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28 Cards in this Set

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  • Back

Risk factors for gallstones

4 F’s


Forty


Female


Fertile


Fat

What is acalculous cholecystitis?

Due to gallbladder stasis, hypoperfusion or infection (CMV)


Seen in critically ill patients

What is Murphy sign?

Inspiratory arrest on RUQ palpation due to pain.


Pain may radiate to right shoulder (due to irritation of phrenic nerve)

What is a Rokitanksy Achoff sinus?

Herniation of gallbladder mucosa into the muscular wall.


Occurs in chronic cholecystitis

What is a porcelain gallbladder?

A shrunken, hard gallbladder due chronic inflammation, fibrosis & dystrophic calcification


Late complication of chronic cholecystitis


Tx - prophylactic cholecystectomy due to high rates of gallbladder carcinoma

What is Charcot’s triad of ascending cholangitis?

Jaundice, fever & RUQ pain

What is Reynolds pentad?

Occurs in ascending cholangitis


Fever, jaundice, right upper quadrant pain (Charcot triad) + altered mental status and shock (hypotension)

What is gallstone ileus?

A gallstone enters and obstructs the small bowel (ileocecal valve-narrowest part)


Due to cholecystitis with a fistula formation between the gallbladder and the small bowel


Can see air in the biliary tree (pneumobilia)

What is Gilbert syndrome?

Due to mildly decreased UDP-glucuronosyltransferase leading to increased unconjugated bilirubin.


Not clinically significant. Jaundice during stress


Autosomal recessive

What is Crigler-Najjar syndrome?

Type 1 is absent UDP-glucuronosyltransferase. Presents early in life, leads to kernicterus, patients die within a few years.


Increased UCB


Tx - plasmapheresis & phototherapy. Liver transplant is curative


Autosomal recessive


(Type II is less severe & responds to phenobarbital which inc liver enzyme synthesis)

What is Dubin-Johnson?

Conjugated hyperbilirubinemia due to deficiency of bilirubin canalicular transport protein (defective liver excretion)


Autosomal recessive


Liver is dark. Not clinically significant

What is Rotor syndrome?

Similar to Dubin-Johnson syndrome but milder without black liver


Due to impaired hepatic uptake & excretion

What are the clinical features of liver cirrhosis?

Portal hypertension leads to:


Ascites, Congestive splenomegaly/hypersplenism, Portosystemic shunts, Hepatorenal syndrome


Decreased detoxification leads to:


Mental status changes, asterixis & eventual coma (due to increased serum ammonia)


Gynecomastia, spider angioma, palmar erythema due to hyperestrinism


Jaundice


Decreased protein synthesis:


Hypoalbuminemia with edema, coagulopathy

What is a Mallory-Denk body?

A cytoplasmic inclusion found in hepatocytes (damaged intermediate filaments)


Highly eosinophilic & classically found in the livers of people suffering from alcholic liver disease

What are the forms of alcoholic liver disease?

Hepatic steatosis


Alcoholic hepatitis


Hepatic cirrhosis

What are possible complications of hemochromatosis?

Classic triad - cirrhosis, secondary diabetes mellitus & skin pigmentation (“bronze diabetes”)


Cardiac arrhythmia, restrictive and dilated cardiomyopathy, hypogonadism, arthropathy.


HCC is a common cause of death

What cause primary hemochromatosis?

Autosomal recessive C282Y mutation on the HFE gene on chromosome 6.


Leads to abnormal iron sensing & increased intestinal absorption


Associated HLA-A3

What causes secondary hemochromatosis?

Chronic transfusion therapy (eg, B-thalassemia major)

What can we use to treat Wilson disease?

Chelation with penacillamine or trientine


Oral zinc

What is Wilson disease?

Autosomal recessive mutations in hepatocyte copper-transporting ATPase (ATP7B gene on chr 13)


Leading to lack of copper transport into bile & lack of copper incorporation into apoceruloplasmin


Copper accumulates esp in liver, brain, cornea, kidneys

What is primary biliary cirrhosis?

Autoimmune granulomatous destruction of intrahepatic bile ducts


Classically arises in middle aged wonen & is associated with other autoimmune diseases


Etiology is unknown. Antimitochondrial antibody is seen

What is secondary biliary cirrhosis?

Caused by extrahepatic biliary obstruction leading to increased pressure in intrahepatic ducts. This leads to injury/fibrosis & bile stasis.


Can be caused by gallstones, biliary strictures, pancreatic carcinoma etc

What is primary sclerosing cholangitis?

Inflammation & fibrosis of intrahepatic and extrahepatic bile ducts.


Leads to periductal “onion skin” fibrosis -> alternating strictures & dilations with “beaded” appearance on ERCP & MRCP


Unknown etiology


Associated with ulcerative colitis


Increased risk of cholangiocarcinoma

What is Reye syndrome?

Rare, often fatal childhood hepatic encephalopathy. Seen in children with viral illness (VZV & influenza esp) who take aspirin.


Reye of sunSHINE:


Steatosis of liver/hepatocytes, Hypoglycemia/Hepatomegaly, Infection, Not awake (coma), Encephalopathy


Plus nausea & vomiting

Risk factors for hepatocellular carcinoma

Chronic hepatitis - HBV, HCV


All causes of cirrhosis - alcoholic & nonalcoholic fatty liver disease, autoimmune disease, hemochromatosis, A1AT deficiency, Wilson disease


Carcinogens like aflatoxin from Aspergillus

What serum marker is associated with HCC?

alpha-fetoprotein (AFP)

Which cancers commonly metastasize to the liver?

Colon, pancreas, lung, breast

What is an angiosarcoma (liver)?

Malignant tumour of endothelial origin.


Associated with exposure to arsenic & vinyl chloride