Hypertensive And Cystic Kidney Diseases

Question 0

Autosomal dominant polycystic kidney disease

Answer 0

Adults Asymptomatic until age 40
PKD1 & PKD2 Polycystins
abnormal tubular growth hence cyst formation
enlarged bilateral kidneys with bubbly cysts
heart valve probs & cerebral aneurysm

Question 1

Simple cysts

Answer 1

1 to 5 cm
no clinical significance
in cortex only

Question 2

Autosomal recessive polycystic kidney disease

Answer 2

Rare but in kids
serious onset at birth causing death otherwise get portal hypertension
PKHD1 Mutation Fibrocystin spongelike cysts cortex & medulla
Enlarged bilateral kidneys with smooth surface

Question 3

Medullary sponge disease

Answer 3

Adults

normal renal function but some hematuria

Question 4

Nephronophthisis & Medullary Cystic Disease

Answer 4

4 variants: infant, juvenile, adolescent nephronopththisis and medullary cystic disease
Nephrocystin involved
initially presents with polyuria & polydipsia
Cysts at corticomedullary junction

Question 5

Acquired cystic disease

Answer 5

Associated with hemodialysis

has calcium oxalate crystals