4. Renal Path 2 Flashcards
(124 cards)
What manifests by hematuria with progression to chronic renal failure, accompanied by nerve deafness and various eye disorders, including lens dislocation, posterior cataracts and corneal dystrophy, XLINKED (85% cases)?
Alport syndrome
Alport syndrome is caused by a defect in collagen IV which is cruicial for function of the GBM, lens of the eye, and cochlea. What is the neumonic ALPORT?
A for alport LP is a record listen to = deafness O is for ocular defects R is for renal failure T thickening of BM and type IV collagen
Alport syndrome is a nephritic syndrome (hematuria), seen on EM as alternating thickening and thinning GBM w lamination of the lamina densa, foci of rarefaction- what type of appearance?
Basket weave appearance (moth eaten or frayed)
in Alport syndrome there may be absent alpha 3,4,5 collagen- which wont stain and thats how you know its alport. Sx appear between ages 5-20 and renal failure between?
20-50years
What is a fairly common hereditary entity manifested clinically by familial asymptomatic hematuria and morphillogically by diffuse THINNING of the GBM?
Thin basement membrane disease
in Thin basement membrane disease, renal function is normal and prognosis is excellent, due to mutation in a3/4 type IV collagen, most people are heterozygotes, if the person is homozygote for the mutation it results in?
alport like syndrome (a5 is present however so no ocurlar or auditory lesions!)
The follow are glomerular syndromes associated with systemic diseases- sceondary renal dz, do they present with nephrotic or nephritic syndrome? Diabetic Neuropathy SLE (15% patients) Hep C-cryoblobulinemia (MPGN type 1) HIV nephropathy (FSGS)
Nephrotic syndrome! = massive proteinuria
The follow are glomerular syndromes associated with systemic diseases- sceondary renal dz, do they present with nephrotic or nephritic syndrome?
SLE (60% patients)
Bacterial endocarditis (acute proliferative glomerulonephritis)
Goodpasture syndrome (RPGN)
Henoch Schonlein Purpura (HSP): IgA nephropathy
Acute nephritic syndrome = HEMATURIA
Hispanics are 1.5-2 times more likely to develop diabetes, which is the leading cause of end stage renal disease, blindness, LE amputations resulting from?
athersclerosis of the arteries
Normal blood glucose is 100mg/dL, diabetics have hyperglycemia, which accounts for most of the long term complications- which include, eyes, nerves, blood vessels, and the?
kidneys!
Metabolic syndrome also can cause kidney failure, which is characterized by HTN, elevated serum glucose, elevated lipid levels and excess abdominal fat which is v bad, what percent of diabetic kidney disease patients have end stage renal failure?
30%
40% of type 1/2 DM patients progress to ESRD, the two major key processes which lead to development of glomerular lesions in DM are metabolic defect linked to hyperglycemia (thickens GBM) and ?
hemodynamic effects assoicated with glomerular hypertrophy (contribute to glomerulosclerosis)
In diabetic nephropathy, you can see diffuse thickening of the GBM and the tubular basement membrane on LM, with deposition of?
glycoproteins
In diabetic nephropathy one can also see on EM diffuse mesangial sclerosis = increased mesangial matrix, along with nodular what on LM?
Nodular glomerulosclerosis (**characteristic aceullular PAS positive nodules)
Along with diabetic neuropathy, one can see advance renal hyaline arteriolosclerosis = markedly thickened, tortuous afferent arteriole with what type of vascular wall?
amorphous or thickened
in endstage diabetic nephrosclerosis, grossly one can see diffuse granular, pitted surface (pebble like), irregulae cortical depressions secondary to pyelonephritis, and marked thinning of the?
renal cortex (all due to DM and HTN)
In summary diabetic nephropathy has three lesions: glomerular lesions, vascular lesions (arteriolosclerosis) and pyelonephritis which is?
infection including necrotizing papillitis
With time diabetic microvascular disease becomes macrovascular causing MI, renal insufficiency, and what are the most common causes of mortality in long standing diabetics?
cerebrovascular accidents
SLE immune complex deposition subendothelial dense deposits, the renal glomerular capillary basement membrane with subendothelial dense deposits known as what can be seen, along with deposits in the mesangium on EM?
wire loop appearance* on light microscopy
In acute/diffuse proliferative lupus nephritis (pattern) you can see marked increase in cellularity, glomerular size greatly enlarged, and appears to be?
Stuffed into bowman’s capsule with resultant decrease in urinary space
What is the difference between diffuse proliverative GN (post infectious) and the proliferation seen in SLE?
Patient has SLE! in post infectious patients root cause is not due to SLE
SLE on IF with anti-IgG antibody, you can see mesangial and capillary wall (subendothelial) IgG localization what type of pattern is seen?
Granular because immune complex deposition not linear antibody
50% of SLE patients have significant renal involvement, what type is the most common and worst prognosis, characterized as being symptomatic with hematuria and proteinuria, HTN and mild to severe renal insufficiency progressing to scaring of the glomeruli?
Class IV - Diffues lupus nephritis
What is a childhood syndrome with skin lesions, abdominal pain, intestinal bleeding, arthralgias and renal abnormalities in 1/3 of the patients?
Henoch-Schonlein Purpura (systemic IgA disease)