HEMATURIA

Definition
1. It is the presence of more than 5 red blood cells/ high power field in sediment of 10ml centrifuged freshly voided urine. 2. Hematuria may be gross seen with naked eye (Figure 6.4) or microscopic.

Causes of hematuria in Children 1. Glomerular diseases

a. Acute post-streptococcal glomerulonephritis b. Membranous glomerulonephritis

c. Membranous proliferative glomerulonephritis

d. Systemic lupus erytheromatosus e. Nephritis of chronic infection

f. Rapidly progressive glomerulonephritis g. Henoch Schonlein purpura h. Hemolytic Uraemic syndrome

i. Interstitial nephritis

j. Post infections glomerulonephritis 2. Urinary tract infection

a. E. coli b. Klebsiella c. T.B d. Bilharsiasis

3. Hematologic causes a. Coagulopathies

b. Thrombocytopenia c. Renal Vein thrombosis d. Sickle cell disease

4. Traumatic
a. Blunt trauma

b. Stones

c. Hypercalciuria 5. Anatomic abnormalities a. Congenital anomalies

b. Vascular abnormalities

c. Polycystic kidney 6. Miscellaneous

a. Tumors b. Violent exercise c. Drugs i. Penicillin ii. Aspirin iii. Heparin

Differential diagnosis of hematuria 1. Hematuria must be differentiated from other causes of dark colored urine. 2. Causes of dark colored urine
a. Dark yellow i. Concentrated urine ii. Bilirubinuria b.Red urine i. Hemoglobinuria; As in acute hemolytic anemia. ii. Myoglobinuria; As in muscular necrosis after severe trauma. iii. Dyes; As colored candies, beet roots and black berries. iv. Drugs Rifampicin Vitamin B complex Desferoxamine Phenolphthalein v. Urates; Pink staining of the diaper, specially in neonates with decreased fluid intake. vi. Porphyria

c.Dark brown or black urine i. Alcaptonuria ii. Melanoma iii. Methemoglobinuria.

Difference between renal & extra renal causes of hematuria Extra renal causes
Color Three tube test RBCS Casts RBCS Clots Blood clots Edema Hyper tension Pink or red RBCS in tube 1 more 3 Absent Normal May be present Absent absent

Renal causes
Brown or smoky Similar in each tube Present Deformed Absent May be present May be present

ACUTE POST STREPTOCOCCAL GLOMERULONEPHRITIS

Definition
1. It is an acute nephritic syndrome characterized by the sudden onset of hematuria, edema, hypertension and acute renal insufficiency. 2. It is the most common glomerular cause of gross hematuria in children.

Etiology
1. It is an auto immune disease following group A beta hemolytic streptococcal infection of the throat or the skin (nephrogenic strains).

Pathology
1. Gross picture. The kidneys appear symmetrically enlarged. 2. Light microscope examination (Figure 6.5). The glomeruli appear enlarged and show diffuse proliferation of mesangial and endothelial cells with an increase in mesangial matrix. 3. Electron microscope examination (Figure 6.6). Electron-dense deposits are observed on the epithelial side of the glomerular basement membrane. 4. Immunofluorecence microscope examination (Figure 6.7). It shows a lumpy-pumpy deposits of immunoglobulin and complement 3 (C3) on the glomerular basement membrane.

Pathogenesis
1. Antibodies formed in response to streptococcal infection combine with their antigens to from immune complexes that are trapped in the glomeruli. 2. These result in complement activation, release of mediators of inflammation and glomerular injury and decreased the glomerular filtration rate.

Clinical manifestations
1. Age. It is most common in children 5-12 years and rare below 3 years. 2. Typical presentation include: a. Hematuria

b. Oliguria c.Edema d.Hypertension 3. However, there is a wide range of presentations from just a symptomatic microscopic hematuria discovered during routine urine examination to acute renal failure. In some cases, the patient presents mainly with one or more of the complications. 4. Hematuria is usually gross with smoky, brown or cola-colored urine but urine usually becomes clear by the end of the first week. 5. Oliguria is present in the majority of cases, very few cases may have anuria and in some cases the urine volume is normal. 6. Edema is usually mild (puffy eyes and slight edema of the extremities) (Figure 6.8, Figure 6.9). Severe edema is seen in cases with severe oliguria, heart failure or renal failure. 7. Hypertension is present in 70% of patients. It may appear at any time during the acute phase and may be as high as 200/120 mm/Hg. It may progress rapidly within few hours, so blood pressure must be measured every 4 hours. Usually blood pressure returns to normal slowly by the end of the first week. 8. Renal insufficiency is usually mild and transient in most cases, but severe renal failure may occur. 9. Non-specific symptoms such as malaise, lethargy, abdominal pain and fever are common. 10. The acute phase generally resolves within 2 months after onset, but urinary abnormalities may persist for more than 1 year.

Laboratory findings
1. Urinanalysis a. Hematuria b. RBCs casts c.Few Leucocytes d. Moderate proteinuria (500-2000 mg/24 hr urine) 2. Blood picture. Mild normochromic anemia due to hemodilution and low grade hemolysis.

3. Serum C3 level is reduced in the acute phase and return to normal in 6-8 weeks. 4. Kidney function. Increased urea and creatinine.

Diagnosis
1. Diagnosis of acute post streptococcal glomerulonephritis is mot likely in a child aged between 5-12 years presenting with acute nephritic syndrome with history of recent streptococcal infection and a low C3 level. 2. Confirmation of the diagnosis requires clear evidence of invasive streptococcal infection. a.Positive throat swab b. Increased Anti-streptolysin O (ASO) titer using the streptozyme test document pharyngeal streptococcal infection. c.Increased deoxyribonuclease B antigen document cutaneous streptococcal infection. 3. Renal biopsy should be considered in the presence of he following: a. Acute renal failure b. Associated nephrotic syndrome (heavy proteinuria) c. Absence of evidence of streptococcal infection. d. Normal C3 level. e. Persistence of condition more than 2 months. 4. Differential diagnosis. See other causes of hematuria.

Complications
1. Acute Heart Failure a. Decreased glomerular filtration rate results in salt and water retention and secondary circulatory overload. b.When severe and combined with hypertension, it may lead to heart failure and pulmonary edema manifested by dyspnea, orthopnea, tachycardia, pulmonary crepitations, and enlarged tender liver. c. Chest x-ray will show enlarged heart and pulmonary congestion (Figure 6.10) 2. Acute renal failure

a. Severe reduction of renal filtration rate results in: i. Reduced urine production ii. Electrolyte hypocalcemia) iii. Acid-base disturbances (acidosis) iv. Impaired excretion of waste products b.Clinically it is manifested by marked oliguria or anuria, acidotic breathing, vomiting, anemia, convulsions and disturbed consciousness (drowsiness, stupor or coma). 3. Hypertensive encephalopathy a. Hypertensive encephalopathy is an emergency. It occurs with rapidly rising blood pressure and consequent loss of auto-regulation of cerebral circulation resulting in dilation of vessels and brain edema. b.Clinically it is manifested by irritability, headache, vomiting, blurring of vision, drowsiness, coma and convulsions. c.It may be wrongly diagnosed as CNS infection, so blood pressure should be measured in any child presenting with altered consciousness or convulsions. disturbances (hyperkalemia, hyperphosphatemia,

Prevention
1. Early systematic antibiotic therapy for streptococcal throat and skin infections does not eliminate the risk of acute post streptococcal glomerulonephritis. 2. Family members of patients with acute post streptococcal glomerulonephritis should be cultured for group A hemolytic streptococci and treated if culture is positive.

Treatment
1. Diet and fluid intake a. Salt and water are restricted in hypertension, oliguria or anuria, and heart failure. b.Fluid intake should be restricted to insensible water loss (400 ml/m 2 /24hr) plus urinary output. c. Protein and potassium intake restriction is indicated only in renal failure, and should be continued until renal function has normalized.

2. Treatment of hypertension a. Diuretics. Furosemide (1 mg/kg/dose IV, every 4-6 hours for rapid decrease in blood pressure; or 1-2 mg/kg oral every 6-12 hr) b.Beta blocker. Propranolol (0.05-1 mg/kg/day, in 3-4 doses, oral/IV) c. Vasodilators. Hydralazine (0.1-0.4 mg/kg/dose, every 2-4 hr, IV) d.Angiotensin converting enzyme (ACE) inhibitors. Captopril (0.1-0.5 mg/kg/dose, every 8-12 hr, maximum 6 mg/kg/day, oral) 3. Benzathine penicillin or oral penicillin for 10 days is recommended to limit spread of the nephrogenic organisms (see treatment of group A streptococci).

Prognosis
1. Complete recovery occurs in 95% of cases 2. Recurrence is extremely rare.