synpharyngitic, normal C3)