Robbins Ch. 20 - The Kidney III

Question 1

affect on calyces

Answer 1

only chronic PN and analgesic nephropathy

Question 2

xanthogranulomatous pyelonephritis

Answer 2

accumulation of foamy macrophages

-proteus infection (weil felix)

Question 3

drug-induced interstitial nephritis

Answer 3

fever, eosinophilia, rash

-PCN, MCN, rifampin, thiazides, NSAIDs

Question 4

prep for colonoscopy

Answer 4

acute phosphate nephropathy

-precipitate of calcium P

Question 5

bence jones

Answer 5

myeloma kidney

-pink blue cast

Question 6

tophus

Answer 6

mononuclear response with giant cells

-chronic urate nephropathy

Question 7

medial/intimal thickening, hyalinzation of artery walls

Answer 7

nephrosclerosis

granular kidney, shrinkage

elderly, blacks, DM - increased

Question 8

malignant nephrosclerosis

Answer 8

BP > 200/120
papilledema, retinal hemorrhage, confusion

hyperplastic ateriosclerosis (onion skinning)

elevated renin levels

Question 9

renal artery stenosis

Answer 9

curable with surgery
-70% caused by atheromatous plaque

• increased renin - leads to ischemic kidney
• causes HTN

-also - fibromuscular dysplasia (string of beads)

Question 10

string of beads

Answer 10

fibromuscular dysplasia

Question 11

bruit on kidney, renin, response to ACE (-)

Answer 11

renal artery stenosis

Tx - 70-80% cured with surgery

Question 12

E. coli 0157/H7

Answer 12

typical HUS

Question 13

mutation in factor H

Answer 13

atypical HUS

also factor I and CD46

Question 14

Tx typical HUS

Answer 14

dialysis - recover in weeks

Question 15

antiphospholipid syndrome

Answer 15

atypical HUS

also pregnancy, systemic sclerosis, chemo, radiation

Question 16

ADAMTS13

Answer 16

TTP

-inhibitory autoantibodies

Question 17

Tx for TTP

Answer 17

plasmapharesis

Question 18

subendo deposits of fibrion and mesangiolysis

Answer 18

TTP

Question 19

reduced NO and prostaglandins

Answer 19

prothrombotic

-in TTP/HUS

Question 20

generalized ischemia to kidney

Answer 20

diffuse cortical necrosis

• during obstetric emergency
• coagulative necrosis of glomeruli and tubules

Question 21

mural thrombi embolizes

Answer 21

renal infarct - 25% CO

-white infarct- wedege shaped

Question 22

sickle cell nephropathy

Answer 22

hematuria/hyposthenuria (can’t concentrate urine)

Question 23

if biopsy kidney or do nephrectomy?

Answer 23

check for U/L kidney

Question 24

hypoplasia

Answer 24

more commonly U/L

Question 25

ectopic kidney

Answer 25

typically in pelvis

Question 26

horseshoe kidney

Answer 26

fusion of lower poles

Question 27

APKD gene

Answer 27

ADPKD

Question 28

bilateral cysts

Answer 28

APKD always is bilateral

Question 29

PKD1

Answer 29

ADPKD - chromosme 16 - cell-cell matrix interactions - worse prognosis - 70% ESRD by 60yo - majority of cases

Question 30

PKD2

Answer 30

``` ADPKD -chromosome 4 -Ca channel -better prognosis - 15% ESRD by 60yo - ```

Question 31

ADPKD clinical

Answer 31

flank pain, stones, UTI, HTN faster in blacks also liver cysts, mitral valve prolapse, colon diverticula

Question 32

berry aneurysms

Answer 32

increased in ADPKD

Question 33

prognosis of ADPKD

Answer 33

40% die of coronary or HTN heart disease

Question 34

perinatal ARPKD

Answer 34

90% cystic collecting ducts -survive few hours after birth MOST COMMON

Question 35

neonatal ARPKD

Answer 35

60% cystic collecting ducts | -survive few months after birth

Question 36

infantile ARPKD

Answer 36

20% cystic collecting ducts | -survive until childhood

Question 37

juvenile ARPKD

Answer 37

10% cystic collecting ducts | -survive to adolescence

Question 38

PKGD

Answer 38

fibrocystin - mutation in ARPKD | -chromosome 6

Question 39

ARPKD

Answer 39

CD dilation - cysts in cortex and medulla (cuboidal) - dilated channels at right angles also - portal fibrosis of liver occurs

Question 40

cysts at corticomedullary junction

Answer 40

nephronophthisis and adult onset medullary cystic disease

Question 41

most common genetic cause of ESRD in children and young adults

Answer 41

nephronophthisis | -polydipsia and polyuria

Question 42

MCKD1 and 2

Answer 42

mutated in adult onset medullary cystic diseases | -autosomal dominant

Question 43

mutations in nephronophthisis

Answer 43

NPHP1-11 | JBTS2, 3, 9, 11

Question 44

NPHP2

Answer 44

inversin - modulates right/left patterning - mutated in nephronophthisis

Question 45

islands of undifferentiated mesenchyme, cartilage, immature CDs

Answer 45

multicystic renal dysplasia U/L - excellent prognosis after removal of affected kidney B/L - ESRD may occur

Question 46

simple cysts

Answer 46

single/multiple - typically in cortex - incidental finding - not clinically significant

Question 47

proteus infection

Answer 47

magnesium ammonium phosphate - struvite stones - staghorn calculi

Question 48

small stones

Answer 48

more hazardous - can enter ureter

Question 49

subtotal intermittent obstruction

Answer 49

hydronephrosis

Question 50

<1cm mass

Answer 50

renal papillary adenoma - benign

Question 51

>1cm mass

Answer 51

low-grade renal cell carcinoma

Question 52

tuberous sclerosis

Answer 52

angiomyolypoma - benign lesions of cerebral cortex, retardation, epilepsy, skin changes, heart tumors

Question 53

TSC1 , 2

Answer 53

mutations in tuberous sclerosis

Question 54

worry with angiomyolypoma

Answer 54

may hemorrhage significantly

Question 55

epithelial cell with increased mitochondria

Answer 55

oncocyte | -in oncocytoma

Question 56

oncocytoma

Answer 56

epithelial neoplasm - large eosinophilic cells, from intercalated cells and CD - mahogany with central scar benign

Question 57

wilms tumor

Answer 57

age 2-5yo asian large abdominal mass pain, hematuria, HTN

Question 58

WAGR

Answer 58

wilms tumor aniridia genital retardation

Question 59

denys drash

Answer 59

with wilms tumor

Question 60

beckwith wiedeman

Answer 60

with wilms tumor

Question 61

nephrogenic rests

Answer 61

precursor lesions in wilms tumor

Question 62

diffuse anaplasia

Answer 62

bad prognosis for wilms tumor

Question 63

older with wilms

Answer 63

better prognosis

Question 64

p53, 11q, 16q deletion with wilms

Answer 64

bad prognosis