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
