Ch. 365 - Cystic Diseases of the Biliary Tract and Liver

Question 1

Usually associated with cystic lesions of the liver and often determines the clinical presentation and prognosis

Answer 1

Cystic renal disease

Question 2

Congenital dilatations of the common bile duct that can cause progressive biliary obstruction and biliary cirrhosis

Answer 2

Choledochal cysts

Question 3

T/F Choledochal cysts are most often intrahepatic

Answer 3

F, extrahepatic

Question 4

Classic triad of choledochal cyst

Answer 4

1) Abdominal pain 2) Jaundice 3) Mass

Question 5

T/F An abdominal mass is commonly palpable if the patient has a choledochal cyst

Answer 5

F

Question 6

The classic triad of choledochal cyst is found in majority of patients

Answer 6

T

Question 7

Diagnosis of choledochal cyst is made by

Answer 7

UTZ

Question 8

Treatment of choice for choledochal cyst

Answer 8

Primary excision and Roux-en-Y choledochojejunostomy

Question 9

Simple drainage for the management of choledchosal cyst is less satisfactory compared to primary excision and Roux-en-Y owing to

Answer 9

Risk of development of carcinoma in the residual cystic tissue

Question 10

Gene mutated in ARPKD

Answer 10

PKHD1

Question 11

More severe, autosomal dominant vs recessive polycystic kidney disease

Answer 11

Autosomal recessive

Question 12

On fetal UTZ, ARPKD appears as

Answer 12

Large echogenic kidneys, describes as “bright” with low or absent amniotic fluid (oligohydramnios)

Question 13

Patients with ARPKD can die in the perinatal period owing to

Answer 13

1) Renal failure 2) Lung dysgenesis

Question 14

In ARPKD, respiratory failure can result from

Answer 14

1) Compression of chest by enlarged kidneys 2) Fluid retention 3) Pulmonary hypoplasia

Question 15

Cystic dilation of the intrahepatic bile ducts

Answer 15

Caroli disease

Question 16

Definitive diagnosis of Caroli disease

Answer 16

Percutaneous transhepatic, endoscopic, or magnetic resonance cholangiography

Question 17

Caroli disease carries a significant risk of developing what cancer

Answer 17

Cholangiocarcinoma

Question 18

Congenital hepatic fibrosis occurs a part of what syndrome

Answer 18

COACH syndrome

Question 19

Congenital hepatic fibrosis has its onset in childhood and presents as

Answer 19

Hepatosplenomegaly OR bleeding secondary to portal hypertension

Question 20

T/F Hepatocellular function is usually well preserved in congenital hepatic fibrosis

Answer 20

T

Question 21

T/F Liver biopsy is rarely required for the diagnosis of congenital hepatic fibrosis

Answer 21

T

Question 22

T/F Serum albumin level and prothrombin time are normal in congenital hepatic fibrosis

Answer 22

T

Question 23

Treatment of congenital hepatic fibrosis should focus on

Answer 23

1) Control of bleeding from esophageal varices 2) Aggressive antibiotic treatment of cholangitis

Question 24

Bleeding in congenital hepatic fibrosis is due to

Answer 24

Varices from portal hypertension

Question 25

APDKD is caused by mutations in 1 of 2 genes

Answer 25

PKD1 or PKD2

Question 26

MC gene mutated in APDKD

Answer 26

PKD1

Question 27

Polycystin-1, protein encoded by mutated gene in ADPKD, functions as

Answer 27

Mechanosensor in cilia

Question 28

Polycystin-2, protein encoded by mutated gene in ADPKD, functions as

Answer 28

Calcium channel

Question 29

T/F Autosomal dominant polycystic liver disease: Girls are more commonly affected than boys, and the cysts often enlarge during pregnancy

Answer 29

T