HematuriaKatie Townes MD, PGY2

Case

8 year old male with episodic hematuria, initially thought to be due to trauma, but persisted for weeks and recurred with fevers. Also with severe anemia requiring multiple blood transfusions. Hematuria seemed to occur when he was febrile……

What is hematuria?

>5 RBCs per high power fieldMicroscopic vs. gross hematuriaMultiple differentials of hematuria

RBCs in urine microscopy

Library.med.utah.edu

Foods or medicines that turn the urine red?Urate crystals in diapersMyoglobinuria?

-positive heme on dipstick -no RBCs seen on microscopy-cause? -muscle breakdown from rhabdomyolysis (crush injury, burns, asphyxia)

Hemoglobinuria can be caused by hemolysis, but again, no RBCs seen on microscopy

Things that are NOT Hematuria

Urate Crystals in Diaper

From newborns.stanford.edu

DifferentialsGlomerular diseaseTubular diseaseUTISchistosomiasisTraumaTumorsStonesPolycystic kidney disease

GlomerulonephritisHistory

-coca-cola colored or smoky urine-edema-malaise

Physical -hypertension -edema

Labs -renal failure-microscopy RBC casts and dysmorphic RBCs

Cyberrounds.com

Formation of RBC casts

-GBM is damaged-RBCs can get through GBM-Going through the tubule, the stick together, forming casts that appear in the urine

Library.med.utah.edu

Casts and dysmorphic RBCs

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Glomerulonephritis Causes

post-strep GNmembranoproliferative GNRPGNIgA nephropathySLEAlport’s disease (not really GN…)

Glomerular Disease- TreatmentPost-Strep GN usually self-resolves in kidsKidney biopsyRPGN, Membranoproliferative GN, IgA

nephropathy, SLE- immunosuppressives (steroids, cyclophosphamide)

Supportive treatment: anti-HTN, fluid restriction, Lasix

Many will develop ESRD and need HD or transplant

Tubular disease ATN

-anoxic injury-rhabdomyolysis-certain medications/toxins?-sometimes reversible with supportive care

Cortical necrosis -usually due to severe anoxia-often irreversible

AIN

-urine eosinophils elevated-usually related to medications- sulfa, ibuprofen -reversible when stopped

UTI Causative organisms?

History-dysuria, frequency, urgency- lower UTI -Fever, back pain, vomiting- upper UTI

Physical-fever- upper-suprapubic pain-CVA tenderness- upper

Labs-CBC-U/A?-urine culture

Catsmeowvets.com

UTI

WBC- see nuclei

WBC cast in pyelonephritis

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UTI Treatment (WHO book)Cotrimoxazole 4mg TMP/20mg sulfa per kg

PO BID x 5d if no signs of upper tract infection

Alternatives: Ampicillin, Amoxicillin, Cephalexin, Ciprofloxacin

If signs of pyelo or in young infants (<2 months), give parenteral Ampicillin and Gentamicin or cephalosporin x10-14 days

Consider sepsis or perinephric abscess if no resolution with antibiotics

Schistosomiasis-PathophysLarval parasites penetrate cutaneouslyMigrate hematogenously to portal system,

where mating occurs over 1-3 monthsS. hematobium organisms migrate to

venous channels in bladder and lay eggsEggs breach vessel walls and enter the

bladder, causing inflammatory response and urinary symptoms

Eggs are then passed back into the water by urination, completing the cycle

Schisto- Clinical Acute sx

-fevers-pruritic rash (swimmer’s itch) -LAN-abdominal pain

Chronic sx -terminal hematuria-dysuria-obstructive uropathy-Chronic inflammation can cause malnutrition, poor growth

Diagnosis: urine microscopy to look for eggs (only in chronic stage)

Treatment- Praziquantel, education (avoid fresh water!)

Schistosomiasis

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Trauma

History -trauma to the back -flank pain

Physical-evidence of trauma-flank bruising

Labs: hematuria without casts

Tumors?Wilms tumor (nephroblastoma)

◦Most common pediatric renal malignancy◦Median age 3-3.5 years, M=F ◦History: hematuria, abdominal mass, fever, abd

pain◦Physical: palpable abdominal mass

Treatment: surgery +/- chemoradiationRhabdomyosarcoma

Wilms Tumor

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Stones

Uncommon in childrenHistory: flank pain, gross hematuriaPhysical: writhing in pain, flank pain,

abdominal pain, can’t get comfortableLabs: Urinalysis with positive heme and

positive RBCs (no casts)Treatment: pain control, fluids. Stones can

usually pass on their own if not too bigComplications: obstruction causing UTI,

abscess, sepsis if stone doesn’t pass

StonesMost common places to obstruct:1) Renal pelvis2) Crossing the pelvic brim3) Entering the bladder

Staghorn calculusCalcium oxalate

All images from google images

Polycystic kidney disease

Autosomal dominant PKD doesn’t affect children (renal failure usually 4th-5th decade)

Autosomal recessive PKD affects children, can be variable (infants-adolescent presentation).

Depending on category, liver disease also part of the syndrome

Clinical- renal failure, palpable flank masses, cystic kidneys on ultrasound

Treatment- ARPKD eventually progresses to ESRD, needing HD or transplant

PKD-Images

Healthjockey.comnature.com

Hemoglobinuria

Due to hemolysis (sickle cell, G6PD def, malaria, TTP, DIC, HUS)

U/A shows positive heme, but no RBCsHemoglobin is toxic to kidneys, causes

ATN

Paroxysmal Nocturnal Hematuria

Rare, autosomal dominant mutation of RBC membrane protein (GPI-anchor), leads to complement deposition and intravascular hemolysis

Clinical triad of intravascular hemolytic anemia, venous thrombosis, and pancytopenia

Median age 46, but children can be affectedChildren tend to have aplastic anemiaHematuria most noticeable in AM when urine

is concentrated, but can occur anytime

Paroxysmal Nocturnal Hematuria History

-Fatigue and jaundice (hemolytic anemia) -coca cola colored urine-abdominal pain, headache, SOB (clots)-bleeding, infections, fatigue (BM failure)

Physical-signs of hemolytic anemia (pallor, tachy, jaundice)-usually no spleen, because intravascular hemolysis-abdominal pain may suggest hepatic or portal vein thrombosis-petechiae or fevers to suggest pancytopenia

Paroxysmal Nocturnal Hematuria

Treatment -medial survival is 10 years, death usually due to thrombosis-immunosuppression with steroids can help with hemolytic anemia-Folic Acid for all children with hemolytic anemia. Why?-beware infection risk….

Back to the case:

So what diagnosis makes the most sense?-UTI?-Trauma?-Glomerulonephritis?-Myoglobinuria?-Paroxysmal nocturnal hemoglobinuria?-Sickle cell anemia?

References

• Besa, Emmanuel. “Paroxysmal Nocturnal Hematuria.” emedicine.com. March 2009

• library.med.utah.edu/WebPath/TUTORIAL/URINE/URINE.html#2

Nelson’s Essentials of Pediatrics. 5th Edition. Chapter 163, page 757-759.

Young, B. Y. et al. Autosomal Recessive Polycystic Kidney Disease. Emedicine.com. April 28, 2010.

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