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Liver disorders Flashcards

1
Q

Isolated unconjugated hyperbilirubinemia with negative workup for hemolysis

A

Gilbert syndrome

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2
Q

AST:ALT ratio in chronic viral hepatitis and NAFLD

A

<1

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3
Q

AST:ALT ratio of >2:1 is suggestive of what?

A

Alcoholic liver disease

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4
Q

AST:ALT ratio of >3:1 is highly suggestive of what?

A

Alcoholic liver disease

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5
Q

Liver enzyme located in the ER and in bile duct epithelial cells. It is elevated in cholestatic damage

A

GGT

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6
Q

Type of steatosis seen in Reye syndrome

A

Microvesicular

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7
Q

Type of steatosis seen in alcoholic fatty liver

A

Combined

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8
Q

Accumulation of green-brown plugs of pile pigment in hepatocytes and dilated canaliculi

A

Cholestasis causing liver injury

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9
Q

Swollen foamy appearance of hepatocyte cytoplasm (feathery degeneration) is caused by what?

A

Accumulation of bile salts in hepatocytes due to cholestasis

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10
Q

Retention of bilirubin, bile salts, and cholesterol associated with elevated levels of conjugated bilirubin, jaundice, and pruritus.

A

Cholestasis

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11
Q

Serum findings in cholestasis

A

Increased ALP and GGT

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12
Q

Hepatocyte swelling, cytoplasmic clearing, and clumping of intermediate filaments associated with alcoholic steatohepatitis and viral hepatitis

A

Ballooning degeneration

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13
Q

Mallory hyaline

A

Prominent clumping of intermediate filaments in hepatocytes

Associated with steatohepatitis

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14
Q

Causes of liver necrosis

A

Ischemic injury
Oxidative stress
Severe viral infection
Autoimmune hepatitis
Secondary vascular insults

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15
Q

Types of liver necrosis

A

Confluent –> zonal loss
Bridging
Panacinar

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16
Q

Causes of abnormal apoptosis of hepatocytes

A

Viral infection
Fatty liver disease
Drug-induced liver injury

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17
Q

Cell involved in scar deposition of the liver by differentiating into myofibroblasts in liver injury

A

Stellate cell

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18
Q

Stain used to identify collagen bundles in liver cirrhosis

A

Masson trichrome staining

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19
Q

Common end point of liver injury characterized by fibrous septa encircling nodules of regenerative hepatocytes

A

Cirrhosis

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20
Q

Amount of functional capacity of liver that must be lost before hepatic failure appears

A

80-90%

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21
Q

Timing of acute liver failure

A

Occurs within 26 wks of initial liver injury

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22
Q

Massive liver necrosis develops in 2-3 wks causing very rapid onset of liver failure

A

Fulminant liver failure

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23
Q

Conditions associated with acute liver failure

A

Encephalopathy
Coagulopathy
Other organ failure

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24
Q

Causes of acute liver failure

A

Drug/toxin
Autoimmune hepatitis
HAV, HBV, or HEV
Galactosemia
Malignancies

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25
Q

Most common malignancies associated with acute liver failure

A

Leukemia
Lymphoma
Breast ca
Colon ca

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26
Q

Liver is small, shrunken, bile-stained, soft, and congested. Microscopy shows broad regions of parenchymal loss in zone 3 with surrounding islands of preserved hepatocytes, and confluent necrosis.

A

Acute liver failure

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27
Q

Causes of acute liver failure without cell death

A

Hepatocellular dysfunction

Diffuse microvesicular steatosis

Non-hepatotropic viruses in immunosuppressed (CMV, HSV, adenovirus)

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28
Q

Symptoms of hepatic encephalopathy

A

Altered consciousness
Fluctuating rigidity and hyperreflexia
Asterixis

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29
Q

Exacerbates hepatic encephalopathy

A

Portal HTN

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30
Q

Early sign of coagulopathy

A

Easy bruisability

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31
Q

Caused by decreased removal of activated coagulation factors from circulation. Possible complication of acute liver failure.

A

DIC

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32
Q

Trigger of hepatorenal syndrome

A

Portal HTN

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33
Q

Conversion of the normal architecture of the liver into structurally abnormal parenchymal nodules surrounded by fibrous bands

A

Cirrhosis

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34
Q

Liver has a bumpy surface with depressed areas of scarring and bulging areas of regenerative nodules.

A

Cirrhosis

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35
Q

Signs of hyperestrogenemia in chronic liver failure (cirrhosis)

A

Palmar erythema
Spider angiomas
Hypogonadism and gynecomastia in males

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36
Q

Prehepatic causes of portal HTN

A

Obstructive thrombosis of portal V

Increased splenic form 2/2 splenomegaly

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37
Q

Common causes of chronic liver failure

A

Chronic HBV or HCV
NAFLD
Alcoholic liver disease

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38
Q

Post-hepatic causes of portal HTN

A

Severe R sided HF
Constrictive pericarditis
Hepatic V outflow obstruction

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39
Q

Accumulation of fluid in the peritoneal cavity

A

Ascites

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40
Q

Possible complication of long-standing ascites

A

Seepage of peritoneal fluid through trans-diaphragmatic may produce hydrothorax (typically R)

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41
Q

Best single test for the classification of ascites into portal HTN and non-portal HTN causes

A

Serum ascites albumin gradient (SAAG) = serum albumin - ascitic fluid albumin

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42
Q

Ascites cause indicated by an SAAG of >1.1 g/dL

A

Portal HTN cause

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43
Q

Ascites cause indicated by an SAAG of <1.1 g/dL

A

Non-portal HTN cause

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44
Q

Infection of ascitic fluid in the absence of an intra-abdominal event or surgically treatable source of infection

A

Spontaneous bacterial peritonitis

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45
Q

Common causal organisms of spontaneous bacterial peritonitis

A

E coli
Klebsiella
Strep pneumoniae

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46
Q

Treatment for spontaneous bacterial peritonitis

A

3rd generation cephalosporins –> cefotaxime or ceftriaxone

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47
Q

Hypersplenism due to sequestration of blood cells in the expanded splenic red pulp. Complication of portal HTN.

A

Congestive splenomegaly

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48
Q

Caused by intrapulmonary vascular dilation producing ventilation-perfusion mismatch leading to hypoxemia. Related to chronic liver failure

A

Hepatopulmonary syndrome

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49
Q

Components of bile

A

Bilirubin
Bile salts
Cholesterol
Phospholipids (phosphatidylcholine)

50
Q

Implicated agent in breast milk jaundice

A

Beta-glucuronidase in milk

51
Q

Genetic anomaly associated with Crigler-Najjar syndrome 1 and 2

A

Deficiency of UGT1A1

52
Q

Type of hyperbilirubinemia in Crigler-Najjar syndromes

A

Unconjugated

53
Q

Deficiency in Dubin-Johnson and Rotor syndromes

A

Deficiency of canalicular membrane transporters

54
Q

Type of hyperbilirubinemia in Dubin-Johnson syndrome

A

Conjugated

55
Q

Congenital hyperbilirubinemia lasting longer than 2 wks associated with almost complete absence of UGT1A1. Does not respond to phenobarbital therapy.

A

Crigler-Najjar syndrome type 1

56
Q

Congenital hyperbilirubinemia lasting longer than 2 wks associated with a decrease in UGT1A1. Responds to phenobarbital therapy.

A

Crigler-Najjar syndrome type 2

57
Q

Adult with mild, chronic conjugated hyperbilirubinemia. Liver enzymes are normal. Liver biopsy shows dark brown pigment in centrilobular hepatocytes.

A

Dubin-Johnson syndrome

58
Q

Classic feature of Dubin-Johnson syndrome

A

Reversal of the ratio of byproducts generated during heme synthesis

59
Q

Reversal in the ratio of urinary coproporphyrins I and III from 1:3 to 1:4 is diagnostic of this hyperbilirubinemia

A

Dubin-Johnson syndrome

60
Q

Inheritance of Crigler-Najjar syndrome type 1

A

Autosomal recessive

61
Q

Inheritance of Crigler-Najjar syndrome type 2

A

Autosomal dominant with variable penetrance

62
Q

Inheritance of Gilbert syndrome

A

Autosomal recessive

63
Q

Inheritance of Dubin-Johnson syndrome and Rotor syndrome

A

Autosomal recessive

64
Q

Causes of physiological jaundice of the newborn

A

Increased RBC with shorter life span
Increased hemolysis after birth
Immature liver enzymes
Lack of normal gut flora

65
Q

Risk factors of bilirubin induced neurologic dysfunction

A

Prematurity
Hemolysis
Birth trauma (cephalhematoma)
Exclusive breastfeeding with excessive weight loss

66
Q

At what bilirubin level is there a risk for kernicterus?

A

> 20 mg/dL

67
Q

Features of acute encephalopathy in BIND

A

Hypertonia
Irritability
Apnea
Seizures

68
Q

Features of chronic encephalopathy in BIND

A

Sensorineural hearing loss
Choreoathetoid movements
Upward gaze palsy

69
Q

Congenital malformations associated with extrahepatic biliary atresia

A

Trisomy 18 and 21

70
Q

Neonatal infections associated with neonatal cholestasis

A

CMV
Bacterial sepsis
UTI
Syphilis

71
Q

Metabolic disease associated with neonatal cholestasis

A

Tyrosinemia
Galactosemia

72
Q

Syndromic paucity of bile ducts

A

Alagille syndrome

73
Q

Type of neonatal jaundice caused by insufficient feeding leading to inadequate quantities of bowel movements

A

Breastfeeding jaundice

74
Q

Spectrum of developmental ductal plate malformations due to persistence of the fetal peripheral ductal plates

A

Fibropolycystic disease of the liver

75
Q

Fibropolycystic disease of the liver is associated with an increased risk of this

A

Cholangiocarcinoma

76
Q

Three types of fibropolycystic disease of the liver

A

Biliary hamartomas
Biliary cysts
Congenital hepatic fibrosis

77
Q

Von Meyenburg complexes

A

Small bile duct hamartomas

Diffuse in fibropolycystic disease of the liver

78
Q

Disease associated with biliary hamartomas type of fibropolycystic disease of the liver

A

Autosomal dominant polycystic kidney disease

79
Q

Isolated biliary cysts is associated with this disease

A

Symptomatic ascending cholangitis

80
Q

Multifocal cystic dilation of the large intrahepatic bile ducts

A

Caroli disease

81
Q

Cystic dilation of the biliary tree with congenital hepatic fibrosis

A

Caroli syndrome

82
Q

Inheritance of biliary cyst form of fibropolycystic disease of the liver

A

Autosomal recessive

83
Q

Autosomal recessive condition characterized by enlargement of portal tracts by broad bands of collagenous septa, irregular hepatic islands, and variable numbers of abnormally shaped bile ducts embedded in the fibrous tissue

A

Congenital hepatic fibrosis type of fibropolycystic disease of the liver

84
Q

Disease associated with congenital hepatic fibrosis type of fibropolycystic disease of the liver

A

Autosomal recessive polycystic kidney disease

85
Q

Three enzymes involved in the metabolism of liver

A

Alcohol dehydrogenase (mainly)
CYP450 isoenzymes
Catalase

86
Q

Affect of CYP2E1 induction in alcoholism

A

Produce ROS that damage cellular proteins, membranes, and mitochondria

87
Q

Frequent cause of death in pts with NASH

A

Cardiovascular disease

88
Q

Characteristic twisted-rope appearance within ballooning hepatocyte

A

Mallory hyaline (Mallory-Denk bodies)

89
Q

Necrosis of perivenular hepatocytes is typical for this type of liver injury

A

Drug or toxin induced

90
Q

HLA alleles associated with autoimmune hepatitis

A

HLA-DR3 (whites)
HLA-DR4 (Japanese)
HLA-DRB1 (S Americans)

91
Q

Serum antibodies associated with type 1 autoimmune hepatitia

A

ANA
ASMA
SLA/LPA
AMA

92
Q

Autoimmune hepatitis more common in children

93
Q

Serum antibodies associated with type 2 autoimmune hepatitis

A

LKM-1 against CYP2D6
LC-1 antibodies

94
Q

Portal inflammatory cells eroding through the limiting plate between the portal tract and liver parenchyma. Formerly called piecemeal necrosis.

A

Interface hepatitis, associated with autoimmune hepatitis

95
Q

Emperipolesis

A

Active penetration of lymphocytes into and through hepatocytes

96
Q

Hepatocyte rosettes

A

Circular arrangement of hepatocytes around a dilated canaliculus

97
Q

Conditions associated with autoimmune hepatitis

A

PBC/PSC
DM type 1
Thyroiditis
Celiac sprue

98
Q

Disease characterized by inflammatory destruction of small and medium sized intrahepatic bile ducts

A

Primary biliary cholangitis

99
Q

AMAs directed against the E2 component of the pyruvate dehydrogenase complex (PDC-E2) is a characteristic finding

A

Primary biliary cholangitis

100
Q

Liver is bile-stained. Microscopy shows florid duct lesion and ductopenia of the intrahepatic bile ducts

A

Primary biliary cholangitis

101
Q

Treatment for PBC

A

Early therapy with ursodeoxycholic acid

102
Q

Inflammation and obliterative fibrosis of extrahepatic and large intrahepatic ducts, and dilation of preserved segments

A

Primary sclerosing cholangitis

103
Q

Atypical pANCA target nuclear envelope protein in bile duct cells. Associated with HLA-B8 and ulcerative colitis

A

Primary sclerosing cholangitis

104
Q

Liver has green color with cholangiectasis and cholangitis abscesses. Microscopy shows injury and neutrophilic infiltrates superimposed on chronic inflammation of the large intra- and extra-hepatic bile ducts. Onion-skin fibrosis of bile ducts.

A

Primary sclerosing cholangitis

105
Q

Beading seen in the large intrahepatic and extrahepatic tree on ERCP/MRCP is diagnostic

A

Primary sclerosing cholangitis

106
Q

Mutation in adult hemochromatosis

A

HFE –> hepcidin deficiency

107
Q

Mutation in juvenile hemochromatosis

108
Q

Features of severe iron overload

A

Micronodular cirrhosis of liver
DM
Abnormal skin pigmentation

109
Q

Liver appearance in early stages of hemochromatosis

A

Chocolate brown micronodular cirrhosis

110
Q

Liver appearance in later stages of hemochromatosis

A

Dark brown to nearly black

111
Q

Bronze diabetes is associated with what genetic condition?

A

Hemochromatosis

112
Q

Inheritance of Wilson disease

A

Autosomal recessive

113
Q

Mutated gene in Wilson disease

114
Q

Inheritance of alpha-1 antitrypsin deficiency

A

Autosomal recessive

115
Q

Gene mutation in alpha-1 antitrypsin deficiency

A

SERPINA1
PiZZ

116
Q

Liver biopsy has round to oval cytoplasmic globular inclusions that are PAS positive and diastase resistant. ER is dilated.

A

Alpha-1 antitrypsin deficiency

117
Q

Circulatory disorder characterized by hepatic outflow obstruction, related to thrombophilic conditions

A

Budd-Chiari syndrome

118
Q

Liver is swollen and red-purple, with a tense capsule. Areas of hemorrhagic collapse alternating with preserved/regenerating parenchyma. Microscopy of affected parenchyma shows severe centrilobular necrosis.

A

Budd-Chiari syndrome

119
Q

Passive process caused by impaired outflow or increased inflow of blood leading to increased blood volume within the liver. Commonly associated with right sided HF.

A

Chronic passive venous congestion

120
Q

Nutmeg appearance of liver. Central V and sinusoids are dilated.

A

Chronic passive venous congestion

GI II (31 decks)

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