Cystic Kidney Dz

Question 1

Renal cystic disease

Answer 1

Characterized by epithelium-lined cavities filled with fluid or semisolid debris within the kidneys.

Question 2

Simple cysts

Answer 2

Often asymptomatic and incidental findings.
Increased frequency with age
50% of pop over 50 has them
US and CT can differentiate malignant from benign.

Question 3

Autosomal Dominant Polycystic kidney dz

Answer 3

Adult PKD
Most common hereditary renal dz in US (500,000)
Characterized by large cysts
Pt’s eventually die from renal failure
Clinical manifestation rarely occur before mid-twenties, so it is often passed on.

Question 4

Autosomal Recessive polycystic kidney dz (ARPKD)

Answer 4

Referred to as infantile or childhood PKD.
Abdominal mass in neonate or infant
Can cause renal failure

Question 5

ADPKD S/S

Answer 5

Acute abd flank pain, back pain
Hematuria
Micro, then gross
Non-specific dull lumbar pain
Sharp localized pain from rupture

Question 6

ADPKD complications

Answer 6

UTI
Pyelonephritis
Cyst infxns
HTN

Question 7

Associated disorders to ADPKD

Answer 7

Multiple hepatic cysts
Mitral valve prolapse
Cerebral aneurysms
Diverticulosis

Question 8

Best imaging study for ADPKD

Answer 8

Renal US
Multiple, bilateral cysts
Renal enlargement

Question 9

ADPKD Tx

Answer 9

HTN tx
Infxn mgmt (bactrim, cipro)
Dialysis for ESRF

Question 10

ARPKD (infantile) Presentation

Answer 10

Hx of oligohydramnios (lack of amniotic fluid)
Respiratory distress
Compromised renal fxn
HTN, UTI, Polyuria

Question 11

Acquired cystic kidney Dz

Answer 11

Development of cysts in pts with chronic renal failure.
Annual screening for pt’s on long-term dialysis
CT scan is imaging of choice

Question 12

Medullary cystic disorders

Answer 12

Rare autosomal dominant dz

Part of a juvenile cystic dz complex

Question 13

Medullary cystic disorder presentation

Answer 13

anemia
nocturia
low urine osmols
eye deformities
short stature
failure to thrive
Low BP
Renal failure (end stage)

Question 14

Medullary cystic disorder prognosis

Answer 14

ESRF during adolescence or early childhood

Question 15

Medullary cystic disorder diagnosis

Answer 15

Very small cysts

Question 16

Medullary cystic disorder Tx

Answer 16

Supportive
Control HTN
Dialysis
Hydration
Transplantation (definitive)

Question 17

Medullary sponge kidney

Answer 17

Common, benign disorder
incidental finding on abd radiography
Results from passage of renal calculus
does NOT result in renal failure

Question 18

Medularry sponge kidney dx and tx

Answer 18

Dx: Intravenous pyelogram

• Bouquet of flowers, bunch of grapes
  treatment: UTI, hydration