12.1 - Congenital kidney and urinary tract pathology

Question 1

horseshoe kidneys are connected at the ________ (upper / lower) pole

Answer 1

lower

Question 2

horseshoe kidney gets caught on which artery as it ascends?

Answer 2

IMA

Question 3

what are the manifestations of potter sequence?

Answer 3

• lung hypoplasia
• flat face
• low set ears
• development defects of extremities

Question 4

noninherited, congenital malformation of the renal parenchyma characterized by cysts and abnormal tissue (especially cartilage) - diagnosis?

Answer 4

dysplastic kidney

Question 5

where are the cysts located in polycystic kidney disease?

Answer 5

renal cortex and medulla

Question 6

what is the inheritance of PKD?

Answer 6

• AR: children

- AD: adults

Question 7

which form of PKD is associated with fibrosis?

Answer 7

AR PKD

Question 8

what are the sequelae of fibrosis seen in AR PKD?

Answer 8

• portal HTN

- HEPATIC cysts

Question 9

how does AD PKD present in adults?

Answer 9

HTN

Question 10

AD PKD is due to mutations in what genes?

Answer 10

• APKD1

- APKD2

Question 11

which form of PKD is associated with berry aneurysms?

Answer 11

AD PKD

Question 12

what is the inheritance of medullary cystic kidney disease?

Answer 12

AD

Question 13

what is the gross appearance of the kidneys in medullary cystic kidney disease? why?

Answer 13

• shrunken

- due to parenchymal fibrosis