5.5 Summary of Cystic DOs of Kidney

Question 1

Disorder:
Inheritance: AD
Morphology: Large multicystic kidneys, liver cysts, berry aneurysms
Clinical: Hematuria, flank pain, UTI, renal stones, HTN
Outcome: Chronic renal failure at 40-60 years
Gross Kidney: enlarged diffuse cysts

Answer 1

Adult polycytic kidney disease APKD

Question 2

Disorder:
Inheritance: AR
Morphology: Enlarged, cystic kidneys at birth
Clinical: Hepatic Fibrosis
Outcome: Variable, death in infancy or childhood
Gross Kidney: Large

Answer 2

Infant/childhood polycystic kidney disease

Question 3

Disorder:
Inheritance: None
Morphology: Medullary cysts on excretory urography
Clinical: Hematuria, UTI, recurrent renal stones
Outcome: Benign
Gross Kidney: Normal

Answer 3

Medullary sponge kidney

Question 4

Disorder:
Inheritance: AR
Morphology: Corticomedullary cysts, shrunken kidneys
Clinical: Salt wasting, polyuria, growth retardation
Outcome: Progressive renal failure in childhood
Gross Kidney: Small

Answer 4

Familial Juvenile Neprhonophthisis

Question 5

Disorder:
Inheritance: AD
Morphology: Corticomedullary cysts, shrunken kidneys
Clinical Features: Salt wasting, polyuria
Outcome: Chronic renal failure-adult onset
Gross: Small

Answer 5

Adult-onset nephronophthisis

Question 6

Disorder:
Inheritance: None
Morphology: Single or several cysts normal sized kidney
Clinical Features: Microscopic hematuria
Outcome:Benign
Gross: Normal size

Answer 6

Simple Cyst

Question 7

Disorder:
Inheritance: None
Morphology: cystic degeneration in end stage kidney
Clinical Features: Hemorrhage, erythrocytosis, neoplasia
Outcome: Dependence on dialysis
Gross: small and shriveled

Answer 7

Acquired renal cystic dz

Question 8

Disorder:
Inheritance: None
Morphology: Irregular kidneys with cysts of various size
Clinical Features: associated with other renal anomalies
Outcome: Renal failure if bilateral, curable if unilateral
Gross: enlarged, normal or smaller than normal

Answer 8

Multicystic renal dysplasia