Renal System-Hematuria By Ryan

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Renal System-Hematuria by Ryan

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Renal System-Hematuria by Ryan

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Define hematuria, gross or microscopic

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gross- blood can be seen in urine
microscopic- blood can be seen only in micoscope

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Red urine can be...

Dark Brown/Black urine can be...

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red- blood, myoglobin, porphyrins, beets, blackberries, rifampin

dark brown/black- blood, homogentisic acid, melanin, tyrosinosis, or methemoglobinemia

*Confirm hematuria with three dipstick measurements over time

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Gross Hematuria Etiologies

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Infectious:
Bacterial UTI
Viral cystitis (adenovirus most frequent cause)

Glomerulonephritis:
Acute post-streptococcal GN
IgA nephropathy (Berger Disease)
Membranoproliferative GN
Anaphalactoid purpura (Henoch-Schonlein purpura)
Lupus nephritis
Hemolytic-uremic syndrome (HUS)

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4 causes for persistent asymptomatic hematuria (in otherwise healthy children)

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-Idiopathic Hypercalcuria
-IgA Nephropathy
-Thin basement membrane disease
-Early Alport syndrome

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Idiopathic Hypercalcuria

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-Presence of excess calcium in the urine without obvious cause.
-Forms stones, which causes hematuria
-Hypercalciuria can occur at any age, including newborns.
-The peak incidence is in children aged 4-8 years.

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Thin basement membrane disease. Hereditary? What's the change in renal function?

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-Hereditary and autosomal dominant
-Not all genetic mutations have been characterized
one mutation in the type IV collagen alpha 4 gene.
-Prevalence 5 to 9%.
-Asymptomatic, usually incidental microscopic hematuria
-mild proteinuria & gross hematuria are occasionally present.
-Renal function is typically normal
-A few patients develop progressive renal failure for unknown reasons.

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Early Alport syndrome. where's the mutation? what is it associated with?

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-Mutations of type IV collagen, which makes up the basement membrane
-80% x-linked, can be recessive or dominant
-Associated with sensorineural defects
-Always think about it in any child with renal problems and a family history of early hearing loss
-No specific treatment

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Lab tests at initial presentation...

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Tests for children at initial presentation:
-CBC
-Urine culture
-Serum creatinine
-Urine Ca:Cr ratio & Urine protein:Cr ratio
-Serum C3 level* look for renal pathologies
-Ultrasound
-If above tests are normal

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How does Acute Poststreptococcal Glomerulonephritis present?

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-Classic nephritic syndrome with gross hematuria, edema, HTN & renal insufficiency

Other organisms can cause post-infectious GN: Other bacteria, Viruses, Parasites

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What do antibiotics do with acute poststrep GN? What's a key clinical finding?

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Antibiotics DO NOT prevent acute GN, but does prevent the spread of the strain of Strep

clinical finding: latency between pharyngitis and GN is 1-2 weeks (ie 1-2 weeks after the infection). With skin infections, latency can be longer (3-6 wks)

Edema & gross hematuria: "smoky" or "cola" colored urine (for hematuria in general)

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key lab finding for acute poststrep GN

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Key finding is low serum C3
-Low for 6-12 weeks
-Be aware that low C3 is also seen in MPGN and SLE

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The most common cause of gross hematuria is...

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IgA nephropathy(Berger nephropathy).

-Most common between 10-30 years of age
-Usually idiopathic
-Occurs with increased frequency in systemic diseases

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Clinical findings of IgA Nephropathy... how's the latency period?

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-Classically presents as recurrent episodes of painless macroscopic hematuria
-Latency period is much shorter for IgA nephropathy*** (this is the key to differentiate)
-Usually concurrent with an infection
-Gross hematuria lasts several days
-Interval between recurrences can vary from months to years

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Laboratory for IgA nephropathy. What do you want to check to rule out similar diseases

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usually check C3 and ASO because presents similar to APSGN (which is low C3, elevated ASO)

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General guidelines for treatment

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-ACE inhibitors when hypertensive or proteinuria
-Omega-3 fatty acids may slow progression to renal insufficiency
-Steroids reserved for florid or progressive cases
-Intensive immunosuppressives for crescenteric disease

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Henoch-Schonlein Purpura Nephritis. Four Classic Features include...

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Four classic features:
-Purpuric rash
-Abdominal pain
-Arthralgias
-Glomerulonephritis with IgA deposition
On biopsy, renal lesion looks just like IgA nephropathy, but it goes away.

90% full recovery

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Membranoproliferative Glomerulonephritis Mechanism

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-Thickening of glomerular basement membrane and hypercellularity
-Most common cause of chronic glolerulonephritis in older children & adults

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Why is there thickening and hyperproliferation?

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Thickening due to immune complex deposition and/or interposition of mesangial cell cytoplasm in the GBM

Hypercellularity due to proliferation of mesangial cells and influx of WBCs

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What do you need in order to diagnose MPGN?

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*Must have renal biopsy for diagnosis
-Type I (and usually Type III) have low C3 & borderline or low C4
-Type II has low C3, normal C4
-C3 returns to normal in 6-8 weeks with APSGN, but stays low in MPGN
-50% have a normocytic, normochromic, Coombs’ negative anemia

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What are the three groups of crescentic GNs?

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1. Immune complex nephritis (80-85%)
-APSGN, Berger, MPGN, SLE
2. Pauci-immune disease (13-15%)
-Wegener’s, polyarteritis, Churg-Strauss
3. Anti-GBM disease (5-7%)
-Goodpasture, anti-GBM nephritis

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Anti-GBM disease, who gets it, presentation, antibody found, renal biopsy

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-Most common in young men
-Can have isolated renal disease
-Usually have pulmonary hemorrhage – Goodpasture Syndrome
-Present with acute nephritis and progress to renal failure within weeks
-Will find anti-GBM antibody in 90% of patients
-Renal biopsy: crescentic nephritis & linear deposits of IgG and C3 in the GBM

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What's the therapy for Anti-GBM Disease?

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-Plasmapharesis is mainstay of therapy
-May also benefit from immunosuppressive therapy
-In most patients, it is short lived (8-14 weeks)
-Quickly causes irreversible renal injury
-ESRD is inevitable if Cr > 6 mg/dL

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What do you usually see in kids with Lupus Nephritis?

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Happens, but rare in children
-Seen usually in kids with complement deficiencies (C2, C3, C4) and IgA deficiency

Caries from asymptomatic microscopic hematuria to RPGN

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What are the three types of ANCA-Positive GNs? Btw, just a hint, they are all rare in children.

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1. Churg-Strauss (allergic asthma followed by vascultitis)

2. Wegener’s granulomatosis (very rare in kids)
Remember cANCA!

3. Microscopic polyarteritis
No granulomas, but still with pulmonary hemorrhage
pANCA elevated, cANCA is not

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Ok, tell me, what is Hemolytic Uremic Syndrome. What is it the most common cause in?

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-Complicated sequence of events that begins with endothelial and glomerular injury, ultimately results in microangiopathic hemolytic anemia, thrombocytopenia and renal failure

-Most common cause of acute renal failure in previously healthy children

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What do 90% of kids have first? How did they get it?

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90% of children have a preceding diarrhea
-Usually E. coli O157:H7 (Shigella-like toxin)
-Also associated with S. pneumoniae, HIV, drug exposure or systemic disease

Source for E. coli can be contaminated beef, lakes, swimming pools, fruits, vegetables & raw milk

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Clinical Manifestations of HUS

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-Initially have gastroenteritis (vomiting/diarrhea), low grade fever
-HUS develops with 2-14 days after illness begins
-½ require dialysis, renal failure lasts about 2 weeks
-Anemia and thrombocytopenia vary in severity
-Neurologic involvement is common (scary)

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Laboratory for HUS

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-Elevated Cr, markers of microangiopathic hemolytic anemia
-PT & PTT usually normal
-UA: hematuria and proteinuria
-**Elevated WBCs (>20 = worse prognosis)
-Obtain stool cultures to look for E. coli

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Treatment for HUS. What don't you give?

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-Supportive care & dialysis have reduced mortality to <5%
-75% require blood transfusions
-DO NOT GIVE antidiarrheals or antibiotics
-Prolongs shedding
-Remember, it’s a TOXIN – Abx make it worse
-Only 60% have normal renal function after HUS