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CRRAB II- Week 2 > Warren- Glomerular Disease > Flashcards

Warren- Glomerular Disease Flashcards

1
Q

What is primary COD in 4-10% of PCKD pts?

A

Intracranial berry aneurysm

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2
Q

What is the inheritance pattern for childhood PKD?

A

Autosomal recessive!

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3
Q

What is seen both grossly in childhood PKD?

A

gross: ENLARGED SMOOTH externally–cut section shows SMALL CYSTS in CORTEX and MEDULLA

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4
Q

Does childhood PCKD occur unilaterally or bilaterally?

A

Bilaterally

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5
Q

Liver cysts and bile duct proliferation is seen in almost ALL pts w/ what disease?

A

childhood PCKD

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6
Q

If children w/ PCKD survive infancy, what may you observe?

A

congenital hepatic fibrosis–periportal fibrosis and proliferation of bile ductules

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7
Q

What is medullary sponge kidney? Who does it commonly affect?

A

Cystic dilations of collecting ducts in the medulla>

HEMATURIA, infection, stones>

MAINTAIN NORMAL kidney fxn

ADULTS

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8
Q

What will you observe grossly in medullary sponge kidney?

A

Dilated papillary ducts in the medulla

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9
Q

What is nephronophthisis?

A

onset in CHILDHOOD>

Cysts in the medulla>
cortical TUBULAR ATROPHY and interstitial fibrosis>

child presents w/ polyuria and polydipsia d/t tubular defect

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10
Q

What is acquired cystic disease and why can it be bad?

A

Usually asymptomatic but 7% of dialysis pts will develop RENAL CELL CARCINOMA w/in the cysts (after about 10 yrs)

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11
Q

What is the clinical significance of finding a single simple cyst in a kidney? Where are they usually located and what do they look like?

A

Usually no clinical significance but you have to differentiate it from a tumor

1-5 cm filled with clear fluid, single layer of cuboidal or flattened epithelium line the cysts

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12
Q

What is glomerulonephrITIS?

A

IMMUNE MEDIATED DISEASE

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13
Q

What are examples of primary glomerulonephritis?

A

Minimal change

membranous

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14
Q

What are examples of secondary glomerulonephritis?

A

Diabetes mellitus
SLE
Vasculitis
AMyloidosis

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15
Q

What are the three histological patterns seen with glomerular injury?

A
  1. HYPERCELLULARITY
  2. BASEMENT MEMBRANE THICKENING (BM material or deposited material Ag-Ab)
  3. HYALINIZATION AND SCLEROSIS
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16
Q

What causes the formation of crescents?

A

Proliferating epithelial parietal cells next to infiltrating WBCs

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17
Q

What tools are used to evaluate renal disease?

A

H and E
PAS- magenta and pink
Trichrome- blue green
silver- black

IF
EM

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18
Q

What type of immune response occurs in anti-GBM Glomerulonephritis?

A

IN SITU COMPLEX CEPOSITION

Ab are directed against normal components of the GBM

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19
Q

What is indicative of anti-GBM glomerulonephritis on IF?

A

Homogenous, diffuse LINEAR pattern

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20
Q

How does anti-GBM glomerulonephritis relate to Good pasture’s syndrome?

A

Ab can cross react w/ other basement membranes like those in the alveoli> hemoptysis

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21
Q

The Ag associated with anti-GBM Glomerulonephritis and Good Pasture’s syndrome is part of what component of the BM?

A

NC1 domain of the alpha 3 chain of Type IV collagen

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22
Q

Heymann Nephritis is associated w/ what immune mediated response?

A

IN SITU COMPLEX DEPOSITION

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23
Q

What Ag is Heymann Nephritis associated with?

A

M type phospholipase A2 receptor

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24
Q

What is commonly seen on IF and EF in a pt with Heymann nephritis?

A

IF: granular and interrupted pattern

EM: electron dense deposits

Ab bind ag>
activates complement>
complexes shed and aggregate

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25
Q

What are “planted antigens”?

A

IN SITU complex deposition

Ags from NON-GLOMERULAR ORIGIN localize in the kidney>
Abs form against them

(DNA, bacterial products, aggregated Igs)

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26
Q

What is circulating Immune Complex Nephritis? What type of injury is this?

A

Circulating Ag-Ab complexes get trapped in the glomerulus (usually SUBENDOTHELIAL) and cause glomerular injury.

Type III hypersensitivity rxn

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27
Q

What is hte source of Ags associated with circulating immune complex nephritis?

A

Endogenous (SLE)

Exogenous (streptococci)

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28
Q

Once GFR is reduced to 30-50% of normal progression to end stage renal failure proceeds at a steady rate. What histological findings are associated with the progression of glomerular disease?

A

Focal segmental glomerulosclerosis

Tubulointerstitial fibrosis

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29
Q

What is FSGS?

A

Loss of function of nephron>
adaptive changes in the glomerulus>
hypertrophy of gloms to make up for rest of nephron>
increased flow/filtration/transcapillary pressure in individual glomeruli>
SEGMENTAL SCLEROSIS in those glomeruli over time

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30
Q

What is the one thing that can delay or slow down FSGS?

A

ACE inhibitors

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31
Q

What is tubulointerstitial fibrosis?

A

Glomerularnephritides>

ISCHEMIC TUBULES downstream from sclerotic glomeruli

PROTEINURIA directly toxic to downstream tubular cells

Increased acute and chronic inflammation> SCARRING

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32
Q

What are the key features of a nephritic syndrome?

A

INFLAMMATORY AND PROLIFERATIVE

hematuria and red cell casts
some proteinuria
azotemia
HTN

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33
Q

What are key features of nephrotic syndrome?

A

PROTEINURIA > 3.5 G/24 HRS>
hypoalbumininemia>
edema

Hyperlipidemia>
hyperlipiduria (fat in urine)

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34
Q

What is a common finding associated w/ nephrotic glomerulonephritis?

A

damage to visceral epithelial cells (PODOCYTES)

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35
Q

What is Acute poststreptococcal glomerulonephritis? What population is it common in?

A

Group A beta hemolytic streptococci are nephritogenic>
1-4 wks post strep pharyngeal or skin infection>
Acute poststreptococal glomerulonephritis

KIDS

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36
Q

If a child comes in who you think has acute poststreptococcal glomerulonephritis, how do you assess them?

A

Usually don’t need a kidney biopsy.

C3 levels LOW

Serum + for:
Antistreptolysin O
antiDNase B

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37
Q

What is seen histologically wtih APS GN?

A

Large hypercellular glomeruli (proliferation of endo/mesang cells and infiltration of WBC)

38
Q

What is seen on IF AND EM of APS GN?

A

IF: VERY GRANULAR glomeruli

EM: SUBEPITHELIAL HUMPS! (CAMEL)

39
Q

If a child presents with SMOKY URINE, red cell casts, mild proteinuria, PERIORBITAL EDEMA, and mild HTN what might they have? How does this differ from an adult presentation?

A

APS GN > 95% children recover

Adults often have more atypical presentation–only 60% recover

40
Q

What is rapidly progressive (cresentic) GN?

A

SEVERE injury to the glomerulus

> 50% have crescents

*Crescents are proliferations of parietal epithelial cells of bowmans capsule + inflammaotry cells

41
Q

What are the three classifications of RPGN?

A

I: anti-GBM GN–> linear deposits

II: immune complex mediated–> lumpy- bumpy (PSGN, SLE, IgA)

III: Pauci- immune type–>can’t see deposits, but most pts have p or c-ANDA in peripheral blood

42
Q

How does RPGN present histologically?

A

Large pale kidneys w/ PETECHIAE

CRESCENT FORMATION >50% of glomeruli

RUPTURES IN GBM

43
Q

What is the clinical presentation for RPGN?

A

Typical Nephritic (hemturia + red cell casts, mod proteinuria, HTN, edema)

BUT it progresses over several weeks w/ SEVERE OLIGURIA

44
Q

What pathophysiology is associated w/ nephrotic syndrome?

A

Increased permeability of GBM to proteins–> MASSIVE PROTEINURIA> hypoalbuminemia–> decreased osmotic pressure>
edema

Increased liver synthesis of LIPOPROTEINS

Increased risk for INFECTIONS d/t loss of Ig and complement

HYPERCOAGUABLE STATE d/t loss of anticoagulants

45
Q

What is the primary cuase of nephrotic syndrome in children?

A

MCD

46
Q

What is the primary cuase of nephrotic syndrome in adults?

A

FSGS

47
Q

What percent of membranous GN are idiopathic and what are common secondary causes?

A

85%

Drugs: NSAIDS
Malignancy
SLE
Infections: HBV, HCV

48
Q

What is the pathogenesis of membranous GN?

A

CHronic Ag-Ab mediated disease–>

complement mediated damage to GBM

49
Q

What is microscopic evidence of membranous GN?

A

Diffuse thickening of capillary wall CHEERIOS

SPIKES (basement membrane material laid down between the deposits)

50
Q

How do subepithelial deposits occur in membranous GN?

A

Subepithelial deposits (IgG and C3)>
Spikes on basement membrane>
thickened GBM w/ lucent defects (deposits are resorbed)>
effacement of foot processes

51
Q

What is the typical course of membranous GN?

A

chronic proteinuria with SLOW deterioration

52
Q

What is the MCC of nephrotic syndrome in children 2-6 years? How is it treated?

A

Minimal Change disease

CORTICOSTEROIDs

53
Q

What is MCD associated wtih?

A

Atopy (eczema)

URI
Immunization

HOdgkin lymphoma

54
Q

What is the pathogenesis of MCD?

A

Visceral epithelial cell injury—>
podocytes become detached–>
appear flattened out

55
Q

What disease has NORMAL GLOMERULI w/ EM showing diffuse EFFACEMENT OF FOOT PROCESSES OF visceral epithelial cells and NO deposits?

A

MCD

56
Q

What is the clinical course of MCD? Can it recur? Can the damaged be reversed by steroid therapy?

A

MASSIVE PROTEINURIA that is mostly albumin

No renal failure, no HTN

YES

YES

57
Q

What is FSGS?

A

Sclerosis of SOME gloms (FOCAL) in a PORTION of the glomerulus (SEGMENTAL)

58
Q

What is FSGS associated with?

A

HIV
heroine
sickle cell
morbid obesity

59
Q

What is the pathogenesis of FSGS?

A

Genetic abnormalities of proteins which localize to the slit diaphragm (nephrin and podocin)

60
Q

What might you see on an IF of FSGS? Microscopically?

A

Mesangial deposits of IgM and C3

Foam cell

61
Q

What is hte clinical course in FSGS?

A

Nephrotic syndrome, HTN, reduced GFR>
poor response to corticosteroids>
at least 50% have ESRD in 10 yrs

*often recurs quickly w/ transplant

62
Q

What may worsen the prognosis of FSGS?

A

HIV nephropathy

63
Q

What is seen on EM of HIV nephropathy?

A

TUBULORETICULAR INCLUSIONS in endothelial cells (also seen w/ lupus)

64
Q

What is membroproliferative GN characterized by?

A

Proliferation of glomerular cells and leukocyte infiltration–>
damaged GBM

May also present with a mild nephrotic syndrome

65
Q

What is MPGN associated with?

A

SLE
Hep B adn C
Endocarditis

*Partial lipodystrophy

Alpha-1 antitrypsin def

Malignancy

66
Q

What is seen microscopically in type II and II MPGN?

A

Large hypercellular glomeruli w/ lobular arcthitecture

thickened GBM (train track/double contour)

67
Q

What is unique to type I MPGN in terms of IF?

A

IF: C3 and C1q

68
Q

What is the pathogenesis of type I MPGN?

A

Immune comlex disease–> activation of classic and alternative comlement pathways>
subendothelial deposits

2/3 of cases of MPGN

69
Q

What is type II MPGN known as?

A

DENSE DEPOSIT DISEASE–> lamina densa of GBM is ribbon like

usually d/t excessive acativation of alternative complement pathway

70
Q

What is the clinical course for MPGN?

A

50% develop chronic renal failure in 10 yrs

steroids not helpful

recurs after transplant (esp type II)

71
Q

What is the MC type of glomerulonephritis world wide?

A

IgA Nephropathy Berger disease?

72
Q

What might you see in a pt with IgA nephropathy?

A

Recurrent hematuria

proteinuria (can be in nephrotic range)

73
Q

What is IgA nephropathy associated with?

A

gluten enteropathy

liver disease

74
Q

What does IgA nephropathy have overlapping features with?

A

Henoch Schonlein purpura

75
Q

What is the pathogenesis of IgA nephropathy?

A

IgA is IG of secretions and it is normally low in the serum

Increase in polymeric IgA in the serum>
aberrantly glycoslyated>
IgA IC deposit or are formed in MESANGIUM

76
Q

What is seen on IF and EM of IgA nephropathy?

A

IF: mesangial deposition of IgA

EM: mesangial deposits

77
Q

A pt presents w/ hematuria following a URI, GI infection or UTI. The hematuria lasts a few days, disappears then recurs. What does this pt have?

A

IgA nephropathy

78
Q

What is herediatry nephritis?

A

Herediatry diseases associated with glomerular injury

alport syndrome
thin membrane disease

79
Q

What characterizes alport syndrome?

A

nephritis
nerve deafness
eye disorders

80
Q

Who is most commonly affected w/ alport syndrome? WHy?

A

Males

Inheritance is usually X-linked dominant

can be autosomal recessive or autosomal dominant

81
Q

What is commonly seen on an EM of alport syndrome?

A

Irregular thick and thin GBM with SPLITTING of the lamina densa

82
Q

What is the pathogenesis of alport syndrome?

A

DEFECTIVE GBM synthesis

mut in gene encoding aklpha chain of type IV collagen

83
Q

What is the clinical course of alport syndrome?

A

Hematuria at 5-20 yrs (may also have proteinuria)>

renal failure by age 20-50

84
Q

What is thin membrane disease?

A

Common disease that presents w/ hematuria

Diffuse thinning of GBM d/t abnormal genes encoding collagen chains

Usually good prognosis

85
Q

What is chronic glomerulonephritis? How does it appear gross and micro?

A

End result of many types of GN

Small, diffusely granular kidneys

globally hylanized glomeruli

86
Q

What two diseases most commonly progress to chronic GN?

A

RPGN (cresenteric)

FGS

87
Q

If an SLEL pt has kidney involvement, how does it appear on an IF and EM evaluation?

A

IF- fullhouse, stains everything

EM- wire loop lesion, thickening of capillary wall by subendothelial deposits

88
Q

A child that is 5 years old presents w/ abdominal pain, vomitting and purpuric skin lesions on his arms, legs and buttocks. He also has some hematuria. What might this pt have?

A

Henoch schonlein purpura

89
Q

What causes HS purpura?

A

IgA deposited in the mesangium

child 3-8

90
Q

What is diabetic nephroaphty?

A

Proteinuria occurs in 50% of type I and II diabetics 12-22 yrs after dx of DM

91
Q

What do you see microscopically w/ diabetic nephropathy?

A
  1. capillary basement membrane thickening
  2. diffuse mesangial sclerosis
  3. **nodular glomerulosclerosis–hyaline masses at the periphery of the glomerulus (vascular pole of glomerulus is often thickened and hylanized)
92
Q

What is the pathogenesis of diabetic nephroapthy?

A
  1. metabolic defect- increase in type IV collagen, NE glycosylation of proteins–> thickened GBM and increased mesangial matrix
  2. Hemodynamic effect- increased GFR and glomerular hypertrophy> increased filtration> glomerulosclerosis

CRRAB II- Week 2 (9 decks)

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