Liver disorders Flashcards
1
Q
Isolated unconjugated hyperbilirubinemia with negative workup for hemolysis
A
Gilbert syndrome
2
Q
AST:ALT ratio in chronic viral hepatitis and NAFLD
A
<1
3
Q
AST:ALT ratio of >2:1 is suggestive of what?
A
Alcoholic liver disease
4
Q
AST:ALT ratio of >3:1 is highly suggestive of what?
A
Alcoholic liver disease
5
Q
Liver enzyme located in the ER and in bile duct epithelial cells. It is elevated in cholestatic damage
A
GGT
6
Q
Type of steatosis seen in Reye syndrome
A
Microvesicular
7
Q
Type of steatosis seen in alcoholic fatty liver
A
Combined
8
Q
Accumulation of green-brown plugs of pile pigment in hepatocytes and dilated canaliculi
A
Cholestasis causing liver injury
9
Q
Swollen foamy appearance of hepatocyte cytoplasm (feathery degeneration) is caused by what?
A
Accumulation of bile salts in hepatocytes due to cholestasis
10
Q
Retention of bilirubin, bile salts, and cholesterol associated with elevated levels of conjugated bilirubin, jaundice, and pruritus.
A
Cholestasis
11
Q
Serum findings in cholestasis
A
Increased ALP and GGT
12
Q
Hepatocyte swelling, cytoplasmic clearing, and clumping of intermediate filaments associated with alcoholic steatohepatitis and viral hepatitis
A
Ballooning degeneration
13
Q
Mallory hyaline
A
Prominent clumping of intermediate filaments in hepatocytes
Associated with steatohepatitis
14
Q
Causes of liver necrosis
A
Ischemic injury
Oxidative stress
Severe viral infection
Autoimmune hepatitis
Secondary vascular insults
15
Q
Types of liver necrosis
A
Confluent –> zonal loss
Bridging
Panacinar
16
Q
Causes of abnormal apoptosis of hepatocytes
A
Viral infection
Fatty liver disease
Drug-induced liver injury
17
Q
Cell involved in scar deposition of the liver by differentiating into myofibroblasts in liver injury
A
Stellate cell
18
Q
Stain used to identify collagen bundles in liver cirrhosis
A
Masson trichrome staining
19
Q
Common end point of liver injury characterized by fibrous septa encircling nodules of regenerative hepatocytes
A
Cirrhosis
20
Q
Amount of functional capacity of liver that must be lost before hepatic failure appears
A
80-90%
21
Q
Timing of acute liver failure
A
Occurs within 26 wks of initial liver injury
22
Q
Massive liver necrosis develops in 2-3 wks causing very rapid onset of liver failure
A
Fulminant liver failure
23
Q
Conditions associated with acute liver failure
A
Encephalopathy
Coagulopathy
Other organ failure
24
Q
Causes of acute liver failure
A
Drug/toxin
Autoimmune hepatitis
HAV, HBV, or HEV
Galactosemia
Malignancies
25
Most common malignancies associated with acute liver failure
Leukemia
Lymphoma
Breast ca
Colon ca
26
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.
Acute liver failure
27
Causes of acute liver failure without cell death
Hepatocellular dysfunction
Diffuse microvesicular steatosis
Non-hepatotropic viruses in immunosuppressed (CMV, HSV, adenovirus)
28
Symptoms of hepatic encephalopathy
Altered consciousness
Fluctuating rigidity and hyperreflexia
Asterixis
29
Exacerbates hepatic encephalopathy
Portal HTN
30
Early sign of coagulopathy
Easy bruisability
31
Caused by decreased removal of activated coagulation factors from circulation. Possible complication of acute liver failure.
DIC
32
Trigger of hepatorenal syndrome
Portal HTN
33
Conversion of the normal architecture of the liver into structurally abnormal parenchymal nodules surrounded by fibrous bands
Cirrhosis
34
Liver has a bumpy surface with depressed areas of scarring and bulging areas of regenerative nodules.
Cirrhosis
35
Signs of hyperestrogenemia in chronic liver failure (cirrhosis)
Palmar erythema
Spider angiomas
Hypogonadism and gynecomastia in males
36
Prehepatic causes of portal HTN
Obstructive thrombosis of portal V
Increased splenic form 2/2 splenomegaly
37
Common causes of chronic liver failure
Chronic HBV or HCV
NAFLD
Alcoholic liver disease
38
Post-hepatic causes of portal HTN
Severe R sided HF
Constrictive pericarditis
Hepatic V outflow obstruction
39
Accumulation of fluid in the peritoneal cavity
Ascites
40
Possible complication of long-standing ascites
Seepage of peritoneal fluid through trans-diaphragmatic may produce hydrothorax (typically R)
41
Best single test for the classification of ascites into portal HTN and non-portal HTN causes
Serum ascites albumin gradient (SAAG) = serum albumin - ascitic fluid albumin
42
Ascites cause indicated by an SAAG of >1.1 g/dL
Portal HTN cause
43
Ascites cause indicated by an SAAG of <1.1 g/dL
Non-portal HTN cause
44
Infection of ascitic fluid in the absence of an intra-abdominal event or surgically treatable source of infection
Spontaneous bacterial peritonitis
45
Common causal organisms of spontaneous bacterial peritonitis
E coli
Klebsiella
Strep pneumoniae
46
Treatment for spontaneous bacterial peritonitis
3rd generation cephalosporins --> cefotaxime or ceftriaxone
47
Hypersplenism due to sequestration of blood cells in the expanded splenic red pulp. Complication of portal HTN.
Congestive splenomegaly
48
Caused by intrapulmonary vascular dilation producing ventilation-perfusion mismatch leading to hypoxemia. Related to chronic liver failure
Hepatopulmonary syndrome
49
Components of bile
Bilirubin
Bile salts
Cholesterol
Phospholipids (phosphatidylcholine)
50
Implicated agent in breast milk jaundice
Beta-glucuronidase in milk
51
Genetic anomaly associated with Crigler-Najjar syndrome 1 and 2
Deficiency of UGT1A1
52
Type of hyperbilirubinemia in Crigler-Najjar syndromes
Unconjugated
53
Deficiency in Dubin-Johnson and Rotor syndromes
Deficiency of canalicular membrane transporters
54
Type of hyperbilirubinemia in Dubin-Johnson syndrome
Conjugated
55
Congenital hyperbilirubinemia lasting longer than 2 wks associated with almost complete absence of UGT1A1. Does not respond to phenobarbital therapy.
Crigler-Najjar syndrome type 1
56
Congenital hyperbilirubinemia lasting longer than 2 wks associated with a decrease in UGT1A1. Responds to phenobarbital therapy.
Crigler-Najjar syndrome type 2
57
Adult with mild, chronic conjugated hyperbilirubinemia. Liver enzymes are normal. Liver biopsy shows dark brown pigment in centrilobular hepatocytes.
Dubin-Johnson syndrome
58
Classic feature of Dubin-Johnson syndrome
Reversal of the ratio of byproducts generated during heme synthesis
59
Reversal in the ratio of urinary coproporphyrins I and III from 1:3 to 1:4 is diagnostic of this hyperbilirubinemia
Dubin-Johnson syndrome
60
Inheritance of Crigler-Najjar syndrome type 1
Autosomal recessive
61
Inheritance of Crigler-Najjar syndrome type 2
Autosomal dominant with variable penetrance
62
Inheritance of Gilbert syndrome
Autosomal recessive
63
Inheritance of Dubin-Johnson syndrome and Rotor syndrome
Autosomal recessive
64
Causes of physiological jaundice of the newborn
Increased RBC with shorter life span
Increased hemolysis after birth
Immature liver enzymes
Lack of normal gut flora
65
Risk factors of bilirubin induced neurologic dysfunction
Prematurity
Hemolysis
Birth trauma (cephalhematoma)
Exclusive breastfeeding with excessive weight loss
66
At what bilirubin level is there a risk for kernicterus?
>20 mg/dL
67
Features of acute encephalopathy in BIND
Hypertonia
Irritability
Apnea
Seizures
68
Features of chronic encephalopathy in BIND
Sensorineural hearing loss
Choreoathetoid movements
Upward gaze palsy
69
Congenital malformations associated with extrahepatic biliary atresia
Trisomy 18 and 21
70
Neonatal infections associated with neonatal cholestasis
CMV
Bacterial sepsis
UTI
Syphilis
71
Metabolic disease associated with neonatal cholestasis
Tyrosinemia
Galactosemia
72
Syndromic paucity of bile ducts
Alagille syndrome
73
Type of neonatal jaundice caused by insufficient feeding leading to inadequate quantities of bowel movements
Breastfeeding jaundice
74
Spectrum of developmental ductal plate malformations due to persistence of the fetal peripheral ductal plates
Fibropolycystic disease of the liver
75
Fibropolycystic disease of the liver is associated with an increased risk of this
Cholangiocarcinoma
76
Three types of fibropolycystic disease of the liver
Biliary hamartomas
Biliary cysts
Congenital hepatic fibrosis
77
Von Meyenburg complexes
Small bile duct hamartomas
Diffuse in fibropolycystic disease of the liver
78
Disease associated with biliary hamartomas type of fibropolycystic disease of the liver
Autosomal dominant polycystic kidney disease
79
Isolated biliary cysts is associated with this disease
Symptomatic ascending cholangitis
80
Multifocal cystic dilation of the large intrahepatic bile ducts
Caroli disease
81
Cystic dilation of the biliary tree with congenital hepatic fibrosis
Caroli syndrome
82
Inheritance of biliary cyst form of fibropolycystic disease of the liver
Autosomal recessive
83
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
Congenital hepatic fibrosis type of fibropolycystic disease of the liver
84
Disease associated with congenital hepatic fibrosis type of fibropolycystic disease of the liver
Autosomal recessive polycystic kidney disease
85
Three enzymes involved in the metabolism of liver
Alcohol dehydrogenase (mainly)
CYP450 isoenzymes
Catalase
86
Affect of CYP2E1 induction in alcoholism
Produce ROS that damage cellular proteins, membranes, and mitochondria
87
Frequent cause of death in pts with NASH
Cardiovascular disease
88
Characteristic twisted-rope appearance within ballooning hepatocyte
Mallory hyaline (Mallory-Denk bodies)
89
Necrosis of perivenular hepatocytes is typical for this type of liver injury
Drug or toxin induced
90
HLA alleles associated with autoimmune hepatitis
HLA-DR3 (whites)
HLA-DR4 (Japanese)
HLA-DRB1 (S Americans)
91
Serum antibodies associated with type 1 autoimmune hepatitia
ANA
ASMA
SLA/LPA
AMA
92
Autoimmune hepatitis more common in children
Type 2
93
Serum antibodies associated with type 2 autoimmune hepatitis
LKM-1 against CYP2D6
LC-1 antibodies
94
Portal inflammatory cells eroding through the limiting plate between the portal tract and liver parenchyma. Formerly called piecemeal necrosis.
Interface hepatitis, associated with autoimmune hepatitis
95
Emperipolesis
Active penetration of lymphocytes into and through hepatocytes
96
Hepatocyte rosettes
Circular arrangement of hepatocytes around a dilated canaliculus
97
Conditions associated with autoimmune hepatitis
PBC/PSC
DM type 1
Thyroiditis
Celiac sprue
98
Disease characterized by inflammatory destruction of small and medium sized intrahepatic bile ducts
Primary biliary cholangitis
99
AMAs directed against the E2 component of the pyruvate dehydrogenase complex (PDC-E2) is a characteristic finding
Primary biliary cholangitis
100
Liver is bile-stained. Microscopy shows florid duct lesion and ductopenia of the intrahepatic bile ducts
Primary biliary cholangitis
101
Treatment for PBC
Early therapy with ursodeoxycholic acid
102
Inflammation and obliterative fibrosis of extrahepatic and large intrahepatic ducts, and dilation of preserved segments
Primary sclerosing cholangitis
103
Atypical pANCA target nuclear envelope protein in bile duct cells. Associated with HLA-B8 and ulcerative colitis
Primary sclerosing cholangitis
104
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.
Primary sclerosing cholangitis
105
Beading seen in the large intrahepatic and extrahepatic tree on ERCP/MRCP is diagnostic
Primary sclerosing cholangitis
106
Mutation in adult hemochromatosis
HFE --> hepcidin deficiency
107
Mutation in juvenile hemochromatosis
HJV
108
Features of severe iron overload
Micronodular cirrhosis of liver
DM
Abnormal skin pigmentation
109
Liver appearance in early stages of hemochromatosis
Chocolate brown micronodular cirrhosis
110
Liver appearance in later stages of hemochromatosis
Dark brown to nearly black
111
Bronze diabetes is associated with what genetic condition?
Hemochromatosis
112
Inheritance of Wilson disease
Autosomal recessive
113
Mutated gene in Wilson disease
ATP7B
114
Inheritance of alpha-1 antitrypsin deficiency
Autosomal recessive
115
Gene mutation in alpha-1 antitrypsin deficiency
SERPINA1
PiZZ
116
Liver biopsy has round to oval cytoplasmic globular inclusions that are PAS positive and diastase resistant. ER is dilated.
Alpha-1 antitrypsin deficiency
117
Circulatory disorder characterized by hepatic outflow obstruction, related to thrombophilic conditions
Budd-Chiari syndrome
118
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.
Budd-Chiari syndrome
119
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.
Chronic passive venous congestion
120
Nutmeg appearance of liver. Central V and sinusoids are dilated.
Chronic passive venous congestion
