Cystic Kidney Diseases

Question 0

What do gross sections of Childhood PCKD look like in comparison to adult PCKD?

Answer 0

Smaller and smoother

Question 1

What is the inheritance pattern of childhood polycystic kidney disease?

Answer 1

Autosomal recessive

Question 2

What is dilated in childhood PCKD?

Answer 2

Collecting tubules

Question 3

What is the most important organ affected by childhood PCKD other than the kidneys? What complication arises?

Answer 3

Liver, leads to hepatic fibrosis

Question 4

What disease consists of dilation in collecting ducts of the medulla?

Answer 4

Medullary Sponge Kidney

Question 5

What do the cysts look like on micro examination in Medullary Sponge disease?

Answer 5

Lined by cuboidal or transitional epithelium

Question 6

What is one of the more common causes of kidney failure in children, caused by medullary cysts, cortical tubular atrophy and interstitial fibrosis?

Answer 6

Nephronophthisis-Uremic Cystic Disease Complex

Question 7

How do children with NUMCDC present?

Answer 7

Polyuria polydipsia

Question 8

What kidney disease can result in a renal cell carcinoma rate of 7% within 10 years?

Answer 8

Acquired (dialysis associated) cystic disease

Question 9

What is the morphology of a simple kidney cyst? (size, substance, lining)

Answer 9

1-5 cm
Fluid filled
Lined with simple cuboidal or flattened epithelium