Renal Cystic Diseases Flashcards Preview

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Flashcards in Renal Cystic Diseases Deck (14):
1

What is the most common form of inheritance for inherited polycystic kidney disease?

autosomal DOMINANT (ADPKD)

less common is autosomal recessive (ARPKD)

2

What is the most common genetic cause of CKD?

ADPKD

3

Common clinical presenting features of ADPKD

abdominal pain, palpable kidneys, HTN!!!!, infection, kidney stones

4

Complications of ADPKD

BERRY ANEURYSM!!!!!!!!!!!!
infection/bleeding of renal cysts
renal failure (advanced disease)
heart valve abnormalities (mitral valve prolapse)
cysts in other organs
diverticula
hernias (abdominal/inguinal)

5

How to dx ADPKD

renal U/S (multiple cysts on kidney)
can do CT or MRI

6

Treatment for ADPKD

No cure :(
just drain cysts if symptomatic or treat infections that happen. Also control HTN

7

Other name for autosomal recesssive PKD?

infantile polycystic kidney disease (usually seen in newborns)

8

Clinical features of ARPKD

-renal cysts predominantly in renal colecting ducts
-ALWAYS HAS LIVER INVOLVEMENT (portal HTN, hepatic fibrosis, cholangitis)
- abdominal distension due to increased kidney size
- HTN
- enlarged kidneys -> pulmonary hypoplasia + limited diaphragmatic development in newborns
- Potter syndrome (oligohydramnios, limb hypoplasia, abnormal facies)

9

How to dx ARPKD

ultrasound during pregnancy (finding of cysts when none of parents have cysts)

10

How to treat ARPKD

no cure
manage respiratory complications and ESRD with renal replacement

11

Having medullary sponge kidney puts you at risk for what?

kidney stones, UTIs, or hematuria

BUT CAN ALSO BE ASYMPTOMATIC

12

How to diagnose medullary sponge kidney

IVP!!

will show cystic dilation of collecting ducts

13

Medullary sponge kidney is associated with what endocrine abnormalities

hyperparathyroidism and parathyroid adenoma

14

What is a common finding on renal ultrasound (more than 50% of people have them over age 50)

simple renal cysts

no treatment necessary