Renal Path, IL

Question 1

Name the different section of the nephron

Answer 1

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

Question 2

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

Answer 2

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

Question 3

The end result of all chronic renal parenchymal disease

Answer 3

CKD

Question 4

Requires chronic dialysis

Answer 4

ESRD

Question 5

is often reversible

Answer 5

AKI

Question 6

focal and segmental: defn

Answer 6

focal some of the glomeruli affected

Segmental: within an individual glomerulus, different parts affected

Question 7

The presence of inflammatory cells in the glomerulus

Answer 7

light microscopy

Question 8

effacement of podocytes

Answer 8

EM

Question 9

Deposition of antibodies on the BM

Answer 9

IF

Question 10

Sclerosis of the glomerulus

Answer 10

light microscopy

Question 11

Reduplication of BM

Answer 11

EM

Question 12

Clinical significance of fetal lobulation in adulthood

Answer 12

none

Question 13

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

Answer 13

PKD1 - chr 16 - Polycystin-1

PKD2 - chr 4 - Polycystin-2

Question 14

More severe of ADPKD

Answer 14

PKD1 - age of onset of ESRD: 53

PKD2 is 69 yo

Question 15

What tissue becomes cystic in ADPKD

Answer 15

tubules of the nephron

Question 16

What may cause pain in ptns with ADPKD

Answer 16

Expanding cysts

Passing blood clots

Question 17

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

Answer 17

Hepatic cysts
Berry aneurysm
Cardiac valve anomalies

Question 18

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

age group affected

Answer 18

PKHD1
Chr 6
fibrocystin

Pediatric

Question 19

What tissue becomes cystic in ADPKD?

Answer 19

Collecting ducts

Question 20

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

Answer 20

AD: cystic

AR: smooth

Question 21

What extrarenal organ is almost always affected in ARPKD?

Answer 21

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

Question 22

Name the four subcategories of ARPKD

most common?

Answer 22

Perinatal - MC
Neonatal - MC
Infantile
Juvenile

Question 23

Is nephronophthisis AD or AR?

Answer 23

AR

Question 24

Three variants of nephronophthisis

most common?

Answer 24

Sporadic, nonfamilial
Familial juvenile - MC
Renal-retinal dysplasia

Question 25

three genes involved in juvenile variant of neprhonophthisis

Answer 25

NPH1 NPH2 NPH3

Question 26

Nephronophthisis | Where does the cyst occur?

Answer 26

Corticomedullary junction

Question 27

What changes are seen in the cortex? | Nephronophthisis

Answer 27

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

Question 28

Name extrarenal tissues that may be affected by Nephronophthisis

Answer 28

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

Question 29

Which two renal cystic dz causes liver fibrosis? who does it affect?

Answer 29

Nephronophthisis ARPKD (periportal fibrosis) Children

Question 30

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

Answer 30

Renal cell carcinoma

Question 31

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

Answer 31

Nephronophthisis

Question 32

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

Answer 32

Smooth edges Avascular Fluid signal rather than solid signal

Question 33

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

Answer 33

Additional findings of hepatic cysts and hepatic fibrosis (ARPKD)

Question 34

61 yo with diabetes mellitus on dialysis for 12 yrs

Answer 34

Multiple 1-2 cm cortical and medullary cysts within small kidneys bilaterally (acquired/Dialysis-associated cysts)

Question 35

Multiple cystic dilations of medullary collecting ducts

Answer 35

Medullary sponge kidney

Question 36

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

Answer 36

Multicystic renal dysplasia

Question 37

Markedly enlarged kidneys with multiple cysts along with a polycystic liver

Answer 37

ADPKD, ARPKD

Question 38

Bilaterally enlarged cystic kidneys along with congenital hepatic fibrosis

Answer 38

ARPKD