Renal Path 1

Question 1

synthesize and maintain the GBM

Answer 1

glomerular endothelial cells

Question 2

birth presentation ARPKD

Answer 2

perinatal mortality

potter’s facies

pulmonary hypoplasia

Question 2

extra-renal manifestations ARPKD

Answer 2

hepatic fibrosis

cholangitis - (infxn of billiary tract)

portal HTN > esophogeal varices, gi bleeding

Question 2

cyst morpholohy AD PKD

Answer 2

abnromal differentiation of epithelial cells

high proliferation

secretion of fluid into cyts > loss of connection to functioning neprhons

abnormal matrix and fibrosis

Question 4

genetics of autosomal recessive PKD

Answer 4

PKHD1 on chromosome 6p21

Question 5

treatment goals ADPKD

Answer 5

slow progress to ESRD (HTN, treat infections)

control pain + symptoms

renal replacement

Question 6

renal manifestations ADPKD

Answer 6

hematuria+ mild proteinuria

HTN

progressive kidney failure (50% ESRD by 57)

infection

stones

pain

Question 7

presentation medullary spogne kidney

Answer 7

dilated medullary and papillary collecting duct

spongy appearance on pyelogram

reccurent UTI

kidney stones

hematuria

Question 8

diagnosis autosomal recessive PKD

Answer 8

in utero ultrasound

large hyperechoic kdineys,

oligohydramios

decreased urine in fetal bladder

Question 9

extra renal manifestations ADPKD

Answer 9

hepatic cyst

intracranial naeurysm

cardiac vavular abnml (mitral prolapse)

IVC thrombosis

inguinal and umbilical hernias

pancreatic cysts

(all due to abnml basement membranes)

Question 10

morphology acquired cystic diseasse

Answer 10

clear, fluid filled cysts

uni or miltiocular cysts

cortext usually, though may involve medulla or c-m juntion

Question 12

genetic AD PKD

Answer 12

PKD1 on chr 16 (90%)

PKD2 on chr 4

(2 progresses to renal failure at a later age)

Question 13

inheiritance medulalry sponge kidney

Answer 13

none

Question 14

contrindications renal biopsy

Answer 14

bleeding disorders

abnormalities (eg solitary kidney)

Question 15

diagnosis and presentation ADPKD

Answer 15

systematic presentaion: flank pain and hematuria

bilateral cysts on imaging study

screening with ultrasound (<30 = 2 cysts, 30-59 at least two in each kidney, >60 at least 4 bilaterally)

Question 16

kidney morphology ARPKD

Answer 16

smooth kidney with numerous small cysts

cylindrical cysts extend radially thorugh cortext

cysts linned with cuboidal epithelium

posible epithelial hyperplasia

normal glomeruli

Question 17

presentation acquired cystic disease

Answer 17

usually asymptomatic

bleeding or pain

Question 18

association horsekidney

Answer 18

increased incidence with turner syndrome

increased risk of infection and kidney stones (due to ureter orientation)

Question 19

association acquired kidney disease

Answer 19

increased risk of paillary cell carcinoma

Question 20

indications renal biopsy

Answer 20

persistent glomerular hematuria

peristent nephrotic range proteinuria

unexplained renal failure

renal transplant rejection