M2010.03.30, 04.01 & 04.05- Renal Path

Front 1

3 components of the glomerular filtration barrier

Back 1

1. fenenstrated capillary endothelium (size)

2. fused BM w/ heparin sulfate (neg charge)

3. podocyte foot processes on epithelium

Front 2

Contrast:
1. azotemia
2. uremia

Back 2

1. elevated serum values of things that the kidney should filter/secrete (BUN, creatinine)

2. azotemia + s/s & biochem abNLities

Front 3

3 roles of mesangial cells of the glomerulus

Back 3

1. contraction

2. phagocytic

3. proliferation

Front 4

Describe 2 types of immune complex deposition

Back 4

1. fixed intrinsic tissue Ags (anti GBM nephritis, membranous nephropathy)

2. planted Ags (exo or endogenous)

Front 5

Contrast:
1. type II
2. type III immunity

Back 5

1. II = Ab mediated injury: Ags on surface, matrix

2. III = IC mediated, activating complement

Front 6

4 common presenting sxs of nephritic syndrome

Back 6

1. hematuria

2. oliguria w/ azotemia

3. HTN

4. mild proteinuria

Front 7

1. Causative agent of renal pathology in poststreptococcal GN & location where they do damage
2. 2 lab findings

Back 7

1. circulating ICs w/ ASO in subepithelium

2. high ASO, low C3

Front 8

1. Contrast recovery of children and adults w/ poststreptococcal GN
2. 2 outcomes in adults that don't recover

Back 8

1. kids: >95% totally recover; adults: 60% recover

2. chronic GN, RPGN

Front 9

1. Clinical presentation of rapidly progressive GN (RPGN)
2. Classic histological finding
3. 3 diverse etiologies of RPGN
4. What is pauci-immune type?

Back 9

1. nephritic synd + rapid & progressive loss of renal fxn

2. crescent moon shape in Bowman's space

3. IC deposition, autoAbs against GBM, c-ANCA

4. No IC or Abs

Front 10

What are the crescent moon of RPGN composed of?

Back 10

1. fibrin
2. proliferating parietal epithelial cells
3. M0s

Front 11

Contrast the immunofluorescent appearance of:
1. IC based RPGN
2. Goodpasture syndrome

Back 11

1. Granular

2. Linear

Front 12

1. 2 classic sxs of Goodpasture synd
2. Cause
3. Gender more commonly affected

Back 12

1. hematuria & hemoptysis

2. Abs against alveolar & GBM

3. Males

Front 13

Pauci-immune RPGN:
1. common Abs responsible
2. result
3. other sxs present
4. Another name for condition

Back 13

1. C-ANCA

2. may induce N0 degranulation

3. systemic vasculitis

4. Wegner's granulomatosis (ELK)

Front 14

List 5 common sxs of nephrotic synd

Back 14

1. proteinuria (>3.5 g/day)

2. hypoalbuminemia

3. edema

4. hyperlipidemia

5. loss of antithrombin III increases thromboembo risk

Front 15

1. General cause of membranous nephropathy
2. 1 key EM finding
3. 1 key LM finding

Back 15

1. autoimmune damage to podocytes

2. subepithelial deposits

3. GMB thickening

Front 16

1. Key EM finding in minimal change dz (MCD) of nephrotic synd
2. Common patients affected
3. Selective protein lost & why?
4. When do sxs resolve?

Back 16

1. effacement of foot processes

2. children 2-6 yo

3. albumin b/c of loss of charge barrier in GBM

4. after puberty

Front 17

Contrast presentation of MCD and FSGS in children

Back 17

MCD: several nephrotic episodes that are steroid sensitive

FSGS: nephrotic episodes that are not sensitive to steroids

Front 18

1. Describe course of focal segmental glomerulosclerosis
2. Common glomerular dz in what pt group?
3. Response to steroid tx?

Back 18

1. initially only rare GS but increases w/ time

2. HIV pts

3. Poor response

Front 19

Gene mutation tied to MCD & FSGS

Back 19

NPHS1 & 2

Front 20

3 types of podocytopathies and the dzs assoc'd with each

Back 20

1. Effacement of foot processes (MCD)

2. Apoptotic path (FSGS)

3. dedifferentiation & proliferation (collapsing FSGS)

Front 21

Membranoproliferative GN:
1. type 1
2. type 2
3. typical presentation

Back 21

1. IC assoc'd, class complement

2. alt complement assoc'd (recurrence in transplants)

3. nephrotic synd + hematuria

Front 22

Berger dz:
1. Cause
2. Typical presentation

Back 22

1. increased IgA synth in response to infection

2. recurrent hematuria flares w/ URI or gastroenteritis

Front 23

4 components of Henoch-Schoenlein purpura

Back 23

1. systemic IgA deposits in purpura
2. abd pain/GI bleeding
3. arthalgia
4. hematuria

Front 24

1. Defect found in Alport syndrome
2. Result in kidney
3. 3 other types of problems

Back 24

1. mutated type IV collagen synth

2. split GBM

3. nerve, eye, auditory probs

Front 25

Diabetic nephropathy:
1. 2 general causes of path
2. Effect on GBM
3. Effect on GFR
4. Characteristic finding

Back 25

1. atherosclerosis, nonenzymatic glycosylation

2. thickened, increase permeability

3. increased GFR, expanded mesangium (v'const eff arteriole)

4. Kimmelstiel-Wilson lesion (nodular GS)

Front 26

Most common cause of acute renal failure

Back 26

acute kidney injury

Front 27

2 types of pathogenesis of acute tubular necrosis

Back 27

1. ischemic -- any prolonged "pre-renal" state (hypoTN, HF, sepsis)

2. toxic (NSAIDs, aminoglycosides)

Front 28

1. Cardiac complication in acute tubular necrosis
2. How does it occur?

Back 28

1. cardiac standstill

2. hyperkalemia during initial oliguric phase

Front 29

Acute pyelonephritis:
1. regions of kidney affected and spared
2. Most common cause
3. List common causes in DM & IC'd pts

Back 29

1. hits cortex, spares vasculature

2. Gram neg rods

3. Fungi in DM, Viruses in IC'd

Front 30

4 common presenting sxs of acute pyelonephritis

Back 30

1. fever

2. CVA tenderness

3. Nausea

4. Vomiting

Front 31

1. 2 etiologies of acute pyelonephritis
2. which is most common?
3. Appearance of kidney

Back 31

1. ascending from bladder/ureter, hematogenous

2. ascending

3. cortical abscesses

Front 32

What is vesicoureteral reflux?

Back 32

incompetence of the vesicoureteral valve

Front 33

1. 2 causes of chronic pyelonephritis
2. 2 gross kidney findings essential for dx
3. 2 histological findings

Back 33

1. --chr urinary tract obstruction
--repeated bouts of acute PN

2. --coarse, asymmetric corticomed scarring
--blunted calyces

3. --interstitial fibrosis
--tubular atrophy w/ E0 casts (thyroidization of kidney)

Front 34

Renal papillary necrosis is associated w/ what 5 conditions?

Back 34

1. DM

2. acute pyelonephritis

3. chronic acetaminophen use

4. sickle cell

5. TB

Front 35

2 ways NSAIDs can cause renal pathology

Back 35

1. acute hemodynamic disturbance (block PGE2 synthesis)

2. acute hypersensitivity interstitial nephritis

Front 36

1. Mechanism by which some drugs are able to cause acute renal interstitial fibrosis
2. type of reaction
3. 5 types of drugs that can do this
4. 4 presenting sxs

Back 36

1. act as haptens, becoming antigenic

2. type I hypersensitivity (IgE)

3. PCN derivs, diuretics, sulfonamides, NSAIDs, rifampin

4. rash, fever, hematuria, CVA tenderness

Front 37

Contrast causes of acute and chronic urate nephrolithiasis

Back 37

acute: leukemia/lymphoma (rapid cell turnover)

chronic: gout

Front 38

Effect of thrombotic microangiopathies on kidneys

Back 38

lead to fibrin thrombi in glomeruli causing ARF

Front 39

3 embryological tissues that contribute to the kidney
--which is definitive?

Back 39

1. pronephros

2. mesonephros (interim for 1st tri)

3. metanephros (permanent)

Front 40

Etiology of cystic renal dysplasia

Back 40

abnormality of metanephric differentiation w/ persistent immature elements

Front 41

Adult polycystic kidney dz:
1. another name
2. 2 gene findings (which more common?)
3. distribution of cysts
4. eventual result
5. 4 extrarenal locations
6. Assoc'd w/ what other 2 conditions?

Back 41

1. autosomal dominant PKD

2. mutation in APKD1 (85%) & APKD2

3. b/L

4. destruction of parenchyma

5. liver, spleen, pancreas, lungs

6. mitral valve prolapse, berry aneurysms

Front 42

Functions of:
1. APKD1
2. APKD2 in ADPKD

Back 42

1. cell-cell or cell-matrix interactions

2. regulate intracell Ca2+

Front 43

Infantile polycystic kidney dz:
1. another name?
2. Result of severe dz in utero
3. 3 concerns post-neonatal
4. assoc'd w/ what other condition?

Back 43

1. ARPKD

2. Potter's syndrome

3. HTN, portal HTN, progressive renal insuff

4. congenital hepatic fibrosis

Front 44

4 complications of Medullary sponge kidney:

Back 44

1. hematuria,
2. infection,
3. calculi,
4. calcifications

Front 45

Acquired cystic dz of the kidney:
1. what cause?
2. increases risk of what condition?
3. 3 characteristics

Back 45

1. long-standing dialysis

2. RCC

3. scarring, multiple cysts, glomerular/tubular atrophy

Front 46

Simple renal cysts:
1. Location
2. 3 sxs
3. need to rule out?

Back 46

1. cortex

2. hemorrhage, pain, calcification

3. RCC

Front 47

2 conditions that produce renal cysts

Back 47

1. Von-Hippel-Lindau dz

2. Tuberous sclerosis

Front 48

3 types of benign renal tumors and their origins

Back 48

1. papillary adenoma - renal tubules

2. oncocytoma - intercalated cells of CD

3. angiomyolipoma - vessels/smM/fat

Front 49

Renal cell carcinoma (RCC):
1. most common in what gender, what age range?
2. risk increases w/ what 2 characteristics?
3. How does it metastasize & 2 common locations?
4. Most common type of RCC

Back 49

1. men, 50-70yo

2. smoking, obesity

3. invades IVC to lungs, bone

4. Clear cell

Front 50

6 clinical manifestations of renal cell carcinoma

Back 50

1. flank pain
2. hematuria
3. fever
4. palpable mass
5. secondary polycythemia
6. weight loss

Front 51

4 paraneoplastic syndromes assoc'd w/ renal cell carcinoma

Back 51

1. EPO
2. ACTH
3. PTHrP
4. PRL

Front 52

Most common renal malignancy

Back 52

renal cell carcinoma

Front 53

Most common renal malignancy of childhood

Back 53

Wilms' tumor (nephroblastoma)

Front 54

1. Gene responsible for development of sporadic & familial clear cell RCC
2. Function of gene
3. What happens in CCRCC pts?

Back 54

1. VHL tumor suppressor

2. ubiquinates HIF (promotes cell survival in hypoxic environments, angiogenesis & metastasis)

3. deletion, mutation, hypermethylation of chrom 3p, loss of VHL activity

Front 55

Wilms' tumor:
1. origin
2. 2 common presentations
3. Genetic component
4. What is the WAGR complex?

Back 55

1. primitive metanephric tissue - immature glomerular structures

2. palpable flank mass, hematuria

3. deletion of WT1&WT2 tumor suppressors on chrom 11

4. all from WT1 deletion: Wilms' tumor, Aniridia, GU malform, mental/motor Retardation

Front 56

Urothelial (transitional cell) carcinoma:
1. Can occur in what 4 locations?
2. Suggestive sx
3. Assoc'd w/ what 4 factors*

Back 56

1. renal pelvis, calyces, ureters, bladder

2. painless hematuria

3. Phenacetin (acetaminophen), Smoking, Aniline dye, Cyclophosphamide

Hint 56

Problems in the Pee SAC