Hematuria is defined as the presence of at least 5 red
blood cells (RBCs) per microliter. In the clinical setting, qualitative estimates are provided by a urinary dipstick that uses a very sensitive peroxidase chemical reaction between hemoglobin (or myoglobin) and a colorimetric chemical indicator impregnated on the dipstick. Chemstrip (Boehringer Mannheim), a common commercially available dipstick, is capable of detecting 3-5RBCs/?L of unspun urine; significant hematuria is suggested by >50RBCs/?L.
False-negative results can occur in the presence of formalin
(used as a urine preservative) or high urinary concentrations of ascorbic acid (i.e., in patients with vitamin C intake >2000mg/ day). False-positive results may be seen in a child with an alkaline urine (pH > 9), or more commonly following contamination with oxidizing agents such as hydrogen peroxide used to clean the perineum before obtaining a specimen. Microscopic analysis of 10-15mL of freshly centrifuged urine is essential in confirming the presence of RBCs suggested by a positive dipstick.
Heme-positive urine without RBCs is caused by
the presence of either hemoglobin or myoglobin. Hemoglobinuria without hematuria can occur in the presence of hemolysis. Myoglobinuria without hematuria occurs in the presence of rhabdomyolysis resulting from skeletal muscle injury and is generally associated with a 5-fold increase in the plasma concentration of creatine kinase.
Rhabdomyolysis can occur secondary to viral
myositis, crush injury, severe electrolyte abnormalities (hypernatremia, hypophosphatemia), hypotension, disseminated intravascular coagulation, toxins (drugs, venom), metabolic disorders of muscles, and prolonged seizures. Heme-negative urine can appear red, cola colored, or burgundy, owing to ingestion of various drugs, foods (blackberries, beets), or food dyes, whereas dark brown (or black) urine can result from various urinary metabolites.
Upper urinary tract sources of hematuria originate
within the nephron (glomerulus, convoluted or collecting tubules, and interstitium). Lower urinary tract sources of hematuria originate from the pelvocalyceal system, ureter, bladder, or urethra. Hematuria from within the glomerulus is often associated with brown, cola or tea-colored, or burgundy urine, proteinuria >100mg/dL via dipstick, urinary microscopic findings of RBC casts, and deformed urinary RBCs (particularly acanthocytes).
Hematuria originating within the convoluted or
collecting tubules may be associated with the presence of leukocytes or renal tubular epithelial cell casts. Lower urinary tract sources of hematuria may be associated with gross hematuria that is bright red or pink, terminal hematuria (gross hematuria occurring at the end of the urine stream), blood clots, normal urinary RBC morphology, and minimal proteinuria on dipstick (<100mg/dL).
Tea- or cola-colored urine, facial or body edema,
hypertension, and oliguria are classic symptoms of acute nephritic syndrome. Diseases commonly manifesting as acute nephritic syndrome include postinfectious glomerulonephritis, immunoglobulin A (IgA) nephropathy, membranoproliferative glomerulonephritis, HenochSchonlein purpura (HSP) nephritis, systemic lupus erythematosus (SLE) nephritis, Wegener granulomatosis, microscopic polyarteritis nodosa, Goodpasture syndrome, and hemolytic-uremic syndrome.
Hereditary glomerular diseases include
hereditary nephritis (Alport syndrome), thin glomerular basement membrane disease, SLE nephritis, and IgA nephropathy (Berger disease). Other hematuric renal disorders with a hereditary component include polycystic kidney disease (PKD) (both autosomal recessive [ARPKD] and autosomal dominant [ADPKD]), urolithiasis, and sickle cell disease/trait.
Abdominal masses may be caused by bladder
distention in posterior urethral valves, hydronephrosis in ureteropelvic junction obstruction, PKD, or Wilms tumor. Hematuria seen in patients with neurologic or cutaneous abnormalities may be the result of renal cystic disease or tumors associated with several syndromes, including tuberous sclerosis, von Hippel-Lindau syndrome, and Zellweger (cerebrohepatorenal) syndrome.
The most common cause of gross hematuria is bacterial
urinary tract infection. Urethrorrhagia, which is urethral bleeding in the absence of urine, is associated with dysuria and blood spots on underwear after voiding. Recurrent episodes of gross hematuria suggest IgA nephropathy, Alport syndrome, or thin glomerular basement membrane disease. Dysuria and abdominal or flank pain are symptoms of idiopathic hypercalciuria, or urolithiasis.
Anemia in this setting may be caused by intravascular dilution
secondary to hypervolemia associated with acute renal failure; decreased RBC production in chronic renal failure; hemolysis from hemolytic-uremic syndrome or SLE; or blood loss from pulmonary hemorrhage, as seen in Goodpasture syndrome, or melena in patients with Henoch-Schonlein purpura or hemolytic-uremic syndrome. Inspection of the peripheral blood smear might reveal a microangiopathic process consistent with the hemolytic-uremic syndrome. The presence of autoantibodies in SLE can result in a positive Coombs test, the presence of antinuclear antibody, leukopenia, and multisystem disease.
Thrombocytopenia can result from decreased
platelet production (malignancies) or increased platelet consumption (SLE, idiopathic thrombocytopenic purpura, hemolytic-uremic syndrome, renal vein thrombosis). Although urinary RBC morphology may be normal with lower tract bleeding and dysmorphic from glomerular bleeding, cell morphology is not reliable to unequivocally delineate the site of hematuria.
A voiding cystourethrogram is only required
in patients with a urinary tract infection, renal scarring, hydroureter, or pyelocaliectasis. Renal biopsy is indicated for some children with persistent microscopic hematuria and most children with recurrent gross hematuria associated with decreased renal function, proteinuria, or hypertension.