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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
What are "planted antigens"?
IN SITU complex deposition Ags from NON-GLOMERULAR ORIGIN localize in the kidney> Abs form against them (DNA, bacterial products, aggregated Igs)
26
What is circulating Immune Complex Nephritis? What type of injury is this?
Circulating Ag-Ab complexes get trapped in the glomerulus (usually SUBENDOTHELIAL) and cause glomerular injury. Type III hypersensitivity rxn
27
What is hte source of Ags associated with circulating immune complex nephritis?
Endogenous (SLE) Exogenous (streptococci)
28
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?
Focal segmental glomerulosclerosis Tubulointerstitial fibrosis
29
What is FSGS?
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
30
What is the one thing that can delay or slow down FSGS?
ACE inhibitors
31
What is tubulointerstitial fibrosis?
Glomerularnephritides> ISCHEMIC TUBULES downstream from sclerotic glomeruli PROTEINURIA directly toxic to downstream tubular cells Increased acute and chronic inflammation> SCARRING
32
What are the key features of a nephritic syndrome?
INFLAMMATORY AND PROLIFERATIVE hematuria and red cell casts some proteinuria azotemia HTN
33
What are key features of nephrotic syndrome?
PROTEINURIA > 3.5 G/24 HRS> hypoalbumininemia> edema Hyperlipidemia> hyperlipiduria (fat in urine)
34
What is a common finding associated w/ nephrotic glomerulonephritis?
damage to visceral epithelial cells (PODOCYTES)
35
What is Acute poststreptococcal glomerulonephritis? What population is it common in?
Group A beta hemolytic streptococci are nephritogenic> 1-4 wks post strep pharyngeal or skin infection> Acute poststreptococal glomerulonephritis KIDS
36
If a child comes in who you think has acute poststreptococcal glomerulonephritis, how do you assess them?
Usually don't need a kidney biopsy. C3 levels LOW Serum + for: Antistreptolysin O antiDNase B
37
What is seen histologically wtih APS GN?
Large hypercellular glomeruli (proliferation of endo/mesang cells and infiltration of WBC)
38
What is seen on IF AND EM of APS GN?
IF: VERY GRANULAR glomeruli EM: SUBEPITHELIAL HUMPS! (CAMEL)
39
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?
APS GN > 95% children recover Adults often have more atypical presentation--only 60% recover
40
What is rapidly progressive (cresentic) GN?
SEVERE injury to the glomerulus >50% have crescents *Crescents are proliferations of parietal epithelial cells of bowmans capsule + inflammaotry cells
41
What are the three classifications of RPGN?
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
How does RPGN present histologically?
Large pale kidneys w/ PETECHIAE CRESCENT FORMATION >50% of glomeruli RUPTURES IN GBM
43
What is the clinical presentation for RPGN?
Typical Nephritic (hemturia + red cell casts, mod proteinuria, HTN, edema) BUT it progresses over several weeks w/ SEVERE OLIGURIA
44
What pathophysiology is associated w/ nephrotic syndrome?
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
What is the primary cuase of nephrotic syndrome in children?
MCD
46
What is the primary cuase of nephrotic syndrome in adults?
FSGS
47
What percent of membranous GN are idiopathic and what are common secondary causes?
85% Drugs: NSAIDS Malignancy SLE Infections: HBV, HCV
48
What is the pathogenesis of membranous GN?
CHronic Ag-Ab mediated disease--> | complement mediated damage to GBM
49
What is microscopic evidence of membranous GN?
Diffuse thickening of capillary wall CHEERIOS SPIKES (basement membrane material laid down between the deposits)
50
How do subepithelial deposits occur in membranous GN?
Subepithelial deposits (IgG and C3)> Spikes on basement membrane> thickened GBM w/ lucent defects (deposits are resorbed)> effacement of foot processes
51
What is the typical course of membranous GN?
chronic proteinuria with SLOW deterioration
52
What is the MCC of nephrotic syndrome in children 2-6 years? How is it treated?
Minimal Change disease CORTICOSTEROIDs
53
What is MCD associated wtih?
Atopy (eczema) URI Immunization HOdgkin lymphoma
54
What is the pathogenesis of MCD?
Visceral epithelial cell injury---> podocytes become detached--> appear flattened out
55
What disease has NORMAL GLOMERULI w/ EM showing diffuse EFFACEMENT OF FOOT PROCESSES OF visceral epithelial cells and NO deposits?
MCD
56
What is the clinical course of MCD? Can it recur? Can the damaged be reversed by steroid therapy?
MASSIVE PROTEINURIA that is mostly albumin No renal failure, no HTN YES YES
57
What is FSGS?
Sclerosis of SOME gloms (FOCAL) in a PORTION of the glomerulus (SEGMENTAL)
58
What is FSGS associated with?
HIV heroine sickle cell morbid obesity
59
What is the pathogenesis of FSGS?
Genetic abnormalities of proteins which localize to the slit diaphragm (nephrin and podocin)
60
What might you see on an IF of FSGS? Microscopically?
Mesangial deposits of IgM and C3 Foam cell
61
What is hte clinical course in FSGS?
Nephrotic syndrome, HTN, reduced GFR> poor response to corticosteroids> at least 50% have ESRD in 10 yrs *often recurs quickly w/ transplant
62
What may worsen the prognosis of FSGS?
HIV nephropathy
63
What is seen on EM of HIV nephropathy?
TUBULORETICULAR INCLUSIONS in endothelial cells (also seen w/ lupus)
64
What is membroproliferative GN characterized by?
Proliferation of glomerular cells and leukocyte infiltration--> damaged GBM May also present with a mild nephrotic syndrome
65
What is MPGN associated with?
SLE Hep B adn C Endocarditis *Partial lipodystrophy Alpha-1 antitrypsin def Malignancy
66
What is seen microscopically in type II and II MPGN?
Large hypercellular glomeruli w/ lobular arcthitecture thickened GBM (train track/double contour)
67
What is unique to type I MPGN in terms of IF?
IF: C3 and C1q
68
What is the pathogenesis of type I MPGN?
Immune comlex disease--> activation of classic and alternative comlement pathways> subendothelial deposits 2/3 of cases of MPGN
69
What is type II MPGN known as?
DENSE DEPOSIT DISEASE--> lamina densa of GBM is ribbon like | usually d/t excessive acativation of alternative complement pathway
70
What is the clinical course for MPGN?
50% develop chronic renal failure in 10 yrs steroids not helpful recurs after transplant (esp type II)
71
What is the MC type of glomerulonephritis world wide?
IgA Nephropathy Berger disease?
72
What might you see in a pt with IgA nephropathy?
Recurrent hematuria | proteinuria (can be in nephrotic range)
73
What is IgA nephropathy associated with?
gluten enteropathy | liver disease
74
What does IgA nephropathy have overlapping features with?
Henoch Schonlein purpura
75
What is the pathogenesis of IgA nephropathy?
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
What is seen on IF and EM of IgA nephropathy?
IF: mesangial deposition of IgA EM: mesangial deposits
77
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?
IgA nephropathy
78
What is herediatry nephritis?
Herediatry diseases associated with glomerular injury alport syndrome thin membrane disease
79
What characterizes alport syndrome?
nephritis nerve deafness eye disorders
80
Who is most commonly affected w/ alport syndrome? WHy?
Males Inheritance is usually X-linked dominant can be autosomal recessive or autosomal dominant
81
What is commonly seen on an EM of alport syndrome?
Irregular thick and thin GBM with SPLITTING of the lamina densa
82
What is the pathogenesis of alport syndrome?
DEFECTIVE GBM synthesis | mut in gene encoding aklpha chain of type IV collagen
83
What is the clinical course of alport syndrome?
Hematuria at 5-20 yrs (may also have proteinuria)> | renal failure by age 20-50
84
What is thin membrane disease?
Common disease that presents w/ hematuria Diffuse thinning of GBM d/t abnormal genes encoding collagen chains Usually good prognosis
85
What is chronic glomerulonephritis? How does it appear gross and micro?
End result of many types of GN Small, diffusely granular kidneys globally hylanized glomeruli
86
What two diseases most commonly progress to chronic GN?
RPGN (cresenteric) FGS
87
If an SLEL pt has kidney involvement, how does it appear on an IF and EM evaluation?
IF- fullhouse, stains everything EM- wire loop lesion, thickening of capillary wall by subendothelial deposits
88
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?
Henoch schonlein purpura
89
What causes HS purpura?
IgA deposited in the mesangium child 3-8
90
What is diabetic nephroaphty?
Proteinuria occurs in 50% of type I and II diabetics 12-22 yrs after dx of DM
91
What do you see microscopically w/ diabetic nephropathy?
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
What is the pathogenesis of diabetic nephroapthy?
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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