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

Nelson - The Digestive System > Ch. 365 - Cystic Diseases of the Biliary Tract and Liver > Flashcards

Flashcards in Ch. 365 - Cystic Diseases of the Biliary Tract and Liver Deck (29):
1

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

Cystic renal disease

2

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

Choledochal cysts

3

T/F Choledochal cysts are most often intrahepatic

F, extrahepatic

4

Classic triad of choledochal cyst

1) Abdominal pain 2) Jaundice 3) Mass

5

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

F

6

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

T

7

Diagnosis of choledochal cyst is made by

UTZ

8

Treatment of choice for choledochal cyst

Primary excision and Roux-en-Y choledochojejunostomy

9

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

Risk of development of carcinoma in the residual cystic tissue

10

Gene mutated in ARPKD

PKHD1

11

More severe, autosomal dominant vs recessive polycystic kidney disease

Autosomal recessive

12

On fetal UTZ, ARPKD appears as

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

13

Patients with ARPKD can die in the perinatal period owing to

1) Renal failure 2) Lung dysgenesis

14

In ARPKD, respiratory failure can result from

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

15

Cystic dilation of the intrahepatic bile ducts

Caroli disease

16

Definitive diagnosis of Caroli disease

Percutaneous transhepatic, endoscopic, or magnetic resonance cholangiography

17

Caroli disease carries a significant risk of developing what cancer

Cholangiocarcinoma

18

Congenital hepatic fibrosis occurs a part of what syndrome

COACH syndrome

19

Congenital hepatic fibrosis has its onset in childhood and presents as

Hepatosplenomegaly OR bleeding secondary to portal hypertension

20

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

T

21

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

T

22

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

T

23

Treatment of congenital hepatic fibrosis should focus on

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

24

Bleeding in congenital hepatic fibrosis is due to

Varices from portal hypertension

25

APDKD is caused by mutations in 1 of 2 genes

PKD1 or PKD2

26

MC gene mutated in APDKD

PKD1

27

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

Mechanosensor in cilia

28

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

Calcium channel

29

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

T