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if a fetus has a single kidney with a congenital malformation of the renal parenchyma that includes abnormal tissue (cartilage) and cysts, what is the risk of the parents having another child with the same disorder?
same as baseline risk- dysplastic kidney is a sporadic disease (not inherited)
what are other associations with ARPKD? ADPKD?
-hepatic cysts with congenital hepatic fibrosis
-HTN, hematuria, berry aneurism, hepatic cysts, mitral valve prolapse
medullary cystic kidney disease
-cysts in the medullary collecting ducts of the kidney
-parenchymal fibrosis results in shrunken kidneys and worsening renal failure
in pre-renal azotemia-
the BUN:Cr ratio is:
the FENa is:
urine osm is:
in post-renal azotemia, what happens in the early and late stage for-
the BUN:Cr ratio:
what is responsible for the change?
> 15, < 15
< 1%, > 2%
> 500, < 500
damage to the tubule
in acute tubular necrosis-
the BUN:Cr ratio is:
the FENa is:
urine osm is:
how long does it take tubular cells to regenerate?
2-3 weeks (they are stable cells)
what are the most common causes of acute interstitial nephritis?
drugs: NSAIDs, PCN, diuretics
what are causes of renal papillary necrosis?
-chronic analgesic abuse (NSAIDs or phenacetin)
-sickle cell trait or disease
-severe acute pyelonephritis
--also caused by interstitial nephritis
what are toxic causes of acute tubular necrosis?
-heavy metals (lead)
-urate (tumor lysis syndrome)
what is the definition of nephrotic syndrome?
proteinuria (> 3.5 g/day)
are people with nephrotic syndrome in a hyper- or hypo- coagulable state? why?
hyper, they lose antithrombin III
what is the lipid profile of someone with nephrotic syndrome?
hyperlipidemia and hypercholesterolemia
what nephrotic syndrome is associated with Hodgkin's lymphoma? how does this make sense?
MCD, the massive amount of cytokines produced damage the foot processes of the glomerulus
what proteins are lost in MCD?
albumin, not immunoglobulin
what is the treatment of MCD? how effective is it? why?
-damage is mediated by cytokines from T cells
a patient with Wilson's disease develops a nephrotic syndrome- which one is it?
membranous nephropathy (penicillamine is a common cause and used to treat Wilson's disease)
what causes the thickening of the membrane in membranous nephropathy? how would it appear on immunofluorescence?
-immune complex deposition
what are the differences between Type I and Type II membranoproliferative glomerulonephritis?
Type I: subendothelial deposits, Hep B and C association, more often tram tracks
Type II: within the basement membrane, C3 nephritic factor (Ab that stabilizes C3 convertase-> overactivation of compliment)
in membranous nephropathy, the deposits are where?
under the epithelium
describe the process of diabetes nephropathy
-efferent is preferentially affected by non-enzymatic glycosylation, leading to hyaline arteriolosclerosis, which results in narrowing of the lumen
-increased GFR leads to microalbuminuria and eventual sclerosis of the mesangium leading to nephrotic syndrome
Why do ACE inhibitors slow the progression of diabetes nephropathy?
-because they block the effects of angiotensin II, which preferentially squeezes down on the efferent arteriole
where does amyloid deposit in the glomerulus?
what defines whether or not a strep infection will cause a glomerular nephritis? what is the treatment?
-M protein virulence factor
what is the histological hallmark of post-strep glomerulonephritis on EM?
crescents in RPGN are comprised of:
fibrin and macrophages
what are possible causes of EM of RPGN that appears:
-immune complex: PSGN or diffuse proliferative glomerular nephritis (most common in SLE)
-Wegener, microscopic polyangiitis, Churg-Strauss
where are the complexes in diffuse proliferative glomerulonephritis?
how are the symptoms of Goodpasture syndrome different than Wegener granulomatosis?
Wegener has nasopharyngeal symptoms like sinusitis, which Goodpasture doesn't
what pauci-immune RPGNs have a p-ANCA? how can they be distinguished?
--Churg-Strauss has granulomatous inflammation, eosinophilia and asthma
-inherited defect in type IV collagen
-thinning and splitting of the basement membrane
-isolated hematuria, hearing loss, ocular disturbances
at what point is pulmonary vascular resistance the lowest? why?
-increased lung volumes stretch the pulmonary vessels, narrowing them
-decreased lung volumes have decreased radial traction
mucicarmine stain is used to identify:
smoking predisposes infants for:
-sudden infant death syndrome
what can cause a total white-out of one lung which shift of the trachea toward it?
collapsed lung due to bronchial obstruction
where is most filtered water reabsorbed in the nephron?
-proximal tubule (regardless of hydration status)
where does the blood supply of the following sections of the ureter come from:
-common iliac, internal iliac, vesical artery
how do you calculate filtration fraction?
GFR/RPF (creatinine clearance/PAH clearance)
-creatinine approximates GFR because it is filtered but not secreted
-PAH approximates renal flow because it is filtered and secreted
how do you calculate the clearance of something?
urine concentration x urine flow rate
why do patients initially get an increased creatinine when starting an ACE inhibitor?
because it preferentially dilates the efferent arteriole, leading to a reduced GFR
what are the two most important acid buffers in the urine?
HPO4 and NH3
eosinophilic casts in an old man with fatigue and constipation is suggestive of:
which ribs overly the spleen? kidneys? liver?
-9, 10, 11
-8, 9, 10, 11
what is an enlarged, hard left supraclavicular node suspicious for?
intra-abdominal cancer, usually GI
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