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

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Flashcards in 05.20 - Kidney, Systemic 2 (Nichols, Hastings) - PP + Handout Deck (74):
1

Most amyloiogenic proteins are made in

Hepatocytes or plasma cells

1

Median age at dx of AL amyloidosis

64

1

When to suspect Light Chain Disease

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

2

Eprodisate

Limits deposition of AA fibrils

2

What percent of patients with HgB SS will develop ESRD

4-12%

3

Presentation of HIV Nephropathy

Progressive Azotemia, Significant Proteinuria, Minimal peripheral edema

3

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

Glomerulomegaly, Mesangial expanion, Segmental sclerosisi

4

AA amyloidosis is due to

Chronic inflammatory diseases such as RA, IBD, etc.

5

Light chain disease presents with

Proteinuria and Renal Failure

5

Later Stages of Sickle cell nephropathy

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

6

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

Membranoproliferative

7

Tubular and insterstitial deposits of amyloid may lead to

Tubular Atrophy and Interstitial fibrosis

9

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

Sickle Cell Nephropathy

9

Concentrating ability in Sickle Nephropathy

Diminished

9

Significant proteinuria but only minimal peripheral edema

HIV Nephropathy

11

Advanced renal amyloidosis results in what kind of glomerulopathy

non-proliferative, non-inflammatory

11

Kidney symptoms in AL amyloidosis

Nephrotic syndrome, Renal insufficiency w/ enlarged kidneys

11

Where in kidney does sickling usually occur

Vasa Recta

12

HIV Nephropathy Histology

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

13

Susceptibility gene for HIV Nephropathy

APOL1

14

What distinguishes HIV-associated nephropathy from idiopathic collapsing FSGS

Accompanying prominent tubular dilation and interstitial inflammation

14

GFR in sickle cell

Increased until 4th decade, then decreased

15

Big kidneys on US

Diabetic Nephropathy, Amyloidosis

16

Ig capable of binding IgG

RF

18

Protein in AL amyloidosis

Ig light chain

18

Median survival of AL amyloidosis

10 months

18

Proteins in Cryoglobulinemia

Ig's, Complement, RF

19

Most patients with AL just have

Monoclonal Gammopathy, instead of Multiple Myeloma

20

Question to ask with Collapsing FSGS

HIV Test?

21

AL amyloidosis can present with

Nephrotic syndrome, Restrictive CM, Diarrhea, and nonspecific

21

Cryoglobulins can be polyclonal Ig's produced by

Chronic Inflammation

22

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

Cryoglobulinemia

24

Early stages of sickle cell nephropathy

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

25

IF of Light Chain Disease

Positive in glomerular capillary loop, mesangium, and tubules

26

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

Hep C

26

Coinfection of HIV and Hep C

Mixed Cryoglobulinemia

27

Characteristic LM appearance of Light Chain Disease in Glomerulus

Nodular glomerulosclerosis, w/ expansion of MM

28

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

Renal Papillary Infarcts

29

Amyloidosis seen in long-term hemodialysis

Beta-2 Microglobulin

29

Pattern of injury in Cryoglobulinemia

Membranoproliferative

30

Cryoglobulinemia

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

32

AA amyloid is derived from

SAA protein

34

Mixed Cryoglobulinemia most often produces what types of symptoms

Constitutional and Nonspecific

35

End Stage of Sickle Cell Nephropathy

Glomerular Enlargement and FSGS

36

Cryoglobulinemia can be asymptomatic or result in

Immune-complex-mediated GN or Vasculitis

38

Limits deposition of AA fibrils

Eprodisate

39

Tx of AL Amyloidosis

Melphalan and Dexamethasone

41

Ages for Amyloidosis

Late middle age and older

42

Triopathy

Retinopathy, Neuropathy, Retinopathy in Diabetes

44

Gross appearance of advanced amyloidosis

Enlarged, pale organs with waxy texture

45

How to distinguish Diabetic Retinopathy from Light Chain Disease

IF shows only lambda or kappa positivity in light chain disease

46

What is necessary for amyloidosis

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

47

Light Chain Disease glomerular LM strongly resembles

Diabetic Nephropathy

49

Beta 2 Amyloid occurs with

long-term hemodilaysis

50

Beta 2 microglobulin is a component of

MHC class 1 molecules

51

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

Mixed Cryoglobulinemia

53

Most common renal manifestation of AA amyloidosis

Nephrotic syndrome

55

How does Sickle Cell Nephropathy most commonly manifest?

Proteinuria

56

Hereditary Amyloidosis

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

57

Pseudothrombi in capillary lumens in glomerulus

Cryoglobulinemia

59

Monoclonal cryoglobulins typically produce signs and symptoms related to

Hyperviscosity and/or thrombosis

60

Pathology of HIV Nephropathy

Collapsing Form of FSGS

62

Most commonly dx type of amyloidosis

AL

63

Main target organ of AA amyloidosis

Kidney

64

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

Bence Jones

65

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

Sickle Cell Nephropathy

66

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

Microcystic Tubular Dilatation

68

Light chain disease vs AL amyloid

deposits are congo red negative and have no fibrillar organization

69

Tx of AA amyloidosis

Eprodisate

70

Beta 2 amyloid tends to deposit

Synovium, joints, and tendon sheaths

71

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

Mixed Cryoglobulinemia

72

HIV-associated nephropathy results from

Direct infection of renal epithelial cells in genetically susceptible host

73

Podocyte proliferation

HIV Nephropathy

74

Cryoglobulins can be monoclonal Ig's produced by

Multiple Myeloma