XVI - The Liver, Gallbladder and Biliary Tree Flashcards Preview

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Flashcards in XVI - The Liver, Gallbladder and Biliary Tree Deck (153)
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121

An autosomal disorder caused by mutation of ATP7B gene resulting in impaired copper excretion into bile and failure to incorporate copper into ceruloplasmin

Wilson Disease(TOPNOTCH)Robbins Basic Pathology, 9th ed, p. 849

122

Presents with movement disorders (tremor, chorea, tremor),rigid dystonia, psychiatric symptoms, hemolytic anemia, green to brown deposits in Descemet membrane in the limbus

Wilson Disease(TOPNOTCH)Robbins Basic Pathology, 9th ed, p. 849

123

Most sensitive and accurate test for Wilson disease

Increase in hepatic copper content(TOPNOTCH)Robbins Basic Pathology, 9th ed, p. 850

124

Most specific screening test for Wilson Disease

Increased urinary excretion of copper copper content(TOPNOTCH)Robbins Basic Pathology, 9th ed, p. 850

125

Most characteristic laboratory finding in primary biliary cirrhosis

Antimitochondrial antibodies(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 858

126

Mechanism of physiologic jaundice of the newborn

Impaired bilirubin conjugation(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 853

127

Hereditary unconjugated hyperbilirubinemia caused by severe UGT1A1 deficiency and is fatal around the time of birth

Crigler-Najjar syndrome type 1(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 853

128

An autosomal recessive disorder caused by impaired biliary excretion of bilirubin glucoronides due to mutation in canalicular multidrug resistance protein 2(MRP2)

Dubin-Johnson syndrome(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 854

129

Most common cause of bile duct obstruction in adults

Extrahepatic cholelithiasis(gallstones)(TOPNOTCH)Robbins Basic Pathology,9th ed., p. 854

130

Histologic hallmark: influx of periductular neutrophils directly into the bile duct epithelium and lumen

Ascending cholangitis(TOPNOTCH)Robbins Basic Pathology,9th ed., p. 854

131

Most common form of cholestasis of sepsis wherein bile plugs within predominantly centrilobular canaliculi

Canalicular cholestasis(TOPNOTCH)Robbins Basic Pathology,9th ed., p. 855

132

Disorder of intrahepatic gallstone formation leading to repeated bouts of ascending cholangitis; has pigmented calcium bilirubinate stones in distended intrahepatic bile ducts

Hepatolithiasis(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 856

133

Presents with neonatal cholestasis, normal birthweight and postnatal weight gain, initially normal stools change to acholic stools.

Biliary atresia(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 857

134

Morphology: Inflammation and fibrosing stricture of the hepatic or common bile ducts; cirrhosis develops within 3-6 mos of birth if uncorrected

Biliary atresia(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 857

135

Florid duct lesion by liver biopsy, elevated alkaline phosphatase and gamma-glutamyltransferase, hypercholesterolemia,

Primary biliary cirrhosis(TOPNOTCH)Robbins Basic Pathology, 9th ed. P. 858

136

Presents with characteristic beading on radiographs of intrahepatic and extrahepatic biliary tree; strong association with IBD particularly ulcerative colitis

Primary biliary cirrhosis(TOPNOTCH)Robbins Basic Pathology, 9th ed. P. 861

137

Presents most often in children before age 10 as jaundice, recurrent abdominal pain, symptoms that are typical of biliary colic; caused by congenital dilations of CBD

Choledochal cyst(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 861

138

Presents with liver enlargement, pain, and ascites due to obstruction of two or more hepatic veins.

Budd-Chiari syndrome(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 863

139

Most important premalignant lesions for cholangiocarcinoma

Biliary intraepithelial neoplasias(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 874

140

Most common malignancy of the extrahepatic biliary tract

Carcinoma of the gallbladder(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 879

141

Most important risk factor for gallbladder cancer

Gallstones(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 879

142

Atrophic, chronically obstructed, often dilated gallbladder, containing clear secretions

Hydrops of the gallbladder(TOPNOTCH)

143

Patients with chronic hemolytic anemias, severe ileal dysfunction or bypass, and bacterial contamination of the biliary tree are at risk for developing what kind of gallbladder stones?

Pigment stones(TOPNOTCH)

144

In severe form of this condition, patient presents with micronodular cirrhosis, diabetes mellitus, and abnormal skin pigmentation.

Hemochromatosis(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 847

145

A 28 year old man with a history of IV drug abuse develops jaundice and malaise. Which of the following liver biopsy findings particularly suggests a hepatitis C infection? (A) ballooning degeneration of hepatocytes (B) ground glass appearance of hepatocyte cytoplasm, with "sanded" nuclei (C) bile duct proliferation and lymphoid aggregate formation (D) tangled skeins of pink cytoplasmic inclusions in degenerating hepatocytes, with neutrophilic infiltrates

bile duct proliferation and lymphoid aggregate formation (TOPNOTCH)Robbins Basic Pathology, 8th ed, p646-647

146

A 25 year old medical intern draws blood from a chronic hepatitis B patient sustains a needlestick injury. He forgets to consult the infection control unit, and develops jaundice 2 months later. A biopsy of his liver will show (A) hemosiderin-laden periportal hepatocytes ((B) ground glass appearance of hepatocyte cytoplasm, with "sanded" nuclei (C) bile duct proliferation and lymphoid aggregate formation (D) tangled skeins of pink cytoplasmic inclusions in degenerating hepatocytes, with neutrophilic infiltrates

ground glass appearance of hepatocyte cytoplasm, and "sanded" nuclei (TOPNOTCH)Robbins Basic Pathology, 8th ed, p646-647

147

A 45 year old male during a drinking spree gets into a fight, and is stabbed. A medicolegal autopsy is performed. Given his chronic alcoholism, his liver will likely show (A) hemosiderin-laden periportal hepatocytes (B) ground glass appearance of hepatocyte cytoplasm, with "sanded" nuclei (C) bile duct proliferation and lymphoid aggregate formation (D) tangled skeins of pink cytoplasmic inclusions in degenerating hepatocytes, with neutrophilic infiltrates

tangled skeins of pink cytoplasmic inclusions in degenerating hepatocytes (Mallory bodies), with neutrophilic infiltrates (TOPNOTCH)Robbins Basic Pathology, 8th ed, p 649-650

148

Which of the following is most likely to have nonalcoholic fatty liver disease? (A) 28 year old overweight man with dyslipidemia and family history of Type 2 DM (B) 14 year old student with alpha thalassemia with history of multiple blood transfusions since childhood (C) 33 year old teacher on his 1st month of taking anti-Koch's medication (D) 44 year old architect with a 30 pack year smoking history

28 year old overweight man with dyslipidemia and family history of Type 2 DM (insulin resistance) (TOPNOTCH)Robbins Basic Pathology, 8th ed, p 654

149

A 40 year old man with beta thalassemia and long history of blood transfusions dies of congestive heart failure. At autopsy, his liver, heart, and pancreas appear dark brown. Histologic examination of his liver shows hepatocytes with golden brown cytoplasmic granules. These granules will stain with (A) prussian blue (B) rhodanine (C) periodic acid schiff (D) hematoxylin

Prussian blue (iron stain)(TOPNOTCH)Robbins Basic Pathology, 8th ed, p 654-655

150

Patients with hemochromatosis have a 200-fold higher risk of developing which malignancy compared to the normal population?

hepatocellular carcinoma (TOPNOTCH)Robbins Basic Pathology, 8th ed, p 656