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CPR II Exam 3 > Renal Pathology pt. 2 > Flashcards

Renal Pathology pt. 2 Flashcards

1
Q

What is the etiology for acquired shiga-toxin mediated HUS?

A

Shiga toxin producing E coli, shigella dysenteriae serotype 1

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2
Q

What is the etiology for inherited atypical HUS?

A

complement dysregulation due to genetic abnormalities (relatively common)

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3
Q

What is the etiology for acquired atypical HUS?

A

Acquired complement dysregulation due to Auto-Abs

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4
Q

What is the etiology for inherited TTP?

A

Genetic ADAMTS13 deficiency (Rare)

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5
Q

What is the etiology for acquired TTP?

A

ADAMTS13 deficiency due to auto-Abs (relatively common)

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6
Q

What is the inheritance for adult polycystic kidney disease?

A

autosomal dominant

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7
Q

What are the pathologic features for ADPKD?

A

large multi cystic kidneys, liver cysts, berry aneurysms

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8
Q

What are the clinal features or complications associated with ADPKD?

A

hematuria, flank pain, UTI, renal stones, HTN

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9
Q

What is the typical outcome for ADPKD?

A

chronic renal failure beginning at 40-60 years old

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10
Q

What is the inheritance pattern for childhood polycystic kidney disease?

A

autosomal recessive

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11
Q

What are the pathologic features for ARPKD?

A

enlarged, cystic kidneys at birth

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12
Q

What are the clinical features or complications for ARPKD?

A

hepatic fibrosis

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13
Q

What is the typical outcome for ARPKD?

A

variable, death in infancy or childhood

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14
Q

What is the inheritance pattern for medullary sponge kidney?

A

none

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15
Q

What are the pathologic features for medullary sponge kidney?

A

medullary cysts on excretory urography

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16
Q

What are the clinical features or complications for medullary sponge kidney?

A

hematuria, UTI, recurrent renal stones

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17
Q

What is the typical outcome for medullary sponge kidney?

A

benign

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18
Q

What is the inheritance pattern for familial juvenile nephronophthisis?

A

autosomal recessive

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19
Q

What are the pathological features for familial juvenile nephronophthisis?

A

corticomedullary cysts, shrunken kidneys

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20
Q

What are the clinical features or complications for familial juvenile nephronophthisis?

A

salt wasting, polyuria, growth retardation, anemia

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21
Q

What is the outcome for familial juvenile nephronophthisis?

A

progressive renal failure beginning in childhood

22
Q

What is the inheritance pattern for multicystic renal dysplasia?

23
Q

What are the pathologic features for multicystic renal dysplasia?

A

irregular kidneys with cysts of variable size

24
Q

What are the clinal features or complications for multicystic renal dysplasia?

A

association with other renal anomalies

25
What is the outcome for multicystic renal dysplasia?
renal failure if bilateral, surgically curable if unilateral
26
What is the inheritance pattern for simple cysts?
none
27
What are the pathologic features for simple cysts?
single or multiple cysts in normal sized kidneys
28
What is the clinical feature for simple cysts?
microscopic hematuria
29
What is the outcome for simple cysts?
benign
30
What is the PKD1 gene?
Located on chromosome 16p13.3 and encodes for large integral membrane protein named polycystin 1
31
Where is polycystin-1 expressed?
tubular epithelial cells
32
Mutations in PKD1 gene account for about 85% of cases involving what?
ADPKD
33
What is the PKD2 gene?
Located on chromosome 4q21 and accounts for the remaining causes of ADPKD; its product is polycystin 2 which is expressed in all segments of renal tubules
34
What is the function of the apical cilia on renal tubule cells?
Function in the kindly tubule as a mechanosensor to monitor changes in fluid flow and shear stress while intercellular junctional complexes monitor forces between cells, and focal adhesions sense attachment to ECM
35
What does the PKHD1 gene encode for?
Fibrocystin which like polycystins 1 and 2 has been localized to primary cilium of tubular cells
36
Which gene loci are associated in the juvenile forms of nephronophthisis?
16 responsible gene loci including NPHP1 to NPHP11 (that encode proteins called neprhocystins, JBTS2, JBTS3, JBTS9, and JBTS11); these proteins are present in primary cilia, basal bodies attached to these cilia or the centrosome organelle from which the basal bodies originate
37
What is the morphology of nephronophthisis on renal US?
Demonstrates increased echogenicity, loss of corticomedullary differentiation, and the presence of corticomedullary cysts, kidneys are not enlarged
38
What is the morphology of nephronophthisis on renal histology?
Shows characteristic triad of renal tubular cysts, tubular membrane disruption, and tubulointerstitial cell infiltrates with interstitial fibrosis and periglomerular fibrosis
39
What is distal tubular acidosis?
Hypokalemia --> intercalated cells unable to secrete H+ in DCT --> less bicarb in the blood --> increased Ca in urine (renal stones) --> metabolic acidosis --> osteopenia; urinary pH >5.5 because DCT unable to acidify urine
40
What are the risk factors for increased serum uric acid?
male, age and obesity
41
What are the causes of overproduction of serum uric acid?
Dietary purine (meat, seafood, beer), endogenous purine synthesis (malignancy, TLS), purine salvage (HGPRT or PRPS deficiency), purine breakdown (glycogen storage dz)
42
What are the causes of an underexcretion of uric acid (more common than overproduction)?
Urinary excretion (diuretics, renal failure), urinary reabsorption (alcohol or genetic defects)
43
What are the cytogenetics for sporadic papillary renal cell carcinoma?
trisomy 7 and 17, loss of Y
44
What are the cytogenetics for hereditary papillary RCC?
trisomy 7
45
What are the genetics for both hereditary and sporadic papillary RCC?
mutated activated MET
46
What are the cytogenetics for both sporadic and hereditary clear cell RCC?
deletions on chromosome 3
47
What are the genetics for both hereditary and sporadic clear cell RCC?
loss of VHL, inactivated and mutated VHL, hypermethylation of VHL
48
What is the MC primary renal tumor of childhood and the 4th MC pediatric malignancy in the US?
Wilms tumor
49
Wilms tumors are microscopically characterized by what?
recognizable attempts to recapitulate different stages of neprhogenesis
50
What characterizes the blastemal component of Wilms tumor?
Sheets of small blue cells with few distinctive features

CPR II Exam 3 (8 decks)

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