Hematuria

Fatma Al-Abri 125473

Husain Al-Aswami 132017
Outline
• Definition of hematuria
• Classification
• History and physical examination
• Differential diagnosis
• Investigation
• Management
Hematuria
• The presence of red blood cells (RBCs) in the urine.
• The presence of more than 3-5 RBCs per high power field on freshly voided and
centrifuged urine.
• Can be either gross (overtly bloody, smoky, tea-colored urine), or microscopic.
• Isolated asymptomatic microscopic hematuria is found in up to 4% of healthy children
• Hematuria may originate from any site in the urinary system.
• Pathophysiology:
• Structural disruption in the integrity of the glomerular basement membrane caused
by inflammatory or immunologic processes
• Toxic disruption of the renal tubules
• Mechanical erosion of mucosal surfaces in the genitourinary tract.
False positive hematuria
• Red urine is not always hematuria
• The first step in evaluating the patients is to perform
a standard urinalysis, focusing on.
a) If the dipstick turns heme-positive
b) If there are red blood cells visualized on
microscopic examination
• Heme-positive urine specimens in which RBCs are
NOT seen on microscopic exam suggest the
possibility of either myoglobinuria ( rhabdomyolysis)
or hemoglobinuria ( hemolytic anemia, paroxysmal
nocturnal hemoglobinuria)
Differential diagnosis of red urine
• Hemoglobinuria and myoglobinuria
• Both conditions cause red, amber, or Coca-Cola-
colored transparent urine that remains
pigmented after centrifugation.
• Porphyrias
• tea-colored urine in Porphyria tarda.
• Red-purple urine in acute intermittent
porphyria.
• Beeturia: red discoloration of urine after eating
beetroot
• Drug- induce urine discoloration
• Rifampin: harmless red-orange discoloration of
bodily fluids ( urine, sweats, tears)
• Phenazopyridine: orange discoloration of urine
Classification
Classification
Classification
According To Quantity
• Macroscopic hematuria ( gross hematuria)
• Frank blood in urine resulting in visible red/brown discoloration on the urine.
• Suggests damage to the kidneys, upper/lower urinary tract ( Nonglomerular
hematuria)
• Microscopic hematuria
• RBCs are present in the urine sediment but no urine discoloration is visible to the
naked eye.
• Can indicate damage to the glomeruli
• However, a number of conditions can cause Nonglomerular microscopic
hematuria( UTI,BPH, urolithiasis, malignancies, menstruation
Classification
According to an occurrence during voiding
• Initial hematuria
o Gross hematuria occurs at the beginning of micturition and clears
by the end of it.
o Typically suggests urethral damage.
• Terminal hematuria
o Passage of blood or clots in urine during the last part of micturition
(when the bladder neck contract).
o Suggests damage to the bladder neck, prostate, or trigonal area.
Classifications
According to an occurrence during voiding:
• Total hematuria
o Passage of blood or clots throughout the entire micturition.
o Suggests damage to the bladder, ureters or kidney.
• Painless hematuria
o Passage of blood or clots in urine in the absence of renal or
urinary symptoms.
o Suggests malignancy.
Classifications
According to origin:
• Glomerular hematuria
• Nephritic syndrome
• Characteristic of glomerulonephritis.
• Hematuria with proteinuria is considered glomerulonephritis until proven otherwis
• Isolated hematuria: the presence of RBCs in the urine with no other urinary abnorma
• Transient isolated hematuria
• Strenuous exercise (i.e., exercise-induced hematuria)
• Infections
• Persistent isolated hematuria
• Most likely IgA nephropathy
• Alport syndrome
Classification
According to origin:
• Nonglomerular hematuria
o Urolithiasis
o Infection: cystitis, urethritis, prostatitis
o Malignancy: urothelial cancer, renal cell carcinoma, prostate cancer, WILMS TUMOR
o Coagulation disorders: platelet dysfunction, hemophilia
o Urinary tract obstruction: congenital anomalies, BPH
o Polycystic kidney disease
o Renal papillary necrosis: sickle cell disease, acute pyelonephritis, DM
o Trauma
o Drugs: warfarin, heparin…
Classification
History and physical
examination
Case
• A 7 years old boy was referred to the paediatric clinic with complaints of dark urine for 2 days. You are
based at the clinic for 1 week. The consultant in charge has asked you to take a full history and provisionally
evaluate the child, urine dipstick performed at the clinic showed 2+ blood, nil protein.

• What further points in history you should ask?

• What are the related clinical signs you should look for?
• What are the differential diagnoses of hematuria?
• What related investigation you will order and how it helps you?
• The presence of absence of RBC on urine microscopy is needed to rule out which conditions?
• Outline your management.
• Outline the important aspect of counseling the parents.
History
• History of presenting complaint
• Quantity
• Occurrence during voiding
• Associated symptoms
• Flank pain, lower abdominal pain, painful urination, urinary urgency or
frequency, fever, passing stone or grits, recent throat or sick infection
• Joint pains, oral ulcer, rash, hemoptysis, leg swelling, hearing loss, flank mass,
back pain
• Constitutional symptoms ( weight loss, anorexia, cachexia)

• Menstruation
• Antenatal history
History
• Past medical history:
o Sickle cell can cause papillary necrosis, which can lead to hematuria
o Kidney disease
• Recent trauma
• Medications:
o Anticoagulants (Warfarin and DOAC)
o nephrotoxic agents (NSAIDS, aminoglycosides)
o Rifampin
• Social History
• Family history
History Suggestion
History
Recent vigorous exercise or trauma Exercise-induced hematuria

New onset of incontinence, dysuria, UTI

frequency, or urgency

u/l flank pain radiate to groin suggests obstruction (calculus / blood clot)

Flank pain w/o radiation but w/ fever, acute pyelonephritis

dysuria, and frequency and/or urgency

Timing of hematuria during micturition • Initial hematuria: urethral bleeding

• Continuous bleeding: bleeding in the bladder, ureter or kidneys
• Terminal bleeding: bladder disease

Color of urine • Glomerulonephritis: brown urine.

• LUT bleeding: pink/red urine, +/- blood clots.
History
Hx Suggestion

Pharyngitis/impetigo (2-3 weeks Poststreptococcal glomerulonephritis

earlier)

Joint pains, sick rashes, prolonged fever Henoch-schonlein purpura, SLE

URTI (1-2 days earlier) IgA nephropathy.

Predisposing clinical conditions SCD/ Coagulopathy (e.g. Hemophilia)

Physical examination
• Vitals
• General examination
• Skin examination
• Abdominal examination
• Genital examination
• Ophthalmological evaluation
Important signs to look for
• Febrile
• Hypertension
• Periorbital edema
• Presence of pallor, icterus, oral ulcer, or rash
• Hearing impairment
• Generalized lymphadenopathy
• Joints swelling
• Flank mass
• Palpable enlarged cystic kidneys
• Costovertebral angle tenderness
• Pubic tenderness
• Urethral discharge or tear
• Lower extremity edema
Differential diagnosis
Glomerular causes
Post Streptococcal Glomerulonephritis
(PSGN)
• The most common form of acute GN
• Epidemiology :
• Children 3-12 years of age .
• Course:
• Occurs after 1-3 weeks after group A beta haemolytic streptococcal infection .
• Pharyngitis /tonsilitis: 1-2 weeks after infection.
• Impetigo skin infection: 3-4 weeks after infection .

• Clinical features :
• Tea colored / cola urine
• Hypertension
• Edema
• Oliguria

• Prognosis: restitution of kidney function in > 90% of case

• Feared Complication: AKI, RPGN, Nephrotic syndrome
Diagnostics Treatment
• Urinalysis: Red cells are
• Management of edema and
dysmorphic with or without red volume overload
blood cell casts • Low-sodium diet:
• Culture: 25% positive throat or • Fluid restriction
skin culture • Loop diuretics
• Serology: Raised Anti-streptolysin
• Antihypertensives
(ASO) - Raised Anti-DNase B
antibodies . • Indicated for hypertension
• Complement: low C3 refractory to management
of edema and volume
• LM: Enlarged and hypercellular
overload
glomeruli
• Calcium channel blockers
• IF: “Starry Sky”, “Lumpy bumpy”
granular appearance • ACE inhibitors or ARBs
• EM: Subepithelial humps
IgA Nephropathy (Berger disease)
• The most common cause of chronic GN
• The course of the disease is highly variable and can manifest in the following forms:
• Asymptomatic
• Recurring episodes of:
• Gross or microscopic hematuria
• Flank pain
• Low-grade fever
• And/or nephritic syndrome (including hypertension)
• Usually during or immediately following a respiratory or gastrointestinal infection
• Diagnostics: Normal C3 with IgA deposits on IF
• Management: Glucocorticoids, ACEi, May slowly progress to ESRD
Henoch-Schönlein Purpura (HSP)
• IgA vasculitis affecting the small blood vessels of the skin, joints,
intestines, and kidneys. Resulting in their inflammation and bleeding.
• Commonly developed after an upper respiratory infection, such as a
cold. Other triggers include chickenpox, strep throat, measles,
hepatitis, certain medications, food and insect bites.
• Most common in children under 10 years. More common in males.
• Self limiting.
Henoch-Schönlein Purpura (HSP)
• Reddish-purple Rash (Purpura), in the buttocks, legs, and feet.
Also on arms, face and trunk, and areas of pressure (Sock line and
waistline)
• Arthritis (Swollen and sore joints) of knees and ankles. After the
rash by a week or two.
• GI symptoms as abdominal pain, nausea, hematemesis and
melena before the rash.
• Renal involvement: Proteinuria or/and Hematuria (Gross or
microscopic)
• Complications: Need for dialysis or Transplant. Intussusception
• TTT: Supportive. IV methyl prednisone for severe disease
Alport Syndrome (Hereditery Nephritis)
• Hereditary X-linked dominant disease
• Mutation in Collagen type IV
• Clinical Presentation
• Renal involvement:
• Persistent Microscopic Hematuria from childhood and with Gross Hematuria and Proteinuria as illness
progresses.
• Proteinuria.
• Renal insufficiency or renal failure.
• Hypertension.
• Ocular Involvement:
• Anterior Lenticonus (Posterior lenticonus is less common).
• Leads to progressive myopia.
• Hearing Defect:
• Progressive sensorineural hearing loss.

“Can’t Pee; Can’t See; Can’t hear a bee”

Alport Syndrome (Hereditery Nephritis)
• Diagnosis:
• Renal Biopsy + Clinical presentation + Family History
• Genetic Analysis
• Treatment:
• Supportive treatment (Antihypertensives and ACEi)
• Renal Transplant (in ESRD)
• Intraocular lens implantation (Lenticonus)
Thin Basement Membrane Nephropathy (Familial
Hematuria)
• Diffuse thinning of the glomerular basement membranes (GBM).
• Associated with heterozygous variants in COL4A3 or COL4A4, the genes that encode
the alpha-3 and alpha-4 chains of type IV collagen, respectively

• Presentation:
• Microscopic Hematuria
• Gross Hematuria and flank pain
• Acute Kidney Injury

• Diagnosis:
• Kidney Biopsy
Good Pasture’s Syndrome (Anti-GBM disease)
• Type II hypersensitivity reaction where antibodies are directed
primarily against renal glomerular basement membrane alpha-3
chain of collagen type IV. This auto-antibody cross-reacts with
alveolar basement membrane also. It is a pulmonary-renal syndrome.

• Presentation:
• Hemoptysis
• Hematuria and proteinuria
• Cough and Dyspnea
• Oliguria and uremia symptoms
Good Pasture’s Syndrome (Anti-GBM
disease)
• Diagnosis:
• Microscopic hematuria/ RBC
casts or gross
• Renal biopsy – linear deposits
of immunoglobulins at
glomerular basement
membrane and alveolar septa.
• LM: Glomerular crescent
formation
• IF: Linear deposition of anti-
GBM ABs
• Granulomatosis with polyangiitis c-ANCA
• Churg-Strauss disease p-ANCA
• Microscopic polyangiitis p-ANCA
Non-glomerular
casues
Lower Urinary Tract Hematuria
• Inflammation
• Cystitis
• Hemorrhagic cystitis
• Urethritis
• Injury
• Trauma
• Kidney Stones
• Hypercalciuria
• Familial hypocalcemic
hypercalciruia
Tubulointerstitial/Parenchymal
Hematuria
• Inflammation
• Interstitial Nephritis
• Pyelonephritis

• Vascular pathology
• SCT/D
• Coagulopathies
• Nutcracker Syndrome

• Structural
• Cyst rupture
• Wilms Tumor
• UT-Obstruction
• Renal Trauma
Nutcracker syndrome
• The compression of the left Renal Vein by two arteries
(Abdominal Aorta and SMA) or (Abdominal Aorta and spinal
bones) leading to Renal Venous Hypertension > Leading to
rupture of thin-wall veins into the collecting system > Hematuria.
• Presentation:
• Left flank pain / Pelvic pain
• Hematuria
• Varicocele
• Association with thin body habitus
• Complications:
• Renal Vein Thrombosis
• Treatment:
• Surgical correction
Investigations and
Management
Investigations
• Complete blood count
• Urea creatinine electrolytes
• Urinary microscopy and urinary culture
• Urine protein to creatinine ratio
• Urine calcium to creatinine ratio
• ASO titer, complements C3, C4, IgA, ANA, ds-DNA, ANCA
• Hepatitis B and C
• Abdominal ultrasound
• Renal biopsy
• CT scan abdomen for renal masses or stones.
• Cystoscopy
Imaging
• Renal and bladder ultrasound
• Detect structural abnormalities leading to hematuria, such as renal stone, neoplasm,
cystic lesion, hydronephrosis, dilated urinary tract, and bladder abnormalities.
• Indication: gross hematuria without proteinuria or RBC cast
• Renal biopsy indications:
• Nephritic and/or nephrotic syndrome with no apparent underlying disease: allows
diagnosing type of glomerulonephritis
• Suspected lupus nephritis
• Rapidly progressive glomerulonephritis
• Renal transplant rejection or dysfunction
• Unexplained acute kidney injury
• Skin biopsy immunostaining (X linked Alport syndrome)
Refernces
• Nelson essentials of pediatrics 9th edition
• Amboss medical library
• Illustrated textbook of paediatrics (Sunflower)