APPROACH TO HEMATURIA

BY
DR ASHWINI

Presence of Atleast 5 red blood cells (RBCs)

per microliter of urine.
urine analysis confirming the diagnosis of
hematuria suggested by a positive dipstick.

Heme-positive urine without RBCs is

caused by the presence of either hemoglobin
or myoglobin.

Rhabdomyolysis may occur secondary to

viral myositis, crush injury, severe electrolyte
abnormalities (hypernatremia,
hypophosphatemia).

Heme-negative urine may appear red, cola

colored, or burgundy, owing to ingestion of
various drugs, foods (blackberries, beet).

Causes of Hematuria in Children:

Glomerular hematuria:
Isolated Renal Disease
IgA nephropathy
Alport syndrome (hereditary nephritis)
Thin glomerular basement membrane nephropathy
Postinfectious GN
Membranous nephropathy
MPGN
FSGN
GBM disease
SLE nephritis
HSP nephritis
Wegener granulomatosis , Polyarteritis nodosa
HUS, Goodpasture syndrome
Sickle cell glomerulopathy , HIV nephropathy

EXTRAGLOMERULAR HEMATURIA
Upper Urinary Tract
Tubulointerstitial
Pyelonephritis
Interstitial nephritis
ATN ,Papillary necrosis
Nephrocalcinosis ,Vascular Arterial/venous thrombosis
Malformations (aneurysms, hemangiomas)
Nutcracker syndromEHemoglobinopathy (sickle cell trait/disease, SC
hemoglobin)
Hydronephrosis
Polycystic kidney disease
Tumor (Wilms, rhabdomyosarcoma, angiomyolipoma)
Trauma
Lower urinary tract
Inflammation
Cystitis Urethritis
Urolithiasis
Trauma
Coagulopathy ,Heavy exercise.

Common Causes of Gross Hematuria:

Urinary tract infection
Meatal stenosis
Perineal irritation, Trauma
Urolithiasis/hypercalciuria
Coagulopathy, Tumor
IgA nephropathy
Alport syndrome
Thin glomerular basement membrane disease
PIGN
HSP nephritis
Systemic lupus erythematosus nephritis

6 year old boy brought with c/o recurrent

hematuria precipitated by URI 2- 3 days
prior to onset of hematuria.c3- normal
Past h/o similar complaints.ur diagnosis?

IGA NEPROPATHY
most common chronic glomerular disease .
IgA within mesangial deposits of the
glomerulus and subendothelial cells.
5-7 days after URI/GIT symptoms.
more common in males than in females.
Gross hematuria, edema ,HTN,
nepritic/neprotic syndrome.
Normal c3 levels.
Serum IgA levels have no diagnostic value
because they are elevated in only 15% of
patients.

Only 20-30% of children at 15 -20 yrs after

disease onset may develop progressive renal
disease.
Poor prognosispersistent hypertension, diminished renal
function, and heavy or prolonged proteinuria.
worse prognosisdiffuse mesangial proliferation, extensive
glomerular crescents, glomerulosclerosis,
and tubulointerstitial changes, including
inflammation and fibrosis.

MANGEMENT:
HTN control.
Fish oil( omega 3 fatty acids) decreases the
rate of renal progression.
Immunosuppressive therapy with alternateday corticosteroids .
Angiotensin-converting enzyme inhibitors
and angiotensin II receptor antagonists are
effective in reducing proteinuria .
Patients with IgA nephropathy may undergo
successful renal transplantation.

4 year old boy c/o hematuria,proteinuria 1+

Urea & creatinine- normal.His ophthal & ent
examination had specific abnormalities.
Family h/o ESRD & renal transplantation
present.DIAGNOSIS?

ALPORTS SYNDROME:

Hereditary nephritis
X linked inheritance, mutations in COL4A5
GENE encoding the type IV collagen.
Microscopy-mesengial proliferation,capillary
wall thickening, thinning , splitting & layering
of glomerular & tubular basement membrane.
Clinical manifestations-microscopic hematuria
,recurrent episode of gross
hematuria,proteinuria in boys.
B/L SENSORINEURAL HEARING LOSS
OCULAR ABNORMALITIES-ANTERIOR
LENTICONUS, MACULAR FLECKS, CORNEAL
EROSIONS.

Leiomyomatosis of oesophagus,
tracheobronchial tree,female genitals.
GBM thickening & thinning, platelet
abnormalities.
Progress to ESRD 75 % OF X LINKED AS
treated with dialysis & kidney
transplantation.
ACE & ARB INHIBITORS reduce the rate of
progression and control of hypertension.

5 year old boy with c/o cola coloured urine for

2 days with oliguria & edema.
BP-120/80mmhg.he has healed impetigo
lesion over legs.Urine examinationdysmorphic RBC, minimal proteinuria, PMN
present.ADNB increased, c3 levels
decreased.ur DIAGNOSIS?

PSGN( NEPHRITIC
SYNDROME)

PSGN follows infection of the throat or skin by

certain nephritogenic strains of group A hemolytic streptococcus.
PSGN commonly occur after streptococcal
pharyngitis in 1-2 weeks & pyoderma in 3-6
weeks.
Most common Strains- throat (serotype 12)
and skin (serotype 49) infections.
Sporadic, Common in children 5- 12year of
age.

PATHOLOGY:
B/L Kidney enlarged.
All glomeruli appear enlarged & show
diffuse mesangial cell proliferation .
Polymorphonuclear leukocytes.
Immunofluorescence microscopy reveals
lumpy-bumpy deposits of IG in GBM.

Clinical manifestations:
edema, hypertension, and oliguria.
Edema -salt and water retention; nephrotic
syndrome may develop in 1020% of cases.
malaise, lethargy, abdominal or flank pain,
and fever are common.

DIAGNOSIS :

Urinalysis -RBC, RBC casts, proteinuria, PMN.

mild normochromic anemia present.
C3 level reduced.
ASO & ADNB increased.
Renal biopsyacute renal failure, nephrotic syndrome,
absence of evidence of streptococcal
infection, or normal complment levels & in
hematuria and proteinuria, diminished renal
function, and/or a low C3 level persist more
than 2 mo after onset.
Other organism-cogulase + ,
staph/pneumoccous.

COMPLICATION :
Hypertension 60%
heart failure
hyperkalemia, hyperphosphatemia,
hypocalcemia. Acidosis.
seizures, and uremia.
TREATMENT:
10-day course of systemic antibiotic therapy
with penicillin .
Sodium restriction
IV LASIX.
calcium channel antagonists, vasodilators,
ACE inhibitors used to treat hypertension.

MEMBRANOUS
GLOMERULOPATHY

Most common cause nephrotic syndrome in

adult.
It is associated with systemic illnesses such
as SLE or chronic ITP, sarcoidosis,
neuroblastoma, gonadoblastoma, gold or
penicillamine therapy, syphilis, and hepatitis
B and C virus infections.
Neonatal-onset membranous glomerulopathy
is characterized by maternal antineutral
endopeptidase (NEP) antibodies, which cross
the placenta from mothers genetically
deficient in the NEP antigen.

nephrotic syndrome , microscopic

hematuria , hypertension.
C3 levels are normal
diagnosis is confirmed only by kidney biopsy.
indications for biopsy include the nephrotic
syndrome , usually older than 10 yr, or the
presence of unexplained hematuria and
proteinuria.
increased risk of renal vein thrombosis.

TREATMENT:

The nephrotic state is best controlled with

salt restriction and diuretic agents.
Proteinuria may be decreased by ACE
inhibitors or ARB.
Immunosuppressive therapy with
prednisone in conjunction with chlorambucil
or cyclophosphamide reduce progression of
disease.

15 year old boy with acute nephritic picture

with decreased c3 levels.Renal biopsy shows
tram tracking appearance with accentuated
lobular pattern due to increased mesangial
matrix. C3 remain depressed even after 2
months. Ur diagnosis?

MPGN:
MPGN is the most common cause of chronic
glomerulonephritis in older children and young
adults.
hypocomplementemia results from an antibody,
referred to as C3 nephritic factor, that activates
the alternative complement pathway.
Three histologic types of MPGN TYPE 1-most common form, The glomeruli
reveal an accentuation of the lobular pattern
owing to a generalized increase in mesangial
cells
.TRAM TRACK APPEAREANCE

TYPE 2:Dense deposits present.

C3 immunofluroscence prominent without
concomitant immunoglobulin.
Worst prognosis.
nephrotic syndrome present with an acute
nephritic syndrome characterized by gross
hematuria
Renal function may be normal or decreased.
Hypertension.
serum C3 complement level may be
decreased.
50% progressed to ESRD,Poor prognosis.

14 year child with fever, dermatitis, weight

loss , multiple joint pain since 1 year on
treatment. currently c/o hematuria, protein
2+, c3 & c4 depressed. On histological
examination shows wire loop lesion. Ur
diagnosis?

GLOMERULONEPHRITIS WITH SLE

fever, weight loss, rash, hematologic
abnormalities, arthritis, and involvement of
the heart, lungs, central nervous system
80% RENAL disease
SLE are mediated by immune complexes.
Aberrations in both B- and T-cell function are
noted.
KIDNEY BIOPSY & EVALUATION OF
RENAL HISTHOPATHOLOGY is definite
diagnosis of SLE NEPHRITIS.

WHO CLASSIFICATION OF LUPUS NEPHRITIS

CLASS 1-Minimal
mesangial LN
II-Mesangial proliferative
LN
III-Focal proliferative LN
(<50% Glomeruli
involved)-active,chronic
Active & chronic
IV-diffuse proliferative
lupus nephritis( >50%
glomeruli involved)
V-membranous lupus
nephritis
VI-Advance sclerosing LN

C/F-No renal findings

II-Mild renal
disease,proteinuria
III-proteinuria,HTN some
evolve into class IV
IV-Most severe renal
involvement,HTN,
Heavy proteinuria,wire
loop lesions
V-significant proteinuria
VI-> 90%
Glomerulosclerosis

The diagnosis of SLE is suggested by the detection of circulating

ANA and double-stranded DNA.

C3 and C4 levels are depressed.

renal biopsy

Immunosuppressive therapy is initiated in all patients with

prednisone at a dose of 12 mg/kg/day divided into 2 or 3 doses
followed by a slow steroid taper over 46 mo beginning 46 wk
after achieving a serologic remission.

For patients having more severe forms of nephritis (WHO classes III
and IV), 6 consecutive monthly intravenous infusions of
cyclophosphamide at a dose of 5001,000 mg/m2 followed by
dosing every 3 mo for 18 mo appears to reduce the risk of
progressive renal dysfunction.

Azathioprine at a single daily dose of 1.52.0 mg/kg may be used

as a steroid-sparing agent in patients with WHO class I or II lupus
nephritis.

8 year old boy present with abdominal pain ,

arthritis,purpuric rashes b/l lower
limbs,hematuria.urine analysis-blood+.ur
diagnosis?

HSP NEPHRITIS
HSP nephritis is a small vessel vasculitis
characterized by a purpuric rash, arthritis,
abdominal pain, microscopic
hematuria,proteinuria,HTN.
Vessel wall infiltration IGA deposits in
mesangium.
1-3 week after URI symptoms.
Urinary abnormalities occur by 3 month after
onset of HSP
Urinalysis should performed weekly in
patient.
Prognosis is favourable progressed to CKD 25%

There are no studies demonstrating that

steroids administered after onset of HSP on
prevent nephritis.
Uncontrolled studies suggest high-dose
corticosteroid and cytotoxic therapy with
cyclophosphamide or azathioprine in
patients with crescentic glomerulonephritis
or significant proteinuria.

RPGN ( CRESENTRIC)

CLASSIFICATION:
Crescents may be found in several types
(1) the immune complexmediated forms of
GN: PSGN, lupus nephritis,
membranoproliferative GN, and HenochSchnlein purpura/IgA nephritis;
(2) antiglomerular basement membrane
mediated GN such as Goodpasture disease;
and
(3) (ANCA)mediated GN: microscopic
polyarteritis nodosa and Wegener
granulomatosis.

Histopathology-crescent in glomeruli
Hemturia, HTN, RENAL
insufficiency,proteinuria, occ late in course
of disease with oliguric renal failure.
Diagnosed by renal biopsy
Therapy combining pulse
methylprednisolone and oral
cyclophosphamide may be effective,
particularly in patients with Wegener
granulomatosis.
Plasmaphresis benefit in pt with ANCA
associated CGN.

GOOD PASTURE DISEASE

Pulmonary hemorrhage & glomerulonephritis
Histology-linear deposition of IGA along GBM
C3 LEVEL normal
ANCA elevated
It include systemic lupus erythematosus, HenochSchnlein purpura, polyarteritis nodosa, and
Wegener's granulomatosis,nephrotic syndrome
with pulmonary embolism, microscopic
polyangitis.
The diagnosis is suggested by kidney biopsy.
Prognosis poor.High dose iv methypredinosolone,
cyclophosphamide,plasmaparesis.
Progress to end stage renal failure despite of
treatment.

HEMOLYTIC UREMIC SYNDROME

It is the most common cause of acute renal

failure in young children.
It is characterized by the triad of
microangiopathic hemolytic anemia,
thrombocytopenia, and renal insufficiency.
acute enteritis with diarrhea caused by Shigalike toxinproducing Escherichia coli 0157.
HUS is also associated with Shigella ,bacterial
(Salmonella, Campylobacter, Streptococcus
pneumoniae, Bartonella) and viral
(coxsackievirus, echovirus, influenza, varicella,
HIV, Epstein-Barr) infections.
use of oral contraceptives, mitomycin, or
cyclosporine.

endothelial cell injury lead to formation of

clots-coagulation factors consumedthrombocytopenia.
fever ,vomiting ,abdominal pain.
Diarrhoea- bloody, sudden onset of pallor,
irritability,weakness, lethargy, oliguria.
Pneumococci associated HUS with
pneumonia, empyma,bactremia.
May progress to severe or fatal multisystem
disease.
Leukocytosis, hyponatremia,renal failure
CNSirritability,lethargy,seizure,encephalopathy
with HTN.

-microangiopathic hemolytic anemia,

thrombocytopenia.
The blood peripheral smear reveals schistocytes.
Coombs test is negative.
leukocytosis.
urinalysis- microscopic hematuria and proteinuria.
Partial thromboplastin time and prothrombin time
are usually normal.
Stool culture negative in pt diarrhoea with HUS.
fluid and electrolytes, control of hypertension,
aggressive nutrition, and early institution of
dialysis .
Antibiotics should be avoided in patients with acute
enteritis presumed secondary to E. coli 0157.
PLASMA INFUSION in severe HUS with CNS.

NUT CRACKER SYNDROME

Unilateral bleeding of varicose veins of the
left ureter, resulting from compression of
the left renal vein between the aorta and
superior mesenteric artery.
persistent microscopic hematuria
(occasionally recurrent gross hematuria,
proteinuria, lower abdominal pain, flank
pain, or orthostatic hypotension.
Diagnosis is confirmed by Doppler
ultrasonography, CT, or magnetic
resonance angiography

RENAL VEIN THROMBOSIS

In newborns and infants, is commonly
associated with asphyxia, dehydration,
shock, sepsis, congenital hypercoagulable
states, and maternal DM.
In older children, RVT is seen in patients
with nephrotic syndrome, cyanotic heart
disease, inherited hypercoagulable states,
and following exposure to angiographic
contrast agents.
sudden onset of gross hematuria ,
unilateral flank masses, flank pain,
hypertension, or oliguria. . Bilateral RVT
results in acute renal failure.

Usg- shows marked enlargement

Doppler flow studies of the inferior vena
cava and renal vein.
supportive care including correction of fluid
and electrolyte imbalance.

anticoagulation or thrombolytic agents

including streptokinase, urokinase, or
recombinant tissue plasminogen activator .

Children with severe hypertension

refractory to antihypertensive medications
may require nephrectomy.

IDIOPATHIC HYPERCALCURIA
autosomal dominant.
recurrent gross hematuria, persistent microscopic
hematuria, dysuria, or abdominal pain .
hypercalcemia, such as hyperparathyroidism,
vitamin D intoxication, Cushing & william
syndrome, corticosteroid therapy, distal RTA, or
Bartter syndrome.
Dent disease- X-linked
24-hr urinary calcium excretion exceeding 4
mg/kg.
A spot urine calcium to creatinine ratio
>0.2>7month > 0.8 < 7 mo of age.
Oral thiazide diuretics, Potassium citrate at a dose
of 1 mEq/kg/24 hr, Sodium restriction.

AR POLYCYSTIC KIDNEY DISEASE

infantile polycystic disease- The gene for
ARPKD encodes fibrocystin.
b/l flank masses during the neonatal
period,HTN.
It associated with oligohydramnios,
pulmonary hypoplasia, respiratory distress,
and spontaneous pneumothorax
Potter facies - low-set ears, micrognathia,
flattened nose, limb-positioning defects, and
growth deficiency may be present.
oliguria and acute renal failure may be seen.
Transient hyponatremia.
Renal function is usually impaired.

LIVER-(1) ascending cholangitis, varices,

and hypersplenism related to portal
hypertension (2) progressive liver
dysfunction, which rarely leads to overt
liver failure and cirrhosis.
The treatment is supportive.
Aggressive ventilatory support.
Careful management of HTN, fluid and
electrolyte abnormalities.
80% Pt die In neonatal peroid.

AD POLYCYSTIC KIDNEY DISEASE

85% PKD1 gene on chromosome 16, which
encodes polycystin
1015% PKD2 gene on the long arm of
chromosome 4, which encodes polycystin 2.
Cyst formation in multiple
organ(liver,pancreas,spleen & brain)& dev of
cereberal aneurysms.
- gross or microscopic hematuria,b/l flank pain,
abdominal masses,HTN, UTI
- USG- multiple bilateral macrocysts, enlarged
kidney.
- treatment is supportive.
- Control of blood pressure ACE & ARB
inhibitors.

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