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Renal > 05.20 - Kidney, Systemic 2 (Nichols, Hastings) - PP + Handout > Flashcards

05.20 - Kidney, Systemic 2 (Nichols, Hastings) - PP + Handout Flashcards

1
Q

Most amyloiogenic proteins are made in

A

Hepatocytes or plasma cells

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1
Q

Median age at dx of AL amyloidosis

A

64

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1
Q

When to suspect Light Chain Disease

A

Nephrotic Syndrome or Rapidly progressive tubulointersitial nephritis, associated with cardiac diastolic dysfunction and monoclonal Ig in urine or serum

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2
Q

Eprodisate

A

Limits deposition of AA fibrils

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2
Q

What percent of patients with HgB SS will develop ESRD

A

4-12%

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3
Q

Presentation of HIV Nephropathy

A

Progressive Azotemia, Significant Proteinuria, Minimal peripheral edema

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3
Q

Glomular histology is sickle cell patients who present with chronic renal disease and proteinuria

A

Glomerulomegaly, Mesangial expanion, Segmental sclerosisi

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4
Q

AA amyloidosis is due to

A

Chronic inflammatory diseases such as RA, IBD, etc.

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5
Q

Light chain disease presents with

A

Proteinuria and Renal Failure

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5
Q

Later Stages of Sickle cell nephropathy

A

Interstitial inflammation, edema, fibrosis, tubular atrophy, papillary infarcts

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6
Q

In the kidney, mixed cryoglobulinemia most often causes what pattern of injury

A

Membranoproliferative

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7
Q

Tubular and insterstitial deposits of amyloid may lead to

A

Tubular Atrophy and Interstitial fibrosis

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9
Q

Interstitial fibrosis and Vascular Sclerosis with abundant brown pigment (Hb breakdown products)

A

Sickle Cell Nephropathy

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9
Q

Concentrating ability in Sickle Nephropathy

A

Diminished

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9
Q

Significant proteinuria but only minimal peripheral edema

A

HIV Nephropathy

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11
Q

Advanced renal amyloidosis results in what kind of glomerulopathy

A

non-proliferative, non-inflammatory

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11
Q

Kidney symptoms in AL amyloidosis

A

Nephrotic syndrome, Renal insufficiency w/ enlarged kidneys

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11
Q

Where in kidney does sickling usually occur

A

Vasa Recta

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12
Q

HIV Nephropathy Histology

A

Collapsing FSGS, with Microcystic Tubular Dilation, Intersitial inflammaiton and fibrosis

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13
Q

Susceptibility gene for HIV Nephropathy

A

APOL1

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14
Q

What distinguishes HIV-associated nephropathy from idiopathic collapsing FSGS

A

Accompanying prominent tubular dilation and interstitial inflammation

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14
Q

GFR in sickle cell

A

Increased until 4th decade, then decreased

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15
Q

Big kidneys on US

A

Diabetic Nephropathy, Amyloidosis

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16
Q

Ig capable of binding IgG

A

RF

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18
Q

Protein in AL amyloidosis

A

Ig light chain

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18
Q

Median survival of AL amyloidosis

A

10 months

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18
Q

Proteins in Cryoglobulinemia

A

Ig’s, Complement, RF

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19
Q

Most patients with AL just have

A

Monoclonal Gammopathy, instead of Multiple Myeloma

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20
Q

Question to ask with Collapsing FSGS

A

HIV Test?

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21
Q

AL amyloidosis can present with

A

Nephrotic syndrome, Restrictive CM, Diarrhea, and nonspecific

21
Q

Cryoglobulins can be polyclonal Ig’s produced by

A

Chronic Inflammation

22
Q

Presence of proteins in serum, which precipitate w/ cooling serum below normal body temp

A

Cryoglobulinemia

24
Q

Early stages of sickle cell nephropathy

A

Glomerular Hypertrophy, hemosiderin deposits, focal areas of hemorrhage and necrosis

25
Q

IF of Light Chain Disease

A

Positive in glomerular capillary loop, mesangium, and tubules

26
Q

Mixed Cryoglobulinemia is associated with ___ in a very high % of cases

A

Hep C

26
Q

Coinfection of HIV and Hep C

A

Mixed Cryoglobulinemia

27
Q

Characteristic LM appearance of Light Chain Disease in Glomerulus

A

Nodular glomerulosclerosis, w/ expansion of MM

28
Q

Sickle cell disease can cause __, although diabetes is much better known for it

A

Renal Papillary Infarcts

29
Q

Amyloidosis seen in long-term hemodialysis

A

Beta-2 Microglobulin

29
Q

Pattern of injury in Cryoglobulinemia

A

Membranoproliferative

30
Q

Cryoglobulinemia

A

Presence of proteins in serum, which precipitate w/ cooling serum below normal body temp

32
Q

AA amyloid is derived from

A

SAA protein

34
Q

Mixed Cryoglobulinemia most often produces what types of symptoms

A

Constitutional and Nonspecific

35
Q

End Stage of Sickle Cell Nephropathy

A

Glomerular Enlargement and FSGS

36
Q

Cryoglobulinemia can be asymptomatic or result in

A

Immune-complex-mediated GN or Vasculitis

38
Q

Limits deposition of AA fibrils

A

Eprodisate

39
Q

Tx of AL Amyloidosis

A

Melphalan and Dexamethasone

41
Q

Ages for Amyloidosis

A

Late middle age and older

42
Q

Triopathy

A

Retinopathy, Neuropathy, Retinopathy in Diabetes

44
Q

Gross appearance of advanced amyloidosis

A

Enlarged, pale organs with waxy texture

45
Q

How to distinguish Diabetic Retinopathy from Light Chain Disease

A

IF shows only lambda or kappa positivity in light chain disease

46
Q

What is necessary for amyloidosis

A

Extracellular Fibriloogenic Environment: GAG, serum amyloid P, laminin, Collagen IV, apo E

47
Q

Light Chain Disease glomerular LM strongly resembles

A

Diabetic Nephropathy

49
Q

Beta 2 Amyloid occurs with

A

long-term hemodilaysis

50
Q

Beta 2 microglobulin is a component of

A

MHC class 1 molecules

51
Q

B cell lymphoproliferative disorder characterized by deposition of immune complexes containing RF, IgG, HCV RNA, and complement on endothelial surfaces, eliciting vascular inflammation

A

Mixed Cryoglobulinemia

53
Q

Most common renal manifestation of AA amyloidosis

A

Nephrotic syndrome

55
Q

How does Sickle Cell Nephropathy most commonly manifest?

A

Proteinuria

56
Q

Hereditary Amyloidosis

A

Replacement of single AA –> Renders protein prone to fibril formation

57
Q

Pseudothrombi in capillary lumens in glomerulus

A

Cryoglobulinemia

59
Q

Monoclonal cryoglobulins typically produce signs and symptoms related to

A

Hyperviscosity and/or thrombosis

60
Q

Pathology of HIV Nephropathy

A

Collapsing Form of FSGS

62
Q

Most commonly dx type of amyloidosis

A

AL

63
Q

Main target organ of AA amyloidosis

A

Kidney

64
Q

__ proteins can precipitate in tubules where epithelial cells coalesce into a syncytium around them

A

Bence Jones

65
Q

Glomerulomegaly, Mesangial Expansion, Tubular epithelial hemosiderin accumulation, Interstitial fibrosis and vascular sclerosis

A

Sickle Cell Nephropathy

66
Q

___ is a unique feature of the collapsing FSGS associated with HIV

A

Microcystic Tubular Dilatation

68
Q

Light chain disease vs AL amyloid

A

deposits are congo red negative and have no fibrillar organization

69
Q

Tx of AA amyloidosis

A

Eprodisate

70
Q

Beta 2 amyloid tends to deposit

A

Synovium, joints, and tendon sheaths

71
Q

Membranoproliferative pattern of injury with deposition of immune complexes containing RF, IgG, Hep C virus RNA and complement factors on endothelial surfaces and in subendothelial and mesangial locations

A

Mixed Cryoglobulinemia

72
Q

HIV-associated nephropathy results from

A

Direct infection of renal epithelial cells in genetically susceptible host

73
Q

Podocyte proliferation

A

HIV Nephropathy

74
Q

Cryoglobulins can be monoclonal Ig’s produced by

A

Multiple Myeloma

Renal (31 decks)

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