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T&D III exam 3 > Renal Path, IL > Flashcards

Renal Path, IL Flashcards

1
Q

Name the different section of the nephron

A 
Glomerulus
Proximal tubule
Descending (thin) loop of Henle
Ascending (thick) loop of Henle
Distal convoluted tubule
Connecting tubule
Collecting duct
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2
Q

If patient develops fibrinous pericarditis from elevated BUN, does this patient have azotemia or uremia?

A

Uremia (development of signs and sx from circulating nitrogenous compounds)

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3
Q

The end result of all chronic renal parenchymal disease

A

CKD

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4
Q

Requires chronic dialysis

A

ESRD

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5
Q

is often reversible

A

AKI

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6
Q

focal and segmental: defn

A

focal some of the glomeruli affected

Segmental: within an individual glomerulus, different parts affected

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7
Q

The presence of inflammatory cells in the glomerulus

A

light microscopy

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8
Q

effacement of podocytes

A

EM

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9
Q

Deposition of antibodies on the BM

A

IF

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10
Q

Sclerosis of the glomerulus

A

light microscopy

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11
Q

Reduplication of BM

A

EM

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12
Q

Clinical significance of fetal lobulation in adulthood

A

none

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13
Q

Name two genes involved in the development of ADPKD, chromosomal location, protein encoded

A

PKD1 - chr 16 - Polycystin-1

PKD2 - chr 4 - Polycystin-2

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14
Q

More severe of ADPKD

A

PKD1 - age of onset of ESRD: 53

PKD2 is 69 yo

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15
Q

What tissue becomes cystic in ADPKD

A

tubules of the nephron

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16
Q

What may cause pain in ptns with ADPKD

A

Expanding cysts

Passing blood clots

17
Q

Name three extrarenal lesions that may be seen in ptns with ADPKD

A

Hepatic cysts
Berry aneurysm
Cardiac valve anomalies

18
Q

Name the gene involved in the development of ARPKD, location, protein

age group affected

A

PKHD1
Chr 6
fibrocystin

Pediatric

19
Q

What tissue becomes cystic in ADPKD?

A

Collecting ducts

20
Q

What does the surface of the kidney look like in ADPKD? ARPKD?

A

AD: cystic

AR: smooth

21
Q

What extrarenal organ is almost always affected in ARPKD?

A

The liver - hepatic cysts are almost always present.

Also may cause Periportal fibrosis which leads to liver compromise and cirrhosis in children who survive past the neonatal period

22
Q

Name the four subcategories of ARPKD

most common?

A

Perinatal - MC
Neonatal - MC
Infantile
Juvenile

23
Q

Is nephronophthisis AD or AR?

A

AR

24
Q

Three variants of nephronophthisis

most common?

A

Sporadic, nonfamilial
Familial juvenile - MC
Renal-retinal dysplasia

25
Q

three genes involved in juvenile variant of neprhonophthisis

A

NPH1
NPH2
NPH3

26
Q

Nephronophthisis

Where does the cyst occur?

A

Corticomedullary junction

27
Q

What changes are seen in the cortex?

Nephronophthisis

A

Tubular atrophy
Thickening of BM of proximal and distal tubules
Interstitial fibrosis

28
Q

Name extrarenal tissues that may be affected by Nephronophthisis

A

Extraocular muscles - motor abnormalities
Retina - dystrophy
Cerebellum
Liver - fibrosis

29
Q

Which two renal cystic dz causes liver fibrosis?

who does it affect?

A

Nephronophthisis
ARPKD (periportal fibrosis)

Children

30
Q

Name one possible serious complication of acquired (dialysis-assoc) cystic renal disease

A

Renal cell carcinoma

31
Q

What is the most common cause of genetic renal disease in children and young adults?

A

Nephronophthisis

32
Q

What helps distinguish a lesion on CT scan as a cyst rather than a second primary cancer or a metastasis?

A

Smooth edges
Avascular
Fluid signal rather than solid signal

33
Q

Premature infant - autopsy shows kidneys enlarged bilaterally with smooth surfaces and elongated cysts

A

Additional findings of hepatic cysts and hepatic fibrosis (ARPKD)

34
Q

61 yo with diabetes mellitus on dialysis for 12 yrs

A

Multiple 1-2 cm cortical and medullary cysts within small kidneys bilaterally

(acquired/Dialysis-associated cysts)

35
Q

Multiple cystic dilations of medullary collecting ducts

A

Medullary sponge kidney

36
Q

Normal left kidney but enlarged right kidney with variably-sized cysts

A

Multicystic renal dysplasia

37
Q

Markedly enlarged kidneys with multiple cysts along with a polycystic liver

A

ADPKD, ARPKD

38
Q

Bilaterally enlarged cystic kidneys along with congenital hepatic fibrosis

A

ARPKD

T&D III exam 3 (1 decks)

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