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Renal 2 > Kidney Systemic Disease II > Flashcards

Kidney Systemic Disease II Flashcards

1
Q

sickle cell nephropathy - where in the kidney does sickling happen?

A

inner medulla

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2
Q

What 3 factors of the inner medulla make it a prime area (the prefect storm) to cause polymerization of deoxygenated Hgb S and sickling of erythrocytes?

A

1) relative hypoxia
2) acidosis
3) hyperosmolarity

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3
Q

what pathologic, structural functional change does sickle cell nephropathy cause over time in the kdiney?

A

considerable decrease in the cortical vasculature and the vasa recta are virtually absent

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4
Q

pt comes in with microalbuminuria, hyposthenuria (decrease of concentrating ability) - your differential could include nephrogenic DI and what else

A

sickle cell -

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5
Q

sickle cell - what key finding do you see in early childhood into young adulthood with regards to the kidney?
How does this manifest in clinical findings?

A

1) increased GFR in early childhood, young adulthood - hyperfiltration
2) you will see a lower creatinine than expected (in plasma)

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6
Q

sickle cell - what is an impt histo feature you see on biopsy

A

papillary infarcts / necrosis

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7
Q

sickle cell - what other pathological processes will be present seen on biopsy of the later stages

A

interstitial inflammation, edema, tubular atrophy and fibrosis

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8
Q

what does sickle cell nephropathy common progress to as end stage

A

ESRD – FSGS w/ glomerular enlargement

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9
Q

sickle cell - if he has a picture of a LM kidney biopsy slide, what general things would you see

A

a lot of red blood cell occlusions – due to all the sickling, you will see a lot of red, gumming up the glomeruli and peritubular capillaries

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10
Q

glomerulomegaly and mesangial expansion; tubular epithelial hemosiderin acumulation; interstitial fibrosis and vascular sclerosis

A

sickle cell nephropathy

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11
Q

amyloidosis - misfolded proteins - what do they fold into

A

beta pleated sheets

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12
Q

amyloidosis - where do deposits get put first

A

blood vessel walls

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13
Q

amyloidosis - what causes organ failure

A

deposits get into the interstitium, stuff squeezes the cells to death

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14
Q

AL (primary) amyloid - what is the abnormal protein

A

immunoglobulin light chain (usually lambda)

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15
Q

AL amyloid - what is a feature of the disease underlying

A

monoclonal *** gammopathy

I think this will be on test

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16
Q

what do we call light chains that get into the urine

A

Bence Jones proteins

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17
Q

pt comes in with either Rheumatoid Arthritis or Inflammatory Bowel Disease - what are you thinking (amyloid)

A

AA - secondary reactive amyloid

RA and IBD - 2 most common systemic diseases giving rise to this reactive type amyloid

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18
Q

AA - secondary amyloidosis - what is the cause in general

A

a systemic chronic inflammatory disease

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19
Q

what molecule needs to be present in order to have amyloidosis formation (i.e. fibril formation)

A

AEF - amyloid enhancing factor

without this, will not have amyloidosis - just having circulating amyloid is not enough

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20
Q

amyloid kidney - what kind of glomerulopathy is seen? what will you see on urinalysis

A

1) nonproliferative, noninflammatory

2) proteinuria, NOT hematuria

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21
Q

w/ renal amyloidosis, what is the size of the kidneys

A

enlarged

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22
Q

what are the 2 pathological processes in the kidneys that are end result of amyloidosis (bad news bears)

A

tubular atrophy and interstitial fibrosis

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23
Q

what stain do you use for amyloidosis?

A

congo red

24
Q

what is a setback with congo red ?

A

can get false negatives
does not always appear very red
(can look tan, peachy, salmon, some may even say taupe)

25
Q

congo red can never tell you what type of amyloid is present. what test can?

A

immunoperoxidase stain

26
Q

what is a setback of immunoperoxidase stain

A

false positives

27
Q

do you see HTN w/ AL amyloidosis

A

no

28
Q

note that amyloidosis AL is associated with GI symptoms

A

GI dysmotility, malabsorption, hemorrhage or obstruction

29
Q

what is the treatment for AL? what is the prognosis?

A

1) melphalan and dexamethasone (if pt younger than 50, can do autologous stem cell transplant)
2) shitty - median survival 10 months

30
Q

what is a third cause of AA

A

ankylosing spondlyitis

31
Q

what abnormal protein si involved with AA amyloid?

what is it produced in response to?

A

1) serum amyloid A protein

2) acute phase reactant produced by liver in response to IL-1 and IL-6

32
Q

what is a new treatment option for AA

A

eprodisate - limits deposition of amyloid A fibrils by interfering w/ their interaction w/ tissue glycosaminoglycans

33
Q

what is most common cause of death in AA

A

death usually from infection or dialysis related complication - b/c most AA pts end up on dialysis in end

34
Q

what type of amyloidosis occurs with long term hemodialysis

A

Amyloid beta2m

this is beta 2 microglobulin

35
Q

where does amyloid beta2m prefer to deposit

A

synovium, joints, and tendon sheaths

this is unique and he kind of emphasized it

36
Q

amyloidosis type that presents with heart failure and arrhythmia

A

amyloid TTR - transthyretin

37
Q

what is the function of transthyretin

A

transporter protein for thyroxine and retinol (notice how the name “transthyretin” actually describes its function)

38
Q

what is another name for light chain deposition disease

A

nonamyloid monoclonal immunoglobulin deposition disease (MIDD)

39
Q

light chain deposition disease (MIDD) - what underlying disease

A

multiple myeloma

40
Q

sicca syndrome

1) what is it?
2) if you see this in a question stem, what disease are you thinking?

A

1) dry eyes, dry mouth

2) light chain deposition disease (MIDD)

41
Q

for light chain disease, what other disease does the histo resemble?

A

diabetic nephropathy
nodular glomerulosclerosis
expansion of mesangial matrix

42
Q

what is unique about the protein assocaited with light chain disease

A

kappa is more common than lambda (diff from AL)

43
Q

light chain disease on EM - where does it deposit and what is the buzz word for what it looks like

A

1) endothelial cell side (subendothelial)

2) silt

44
Q

if you see the combination of “heavy proteinuria” and “minimal swelling” what are you thinking

A

HIV associated nephropathy (HIVAN)

45
Q

HIVAN - what is the classical glomerulopathy

A

collapsing form of focal segmental glomerulosclerosis

46
Q

what is the buzz word finding on histo for HIVAN

A

microcystic tubular dilatation

47
Q

what is a key susceptibility allele associated with HIVAN

A

APOL1

48
Q

palpable purpura, arthralgia, elevated serum RF in a Hep C patient - what is the diagnosis

A

mixed cryoglobulinemia type II

49
Q

what is the pathogenesis of cryoglobulinemia

A

deposition of immune complexes on endothelial surface, activation of complement and eliciting vascular inflammation

50
Q

what will you see on urinalysis of cryo? (think inflammation)

A

hematuria

51
Q

cryo - what kind of disease picture of the glomerulus seen on renal biopsy

A

MPGN

52
Q

purpura, arthralgia, and weakness - what is this and what disease is it associated with

A

Meltzer’s triad - associated with cryo

53
Q

what kind of immunoglobulin is RF

A

IgM – binds IgG

54
Q

what is seen on EM with amyloidosis

A

randomly oriented fibrils

55
Q

what is a key diagnostic feature of light chain disease? (recall, it is not true amyloidosis)

A

congo red negative

56
Q

cryo - what is contained in the immune complexes

A

RF, IgG, HCV RNA, and complement

Renal 2 (11 decks)

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