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)
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1
Q

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

A

Cystic renal disease

2
Q

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

A

Choledochal cysts

3
Q

T/F Choledochal cysts are most often intrahepatic

A

F, extrahepatic

4
Q

Classic triad of choledochal cyst

A

1) Abdominal pain 2) Jaundice 3) Mass

5
Q

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

A

F

6
Q

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

A

T

7
Q

Diagnosis of choledochal cyst is made by

A

UTZ

8
Q

Treatment of choice for choledochal cyst

A

Primary excision and Roux-en-Y choledochojejunostomy

9
Q

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

A

Risk of development of carcinoma in the residual cystic tissue

10
Q

Gene mutated in ARPKD

A

PKHD1

11
Q

More severe, autosomal dominant vs recessive polycystic kidney disease

A

Autosomal recessive

12
Q

On fetal UTZ, ARPKD appears as

A

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

13
Q

Patients with ARPKD can die in the perinatal period owing to

A

1) Renal failure 2) Lung dysgenesis

14
Q

In ARPKD, respiratory failure can result from

A

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

15
Q

Cystic dilation of the intrahepatic bile ducts

A

Caroli disease

16
Q

Definitive diagnosis of Caroli disease

A

Percutaneous transhepatic, endoscopic, or magnetic resonance cholangiography

17
Q

Caroli disease carries a significant risk of developing what cancer

A

Cholangiocarcinoma

18
Q

Congenital hepatic fibrosis occurs a part of what syndrome

A

COACH syndrome

19
Q

Congenital hepatic fibrosis has its onset in childhood and presents as

A

Hepatosplenomegaly OR bleeding secondary to portal hypertension

20
Q

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

A

T

21
Q

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

A

T

22
Q

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

A

T

23
Q

Treatment of congenital hepatic fibrosis should focus on

A

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

24
Q

Bleeding in congenital hepatic fibrosis is due to

A

Varices from portal hypertension

25
Q

APDKD is caused by mutations in 1 of 2 genes

A

PKD1 or PKD2

26
Q

MC gene mutated in APDKD

A

PKD1

27
Q

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

A

Mechanosensor in cilia

28
Q

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

A

Calcium channel

29
Q

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

A

T