Polycystic Kidney Disease

Question 1

What is the etiology of pkd?

Answer 1

Autosomal dominant Inherited disorder

Question 2

Describe how a pk looks

Answer 2

multiple, bilateral, grape like clusters of fluid filled cysts that enlarge the kidneys

Question 3

How do the cysts affect the kidney?

Answer 3

Cysts eventually compress and replace functioning renal tissue

Question 4

What genes are affected in pkd?

Answer 4

ADPKD1 & ADPKD2

Question 5

What is the most common inherited disease of the kidneys?

Answer 5

PKD

Question 6

How prevalent is PKD?

Answer 6

1 in 800 live births

Question 7

What percentage of ESRD is attributed to PKD?

Answer 7

10%

Question 8

How long can pple live without developing symptoms?

Answer 8

Most patients live for several decades without developing symptoms

Question 9

What structure of the kidney do the cysts grow out of?

Answer 9

Nephrons

Question 10

True/False. The kidneys enlarge along with the cysts

Answer 10

True

Question 11

Approximately how many cysts can be present in a kidney?

Answer 11

THousands

Question 12

How much can a cyst-filled kidney weigh?

Answer 12

20-30 lbs!

Question 13

What disorder commonly occurs along with PKD?

Answer 13

HTN by age 20-30

Question 14

What disorder are pkd pts at a high risk for?

Answer 14

cerebral aneurysm

Question 15

Sxs of PKD

Answer 15

1. Abdominal or Flank Pain 2.Hematuria: Microscopic or gross
2. Family history PKD
3. HTN
4. Palpable kidneys
5. +/- hepatic or pancreatic cysts
6. Most cases are asymptomatic & physical condition appears normal for many years, so the disease can go unnoticed

Question 16

What are the ua results for PKD?

Answer 16

Hematuria

Proteinuria

Question 17

What are the renal us results for pkd?

Answer 17

Confirms diagnosis

1. < 30 yrs: 2 or more cysts has 89% sensitivity
2. 30-59 yrs: 2 or more cysts in each kidney has sensitivity of 100%
3. 60 yrs or >: 4 or more cysts in each kidney has sensitivity of 100%

Question 18

What genetic studies can be performed for pkd?

Answer 18

ADPKD1 and ADPKD2

Question 19

What tx is used for abd or flank pain?

Answer 19

Pain management

Abd or flank pain caused by infection, bleeding or kidney stones

Question 20

What tx is used for hematuria?

Answer 20

Hydration and bed rest

Question 21

What tx is used for bp management?

Answer 21

ACE I or ARB to suppress RAAS

Question 22

What tx is used for infection?

Answer 22

abx prn

Question 23

What is used to rule out cerebral aneurysm in pkd pts?

Answer 23

Neurosurgery

Question 24

What is the eventual tx for all pkd pts?

Answer 24

Kidney transplant or dialysis

Question 25

true/false. Kidney transplants will develop cysts in a pt with pkd

Answer 25

False. Healthy—non-PKD—kidneys transplanted into PKD patients do not develop cysts

Question 26

How long does renal failure appear after symptoms occur?

Answer 26

In most cases of PKD, renal failure develops about 10 years after symptoms appear