4. Renal Path 2 Flashcards

1
Q

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)?

A

Alport syndrome

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2
Q

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
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
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3
Q

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?

A

Basket weave appearance (moth eaten or frayed)

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4
Q

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?

A

20-50years

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5
Q

What is a fairly common hereditary entity manifested clinically by familial asymptomatic hematuria and morphillogically by diffuse THINNING of the GBM?

A

Thin basement membrane disease

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6
Q

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?

A

alport like syndrome (a5 is present however so no ocurlar or auditory lesions!)

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7
Q
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)
A

Nephrotic syndrome! = massive proteinuria

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8
Q

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

A

Acute nephritic syndrome = HEMATURIA

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9
Q

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?

A

athersclerosis of the arteries

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10
Q

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?

A

kidneys!

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11
Q

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?

A

30%

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12
Q

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 ?

A

hemodynamic effects assoicated with glomerular hypertrophy (contribute to glomerulosclerosis)

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13
Q

In diabetic nephropathy, you can see diffuse thickening of the GBM and the tubular basement membrane on LM, with deposition of?

A

glycoproteins

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14
Q

In diabetic nephropathy one can also see on EM diffuse mesangial sclerosis = increased mesangial matrix, along with nodular what on LM?

A

Nodular glomerulosclerosis (**characteristic aceullular PAS positive nodules)

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15
Q

Along with diabetic neuropathy, one can see advance renal hyaline arteriolosclerosis = markedly thickened, tortuous afferent arteriole with what type of vascular wall?

A

amorphous or thickened

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16
Q

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?

A

renal cortex (all due to DM and HTN)

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17
Q

In summary diabetic nephropathy has three lesions: glomerular lesions, vascular lesions (arteriolosclerosis) and pyelonephritis which is?

A

infection including necrotizing papillitis

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18
Q

With time diabetic microvascular disease becomes macrovascular causing MI, renal insufficiency, and what are the most common causes of mortality in long standing diabetics?

A

cerebrovascular accidents

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19
Q

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?

A

wire loop appearance* on light microscopy

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20
Q

In acute/diffuse proliferative lupus nephritis (pattern) you can see marked increase in cellularity, glomerular size greatly enlarged, and appears to be?

A

Stuffed into bowman’s capsule with resultant decrease in urinary space

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21
Q

What is the difference between diffuse proliverative GN (post infectious) and the proliferation seen in SLE?

A

Patient has SLE! in post infectious patients root cause is not due to SLE

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22
Q

SLE on IF with anti-IgG antibody, you can see mesangial and capillary wall (subendothelial) IgG localization what type of pattern is seen?

A

Granular because immune complex deposition not linear antibody

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23
Q

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?

A

Class IV - Diffues lupus nephritis

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24
Q

What is a childhood syndrome with skin lesions, abdominal pain, intestinal bleeding, arthralgias and renal abnormalities in 1/3 of the patients?

A

Henoch-Schonlein Purpura (systemic IgA disease)

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25
Q

Henoch-Schonlein Purpura presents with hematuria, nephritic/nephrotic syndrome, associated with atopy and URI, HSP is systemic while bergers is?

A

localized to the kidney (IgA nephropathy)

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26
Q

Henoch-Schonlein Purpura has renal leasions that vary, diagnosis is made on IF by IgA depositied in the renal mesangium along with what sometimes?2

A

C3
IgG
*sometimes deposits extending into capillary loops

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27
Q

HSP patients present with what on the extensor surfaces of arms and legs and buttocks, which contain supepidermal hemorrhages and necrotizing vasculitis in the small vessels of the dermis?

A

Pruritic skin rashes

onset 3-8 years old - excellent prognosis

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28
Q

Glomerulonephritis associated with bacterial endocarditis particularly occurs with rheumatic fever, endocarditis and infected AV shunts. Circulating antigens cause immune deposition and nephritis ranging from mild to full blown RPGN and in chronic cases can have what pattern?

A

mebranoproliferative glomerulonephritis

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29
Q

Goodpasture, microscopic polyangiitis and granulomatosis with polyangiitis have glomerular lesions that are similar and characterized by foci of glomerular necrosis and?

A

crescent formation

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30
Q

What is a systemic condition in which deposits of cryoglobulins composed of IgG-IgM complexes induce cutaneous vasculitis, synovitis and proliferative GN (MPGN)?

A

Essential mixed cryoglobulinemia

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31
Q

What is a clinicopathologic entity chracterized clinically by acute renal failure and morphologically by damage to tubular epithelial cells in the form of necrosis?

A

Acute tubular injury (ATI)

*MCC of acute renal failure

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32
Q

Acute tubular injury (ATI) is commonly due to ischemia, due to decreased or interrupted blood flow such as due to hypotension and shock, are in diffuse involvement of intrarenal BVs in conditions such as malignant hypertension, TTP, DIC, or?

A

HUS

```
TTP= thrombotic thrombocytopenia purpura
DIC= disseminated intravascular coagulation
~~~

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33
Q

Acute tubular injury (ATI) can also be due to direct toxic injury to the tubules by endogenous or exogenous agents such as?

A

certain drugs or toxins

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34
Q

Tubular injury due to toxic injury or ischemia results in tubular backleak, sloughed cells, and interstitial inflammation, leading to obstruction, and what two main endpoints?

A

Decreased GFR and Decrease urine output

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35
Q

the critical evens in both ischemic and nephrotoxic ATI are believed to be tubular injury and persistent and severe disturbances in ?

A

blood flow

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36
Q

Tubular epithelial cells have high metabolic demand (massive resorption and Na/K ATPase use), ischmia/poisoned cells lose polarity and transfer from basolateral to luminal side causing?

A

abnormal ion transport = increased distal sodium delivery = vasconstriction and tubuloglomerular feedback

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37
Q

Ischemic tubular cells detach from the BM, and obstruct the lumen causing decrease GFR, disturbance of BF causes vasconstriction can also occur - reducing GFR and causing reduced?

A

O2 delivery to the functionally important tubules in the outer medulla (TAL/ straight seg of PT)

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38
Q

the tubular damage in acute tubular injury is different for ischemia and toxicity. In ischemia one can see patchy necrosis in the PCT, PST, and ascending loop of henle straight segments. What is seen in toxic tubular damage?

A

Continuous damage in the PCT, PST, and patchy damage in the ascending loop henle

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39
Q

Occlusion of tubular lumens by casts is common, usually occuring in the DCT and the collecting duct, which contain what protein, which is a urinary glycoprotein normally secreted by the cells of the ascending thick limb and distal tubules?

A

Tamm-Horsfall protein (in the casts)

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40
Q

Proximal tubule cells have a high energy demand and are more susceptible to ischemia, inorder for tubular epithelial cells to regenerate after injury (reversible) what must be intact?

A

the basement membrane

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41
Q

If there is toxic poisoning with ethylene glycol, ballooning and hydropic or what type of degeneration of the PCTs can be seen?

A

vacuolar degeneration

all PCT cells swell and have vacuolization

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42
Q

What type of aminoglycoside causes acut renal failure in 15-20% of patients, which are reserved for life threatening gram negative sepsis?

A

Gentamycin

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43
Q

The clinical course of ATI is highly variable but is divided into three stages. Initiation phase lasts about 36hours in which the nephrotoxic insult has not yet caused acute renal failure, what can be seen?

A

Oliguria

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44
Q

The second stage of ATI is what, which is charcterized oliguric crisis with evidence of uremia, and hyper*kalemia ?

A

Maintenance Phase

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45
Q

The third stage of ATI is recovery (depending on the duration and nature of the precipitating event) can see large urine volumes (3L/day), large loss of water Na/K, susceptibility to infection and what?

A

hypokalemia (can cause arrhythmias and muscle issues)

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46
Q

What is a group of renal disease that involve inflammatory injuries of the tubules and interstitium that are often insidious in onset and are principally manifested by azotemia and inability to concentrate urine (polyuria)?

A

Tubulointerstitial nephritis

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47
Q

There are many causes of Tubulointerstitial nephritis including infection (acute/chronic at vesicourethra reflux), toxins, metabolic dz, physical factors, neoplasms, immunologic reactions and?

A

vascular diseases

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48
Q

Tubulointerstitial nephritis can be acute and chronic, acute has a rapid clinical onset, interstitial edema, WBC infiltration of the interstitium and tubules. In chronic there is infiltration with mononuclear WBCm interstitial fibrosis and?

A

lots of tubular atrophy

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49
Q

Tubulointerstitial nephritis is distinguished from glomerular disease via the presence of defects in tubular function and the absence of?

A

Nephrotic/Nephritic Syndrome!!

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50
Q

What is the most common diseases of the kidney and is defined as inflammation affecting the renal tubules, interstitium, and renal pelvis?

A

Pyelonephritis

51
Q

Cystitis is inflammation (bacterial) of the urinary bladder mucosa, acute pyelnoephritis is due to acute bacterial infection of the kidney and chronic pyelonephritis- reccurent infection with resultant damage to , what ? commonly associated with vesicoureteral reflux

A

damage to pelvis/calyceal system and parenchyma

52
Q

85% of urinary tract infections are caused by what type of bacteria, including E coli. or less commonly: proteus, klebsiella, enterobacter?

A

Gram negative bacilli

53
Q

Pyelonephritis occurs 95% of the time due to ascending infection from the bladder, commonly associated with vesicoureteral or intrarenal reflux, some predisposing conditions include? 3

A

FEMALE* shorter urethra
Diabetes
Pregnancy

54
Q

One mechanism how microbes move from the bladder to the kidneys is urinary obstruction which is more common in males older than 60 with prostate hypertrophy, which causes stasis allowing?

A

the unhindered multiplication of bacteria

55
Q

An incompetent vesicoureteral orifice allows reflux of urine up the to the kidney, usually due to a congential absence or shortening of the intravesical portion of?

A

the ureter (ureter is not compressed during micturition)

56
Q

What occurs in the upper and lower poles of the kidney where papillae have flattened or concave tips rather than the convex pointed type?

A

Intrarenal reflux

57
Q

Instrumentation of the urinary tract (catheters) allow the entrance of bacteria into the bladder, as well as what condition which would be poorly uncontrolled?

A

Diabetes

58
Q

Acute pyelonephritis is almost always the result of ascending cystitis infection combined with a predisposing?

A

anatomical defect (VUR)

59
Q

The most common cause of the reflux is congential, due to partial or complete lack of what?

A

lack of oblique entry/angle of the intravesical portion of the ureter

60
Q

What type of pyelonephritis has a hallmark of patchy interstitial suppurative inflammation (focal abscesses or large wedge like areas), intratubular WBC aggregates (WBC casts on urinalysis) and tubular necrosis?

A

Acute pyelonephritis (upper and lower lobes more)

61
Q

Acute pyelonephritis is glomerular sparing and grossly can see cortical surgace with multiple foci of yellow-grey areas of acute inflammation and abscess formation with mainly what type of infiltrate?

A

PMNs = NEUTROPHILS

62
Q

There are three complications seen with acute pyelonephritis including papillary necrosis, pyonephrosis, and perinephric?

A

abscess

63
Q

Papillary necrosis is seen in diabetics, sickle cell disease, obstruction and analgesic nephropathy, typically bilateral, with the distal pyramid being affected looking grey - white, pap necrosis with acute pyelonephritis =?

A

acute renal failure

64
Q

What occurs when there is total obstruction high in the urinary tract, not allowing the suppurative exudate to to drain thus filling the renal pelvis, calyces and ureter w pus?

A

Pyonephrosis

65
Q

After the acute phase of pyelonephritis, healing occurs via macrophages and fibrosis, causing a pyleonephritic scar which is almost always associated with inflammation, fibrosis and deformation of the?

A

underlying calyx and pelvis

66
Q

In analgesic nephropathy you can see red-brown necrotic papillae sloughed into calyces but in diabetes mellitus how does it look?

A

pale grayish necrosis limited to papillae

67
Q

Acute pyelonephritis presents with costovertebral angle pain and fever and malaise, indications of vladder and urethral irritation such as dysuria, frequency and urgency and the urine may contain?

A

leukocytes (pyuria) - doesnt indicate renal involvement

if leukocyte casts - neutrophil pus casts- you can think renal involvement

68
Q

What is a common viral pathogen causing pyelonephritis in kidney allografts, seen as large nuclear inclusions on LM/EM?

A

Polyomavirus

69
Q

Chronic pyelonephritis is a chronic tubulointerstitial inflammation and renal scarring associated with infection/anatomic deeformation/scarring of pelvis and calyces. *NOTE: Chronic pyelonephritis and analgesic nephropathy are the major entities that affect and damage the?

A

CALYCES

70
Q

VUR with Chronic pyelonephritis frequently results in polar scarring of the kidney (top and bottom) and the major predisposing factors are chronic obstructive pyelonephritis and ?

A

reflux neuropathy

71
Q

What is the most common pyelonephritic scarring occuring in early childhood from superimposition of UTI on congenital vesicouretreal reflux and intrarenal reflux?

A

Reflux neuropathy

may be uni or bilateral

72
Q

Chronic obstructive pyelonephritis prediposes to infection, leading to scarring and Chronic pyelonephritis, usually due to a defective what, which is bilateral?

A

defective posterior urethral valve

73
Q

The hallmarks of Chronic pyelonephritis are coarse, discrete, corticomedullary scars overlying dilated, blunted or deformed calyces and flattening of?

A

the papillae (more common in upper and lower lobes)

74
Q

What can be see on LM showing corticomedullary renal scar with an underlying dilated deformed calyx, with what of the tubules in the cortex?

A

Thyroidization (thyroid colloid)

75
Q

What type of pyelonephritis is a rare chronic form that is characterized by foam cells mixed with plasma cells, giant cells, other WBCs, often due to proteus infection, producing large yellow nodules that look like RCC?

A

Xanthogranulomatous pyelonephritis

76
Q

What is the second most common cause of acute kidney injury (after pyelonephritis)?

A

drug and toxin-induced tubulointerstitial nephritis

77
Q

What is an immune mediated reaction (type 1/4 hypersensitivity) to a variable number of drugs resulting in tubulitis and acute renal failure?

A

Acute (drug induced) tubulointerstitial nephritis

78
Q

Acute (drug induced) tubulointerstitial nephritis is not dose related but related to an immune mechanism where the drug acts as a hapten and activate IgE or?

A

T/B/Plasma cells in the local tubule

79
Q

Acute (drug induced) tubulointerstitial nephritis begins 15 days after drug exposure and one can see fever, eosinophilia, a rash in 25% of patient and interstitial renal?

A

parenchymal infiltrates

80
Q

Renal abnormalities are seen with Acute (drug induced) tubulointerstitial nephritis, including hematuria, proteinuria and?

A

leukocyturia (eosinophils*)

81
Q

in Acute (drug induced) tubulointerstitial nephritis, cessation of the drug and therapy for infection usually stabilzes renal function and improve. What are patients with analgesic nephropathy (d/t phenacetin) more likely to develop?

A

Urothelial carcinoma of the renal pelvis

82
Q

Acute (drug induced) tubulointerstitial nephritis may be complicated by what, which are excreted and may cause gross hematuria or renal colic due to ureteric obstruction?

A

papillary necrosis - usually at the tip / lowest part of the renal pyramid (also seen in DM, analgesia nephropathy, sickle cell trait)

83
Q

Other tubulointerstitial disease such as urate nephropathy occur in patients with hyperuricemic disorders such as gout. What type of nephropathy is d/t precip of uric acid crystals in collecting ducts, obstructing the neprhons and causing ARF, mostly happening in pts with leukemias and lymphomas that get?

A

chemotherapy

84
Q

what type of nephropathy occurs in patients with hyperuricemia, crystals develop in the distal tubules/ collecting ducts forming distinct birefringent needle like crystals?

A

Chronic urate nephropathy (gouty nephropathy)

85
Q

Disorders that involve hypercalcemia can lead to formation of Ca stones and deposition of Ca in the kidney which chronically can lead to tubulointerstitial disease, is characteristic of what?

A

Hypercalcemia and Nephrocalcinosis

86
Q

What nephropathy occurs by accumulation of phosphate in patients that consume oral phosphate solutions in prep for colonoscopy, causing renal insufficiency?

A

Acute phosphate nephropathy

87
Q

Light chain cast nephropathy or myeloma kidney (tubulointerstitial lesions)- has many different types that all end up with proteinuria due to proteins such as bence jones proteins (light chain), non light chain proteinuria sugges what disease?

A

Amyloidosis AL type or light chain deposition disease

88
Q

The most common myeloma kidney clinical features are chronic kidney disease that develops slowly and progresses slowly over months to years, with precipitating factors including dehydration, hypercalcemia, acute infection and tx with ?

A

nephrotoxic antibiotics

bence jones proteinuria presents in 70% of ppl with multiple myeloma

89
Q

What is a hepatorenal syndrome refering to impairment of renal function in patients with acute or chronic liver disease with advanced liver failure, serum bilirubin levels are elevated in jaundiced patients causing bile cast formation?

A

Bile cast nephropathy

90
Q

What is the term used for the renal pathology associated with sclerosis of renal arterioles and small arteries and is strongly associated with hypertension, which can be a cause and a consequence of the term?

A

Nephrosclerosis

91
Q

two processes participate in the arterial lesions inlcuding medial and intimal thickening and hyalinization of?

A

arteriolar walls

92
Q

in Nephrosclerosis, the kidneys are either normal or moderately reduced in size, the cortical surfaces have a fine even granularity that resembles grain leather, the loss of mass is due mainly to?

A

cortical scarring and shrinking

93
Q

in Nephrosclerosis histologically one can see narrowing of lumens and hyaline arteriolosclerosis. There is also patchy ischemic atrophy consisting of tubular atrophy and intersitial fibrosis along with a avariety of ?

A

glomerular alterations

*note: benign nephrosclerosis is a general process not a diagnosis

94
Q

How can you tell the difference between benign and malignant nephrosclerosis?

A

benign with have hyalinosis and intimal thickening while malignant will have onion skinning/concentric lamellations/ hyperplastic change

95
Q

benign nephrosclerosis is caused by increasing age, hypertension and diabetes associated with medial and intimal thickening and?

A

hyaline protein deposition in arteriolar walls

96
Q

clinically, benign nephrosclerosis is not associated with renal insufficiency except in african americans, in severe hypertension or in?

A

diabetic nephropathy

97
Q

What is a renal vascular disorder with atypical changes associated with accelerated hypertension due to vascular damage to the kidneys?

A

Malignant nephrosclerosis

98
Q

in Malignant nephrosclerosis, extreme blood pressure causes endothelial cell damage, increased perm to fibrinogen and plasma porteins, focal death of cells of the vascular wall and platelet activation lead to ?

A

fibrinoid necrosis of arterioles (and intravascular thrombosis)

99
Q

Malignant nephrosclerosis causes ischemic kidneys and elevated renin with a self perpetuating cycle of damage and hypertension leading to?

A

malignant arteriosclerosis

100
Q

Grossly one can seen instead of a grain leather look, can see petechial hemorrhage on the cortical surface also know as a ?

A

flea bitten appearance

101
Q

CLinical feathers of malignant hypertension/ Malignant nephrosclerosis is a BP of over 180/120, renal failure, cardiovascular abnormalities, encephalopathy and what two things in the eyes?

A

papilledema
retinal hemorrhages
(symptoms early due to increase intracranial pressure)

102
Q

The importance of renal artery stenosis is clinically patients often resemble those with essential HTN, but this is a potentially?

A

curable form of hypertension

103
Q

Hypertension secondary to what is caused by increased production of renin from the ischemic kidney?

A

renal artery stenosis

104
Q

renal artery stenosis is uncommon but when it does occur- occurs in old diabetic men, results from atheromas that lead to a decreased renal blood flow, and elaboration of ANGII via ?

A

RAAS- elevated serum renin d/t ischemic kidney

use ACE inhibitor to tx!!

105
Q

What is the second most common cause of renal artery stenosis, which is characterized by fibrous or fibromuscular thickening (intimal/medial/adventitial), more common in women and younger (30-40)?

A

fibromuscular dysplasia (*String of beads)

106
Q

What term encompases clinical syndromes that include thrombotic thrombocytopenia purpura (TTP) and hemolytic-uremic syndrome (HUS) which are caused by diverse insults that lead to excessive activation of platelets, which deposit as thrombi in capillaries and arterioles in various tissue beds, including the kidneys?

A

Thrombotic microangiopathy

107
Q

Consumption of platelets that deposit in the kidney also leads to thrombocytopenia (lack of platelets) that can shear the RBCs and lead to microangiopathic hemolytic?

A

anemia

see schistocytes=fragmented RBC

108
Q

HUS does not do plasmapheresis while TTP does, there are two major triggers including enothelial cell injury and activation (HUS) and what in TTP?

A

TTP= excessive platelet aggregation

109
Q

There are typical endothelial injury triggers including bacterial toxins, cytokines, anti-endothelial antibodies, certain medications and?

A

viruses

110
Q

For typical HUS the trigger is shiga toxin (seen with diarrhea, children), while atypical HUS (inherited forms) the trigger is excessive inappropriate activation of?

A

the complement

111
Q

Typical HUS is epidemic and diarrheal while atypical is not, because it is an inherited mutation of proteins that regulate complement, also seen in acquired cases of endothelial injury in adults like chemo, and other causes like?

A

antiphospholipid syndrome, preg associated, sclerosis

112
Q

Thrombotic thrombocytopenic purpura TTP is often associated with inherited or acquired deificiencies of what, which is a protease that regulates the function of von Willebrand Factor (vWF) via inhibitory autoantibodies?

A

ADAMTS13 = excessive platelet aggregation

**neurologic involvement prominent

113
Q

In hus the main mechanism is endothelial injury..• The injury to the endothelium causes there to be platelet aggregation and thrombosis within microvascular beds
• Reduced endothelial production of PGI2 (prostacyclin) and NO –> platelet aggregation
• increased production of endothelin =

A

vasoconstriction (exacerbates hypoperfusion)

114
Q

Typical HUS is classic, seen in childhood, epidemic and diarrhea positive, E coli in hamburgers (O157:H7) containing shiga like toxin also raw milk and person to person, the toxin causes increase in adhesion molecules of epithelium and decreased?

A

NO synthesis

115
Q

Typical HUS is most common in kids/elderly sudden onset of hematemesis/melena, oliguria/hematuria/hemolytic anemia which is managed by dilaysis which acutely resolves but long term has a?

A

poor prognosis

116
Q

Atypical HUS is diarrhea negative and more common in adults, has a worse prognosis than typical HUS since the underlying cause is chronic, neuro signs and symptoms, what is the most common inhertied mutation of complement regulatory proteins?

A

Factor H

117
Q

Thrombotic thrombocytopenic purpura TTP has a pentad of clinical features including:
1. fever
2. neurologic symptoms ***most dominante
3. Microangiopathic hemolytic anemia
4. thrombocytopenia
5. ?
is it more common in M/F , older/younger adults?

A

Renal failure in 50% pts

usually adults less than 40, FEMALES

118
Q

What renal disease occurs in adults due to bilateral renal artery stneosis causing renal ischemia, HTN may be absent, ANGII is usually elevated, avoid ACE/ARB, surgery needed?

A

Atherosclerotic ischemic renal disease

119
Q

What renal disease occurs from emboli from atheromatous plaques proximal to the renal artery containing cholesterol crystals that appear as RHOMBOID clefts, causes no problems in healthy kidneys but infarcts lead to acute renal failure in diseased kidneys?

A

Atheroembolic renal disease

120
Q
The following are some signs and symptoms of Atheroembolic renal disease: 
flank/abdominal pain
hematuria
arterial HTN
arrhythmia
nausea/vomiting
and ?
A

oliguria/anuria

121
Q

What is seen in both the disease and trait, and is seen as sickling in the vasa recta decreases the concentrating ability and increases thrombosis?

A

Sick Cell Nephropathy

122
Q

Sick Cell Nephropathy usually presents with hematuria and hyposthenuria (diminished concentration ability*), seen with cortical scarring and patchy papillary necrosis, with 30% exhibiting what?

A

proteinuria (sometimes with progressive glomerulosclerosis)

123
Q

What is an uncommon condtion that occurs most frequently after obstetric emergency (abruptio placentae), spetic shock and extensive surguery, leading to systemic hypoperfusion or hypoxia?

A

Diffuse cortical necrosis

124
Q

in Diffuse cortical necrosis, ischemia and coagulative necrosis is limited to the cortex with white (pale) patchy infarcts, angiography shows vascular disruption, and coagulative necrosis of both tubules and?

A

glomeruli