Sketchy Path: Congenital and Cystic Kidney Disease

Question 1

Horseshoe kidney is associated with _____________.

Answer 1

Turner syndrome (like the turning wheel toys behind the horseshoe game) and Wilms tumor (like the kid getting the crab toy next to the Turner wheels)

Question 2

The most common complication of horseshoe kidney is ______________.

Answer 2

hydronephrosis (like the swollen kidney in between the Turner wheels and the horseshoe game)

Question 3

Horseshoe kidney can also cause _____________.

Answer 3

nephrolithiasis, UTIs, and hematuria (like the kid throwing the stone at the dunk tank guy’s flank above the red pit; the guy’s holding the UTI cup)

Question 4

The most common renal disorder in kids is ________________.

Answer 4

renal dysplasia (like the messed up balloon that looks like a kidney)

Question 5

Renal dysplasia can present with _____________ in the kidneys.

Answer 5

mesenchymal tissue like collagen (think of the shark)

Question 6

True or false: renal dysplasia is most often bilateral.

Answer 6

False. It’s bilateral only 20% of the time.

Question 7

Renal agenesis leads to _____________.

Answer 7

Potter sequence (like the bear squished in the oligohydramnios sack)

Question 8

ARPKD is now often detected by ________________.

Answer 8

20-week ultrasounds, which can detect the renomegaly

Question 9

ARPKD results from a mutation in _____________.

Answer 9

fibrocystin (like the mom’s fibrous pants)

Question 10

Where in the kidney do cysts develop in ARPKD?

Answer 10

In the collecting duct (like how the mom and child are stepping into the collecting duct)

This is also why the cysts will be lined with cuboidal epithelium (like the cuboidal tiles lining the duct).

Question 11

If not caught by ultrasound, how can ARPKD be diagnosed?

Answer 11

• Potter syndrome (bear in the duct)
• Bilateral flank masses (kid grabbing mom’s sides)
• HTN (mom fuming)
• Ascites from portal HTN from the obstructed bile duct

Question 12

In addition to renal symptoms, ARPKD also causes ______________.

Answer 12

intrahepatic bile duct cysts (like the cystic balloons in the liver tree right about where the bile duct would be)

Question 13

ADPKD results from defects in which protein?

Answer 13

Polycystin (like the polygons on the man’s sweater)

Question 14

In which tissues of the kidney do cysts appear in ADPKD?

Answer 14

Throughout the nephron (like how the dominant balloons are blocking the nephron map)

Question 15

ADPKD is associated with _______________.

Answer 15

• berry aneurysms that can rupture and caused SAH (like the ruptured balloon on the guy’s head)
• pancreatic cysts (like his pancreas-shaped fanny pack)
• diverticulosis (like the intestine belts he’s got)

Question 16

What is medullary sponge syndrome?

Answer 16

A relatively benign disorder in which cysts develop in the renal medulla (like the happy guy with renal sponges –he’s got no issues)