Transcript Approach_to_Hematuria_and_Proteinuria_in_Children.ppt

Approach to Hematuria and
Proteinuria in Children
Adi Alherbish
Objectives
• To be able to define and recognize hematuria
and proteinuria
• To be able to generate a differential diagnosis of
the commonest and most serious causes of
hematuria and proteinuria
• To have a clinical approach to both conditions.
Case 1
• 14 year old boy presenting with red urine
since last night. Otherwise healthy. Normal
BP, no flank pain, no ankle edema.
• What’s the next step?
Case 1
• Urine dipstick: negative
Case 2
• 5 year old boy presenting with pallor, and
shortness of breath.
Urine dip: SG 1.015, Hg 2+, Prot neg,
Urinalysis: RBC 0, WBC 0
Case 2
• CBC: Hg 80, WBC 5, Plt 180
• Retics: 3%
Hemolytic Anemia
• Send blood for: Hg electrophoresis,
peripheral smear, Coombs test, G6PD
Case 3
• 14 year old girl, healthy
• Regular check up:
Urine dip: SG 1.035, Hg 2+, Prot trace
Urinalysis: RBC 5- 10 /HPF
WBC 0- 5 / HPF
Case 3
• Repeat urinalysis after drinking a bottle of
water:
Urine SG: 1.015
RBC: 1- 5 /HPF
WBC: 0- 5 / HPF
HPF= x 400
Case 5
• 9 year old girl, presenting with fever, rash,
coryza, conjuctivitis, and dark urine.
• Urine dip: SG 1.015, Hg +3, Prot trace
• Urinalysis: RBC > 100/ HPF
WBC 10- 25/ HPF
Case 5
• Urine positive for adenovirus
Case 6
• 14 year old girl, presenting with
intermittent, sudden onset left flank pain
and dark urine.
• Urine SG: 1.015, Hg 3+, Prot neg
• Urinalysis: RBC 100/ HPF, WBC 0
Crystals present
Case 6
• In clinic: send urine for Ca/ Cr ratio, citrate,
oxalate, uric acid, cystine
Case 7
• 14 year old girl, with hypertension, left
knee arthritis, dark urine, malar rash
• Urine dip: SG 1.010, Hg 2+, Prot 2+
• Urinalysis: RBC 10- 25/ HPF
WBC 0
RBC casts
Case 7
• Send blood for:
C3, C4, ANA, anti-ds DNA
Hematuria
• Presence of > 5 RBC/ HPF, on more than
two occasions, in the context of a normal
urine specific gravity
The 3 Vital Questions
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3
Is it true hematuria?
Is it serious (urgent)?
What is the cause?
Is it serious?
Nephritis
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Hematuria
Hypertension
Oliguria
Increased Cr
Nephrosis
• Edema
• Nephrotic range
proteinuria
• Low albumin
• Hypercholestrolemia
Rapidly Progressive
Glomerulonephritis (RPGN)
RPGN
Immune Complex
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Post- strep GN
IgA nephropathy
Lupus
HSP
Pauci- immune
Anti- GBM
• Wegner’s
granulomatosis
• Microscopic
polyangiitis
• Polyartritis nodosa
• Goodpasture’s
disease
RPGN
Immune Complex
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Post- strep GN
IgA nephropathy
HSP
Lupus
• ASO, anti-DNase
• Immunoglobulins
• ANA, anti-ds DNA,
C3, C4
Pauci- immune
Anti- GBM
• Wegner’s
granulomatosis
• Microscopic
polyangiitis
• Polyartritis nodosa
• Goodpasture’s
disease
• ANCA
• Anti- GBM
Post strep Glomerulonephritis
• Strep pharyngitis, or strep skin infection,
followed 10 to 14 days by microscopic
hematuria, nephritis, or nephrosis
• Diagnosis: positive ASO
low C3 which normalize in 8 weeks
• Management: supportive
• Prognosis: Excellent (Vog et. Al: 137 cohortESRD: none, high Cr 10%)
IgA nephropathy
• Typical presentation: intermittent gross
hematuria that happen during colds
• Other: gross hematuria
microscopic hematuria
nephritis
nephrotic syndrome
ESRD
IgA nephropathy
• Diagnosis: clinical suspicion
IgA level 20% sensitivity!
Kidney biopsy- IgA in Immunoflorecence
• Treatment: supportive in mild cases
ACEI in proteinuria
Steroids
Henoch Schonlein Purpura
(HSP)
• Pathology: IgA nephropathy
• Clinical:
- purpuric rash
- arthritis
- intestinal edema
(intussusception)
- hematuria/ nephritis/
nephrosis
Hemolytic Uremic Syndrome
• Pathogenesis:
- typical (d+): E. coli O157:H7 shiga toxin 1
induced vascular injury
- atypical (d-): alternative complement
pathway defect
• Clinical: triad of microangiopathic
hemolytic anemia, thrombocytopenia, ARF
Alport Syndrome
(Hereditary Nephritis)
• Homozygous mutation in
genes encoding type IV
collagen in basement
membrane
• Genetics: 80% X-linked
AR, AD
• Clinical: persistent
microscopic hematuria,
hearing loss, lenticonus
Benign familial hematuria
(thin basment membrane
nephropathy)
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Autosomal dominant
Hetrozygous mutation in type IV collagen
Microscopic hematuria
Screen the parents’ urine
Benign course
Work up for hematuria
(History is important!)
1. Gross hematuria: onset, duration, progression, aggravating, relieving factors,
associated symptoms
2. UTI symptoms: dysuria, frequency, urgency, urge incontinence
3. Food intake: beet
4. Drugs: rifampin, nitrofurantoin, ibuprofen
5. IgA: gross hematuria onset while having colds
6. post strep: history of sore throat, tonsillitis, skin infection
7. HUS: diarrhea, pallor, fatigue, SOB
8. HSP: pupuric skin rash over legs and buttocks (palpable), join swelling/pain, abdominal
pain/bloody stools
9. Goodpasture/Wegners: hemoptysis, cough, SOB
10. SLE/ vasculitis: butterfly rash, discoid rash, mouth ulcers, photosensitivity, CNS
seizures/psychosis, join swelling
11. Kidney stones: renal colic, radiation to groins, past history or family history of stones
12. Anatomic: antenatal U/S, single umbilical artery, abdominal swelling
13 Hereditary: family history of deafness, family member with hematuria
14. Bleeding diathesis: hemarthrosis, epistaxis, petechaie, heavy periods in older girls
15. Problems with high blood pressure
16. Family history: nephritis, kidney failure, transplant, deafness, stones, hematuria,
consanguinity
Work up for hematuria
• Nephritis: ASO, C3, C4, anti-ds DNA,
ANA, ANCA, anti- GBM
• Kidney and bladder U/S
• Stone work up: urine Ca, Cr, oxalate,
citrate, cystine, uric acid
• Urinalysis in both parents
• Bleeding tendency: PT, PTT, INR
Proteinuria (Urine dip)
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Negative
Trace
1+
2+
3+
4+
< 10 mg/dl
10- 20 mg/dl
30 mg/dl
100 mg/dl
300 mg/dl
1000 mg/dl
Proteinuria (Quantitative)
Non- nephrotic
Nephrotic
• Urine prot/cr:
> 20 mg/mmol
• Urine prot/cr
> 200 mg/mmol
• 24 h urine collection:
> 100 mg/m2/day
> 4 mg/m2/hr
• 24 h urine collection:
> 1 g/m2/ day
> 40 mg/m2/hr
The 3 Vital Questions
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2
3
Is it persistent?
Is it nephrotic?
What is the cause?
Case 1
• 15 year old, athletic boy
• Regular check up:
Urine dip: Prot 2+
Urine prot/Cr ratio: 50 mg/mmol
• What next?
Case 1
• 8 am: urine prot/Cr ratio- 10 mg/mmol
• 4 pm: urine prot/Cr ratio- 50 mg/mmol
Orthostatic proteinuria
Non Persistant Proteinuria
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Fever
Strenuous exercise
Cold exposure
Epinephrine infusion
Orthostatic
Case 2
• 1 year old infant with failure to thrive. Both
height and weight are below the 3rd
percentile. He has sings of rickets in
exam.
• Urine dip: Prot 3+ , Glu 2+
Derakhshan Ali et al. Saudi J Kidney Dis Transpl. 2007 Oct-Dec;18(4):585-9.
Fanconi Synrome
• PCT defect
• Proximal renal tubular
acidosis (type II RTA)
• Glucosuria
• Aminoaciduria
• Phosphaturia
• hypokalemia
Proteinuria
Glomerular
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Congenital:
-Finish- type
- TORCH infection
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Nephritis:
- postinfectious GN
- lupus
- Wegner
- HUS
- Goodpasture
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Nephrotic:
- Minimal change
- FSGS
- MPGN
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Drugs: captopril
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Neoplasia
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Renal vein throbosis
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Tubualr absorption
Protein overload
ATN
Fanconi Syndrome
Cystic/dysplastic
Interstial nephritis
Pyelonephritis
• Hemolysis
• Rhabdomyolysis
• Light chain
Proteinuria
Glomerular
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Congenital:
-Finish- type
- TORCH infection
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Nephritis:
- postinfectious GN
- lupus
- Wegner
- HUS
- Goodpasture
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Nephrotic:
- Minimal change
- FSGS
- MPGN
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Drugs: captopril
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Neoplasia
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Renal vein throbosis
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Tubualr absorption
Protein overload
ATN
Fanconi Syndrome
Cystic/dysplastic
Interstial nephritis
Pyelonephritis
• Hemolysis
• Rhabdomyolysis
• Light chain
Urine electrophoresis:
• Glomerular: albumin
•Tubular: other proteins..
Case 3
• 5 year old boy, presenting with puffy eyes,
enlarged tummy, and feet swelling.
• Exam: normal BP, ascites, pitting edema
• Urine dip: Prot 4+
• What’s the next step?
Case 3
• Urine prot/cr 1500 mg/mmol
• Serum albumin 15 g/l
• High cholesterol
Nephrotic Syndrome
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Urine Prot/Cr > 200 mg/mmol
Serum albumin < 25 g/l
Edema
Hyperlipedemia
Nephrotic Syndrome
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Minimal change disease
Focal segmental glomerulosclerosis
Membranoproliferative
Membranous GN
Infection: HIV, hepatits, syphilis
Lupus, Ig A, HSP, post strep
Initial therapy
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Supportive: albumin 25% and lasix prn
Salt restriction
Fluid restriction while nephrotic
Prednisone 60 mg/m2/day for 6 weeks
followed by 40 mg/m2/day for 6 weeks
then wean..
Indications for biopsy
• Steroid resistant: fail to enter remission after 8
weeks of therapy
• Steroid dependent: intially enter remission, but
develping relapse while on therapy, or within 2
weeks of steroid discontinuration
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Hematuria
Increased Cr (when intravasculary repleted)
Low complement
Positive lupus serology