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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?

A

none

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
Q

What is the outcome for multicystic renal dysplasia?

A

renal failure if bilateral, surgically curable if unilateral

26
Q

What is the inheritance pattern for simple cysts?

A

none

27
Q

What are the pathologic features for simple cysts?

A

single or multiple cysts in normal sized kidneys

28
Q

What is the clinical feature for simple cysts?

A

microscopic hematuria

29
Q

What is the outcome for simple cysts?

A

benign

30
Q

What is the PKD1 gene?

A

Located on chromosome 16p13.3 and encodes for large integral membrane protein named polycystin 1

31
Q

Where is polycystin-1 expressed?

A

tubular epithelial cells

32
Q

Mutations in PKD1 gene account for about 85% of cases involving what?

A

ADPKD

33
Q

What is the PKD2 gene?

A

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
Q

What is the function of the apical cilia on renal tubule cells?

A

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
Q

What does the PKHD1 gene encode for?

A

Fibrocystin which like polycystins 1 and 2 has been localized to primary cilium of tubular cells

36
Q

Which gene loci are associated in the juvenile forms of nephronophthisis?

A

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
Q

What is the morphology of nephronophthisis on renal US?

A

Demonstrates increased echogenicity, loss of corticomedullary differentiation, and the presence of corticomedullary cysts, kidneys are not enlarged

38
Q

What is the morphology of nephronophthisis on renal histology?

A

Shows characteristic triad of renal tubular cysts, tubular membrane disruption, and tubulointerstitial cell infiltrates with interstitial fibrosis and periglomerular fibrosis

39
Q

What is distal tubular acidosis?

A

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
Q

What are the risk factors for increased serum uric acid?

A

male, age and obesity

41
Q

What are the causes of overproduction of serum uric acid?

A

Dietary purine (meat, seafood, beer), endogenous purine synthesis (malignancy, TLS), purine salvage (HGPRT or PRPS deficiency), purine breakdown (glycogen storage dz)

42
Q

What are the causes of an underexcretion of uric acid (more common than overproduction)?

A

Urinary excretion (diuretics, renal failure), urinary reabsorption (alcohol or genetic defects)

43
Q

What are the cytogenetics for sporadic papillary renal cell carcinoma?

A

trisomy 7 and 17, loss of Y

44
Q

What are the cytogenetics for hereditary papillary RCC?

A

trisomy 7

45
Q

What are the genetics for both hereditary and sporadic papillary RCC?

A

mutated activated MET

46
Q

What are the cytogenetics for both sporadic and hereditary clear cell RCC?

A

deletions on chromosome 3

47
Q

What are the genetics for both hereditary and sporadic clear cell RCC?

A

loss of VHL, inactivated and mutated VHL, hypermethylation of VHL

48
Q

What is the MC primary renal tumor of childhood and the 4th MC pediatric malignancy in the US?

A

Wilms tumor

49
Q

Wilms tumors are microscopically characterized by what?

A

recognizable attempts to recapitulate different stages of neprhogenesis

50
Q

What characterizes the blastemal component of Wilms tumor?

A

Sheets of small blue cells with few distinctive features

CPR II Exam 3 (8 decks)

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