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67 Cards in this Set
- Front
- Back
What are 2 common etiolgies of gross hematuria?
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infectious or Glomerulonephritis (GN)
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What virus is most commonly found in pediatric cystitis?
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adenovirus
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What are the 6 GN's that cause gross hematuria?
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1. acute post-strep GN
2. IgA nephropathy 3. membranoproliferative GN 4. HSP (anaphylactoid purpura) 5. lupus nephritis 6. HUS |
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Of the 6 GN's that cause gross hematuria, which are seen mostly seen in kids?
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HSP and HUS
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How do you confirm hematuria?
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3 different dipsticks over time
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What can turn urine red?
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blood, myoglobin, porphyrins, beets, blackberries or rifampin
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What can turn urine brown or black?
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blood, homogentisic acid, melanin, tyrosinosis, or methemoglobinemia
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In healthy kids, what are the 4 causes of persistent hematuria?
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1. Idiopathic Hypercalcuria
2. IgA Nephropathy 3. Thin basement membrane disease 4. Early Alport syndrome |
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T or F: newborns can be afflicted by idiopathic hypercalcuria.
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true
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What is the peak incidence of idiopathic hypercalcuria in kids? What is the tx?
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1. 4-8 y/o
2. increase fluids |
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Thin Basement Membrane Disease has a mutation at what gene?
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type IV collagen alpha 4
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Is thin basement membrane disease autosomal dominant or autosomal recessive?
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autosomal dominant
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Is thin basement membrane disease symptomatic or not? Is renal function impaired or not?
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no and no
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What mutations occur in early alport syndrome?
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type IV collagen
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T or F: early alport syndrome is x-linked.
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true
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What are the 2 key features in early alport syndrome?
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renal problems and hearing loss (sensorineural)
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What lab tests would you order when performing hematuria work-up for peds?
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cbc, ua, serum creatinine, urine Ca:Cr and protein:Cr ratios, serum C3, ultrasound if labs are normal
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Is acute post-strep GN nephrotic or nephritic?
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nephritic
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What are sx of acute post-strep GN?
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gross hematuria, edema, HTN & renal insufficiency
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What bacterial strains cause acute post-strep GN?
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s. pyogenes
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Will abx tx acute post-strep GN?
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no, but they will stop the spread of that strain
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What population is highest in acute post-strep GN?
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5-15 y/o males
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What are the clinical findings in acute post-strep GN?
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afebrile, 1-2 wk latency between pharyngitis and GN, edema, smoky/cola colored urine, improve after a week
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What are key lab findings in acute post-strep GN?
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low serum C3, hematuria, proteinuria, red cell casts, elevated ASO or DNase B
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Will an ASO titer be positive in skin infections that develop into acute post-strep GN?
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no, only in pharyngitis-derived
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What is the tx for acute post-strep GN?
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nothing specific: give abx if infection is present to prevent rheumatic fever, if HTN give CCB, steroids don't work
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What is the most common cause of gross hematuria in ages 10-30?
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IgA nephropathy
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What causes IgA nephropathy?
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idiopathic
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What are clinical findings of IgA nephropathy?
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recurrent painless gross hematuria, concurrent w/infection, hematuria lasts for days
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What are lab findings in IgA nephropathy?
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none specific-r/o acute post-strep GN, serum IgA won't help, biopsy if: HTN, renal impairment, or proteinuria >1g/day
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What is the tx for IgA nephropathy?
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nothing specific-ACEI for renal problem/proteinuria, omega-3 will slow progression to renal insufficiency, steroids for bad cases, immunosuppressives for crescenteric cases
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4 features of HSP nephritis?
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Purpuric rash
Abdominal pain Arthralgias Glomerulonephritis with IgA deposition |
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HSP nephritis looks like what other GN under microscope?
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IgA nephropathy
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Clinical findings in HSP nephritis?
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asymptomatic hematuria and mild proteinuria, normal Cr, renal disease in 8 wks, 70% resolve in 4 weeks
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What is tx for HSP nephritis?
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no specific tx: steroids for relief, 90% resolve w/o tx
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Thickening of glomerular basement membrane and hypercellularity describes which GN?
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membranoproliferative GN
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What causes thickening and hypercellularity in MPGN?
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thickening-immune deposits or mesangial cells cytoplasm in GBM; hypercellularity-WBC's and proliferation of mesangial cells
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Most common cause of chronic glolerulonephritis in older children & adults is what?
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membranoproliferative GN
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Of the 3 types of MPGN, which is most common? Which has worst prognosis?
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type I; type II
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What is the main way to separate MPGN from APSGN?
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APSGN is nephritic w/low C3 for 6-8 weeks; MPGN is usually nephrotic (30-50% of the time) w/low C3 indefinitely
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What is the key for diagnosing MPGN?
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renal biopsy
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Which MPGN has low C3 and low C4? Low C3 and normal C4?
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I and III; II
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What hematologic feature is present in 1/2 of MPGN?
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normocytic, normochromic, Coombs’ negative anemia
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What is tx for children w/idiopathic MPGN w/significant proteinuria?
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steroids 3-10 years
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T or F: kids w/MPGN and nephrotic syndrom have a good prognosis.
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false-1/2 will be in ESRD by 10 yrs
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T or F: RPGN is common in kids.
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false-rare
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What are 3 groups of crescenteric GN's in kids?
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immune-complex nephritis, pauci-immune disease, anti-GBM disease
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What is the most common group of crescenteric GN's?
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immune-complex nephritis
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What are the 4 immune-complex GN's?
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APSGN, Berger, MPGN, SLE
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What are the 3 pauci-immune diseases?
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Wegener’s, polyarteritis, Churg-Strauss
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What are the 2 anti-GBM diseases?
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Goodpasture, anti-GBM nephritis
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Anti-GBM disease is most common in what population?
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young men
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Anti-GBM disease is usually seen w/pulmonary hemorrhage. What is this?
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Goodpasture's syndrome
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What are some key features of anti-GBM disease?
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acute nephritis->renal failure in weeks, 90% have anti-GBM antibody, biopsy-crescentic nephritis w/IgG and C3 linear deposition on GBM
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What is mainstay of therapy of anti-GBM disease?
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plasmapheresis (only works for 8-14 weeks)
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ESRD will develop from anti-GBM disease if Cr is above what level?
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6
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What kids (rarely) get lupus nephritis?
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complement and IgA deficiency
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ANCA + GN's are rare in kids, but what are the 3 kinds?
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churg-strauss (asthma followed by vasculitis), wegener's granulomatosis(cANCA), and microscopic polyarteritis (pulmonary hemorrhage and elevated pANCA)
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What is the most common cause of ARF in previously healthy children?
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HUS
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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 describes what disease?
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HUS
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What is the progression seen in HUS?
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endothelial and glomerular injury->microangiopathic hemolytic anemia, thrombocytopenia, and renal failure
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What often preceeds HUS?
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diarrhea from O157:H7 e. coli (shigella-like toxin)
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What are clinical manifestations of HUS?
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GE, low grade fever, HUS 2-14 days after infection, anemia, thrombocytopenia, neuro involvement, bleeding from orifices, (1/2 need dialysis)
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What are lab findings in HUS?
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elevated Cr, hematuria, proteinuria, elevated WBC's
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What do you not give kids w/HUS? Why?
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anti-diarrheals or abx; prolongs shedding, bacteria can lyse and release toxins
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What is tx for HUS?
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supportive care, dialysis, 75% need tranfusion
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What % have normal renal function after HUS?
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60%
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