Liver Disease Associated With Systemic Disorders

Question 1

IBD Associated liver disease……?

Answer 1

sclerosing cholangitis,

autoimmune hepatitis [AIH]),

drug toxicity (thiopurines, methotrexate, 5-ASA,

biologics

Question 2

Risk factors for liver disease in IBD…..?

Answer 2

malnutrition and disordered physiology (fatty liver, cholelithiasis), bacterial translocation and systemic infections (hepatic abscess, portal vein thrombosis), hypercoagulability (infarction, Budd-Chiari), and long-term complications of these liver diseases, such as ascending cholangitis, cirrhosis, portal hypertension, and biliary carcinoma

Question 3

Most common hepatobiliary disease associated with IBD …..?

Answer 3

Sclerosing cholangitis

Question 4

Sclerosing cholangitis is most common in which IBD….?

Answer 4

2–8% of adult patients with ulcerative colitis and less often in Crohn disease

Question 5

What is sclerosing cholangitis……?

Answer 5

Sclerosing cholangitis is characterized by progressive inflammation and fibrosis of segments of the intrahepatic and extrahepatic bile ducts and can progress to complete obliteration

Question 6

Sclerosing cholangitis patients are asymptomatic, and the disease is initially diagnosed by routine liver function testing……?

Answer 6

Elevated serum alkaline phosphatase (AP),

5′-nucleotidase, or γ-glutamyl transpeptidase (GGT) activities

Question 7

Sclerosing cholangitis is strongly associated with whichever hepatobiliary malignancies …..?

Answer 7

cholangiocarcinoma,

hepatocellular carcinoma,

gallbladder carcinoma

Question 8

Symptomatic therapy should be initiated for pruritus ?……..?

Answer 8

rifampin, ursodeoxycholic acid, diphenhydramine

Question 9

What is overlap syndrome or autoimmune sclerosing cholangitis (ASC) …..?

Answer 9

IBD-associated AIH can closely resemble IBD-associated sclerosing cholangitis,

Question 10

Total parenteral nutrition (TPN) can cause a variety of liver diseases which are those …..?

Answer 10

hepatic steatosis, gallbladder and bile duct damage, and cholestasis

Question 11

Risk factors for TPNassociated cholestasis…….?

Answer 11

Prolonged duration of TPN (particularly soy-based lipids), 
prematurity, 
low birthweight, 
sepsis, 
necrotizing enterocolitis, and 
short bowel syndrome

Question 12

Cystic fibrosis (CF) gene …..?

Answer 12

Mutations in the CFTR gene, which impair chloride transport across the apical membranes of epithelial cells in numerous organs (including cholangiocytes)

Question 13

Pathognomonic liver lesion in CF …..?

Answer 13

Focal biliary cirrhosis

Question 14

Pathogenesis of liver disease in Cystic fibrosis…..?

Answer 14

Blockage of biliary ductules secondary to viscid secretions results in periductal inflammation, bile duct proliferation, and increased fibrosis within focal portal tracts.

Question 15

candidate gene modifier for clinical phenotypes of CF-related liver disease that shows a strong association is …..?

Answer 15

SERPINA1.

Question 16

Liver disease is common in patients who have received hematopoietic stem cell transplantation (SCT)……?

Answer 16

Infections (viral, bacterial, or fungal);
toxicity from parenteral nutrition,
chemotherapy, or radiation;
venoocclusive disease (VOD);
graft versus host disease (GVHD); or
hemosiderosis secondary to iron overload from frequent blood transfusions.

Question 17

What is GVHD of the liver ……?

Answer 17

Hepatic GVHD is caused by immunologic reaction to bile duct epithelium, leading to a nonsuppurative cholangiti

Question 18

Lab evidence of hepatic GVHD…..?

Answer 18

In acute hepatic GVHD, serum aminotransferase levels can rise markedly in the absence of elevated bilirubin, AP, and GGT levels, mimicking viral hepatitis.

Acute hepatic GVHD can manifest both early (days 14-21) and late (>day 70) after allogeneic SCT

Question 19

What is VOD of the liver in BMT ……?

Answer 19

VOD of the liver usually develops in the first 3 wk after SCT

VOD is caused by fibrous obliteration of the terminal hepatic venules and small lobular veins, with resultant damage to the surrounding hepatocytes and sinusoids

Question 20

Risk factors for VOD in BMT ……?

Answer 20

Risk factors include trauma, high-dose conditioning regimens, coagulopathies, sickle cell anemia, leukemia, polycythemia vera, thalassemia major, hepatic abscesses, irradiation, GVHD, iron overload, preexisting liver disease, and younger age

Question 21

How VOD is different from Budd-Chiari syndrome …?

Answer 21

It is not associated with thrombus formation, in contrast with Budd-Chiari syndrome, which involves occlusion of the larger hepatic veins or inferior vena cava by a web, mass, or thrombus.

Question 22

Treatment of VOD ….?

Answer 22

Treatment for VOD with defibrotide, an agent with antithrombotic and thrombolytic properties, at doses of 20-40 mg/kg/day