Glomerular And Other Renal Diseases

Front 1

Glomerular diseases induced by NSAIDs (2)

Back 1

-Minimal Change Disease
-Membranous Nephropathy

Front 2

Pulmonary-Renal Syndromes (4)

Back 2

-Goodpastures (Anti-GBM)
-Granulomatous Vasculitis (c-ANCA)
-SLE (Lupus)
-HS Purpura (type of IgA Nephropathy)

Front 3

Renal diseases that primarily affect people over the age of 50

Back 3

Pauci-immune RPGN
MicroPAN
amyloidosis

Front 4

Secondary causes of IgA Nephropathy (4)

Back 4

-HS Purpura
-Chronic liver disease
-Celiac's Disease
-Inflammatory Bowel Disease

Front 5

Presentations of IgA Nephropathy (3)

Back 5

-Macroscopic hematuria coupled to upper respiratory infection
-Asymptomatic microscopic hematuria with <1g proteinuria
-Nephrotic (less common)

Front 6

Renal diseases proceeding upper respiratory infections (3)

Back 6

-IgA Nephropathy
-HS Purpura
-PSGN

Front 7

Viral renal diseases (3)

Back 7

-HIV: FSGS (Collapsing)
-Hep B: Vasculitis (microPAN)
-Hep C: MPGN Type I (Type II is usually primary due to C3 NeF)

Front 8

mechanism of hyperlipidemia in nephrotic syndrome (2)

Back 8

-less albumin = more chol. produced
-LCAT (enzyme that helps chol. return to the liver) lost to filtration

Front 9

Hypercoaguability in nephrotic syndrome (explain)

Back 9

loss of clotting inhibitors (protein S, antithrombin 3) to filtration

Front 10

Clinical presentation: MCD vs FSGS (4)

Back 10

a.Hematuria
b.Renal function (elevated creatinine)
c.HTN
d.Selective vs non-selective proteinuria

Front 11

Non-nephritic causes of hematuria (2)

Back 11

a.Urinary blood clots
b.Kidney stone (post-renal obstruction)

Front 12

Glomerular disease caused by malignancy (3)

Back 12

a.Membranous Nephropathy
b.MCD (lymphoma)
c.MPGN

Front 13

Renal diseases that affect people over 50: (4)

Back 13

a.Amyloidosis
b.C-ANCA + Granulomatous vasculitis (pauci-immune)
c.MicroPAN vasculitis (pauci-immune)
d.Membranous Nephropathy (especially if 2’ to malignancy)

Front 14

Renal diseases that affect males more than females: (6)

Back 14

a.Amyloidosis
b.Fabry’s (X-linked)
c.Membranous
d.IgA Nephropathy
e.MPGN Type I (young male smoker)
f.Alport's (mainly X-linked)

Front 15

Nephrotic Diseases (5)

Back 15

-Minimal Change Disease
-FSGS (HIV Nephropathy)
-Membranous (lupus V)
-Amyloidosis
-MPGN Type I

Front 16

Renal diseases that present with intense pain (3)

Back 16

-PCKD (flank)
-Fabry's (distal limb neuropathy)
-Sickle cell nephropathy (colic if clots obstruct; can present w/ painless hematuria)

Front 17

Disease associated with lysosomal defect

Back 17

Fabry's

Front 18

Complement activation in Membranoproliferative Glomerular Nephritis (MPGN)

Back 18

a.Type I = low levels of C3, C1q, C4
b.Type II = low levels of C3 for a longer time

Front 19

Gender/Age/Race risk factors for Goodpastures

Back 19

a.Gender = Male
b.Age = 30s
c.Race = N/A

Front 20

differences between types I and II Rapidly Progressive Glomerular Nephritis (RPGN) Disease Examples = ?
EM findings = ?
IF findings = ?

Back 20

a.Disease example? I = Goodpastures, II= Lupus
b.EM findings? I = nothing, II = deposits in loops/mesangium
c.IF findings (what location is being stained and how is it staining)? I = linear IgG loops/mesangium, II = granular loops/mesangium

Front 21

Epithelial crescents will be stained and capillary loops will not be stained when _____ is used.

Back 21

Trichome

Front 22

Membranoproliferative Glomerular Nephritis (MPGN) microscopy findings
LM = ?
IF = ?
EM = ?

Back 22

a.LM (2)
i.Lobular appearing capillary loops
ii.“Tram-Tracking” (separation of endothelium and epithelium of GBM)
b.IF: “tree trunks (mesangium) and branches (capillary loops)”
c.EM (Type I and Type II)
i.Type I = mesangial interposition within GBM
ii. Type II = Dense deposits within GBM

Front 23

Clinical differences in the two types of Membranoproliferative Glomerular Nephritis (MPGN)

Back 23

a.Type I = more nephrotic
b.Type II = more nephritic

Front 24

Describe the H/E, IF, and EM findings of PSGN

Back 24

a.H/E: hypercellular glomeruli, WBCs, Diffuse glomerular involvement
b.IF: “camel hump” staining of IgG underneath the podocytes
c.EM: subepithelial deposits

Front 25

Lamellar (zebra) bodies are specific for _____ Disease.

Back 25

Fabry's

Front 26

Lenticonus lens defect is specific for _____ Disease

Back 26

Alport's

Front 27

Membranous Glomerulonephritis: where are the immune complexes made and where do they reside?

Back 27

a.In-situ (made on site)
b.Subepithelial or Membranous Deposits

Front 28

Describe the IF findings of Membranous Glomerulonephritis

Back 28

a.Continuity? Granular (non-continuous)
b.Molecules? IgG and C3

Front 29

Spiky projections of the GBM are seen in which disease

Back 29

Membranous Glomerulonephritis

Front 30

Membranous Glomerulonephritis...
a.Diffuse or Focal?
b.Thickening of what structure due to which molecules? c.Nephrotic/Nephritic Syndrome?

Back 30

a.Diffuse or Focal?
Diffuse
b.Thickening of what structure due to which molecules?
Capillary loops, Immune complexes
c.Nephrotic/Nephritic Syndrome?
Nephrotic

Front 31

Name the diseases caused by mutations in the following…
a.Nephrin?
b.Podocin?
c.Actinin?

Back 31

a.Nephrin? MCD
b.Podocin? FSGS
c.Actinin? FSGS

Front 32

Classic FSGS immunofluoresence findings (2 molecules) within sclerotic segment of glomerulus:

Back 32

a.IgM
b.C3

Front 33

Three major 2’ causes of FSGS

Back 33

a.HIV
b.Obesity
c.HTN

Front 34

Analogy time
_______ : Focal :: Global : _______

Back 34

Diffuse : Focal :: Global : Segmental

Front 35

Pathology signs of MCD (2 LM, 1 EM):

Back 35

a.Loss of brush border in PCT cells (LM)
b.Lipid droplets in tubular cells and urine (LM)
c.Podocyte effacement (EM)

Front 36

________ injury leads to proteinuria.

__________ injury leads to hematuria.

Back 36

_podocytes_ injury leads to proteinuria.

_capillary/mesangium__ injury leads to hematuria.

Front 37

“Full House” meaning in Lupus

Back 37

a.During all classes (especially 2 and above) of lupus, all kinds of Igs are involved in mesangial deposits

Front 38

3. Location of deposits in lupus 3,4,5
3 =
4 =
5 =

Back 38

3 = subendothelial
4 = subendothelial
5 = subepithelial

Front 39

Thrombi, crescents, tubular injury are all signs of disease _________ in lupus, whereas tubular atrophy, progression to fibrous crescents, and progression to interstitial fibrosis are signs of disease _______.

Back 39

a. Activity Level
b. Chronicity

Front 40

Age/Gender/Race risk factors of IgA Nephropathy

Back 40

a.Age = 20s/30s
b.Gender = Male
c.Race = Asian

Front 41

Microscopy findings in IgA Nephropathy

Back 41

a.LM = mesangial hypercellularity
b.IF = Granular IgA deposits
c.EM = mesangial deposits

Front 42

Differences between Henoch-Schonlein Purpurua and IgA Nephropathy

Back 42

a.Age = HSA affects kids; IgA affects 20s/30s
b.Crescent formation and Glomerular necrosis = only common in HSA
c.Extra-renal symptoms = only in HSA

Front 43

9.Infectious endocarditis can present with renal symptoms, most commonly _____

Back 43

a.Hematuria

Front 44

Skin testing in Alport’s disease (to whom does it apply, and describe possible findings)

Back 44

a.Only the X-linked (alpha 5) kind
b.Affected males have almost no staining; female carriers have mosaic staining

Front 45

Thin Membrane Disease is very similar to the recessive forms of Alport’s due to the common defect in _______

Back 45

a.Alpha 3/4 Collagen IV chains

Front 46

Thickening of the GBM, nodules of collagen IV + ECM that stain black, and mesangial hypercellularity are hallmarks of ________

Back 46

Diabetic Glomerular Sclerosis

Front 47

Normal width of GBM

Back 47

350 nm +/- 50 nm

Front 48

Renal presentation/progression in amyloidosis

Back 48

a.Proteinuria
b.Progression to uremia, renal failure

Front 49

2 chemicals that stabilize amyloid fibrils

Back 49

a.SAP (serum amyloid protein, an acute phase c-reactive protein)
b.Gylcosaminoglycans (GAGs)

Front 50

Two forms of AL amyloidosis

Back 50

a.Primary: just messing up of the proteins
b.Immunoproliferative: B cells are spitting out Ab’s like crazy

Front 51

Two forms of PAN (BV Inflammation)

Back 51

a.Classic: medium-small artery involvement; no glomerular disease; could affect kidneys via infarct/aneurysm

b.Micropan: capillaries, more likely to have nephritic presentation; M > F; 60s;

Front 52

strange (isn't seen in any other renal disease we covered) symptom of sickle cell nephropathy

Back 52

increased GFR (may explain proteinuria)

Front 53

2 determining factors for giving treatment to Membranous nephropathy patients

Back 53

1. gender (males at higher risk)
2. proteinuria

Front 54

Diseases that affect African Americans more than other ethnicities (4)

Back 54

-Lupus
-Sickle Cell Nephropathy
-Polycystic Kidney Disease
-FSGS

Front 55

extra-renal presentation of PCKD (4)

Back 55

Diverticulosis
Benign GI cysts
Berry Aneurysms
Mitral Valve Prolapse

Front 56

Diagnostic criteria for PCKD

Back 56

US/CT
2 cysts in each kidney (30-60)
1 cyst in each kidney (under 30)

Front 57

name all the cystic kidney diseases (not including pediatric urinary obstruction disease) (4)

Back 57

-PCKD
-Simple Cysts (aging, benign)
-Acquired (smaller kidneys than PCKD)
-Medullary (teens, ESRD, bad news)

Front 58

Name the cystic kidney diseases (not including pediatric urinary obstruction diseases) (4)

Back 58

PCKD
Simple cysts
Acquired
Medullary

Front 59

Extra-renal PCKD features (4)

Back 59

Berry aneurysms
Diverticulosis
GI cysts
MV prolapse

Front 60

Kidney specific problems in PCKD (4)

Back 60

rupture
bleeding
enlargement of kidney
infection

Front 61

PCKD: age of symptom onset

Back 61

mean = 45 years

Front 62

Medullary cystic KD

Back 62

the worst one; teens with ESRD; autosomal dominant

Front 63

earliest symptom in Alport's (male or female carrier)

Back 63

hematuria (if in female carrier, microscopic)

Front 64

PCKD dx:

Back 64

US/CT

30-60: two cysts in each kidney
under 30: one cyst in each kidney
+60: probably simple aging cysts, benign

Front 65

extra-renal features of fabry's

Back 65

corneal opacities, Left ventricular hypertrophy, purple rashes, distal limb pain (neuropathy)

Front 66

extra-renal features of Alport's

Back 66

hearing loss (90% @ 40)
anterior lenticonus, platelet abnormalities

Front 67

Two factors that, when increased, lead to sickling of RBCs in sickle cell disease

Back 67

1. osmolarity
2. [H+]

Front 68

most consistent, early feature of sickle cell kidney nephropathy

Back 68

conc. defect (max urine osmolarity ~400)

Front 69

Classic signs of allergic (non-NSAID) AIN (3)

Which drugs cause it?

Back 69

-Allergy (eosinophils/rash/fever)
-Mild proteinuria
-Hematuria (microscopic, without RBC casts b/c the glomerulus is fine)
-Recent antibiotic/diuretic/PPI/allopurinol use

Front 70

Distinguishing features of NSAID AIN (from normal allergy AIN) (3)

Back 70

-lymphocytes instead of eosoinophils
-glomerular involvement
-nephrotic-range proteinuria

Front 71

ways to explain high eosinophils in urine/blood (3)

Back 71

1. AIN
2. cholesterol emboli
3. contrast-induced

Front 72

Three general types of CIN (covered in class)

Back 72

1. Analgesia
2. Oxalate
3. Metals

Front 73

Sterile pyruia: what is it and how is it revelant to CIN

Back 73

urine containing pus that doesn't yield a culture sample

found in analgesic CIN

Front 74

presenting factors of analgesic CIN
age?
urine findings?
systemic features?

Back 74

Age = 30-70
Urine findings =
[+] protein
[-] hematuria, bacteria (sterile pyuria)
Systemic = HTN, back/head pain

Front 75

Diagnostic sign of analgesic CIN

Back 75

[imaging] small, lobulated kidneys with papillary calcifications

Front 76

analgesic CIN: synergy of negative effects due to ______ and ______.

What are the negative effects of each analgesic? Where do they occur in the kidney?

Back 76

NSAID, acetominophen

NSAIDs = ischemia due to lower GFR 2' to decreased prostaglandins

Acetaminophen = oxidative damage 2' to reduced levels of glutathione

MEDULLARY fibrosis

Front 77

Conditions that indicate Fanconi's (or a Fanconi-like) syndrome

Back 77

1. Positive glucose dipstick (probably hyperglycemia, but could be Fanconi's if pediatric)
2. Cadmium-induced CIN
3. pRTA

Front 78

where does class II MHC fit into the schematic for AIN?

Back 78

tubular insult > MHC II upregulation > T-lymphocytes/Macrophages > edema, fibrosis, renal decline

Front 79

Negative effects of high [oxalate]

Back 79

1. bone dz
2. arthritis
3. heart conduction problems

Front 80

type II 1' oxalosis

Back 80

hepatic enzyme defect causes increased NAD+, drives glycoxylate metabolism toward oxalate

Front 81

type I 1' oxalosis

Back 81

hepatic enzyme defect prevents conversion of glyoxylate to glycine, shunting it to a different (oxalate) pathway

Front 82

decreased absorption of _____ ties up _____ and allows oxalate to be reabsorbed in the _______

Back 82

decreased absorption of _fat_ ties up _Ca2+_ and allows oxalate to be reabsorbed in the _colon__

Front 83

Analgesic CIN increases one's risk for (3)

Back 83

HTN
Athersclerosis
Urinary tract cancer

Front 84

mechanisms for damage in lead-induced CIN (3)

Back 84

-decreased cellular respiration
-cortical atrophy
-impaired excretion of uric acid

Front 85

clinical presentation of lead-induced CIN (3)

Back 85

HTN
Gout
min. 5-10 years Pb2+ exposure

Front 86

Histology of lead-induced CIN

Back 86

similar to hypertensive nephrosclerosis
-tubular/interstitial changes
-late in course glomerular changes
-PCT damage

Front 87

2 features that would confirm CIN 2' to Cd2+ (assuming prolonged exposure)

Back 87

-lots of metabolites in urine (Fanconi-like)
-kidney stones (hypercalciuria)

Front 88

Analgesic CIN increases one's risk for (3)

Back 88

HTN
Athersclerosis
Urinary tract cancer

Front 89

mechanisms for damage in lead-induced CIN (3)

Back 89

-decreased cellular respiration
-cortical atrophy
-impaired excretion of uric acid

Front 90

clinical presentation of lead-induced CIN (3)

Back 90

HTN
Gout
min. 5-10 years Pb2+ exposure

Front 91

Histology of lead-induced CIN

Back 91

similar to hypertensive nephrosclerosis
-tubular/interstitial changes
-late in course glomerular changes
-PCT damage

Front 92

2 features that would confirm CIN 2' to Cd2+ (assuming prolonged exposure)

Back 92

-lots of metabolites in urine (Fanconi-like)
-kidney stones (hypercalciuria)

Front 93

Analgesic CIN increases one's risk for (3)

Back 93

HTN
Athersclerosis
Urinary tract cancer

Front 94

mechanisms for damage in lead-induced CIN (3)

Back 94

-decreased cellular respiration
-cortical atrophy
-impaired excretion of uric acid

Front 95

clinical presentation of lead-induced CIN (3)

Back 95

HTN
Gout
min. 5-10 years Pb2+ exposure

Front 96

Histology of lead-induced CIN

Back 96

similar to hypertensive nephrosclerosis
-tubular/interstitial changes
-late in course glomerular changes
-PCT damage

Front 97

2 features that would confirm CIN 2' to Cd2+ (assuming prolonged exposure)

Back 97

-lots of metabolites in urine (Fanconi-like)
-kidney stones (hypercalciuria)

Front 98

Analgesic CIN increases one's risk for (3)

Back 98

HTN
Athersclerosis
Urinary tract cancer

Front 99

mechanisms for damage in lead-induced CIN (3)

Back 99

-decreased cellular respiration
-cortical atrophy
-impaired excretion of uric acid

Front 100

clinical presentation of lead-induced CIN (3)

Back 100

HTN
Gout
min. 5-10 years Pb2+ exposure

Front 101

Histology of lead-induced CIN

Back 101

similar to hypertensive nephrosclerosis
-tubular/interstitial changes
-late in course glomerular changes
-PCT damage

Front 102

2 features that would confirm CIN 2' to Cd2+ (assuming prolonged exposure)

Back 102

-lots of metabolites in urine (Fanconi-like)
-kidney stones (hypercalciuria)

Front 103

differentiating acquired from genetic oxalosis

Back 103

acquired = later in life, usually with anatomical bowel resection or increased oxalate intake

genetic = presents earlier in life (childhood)

Front 104

renal disease with prominent lipid droplets in PCT epithelial cells

Back 104

Minimal change disease

Front 105

Minimal change disease IF findings

Back 105

nothing

Front 106

Common (3) and less common (3) 2' causes of FSGS

Back 106

Common = obesity/HTN/sickle cell Dz; Less common = de novo (transplant), UTI, Obstruction

Front 107

mutations in these genes can result in which diseases: alpha-actinin, podocin, nephrin

Back 107

alpha-actinin and podocin = FSGS; nephrin = Minimal Change

Front 108

histological differences between classic and collapsing FSGS (with respect to cap loops and podocytes)

Back 108

Classic = sclerosed cap loops + podocyte loss (peripheral damage); Collapsing = collapsed cap loops + podocyte proliferation (central damage)

Front 109

Etiologies of collapsing FSGS (3)

Back 109

HIV (infects podocytes) mainly, but also CMV and Hep C

Front 110

IF findings of FSGS

Back 110

sclerotic areas + mesangium have IgM and C3

Front 111

Membranous Glomerulonephritis mechanism

Back 111

IgG binding to endogenous glomerular antigens

Front 112

the relatively small fraction of 2' membranous GN cases are due to (3)

Back 112

Hepatitis B, Lupus, Malignancy

Front 113

IF findings of Membranous GN

Back 113

Diffuse, granular deposits of IgG (M+A if lupus) and C3 in a capillary loop pattern

Front 114

EM findings of Membranous GN

Back 114

subepithelial/intramembranous dense deposits + podocyte effacement (loss)

Front 115

Heymann Nephritis

Back 115

animal model for membranous GN; inject PCT brush border antigens (megalin); get anti-megalin Abs

Front 116

negative prognosis predictor in FSGS

Back 116

proteinuria > 10g/day and HTN

Front 117

negative prognosis predictor in APSGN

Back 117

presence/abundance of glomerular crescents

Front 118

glomerular diseases and types of proteinuria (2)

Back 118

MCD is selective (albumin) and FSGS is non-selective

Front 119

FSGS Tx

Back 119

Primary FSGS = corticosteroids; Secondary FSGS = ACEi

Front 120

presentations of FSGS

Back 120

HTN = 30%; microscopic hematuria = 50%; less severe proteinuria/edema than MCD; renal decline = 30%

Front 121

example of a glomerular disease with normal serum complement levels despite local complement activation

Back 121

Membranous

Front 122

Membranous Glomerulonephritis LM findings (2)

Back 122

silver stain shows spikes on GBM; capillary loop thickening;

Front 123

negative prognostic factors for Membranous (3)

Back 123

proteinuria > 10g/day. HTN, male (same as FSGS except for gender)

Front 124

ASPGN LM findings (3)

Back 124

ALL OF THESE ARE DIFFUSE, DAMAGE IS EVERYWHERE: capillary thrombi (maybe); endocapillary proliferation w/ neutrophils; enlarged caps

Front 125

ASPGN IF findings

Back 125

Granular deposits of IgG/M and C3 in mesangium/GBM

Front 126

ASPGN EM findings

Back 126

subepithelial "humps"

Front 127

to be a RPGN, must have this histological finding:

Back 127

LM: diffuse (more than 50%) of glomeruli featuring cellular crescents (indicate acute process)

Front 128

goodpasture's tx (2)

Back 128

cytotoxics (kill lymphocytes) and plasmaphoresis (remove problematic antibodies)

Front 129

common MPGN LM findings (2)

Back 129

endocapillary proliferation and "tram-tracking" (splitting of GBM)

Front 130

MPGN Type I histo findings

Back 130

Type 1 = IgG and C3/4 granular diffuse deposition (IF) and mesangial interposition or subendothelial deposits (EM)

Front 131

MPGN Type II histo findings

Back 131

Type 2 = continuous (non-immune complex) material that looks like another membrane or layer (EM); granular irregular linear C3 deposition (IF)

Front 132

Differences between Types I and II MPGN (age, presentation, pathology, EM, complement, etiology)

Back 132

Type I = children, immune complexes, often due to HepC; nephrotic, C4/1q depression, mesangial interposition and endothelial deposits on EM; Type II = young children; C3 NeF; duplication of GBM on EM; nephritic presentation

Front 133

Lupus EM findings by class

Back 133

3/4 = subendothelial; 5 = subepithelial

Front 134

transition from Lupus II to Lupus III as seen on IF

Back 134

just mesangial (tree trunk) staining on II; III has loop + mesangial staining (more global)