1 Treatment for Criggler Najar Type 2
Phenobarbitone
2Which disease is associated with Ulcerative colitis
Primary sclerosing cholangitis
3Unconjugated Bilirubin __> Conjugated bilirubin,Enzyme
UDP Glucoronyl Transferase
4Radiology findings of PSC
Onion skinning,Prunning of small bile ducts,Strictures and beading of large bile ducts
5Complete deficiency of UGT
Criggler Najar Type 1
6Antibodies seen in PBC
AMA ANA
7Antibodies associated with PSC
p ANCA
8What is the difference between dubin johnson syndrome and rotor syndrome
Dubin johnson has dark pigmented liver due to the presence of Epinephrine whereas non pigmenetd liver is seen in Rotor syndrome
9Which form of hyperbilirubinemia is typically associated with Gilbert syndrome and Crigler-Najjar syndrome?
Unconjugated hyperbilirubinemia (impaired conjugation)
10What are the three main pathophysiological mechanisms of unconjugated hyperbilirubinemia?
Increased production, impaired hepatic uptake, or decreased conjugation
11What is the enzyme responsible for conjugating bilirubin in the liver?
UDP-glucuronosyltransferase (UGT1A1)
12Which hereditary disorder of conjugated hyperbilirubinemia causes the liver to appear black due to dark pigment accumulation?
Dubin-Johnson syndrome
13Which hereditary disorder of conjugated hyperbilirubinemia involves a normal-appearing liver on biopsy and can be distinguished by high urinary coproporphyrin excretion?
Rotor syndrome
14What major clinical risk is associated with very high levels of unconjugated bilirubin in newborns?
Kernicterus (bilirubin-induced encephalopathy)
15In a patient with extrahepatic biliary obstruction (e.g., gallstones in the common bile duct), which form of bilirubin predominates in the serum?
Conjugated hyperbilirubinemia
16What is the effect of administering Phenobarbital in Crigler-Najjar syndrome Type II?
It reduces serum bilirubin levels (by inducing UGT1A1 activity)
17Which form of hyperbilirubinemia is also known as direct bilirubinemia?
Conjugated hyperbilirubinemia
18Which form of bilirubin is water-soluble and excreted in urine?
Conjugated bilirubin
19What is the end stage of all chronic liver diseases?
Cirrhosis (characterized by diffuse hepatic fibrosis and regenerative nodules)
20What is the most common cause of acute liver failure in Western countries?
Acetaminophen (paracetamol) overdose
21What is the primary function of the gallbladder?
Storage and concentration of bile
22Which two viral infections are typically acute and self-limited, rarely progressing to chronic liver disease?
Hepatitis A (HAV) and Hepatitis E (HEV)
23Which viral infections are most likely to progress to chronic hepatitis and potentially cirrhosis/HCC?
Hepatitis B (HBV) and Hepatitis C (HCV)
24What histopathological finding is characteristic of chronic hepatitis B infection?
"Ground-glass" hepatocytes (due to accumulation of HBsAg in the endoplasmic reticulum)
25What is the name for the highly malignant primary liver cancer commonly associated with chronic hepatitis and cirrhosis?
Hepatocellular Carcinoma (HCC)
26What type of gallstones are most likely to form when bile is supersaturated with cholesterol?
Cholesterol gallstones
27What are the three main types of jaundice (hyperbilirubinemia) based on the site of pathology?
Pre-hepatic, Hepatic, and Post-hepatic
28What is the term for scarring of the liver caused by repeated cycles of damage and repair?
Fibrosis
29What is the definition of cirrhosis?
The end stage of chronic liver disease characterized by diffuse fibrosis and the formation of regenerative nodules
30What are the three main morphological patterns of cirrhosis based on nodule size?
Micronodular, macronodular, and mixed
31What is the most common cause of cirrhosis in the Western world?
Alcoholic liver disease (now often co-equal with Non-Alcoholic Steatohepatitis/NASH)
32What is the major complication of portal hypertension that can lead to life-threatening hemorrhage?
Esophageal varices
33What is the most common primary malignant tumor of the liver?
Hepatocellular Carcinoma (HCC)
34What conditions are the major risk factors for developing HCC?
Chronic Hepatitis B (HBV) or C (HCV) infection and cirrhosis (from any cause)
35What is the characteristic serum tumor marker often elevated in HCC?
Alpha-fetoprotein (AFP)
36What is the most common overall malignant tumor found in the liver?
Metastases (from primary cancers elsewhere, such as colon, lung, or breast)
37What is a benign liver tumor composed of blood vessels, and the most common benign liver tumor?
Hepatic hemangioma
38What is a common benign liver tumor associated with the use of oral contraceptives?
Hepatic Adenoma (or Liver Cell Adenoma)
39What is the major clinical consequence of hepatic encephalopathy in cirrhosis?
Neuropsychiatric abnormalities due to the liver's inability to clear ammonia and other toxins
40What is the name for the development of renal failure in patients with severe cirrhosis and portal hypertension?
Hepatorenal syndrome