cystic kidney disease and nephrolithiasis

Question 1

two types of polycystic kidney disease

Answer 1

autosomal recessive PKD

autosomal dominant PKD

Question 2

ARPKD onset

Answer 2

infancy or early childhood

Question 3

ARPKD mutation

Answer 3

PKHD1 gene on chromosome 6

Question 4

most common mutation in ADPKD

Answer 4

PKD1 on chromosome 16

Question 5

other mutation in ADPKD

Answer 5

PKD2 on chromosome 4

Question 6

ADPKD symptom onset

Answer 6

after 30 yrs old usually

Question 7

ADPKD pathophys

Answer 7

abnormal cilia mediated signaling pathways leads to the formation and expansion of cysts in the cortex/medulla

compression of vessels leads to ischemia and destruction of parenchyma

Question 8

most common inherited cause of CKD

Answer 8

ADPKD

Question 9

PKD1

Answer 9

polycystic 1

involved in cell-cell or cell-matrix interactions

Question 10

PKD2

Answer 10

polycystic 2

nonselective cation channel transporting Ca

Question 11

renal manifestation of ADPKD

Answer 11

gross hematuria
flank pain
kidney stones
CKD
UTIs

Question 12

extra-renal manifestation of ADPKD

Answer 12

intracranial aneurysm
hepatic and pancreatic cysts
HTN
diverticulosis

Question 13

renal manifestation of ARPKD

Answer 13

protruding abdomen

chronic renal failure: hematuria, oliguria

Question 14

extrarenal manifestation of ARPKD

Answer 14

HTN

hepatic fibrosis

Question 15

ADPKD US findings

Answer 15

enlarged kidneys with multiple cysts of varying sizes

Question 16

ARPKD US findings

Answer 16

enlarged kidneys with multiple cysts bilaterally of equal size
hepatic cysts

Question 17

diagnostic imaging of choice of PKD

Answer 17

US

Question 18

simple cyst treatment

Answer 18

observation

Question 19

multiple cyst treatment

Answer 19

increase fluid intake
ACEI or ARB for HTN
tolvaptan
avoid nephrotoxic substances

Question 20

tolvaptan

Answer 20

slows growth of kidney cysts and delays progression to ESRD

Question 21

tuberous sclerosis sx

Answer 21

seizures
respiratory compromise
recurrent hematuria
increased risk of Renal cell carcinoma
angiofibromas
skin lesions

Question 22

tuberous sclerosis

Answer 22

autosomal dominant or spontaneous mutation of tumor suppressor genes

Question 23

risk factors of getting kidney stones

Answer 23

hot climate
high protein, high salt, low K/Mg diet
increased risk with metabolic syndrome
age, obesity

Question 24

most kidney stones are

Answer 24

calcium oxalate

Question 25

type of stone that is more common after 55

Answer 25

uric acid

Question 26

type of stones that are more common in the summer

Answer 26

calcium oxalate and uric acid

Question 27

cystine stones

Answer 27

congenital defect in cystine reabsorption

hexagonal crystals

Question 28

treatment of cystine stones

Answer 28

urinary alkalinization, diet modification

Question 29

allopurinol can treat

Answer 29

uric acid stones

Question 30

ph <5 suggest ____ stones

Answer 30

uric acid

Question 31

kidney stone general s/sx

Answer 31

hematuria
colicky flank pain that radiates anteriorly
CVA tenderness
N/V

Question 32

struvite stone risk factors

Answer 32

UTI with urea splitting organisms

Question 33

management of stones < 5 mm

Answer 33

IV fluids and analgesics

tamsulosin

Question 34

management of stones > 5 mm

Answer 34

lithotripsy
ureteroscopy
percutaneous nephrolithotomy

Question 35

imaging test of choice for nephrolithiasis

Answer 35

non contrast CT abdomen and pelvis

Question 36

____ stones are usuals due to high protein foods, gout, chemo

Answer 36

uric acid