SU2M - Renal Cystic Diseases

Question 0

ESRD in ADPKD?

Answer 0

• course of the disease is variable
• 50% develop ESRD by their late 50s or 60s –> the remainder of the pts have a normal life span
• ESRD develops bc of the recurrent episodes of pyelonephritis and nephrolithiasis

Question 1

What is the most common genetic cause of chronic kidney disease?

Answer 1

-autosomal dominant polycystic kidney disease

Question 2

5 things that ADPKD presents with?

Answer 2

1. Pain
2. Hematuria
3. Infection
4. HTN - 50% of cases
5. Kidney stones

Question 3

Complications/associated findings of ADPKD?

Answer 3

1. Intracerebral berry aneurysm –> 5-20% of cases, most do not rupture
2. Infection of renal cysts or bleeding into the cysts
3. Renal failure (later in the dz)
4. Kidney stones
5. Heart valve abnormalities (esp mitral valve prolapse)
6. Cysts in other organs (liver, spleen, pancreas, brain
7. Diverticula
8. Hernias (abdominal/inguinal)

Question 4

ADPKD: tx?

Answer 4

1. No curative tx available
2. Drain cysts if symptomatic
3. Tx infection w/ antibiotics
4. Control HTN

Question 5

5 Clinical features of ARPKD?

Answer 5

1. Liver involvement –> always present, can include portal HTN and cholangitis
2. Increased kidney size –> can cause severe abdominal distention
3. Pulmonary insufficiency –> secondary to pulmonary hypoplasia and enlarged kidneys, which limit diaphragmatic mvmnt
4. HTN
5. Potter’s syndrome –> often present with this

Question 6

Leading cause of morbidity and mortality in the neonatal period with ARPKD?

Answer 6

-pulmonary insufficiency

Question 7

Potter syndrome: 3 features?

Answer 7

1. Hypoplasia of the lungs
2. Limb abnormalities –> ex. Club feet
3. Characteristic abnormal facies
   * * associated with oligohydramnios!

Question 8

ARPKD: tx?

Answer 8

1. No curative tx available
2. Manage respiratory issues in newborns
3. Tx ESRD with renal transplant

Question 9

Medullary sponge kidney: what is it? What is it associated with?

Answer 9

• cystic dilation of the collecting ducts

- thought to be associated with hyperparathyroidism and parathyroid adenoma

Question 10

Medullary sponge kidney: ssx (4)?

Answer 10

1. Hematuria
2. UTIs
3. Nephrolithiasis
4. Asymptomatic

Question 11

Medullary sponge kidney: tx?

Answer 11

• no tx necessary
• tx recurrent UTIs
• prevent renal stone formation

Question 12

Simple renal cysts: what are they? Ssx? Tx?

Answer 12

• very common
• incidence increases with age
• can be single or multiple
• usually asymptomatic
• no tx necessary