Red Urine and Hematuria in children

2. Basic Science
• RBCs may be excreted in the urine by normal
persons.
• It is not known precisely how these cells reach
the urinary tract.
• However, the normal excretion rate is :
• 0.5 to 2 million RBCs/24 hr, or <5 RBCs/hpf
on microscopic examination of a urine
specimen.

3. Hematuria
• Red or brown urine may indicate hematuria and
possible renaldisease.
• Hematuria is defined as the presence of at least 5 red
blood cells (RBCs) per microliter of urine.
• Occurs with a prevalence of 0.5-2.0% among school-
aged children.
• Microscopic hematuria is defined as more than 5 RBCs
per high-power field in the sediment of freshly voided
urine.
• Gross hematuria is visible to the naked eye.
• There are many conditions other than hematuria
causing red or brown discoloration of the urine.

4. Clinical evaluation
• After taking good history and clinical examination you have to
think about important investigations:
• 1- Biochemical test:
• urinary dipstick
• Microscopic analysis of urine
• RFT : Serum Creatinine
• Urine Protein & Protein to Creatinine Ratio
• Antinuclear Antibody, ASO Titer,Serum complement (C3, C4, C50)
• Coagulation Factors: INR , prothrombin time (PT) & Partial
Thromboplastin Time (PTT)
• Collect 24 hour Urine Calcium, Urine Uric Acid
• 2- Radiology: Renal US, Helical CT Urogram, Intravenous Pyelogram,
Cystoscopy.

5. Clinical evaluation
• 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 3-5 RBCs/ML of urine;
• Microscopic analysis of 10-15 mL of freshly centrifuged urine is
essential in confirming the presence of RBCs suggested by a positive
dipstick.

6. Urine Discoloration from Sources Other Than Hematuria
Pink, Red, Cola-Colored, Burgundy
Disease Associated
Hemoglobinuria*
Myoglobinuria*
Porphyrinuria
Associated with Drug or Food Ingestion
Aminopyrine
Anthocyanin
Azo dyes
Beets
Blackberries
Chloroquine
Deferoxamine mesylate
Iron sorbitol
Methyldopa
Nitrofurantoin
Phenazopyridine
Phenolphthalein
Pyridium
Red food coloring
Rifampin
Rhodamine B
Rhubarb
Sulfasalazine
Urates
Dark Brown, Black
Disease Associated
Alkaptonuria
Methemoglobinemia
Homogentisic aciduria
Tyrosinosis
Melanin
Bile pigments
Associated with Food or Drug Ingestion:
Alanine
Resorcinol
Cascara
Thymol

7. • A positive reagent strip (dipstick) in the absence of RBCs
indicates the presence of Hgb or myoglobin.
 Hemoglobinuria occurs with hemolysis. It may occur in hemolytic
anemias, hemolytic-uremic syndrome, mismatched transfusions,
freshwater drowning, septicemia, and paroxysmal nocturnal
hemoglobinuria. It is also associated with carbon monoxide, fava
beans, venoms, mushrooms, naphthalene, quinine, and many other
substances.
 A CBC with smear will often show fragmented cells, and the
reticulocyte count may be elevated.
 Myoglobinuria occurs with rhabdomyolysis after viral myositis and
in children with inborn errors of energy metabolism, often after
exercise.
 The clinical picture as well as elevated muscle enzyme levels may
aid in distinguishing myoglobinuria from hematuria.
 If needed, Hgb and myoglobin may be measured in the urine.

8. Causes of Hematuria in Children
Glomerular
Primary
Acute postinfectious glomerulonephritis
IgA nephropathy*
Mesangial proliferative glomerulonephritis
Membranoproliferative glomerulonephritis
Familial nephritis (Alport syndrome)
Benign familial hematuria - thin basement
membrane disease
Rapidly progressive glomerulonephritis
Vascular
Trauma
Sickle cell disease and trait
Renal artery/vein thrombosis
Arteriovenous malformation
Nutcracker syndrome
Sports- and exercise-induced
hematuria
Hemangioma/hamartoma
Interstitial Disease
Pyelonephritis
Acute interstitial nephritis
Polycystic kidney disease
(autosomal dominant)
Systemic
Henoch-Schönlein purpura
Systemic lupus erythematosus
Hemolytic uremic syndrome
ANCA-associated vasculitis
Goodpasture disease (rare in childhood)
Bacterial endocarditis
Neoplastic
Wilms tumor
Renal cell carcinoma
Uroepithelial tumors
Rhabdoid tumors
Congenital mesoblastic nephroma
Angiomyolipoma
Bleeding Disorders
Hemophilia A or B
Platelet disorder
Thrombocytopenia
Coagulopathy, congenital
or acquired
Urinary Tract
Cystitis .Bacterial .Viral (adenovirus) .Parasitic (schistosomiasis)
Tuberculosis .Cyclophosphamide .Urethritis
Urolithiasis .Idiopathic hypercalciuria without urolithiasis .Trauma
Hydronephrosis, severe .Foreign body

9. Microscopic hematuria
• Microscopic hematuria is often found on routine
screening.
• If the child is asymptomatic, with normal BP and no
proteinuria, the UA should be repeated at least 2 to 3
times over 2 to 3 months. If followup UA is normal, a
diagnosis of isolated asymptomatic hematuria is
made.
• If proteinuria is present, the evaluation is the same as
for gross hematuria.
• If the child is symptomatic (hypertension, edema) and
has proteinuria and a family history of deafness or
kidney disease, a nephrologist should be consulted.

10. Site of bleeding
• It is difficult to localize the site of bleeding by
routine examination of the patient with
hematuria.
• However, certain findings may be very helpful
depend on size & shape of RBCs.

11. Gross hematuria
• Gross hematuria can be localized to the upper or
lower urinary tract.
• Upper tract bleeding causes brown, smoky, or
tea-colored urine.
• Proteinuria suggests glomerular involvement.
• Dysmorphic RBCs due to passage through the
glomerular basement membrane also indicate
upper tract involvement.
• Lower tract bleeding is bright red, may have
clots, rarely contains significant amounts of
protein, and shows isomorphic RBC morphology.

12. • Symptomatic gross hematuria may be due to renal disease.
• UTIs commonly cause gross hematuria and can be
diagnosed by a positive urine culture when bacterial in
origin.
• Hemorrhagic cystitis is often caused by adenovirus.
• Nephrolithiasis is associated with renal colic, positive
family history, and UA that may show crystals as well as
hematuria.
• Stones can be diagnosed by using IV pyelography, spiral CT,
or US. X-rays may not detect radiolucent stones.
• Hypercalciuria, even without the presence of a stone, may
cause abdominal or flank pain, dysuria, and hematuria.
Gross hematuria

13. • Meatal stenosis with ulceration, trauma due to
catheterization, and sexual abuse may cause
hematuria.
• Abdominal and renal traumas are also causes
and require abdominal CT scan with IV contrast
medium enhancement.
• Injury to the bladder and posterior urethra may
be associated with pelvic fractures and may be
diagnosed by retrograde urethrography.
Gross hematuria

14. • Idiopathic hypercalciuria most often occurs as
persistent microscopic hematuria or as recurrent gross
hematuria or dysuria. A calcium-to-Cr ratio above 0.2
is suggestive. If this is present, a 24-hour urine
collection for calcium should be obtained.
• Autosomal dominant polycystic kidney disease often
appears as gross hematuria. Symptoms may begin in
childhood but more often occur in adulthood.
• Renal and bladder tumors may rarely occur as
hematuria.
• Arteriovenous malformations of the kidney may
present as gross hematuria because of rapid transit of
blood down the ureter; localization of bleeding may
require cystoscopy or angiography.

15. • Henoch-Schönlein purpura (HSP) may appear as abdominal pain,
joint pain, and lower extremity rash (palpable purpura). In addition
to hematuria, proteinuria may also be present. There is no specific
laboratory test for HSP; however, serum IgA levels may be elevated.
• Systemic infections such as bacterial endocarditis and shunt
infections may be associated with hematuria and proteinuria.
• Nephrotic syndrome may be associated with hematuria,
particularly in focal segmental sclerosis and membranoproliferative
glomerulonephritis.
• Tubulointerstitial nephritis is often associated with penicillins,
cephalosporins, sulfonamides, rifampin, tetracyclines, nonsteroidal
antiinflammatory drugs, furosemide, thiazides, heavy metals, and
others. It may also be associated with other diseases, such as
systemic lupus erythematosus.

16. Acute Poststreptococcal
Glomerulonephritis
• A 10-year-old boy presents with Coca-Cola–
colored urine and edema of his lower extremities.
• On physical examination, the patient has a blood
pressure of 185/100 mm Hg. He does not appear
to be in any distress. His lungs are clear to
auscultation, and his heart has a regular rate and
rhythm without any murmurs, gallops, or rubs.
• His past medical history is remarkable for a sore
throat that was presumed viral by his physician 2
weeks before.

17. • Etiology
• − Follows infection with nephrogenic strains of group A
beta-hemolytic streptococci of the throat (mostly in
cold weather) or skin (in warm weather)
• − Diffuse mesangial cell proliferation with an increase
in mesangial matrix; lumpybumpy deposits of
immunoglobulin (Ig) and complement on glomerular
basement membrane and in mesangium
• − Mediated by immune mechanisms but complement
activation is mostly through the alternate pathway
Acute Poststreptococcal Glomerulonephritis

18. • Clinical presentation
• Most 5–12 years old (corresponds with typical
age for strep throat)
• 1–2 weeks after strep pharyngitis or 3–6 weeks
after skin infection (impetigo)
• Ranges from asymptomatic microscopic
hematuria to acute renal failure
• Edema, hypertension, hematuria (classic triad)
• Constitutional symptoms—malaise, lethargy,
fever, abdominal or flank pain
Acute Poststreptococcal Glomerulonephritis

19. • Diagnosis
• Urinalysis—RBCs, RBC casts, protein 1–2 +, polymorphonuclear
cells
• Mild normochromic anemia (hemodilution and low-grade
hemolysis)
• Low C3 (returns to normal in 6–8 weeks)
• Need positive throat culture or increasing antibody titer to
streptococcal antigens;best single test is the anti-DNase antigen
• For diagnosis of prior Strep infection, use streptozyme (slide
agglutination), which detects antibodies to streptolysin O, DNase
B, hyaluronidase, streptokinase, and nicotinamide-adenine
dinucleotidase.
• Consider biopsy only in presence of acute renal failure, nephrotic
syndrome, absence of streptococcal or normal complement; or if
present >2 months after onset
Acute Poststreptococcal Glomerulonephritis

20. • Complications
• Hypertension
• Acute renal failure
• Congestive heart failure
• Electrolyte abnormalities
• Acidosis
• Seizures
• Uremia
Acute Poststreptococcal Glomerulonephritis

21. o Treatment (in-patient, if severe)
o Antibiotics for 10 days (penicillin)
o Sodium restriction, diuresis
o Fluid and electrolyte management
o Control hypertension (calcium channel
blocker, vasodilator, or angiotensinconverting
enzyme inhibitor)
o Complete recovery in >95%
Acute Poststreptococcal Glomerulonephritis

22. Hemolytic Uremic Syndrome (HUS)
• A 3-year-old child presents to the emergency
center with history of bloody diarrhea and
decreased urination.
• The mother states that the child’s symptoms
began 5 days ago after the family ate at a fast-
food restaurant.
• At that time the patient developed fever,
vomiting, abdominal pain, and diarrhea.
• On physical examination, the patient appears ill.
He is pale and lethargic.

23. • Most common cause of acute renal failure in
young children
• Microangiopathic hemolytic anemia,
thrombocytopenia, and uremia
• Most from E. coli O157:H7 (shiga toxin–
producing)
• Most from undercooked meat or unpasteurized
milk; spinach
• Also from Shigella, Salmonella, Campylobacter,
viruses, drugs, idiopathic
Hemolytic Uremic Syndrome (HUS)

24.  Pathophysiology
 Subendothelial and mesangial deposits of granular,
amorphous material—vascular occlusion, glomerular
sclerosis, cortical necrosis
 Capillary and arteriolar endothelial injury → localized
clotting
 Mechanical damage to RBCs as they pass through
vessels
 Intrarenal platelet adhesion and damage (abnormal
RBCs and platelets then removed by liver and spleen)
 Prothrombotic state
Hemolytic Uremic Syndrome (HUS)

25. o Clinical presentation
o Most common <4 years old
o Bloody diarrhea
o 5–10 days after infection, sudden pallor, irritability,
weakness, oliguria occur;mild renal insufficiency to acute
renal failure (ARF)
 Laboratory finding:
 hemoglobin 5–9 mg/dL, helmet cells, burr cells,
fragmented cells, moderate reticulocytosis, white blood
cells up to 30,000/mm3, Coombs negative, platelets
usually 20,000–100,000/mm3,
 low-grade microscopic hematuria and proteinuria
Hemolytic Uremic Syndrome (HUS)

26. Complications ; including seizures, infarcts, colitis,
intussusception, perforation,heart disease, death
 Treatment
 Meticulous attention to fluids and electrolytes
 Treat hypertension
 Aggressive nutrition (total parenteral nutrition [TPN])
 Early peritoneal dialysis
 No antibiotics if E. coli O157:H7 is suspected—treatment
increases risk of developing HUS
 Plasmapheresis or fresh frozen plasma—may be beneficial
in HUS not associated with diarrhea or with severe central
nervous system involvement
Prognosis—more than 90% survive acute stage; small
number develop ESRD (end-stage renal disease)
Hemolytic Uremic Syndrome (HUS)

27. THANKS FOR YOUR
ATTENTION