Liver and Gallbladder Disorders Flashcards
1
Q
Hepatic vein obstruction is also called _____.
A
Budd-Chiari syndrome
2
Q
What are the most common causes of hepatic vein obstruction?
A
- hepatic vein thrombosis (MC)
2. hepatic vein occlusion (ex. by a tumor)
3
Q
What are the risk factors of hepatic vein obstruction?
A
- idiopathic
- hyper coagulable states (ex. polycythemia vera, pregnancy, OCPs, Factor V Leiden, malignancy, Protein C and S deficiency)
- women in their 20s and 30s
4
Q
Hepatic vein thrombosis or occlusion causes —>
A
decrease liver drainage and portal HTN and cirrhosis
5
Q
What is the classic triad for hepatic vein obstruction?
A
- ascites
- hepatomegaly
- RUQ abdominal pain
also have rapid development of jaundice and hepatosplenomegaly
6
Q
What are the diagnostic tests of choice for hepatic vein obstruction?
A
Ultrasound– shows occlusion of hepatic vein or IVC
Venography
7
Q
What is the management for mild hepatic vein obstruction?
A
Shunts (ex. TIPS)
8
Q
What is the management for stenosis of the hepatic vein or obstruction of the IVC?
A
Angioplasty with stent
angioplasty = repair of blood vessel by inserting balloon-tipped catheter to unclog the vessel.
9
Q
What is the management of ascites?
A
Diuretics
Low sodium diet
Large volume paracentesis (for refractory ascites)
10
Q
What is cholelithiasis?
A
gallstones in the gall bladder with NO inflammation!
11
Q
90% of gallstones are _______.
A
cholesterol
12
Q
What are the risk factors for cholelithiasis?
A
5Fs: fat, fair, female, forty, fertile
13
Q
What are the clinical manifestations of cholelithiasis?
A
- asymptomatic
- “biliary colic” —> episodic RUQ pain that begins abruptly and resolves slowly that is often precipitated by fatty foods or large meals
14
Q
What is the diagnostic test of choice for cholelithiasis?
A
Ultrasound!
15
Q
What are the management options for cholelithiasis?
A
- asymptomatic —> observe
2. symptomatic —> cholecystectomy
16
Q
What are the complications of cholelithiasis?
A
- choledocholithiasis
- cholangitis
- cholecystitis
17
Q
What is choledocholithiasis?
A
gallstones in the biliary tree (common bile duct) associated with ductal dilation
As liver produces bile/bilirubin it will push against the stone and the biliary tree with expand until bilirubin overflows into the blood and the patient then gets jaundice!
18
Q
Choledocholithiasis can lead to _____ and _______.
A
- gallstone pancreatitis (so want to check lipase and amylase)
- jaundice
19
Q
What is the clinical manifestation of choledocholithiasis?
A
biliary colic and JAUNDICE (increased bilirubin)
20
Q
What are the diagnostic tests of choice for choledocholithiasis?
A
Ultrasound and ERCP
21
Q
What is the management for choledocolithiasis?
A
NPO, IVFs and stone extraction via ERCP
22
Q
What is cholangitis?
A
biliary tree infxn secondary to obstruction by gallstone
23
Q
What are the clinical manifestations of cholangitis?
A
Charcot’s triad!
- fevers/chills
- RUQ pain
- jaundice
24
Q
What is the diagnostic test of choice for cholangitis?
A
ultrasound
25
What is the management of cholangitis?
1. Emergent ERCP
2. Abx: penicillin derivaties, ceftazidime (gram -), ampicillin (gram +), and metronidazole (anaerobic coverage)
3. eventually cholecystectomy
26
What is acute cholecystitis?
Cystic duct obstruction by gallstone causing inflammation and infxn
27
What are the most common bacteria found with cholecystitis and cholangitis?
1. E. coli
2. Klebsiella
3. enterococci
28
What are the clinical manifestations of acute cholecystitis?
1. Biliary colic
2. Fever, N/V, palpable GB, leukocytosis
3. + Murphy's sign
4. + Boas sign
Jaundice is NOT common
29
What is Boas sign?
referred pain to right subscapular area due to phrenic nerve irritation
30
What are the diagnostic tests of choice for acute cholecystitis and what will they show?
1. Ultrasound---> thickened gallbladder, pericholecystic fluid, and gallstones
2. Labs---> increased WBCs, bilirubin, ALP, and LFTs
3. HIDA scan**---> non-visualization of gallbladder is + test
* = gold standard
31
What is the management of acute cholecystitis?
NPO, IVFs, Abx (3rd gen. ceph + Metronidazole)
| Cholecystecomy usually within 72 hours
32
What is chronic cholecystitis?
Associated with gallstones resulting in repeated bouts of acute/subacute cholecystitis
Gallbladder may appear strawberry (interior) or porcelain (pre-malignant condition!)
33
What is Gilbert's syndrome?
common benign hereditary disorder with mildly reduced activity of UGT enzyme, so you have an increase in indirect bilirubin
34
What are the clinical manifestations of Gilbert's syndrome?
most are asymptomatic!
may have transient episodes of jaundice during periods of stress, fasting, ETOH or illness
35
How is Gilbert's syndrome diagnosed?
usually incidental finding when have SLIGHT increase in indirect bilirubin levels with NORMAL liver function values
36
What is the management of Gilbert's syndrome?
None! Benign disease no associated with sequelae
37
What causes PHYSIOLOGIC jaundice in newborns?
increase indirect bilirubin due to deficiency in UGT and increased breakdown of fetal Hgb.
38
Physiologic jaundice in newborns usually occurs ____ days after birth.
3-5
If jaundice on 1st day is pathologic!! NOT physiologic
39
What is the management of physiologic jaundice in newborns?
observation and phototherapy to make the indirect bilirubin water soluble to enhance renal excretion which then lowers the levels.
40
What is Crigler Naijar syndrome?
autosomal recessive disorder.
Type I ---> no UGT activity
Type II ---> very little UGT activity
*basically a more severe form of Gilbert's
41
What is kernicterus?
increased bilirubin in the CNS and basal ganglia
can lead to deafness, lethargy, hypotonia, oculomotor palsy, and death
42
What are the clinical manifestations of Crigler Naijar syndrome?
Type I ---> jaundice in 1st week of life with severe progression in 2nd week leading to kernicterus
death in 15 months if not treated!
43
How is Crigler Naijar syndrome diagnosed?
isolated indirect bilirubin hyperbilirubinemia with NORMAL liver functions
Type I ---> serum bili 20-50 mg/dL
Type II ---> serum bili 7-10 mg/dL (milder form)
44
What is the management of Crigler Naijar syndrome?
phototherapy, liver transplant
plasmapheresis in crisis
45
What is Dubin-Johnson syndrome?
Isolated mild conjugated hyperbilirubinemia due to inability of hepatcytes to secrete conjugated bilirubin (gene mutation)
46
Patients with Dubin-Johnson syndrome will have a _____ liver.
grossly black!
47
What is the management of Dubin-Johnson syndrome?
None!
48
What is Rotor's syndrome?
a mild form of Dubin-Johnson syndrome (increase conjugated bilirubin)
liver is NOT black
49
What is the pathophysiology of hemolytic (pre hepatic) jaundice?
Massive RBC lysis! (hemolytic anemia)
increased indirect bilirubin production ---> overwhelms liver's ability to conjugate it
50
Hemolytic (prehepatic) jaundice results in ________ hyperbilirubinemia.
increased indirect/unconjugated
51
Hemolytic (prehepatic) jaundice results in _____ urine color.
normal
52
What is the pathophysiology of obstructive (post-hepatic) jaundice?
Cholestasis = bile duct blockage!
So conjugated bilirubin is prevented from passing into the intestine and refluxes back into the blood so get increased CONJUGATED bilirubin
53
Obstructive (post-hepatic) jaundice results in _____ hyperbilirubinemia.
increased direct/conjugated
54
What LFTs are markedly elevated with biliary obstruction or intrahepatic cholestasis?
ALP and GGT! ---> GGT more sensitive indicator
55
Dark urine = increased _____ bilirubin
Direct/conjugated
56
What is the pathophysiology of hepatocellular (intra-hepatic) jaundice?
Liver damage (hepatitis)
1. decreases conjugation
2. direct bilirubin not efficiently secreted into bile
57
Hepatocellular (intra-hepatic) jaundice results in increased _____ bilirubin.
BOTH indirect and direct
58
What LFTs are markedly elevated with hepatocellular (intra-hepatic) jaundice?
ALT and AST
59
How does ETOH hepatitis effect AST and ALT?
increased AST:ALT >2:1 (AST usually ETOH causes direct mitochondrial injury ---> increased AST
60
How does Acute hepatitis effect AST and ALT?
increased ALT and AST >1,000
| ALT > AST usually
61
How does Chronic hepatitis effect AST and ALT?
increased ALT:AST but
62
What are the lab tests of TRUE liver function?
1. Prothrombin time (PT)
| 2. Albumin
63
Prothrombin time depends on the synthesis of coagulation factors, so it is ______ dependent.
vitamin K
64
Which "actual" liver function test is an earlier indicator of severe liver injury?
PT
65
Albumin is a marker for overall liver....
protein synthesis
66
Which indicators point towards autoimmune hepatitis?
1. increased ALT > 1,000
2. +ANA
3. + smooth muscle antibodies
4. increased IgG
5. Responds to corticosteroids!
67
Autoimmune hepatitis will respond to ______.
corticosteroids
68
Acute hepatic failure is defined as....
rapid liver failure + hepatic encephalopathy often with coagulopathy
is within 8 wks after onset of liver injury in a previously healthy patient
69
Reye's syndrome is most commonly seen in who?
children and associated with aspirin use during viral infxn
70
What is Reye's syndrome?
fulminant hepatitis (acute hepatic failure) most commonly seen in children after aspirin use during viral infxn
71
Children with Reye's syndrome will most commonly present with?
1. rash (hands and feet)
2. intractable vomiting
3. liver damage
4. encephalopathy
5. dilated pupils
6. multi-organ failure
72
What are the most common causes of acute hepatic failure (fulminant hepatitis)?
1. Acetaminophen overuse or overdose
2. drug runs
3. viral hepatitis
4. Reye's syndrome
5. Budd-Chiari syndrome (hepatic vein obstruction)
6. autoimmune hepatitis
73
What is asterixis?
flapping tremor of the hand with wrist extension
associated with hepatic encephalopathy
74
What are the main diagnostic indicators of fulminant hepatitis (acute hepatic failure)?
1. increased ammonia!**
2. increased PT/INR ---> decreased liver production of clotting factor proteins
3. increased LFTs
4. hypoglycemia
75
How does hepatic encephalopathy present?
1. vomiting
2. coma
3. seizures
4. asterixis (flapping tremor of hand with wrist extension)
5. hyperreflexia
6. increased ICP
76
Why is there increased serum ammonia levels with fulminant hepatitis?
failure of liver to excrete ammonia and reduced ability of converting ammonia to urea. Protein breakdown also leads to increased ammonia
77
Why is there coagulopathy with fulminant hepatitis?
decreases production of coagulation factors
78
What is the management of hepatic encephalopahty?
1. Lactulose ---> neutralizes the ammonia
2. Neomycin---> abx that decreases the bacteria producing ammonia in GI tract
3. Protein restriction---> reduces breakdown of protein into ammonia
79
What is the definitive tx of fulminant hepatitis?
Liver transplant!
80
What are the 2 phases of viral hepatitis?
1. Prodromal phase
| 2. Icteric phase (most don't develop this phase)
81
The prodromal phase of viral hepatitis is associated with....
```
malaise
arthralgia
fatigue
URI sx
N/V
```
82
Hepatitis ___ is the only one associated with spiking fevers during the prodromal phase.
A
83
What is the main clinical manifestation during the icteric phase of viral hepatitis?
jaundice! (usually after the patient's fever breaks)
84
Chronic hepatitis is defined as the disease lasting for...
more than 6 months
85
Which viral hepatitis's are associated with chronic liver disease?
B, C, and D.
86
What % of HCV patients develop chronic disease?
80%
87
What is the transmission of HAV?
feco-oral
Outbreaks with contaminated water/food during international travel
Day-care workers
Homosexuals
88
What is the most common source of HAV in adults?
asymptomatic children
89
Acute HAV is diagnosed by having a positive..
IgM HAV Ab
IgM = acute!
90
Past exposure of HAV is diagnosed by having a positive...
IgG HAV Ab with a negative IgM
91
What is the management of HAV?
symptomatic tx. It is self-limiting
**HAV immune globulin is available for close contacts.
92
What is the transmission of HCV?
parental (ex. IV drug use)
sexual
increased risk if blood transfusion before 1992
93
Which test implies recovery of HCV?
HCV-RNA
*anti-HCV may become negative after recovery!
94
Acute HCV titers will show..
1. (+) HCV RNA
| 2. (+/-) anti-HCV (becomes + in 6 weeks)
95
Resolved HCV titers will show...
1. (-) HCV RNA
| 2. (+/-) anti-HCV
96
Chronic HCV titers will show...
1. (+) HCV RNA
| 2. (+) anti-HCV
97
What is the management of chronic HCV?
interferon AND ribavirin
98
Screenings for hepatocellular carcinoma (HCC) for patients with HCV is done via...
serum alpha-fetoprotein and ultrasound
99
How is HBV typically transmitted?
1. Perinatal
2. Per cutaneous
3. Sexual
4. Parenteral
100
If HBsAg is positive for more than 6 months it means -->
Chronic infection is established
101
What does HBsAg mean?
surface antigen
1st evidence of HBV infection (before symptoms)
102
What does HBsAb or anti-HBs mean?
surface antibody
Means distant resolved infection OR vaccination
103
If patient does NOT develop anti-HBs in 6 months ---->
the patient has chronic infection
104
What are the HBcAb's?
Core antibodies
IgM --> indicates acute infection (1st Ab to appear)
IgG --> indicates chronic infection OR distant resolved infection
105
What does HBeAg mean?
envelope antigen
increased viral replication and increased infectivity
106
If a patient has a positive HBeAg for more than 3 months they have increased likelihood of...
developing chronic HBV
107
What does HBeAb mean?
envelope antibody
indicates waning viral replication and infectivity
108
What is the management of acute HBV?
supportive care
109
When is the HBV vaccination given?
at 0, 1, and 6 months
110
What is the management of chronic HBV?
alpha-interferon 2b, lamivudine, adefovir
111
How is HDV transmitted?
It is a defective virus! Requires HBV (HbsAg) to cause coinfection or superimposed infection
112
How is HEV typically transmitted?
feco-oral
| associated with waterborne outbreaks
113
How is HEV diagnosed?
+ IgM anti-HEV
114
What is the management of HEV?
it is a self-limiting virus
115
Which hepatitis has the highest mortality during pregnancy? especially the 3rd trimester?
HEV!
116
```
Window period for HBV:
HBsAg -->
anti-HBs -->
anti-HBc -->
HBeAg -->
anti-Hbe -->
```
```
HBsAg (-)
anti-HBs (-)
anti-HBc (IgM)
HBcAg (-)
anti-Hbe (-)
```
117
```
Acute HBV:
HBsAg -->
anti-HBs -->
anti-HBc -->
HBeAg -->
anti-Hbe -->
```
```
HBsAg (+)
anti-HBs (-)
anti-HBc (IgM)
HBeAg (+/-)
anti-Hbe (+/-)
```
118
```
Recovery HBV:
HBsAg -->
anti-HBs -->
anti-HBc -->
HBeAg -->
anti-Hbe -->
```
```
HBsAg (-)
anti-HBs (+)
anti-HBc (IgG)
HBeAg (-)
anti-Hbe (-)
```
119
```
Immunization against HBV:
HBsAg -->
anti-HBs -->
anti-HBc -->
HBeAg -->
anti-Hbe -->
```
```
HBsAg (-)
anti-HBs (+)
anti-HBc (-)
HBeAg (-)
anti-Hbe (-)
```
120
```
Chronic HBV replicative:
HBsAg -->
anti-HBs -->
anti-HBc -->
HBeAg -->
anti-Hbe -->
```
```
HBsAg (+)
anti-HBs (-)
anti-HBc (IgG)
HBeAg (+)
anti-Hbe (-)
```
121
```
Chronic HBV non-replicative:
HBsAg -->
anti-HBs -->
anti-HBc -->
HBeAg -->
anti-Hbe -->
```
```
HBsAg (+)
anti-HBs (-)
anti-HBc (IgG)
HBeAg (-)
anti-Hbe (+)
```
122
What is the pathophysiology of cirrhosis?
irreversible liver fibrosis with nodular regeneration secondary to chronic liver disease. Nodules cause increased portal pressure!
123
What is the most common cause of cirrhosis of the liver in the U.S.?
ETOH
124
What are other causes of cirrhosis besides ETOH?
1. chronic viral hepatitis (esp. HCV*, HBV, HDV)
2. non-alcohol fatty liver disease
3. hemochromatosis
4. primary sclerosing cholangitis
125
What are the skin manifestations as a result of cirrhosis?
1. spider angioma
2. caput medusa (distended and engorged superficial epigastric veins, which are seen radiating from the umbilicus across the abdomen)
3. jaundice
4. Dupuytren's contractures
5. pruritus
126
What are the diagnostic studies of choice for cirrhosis?
Ultrasound and liver bx
127
What are the screening tests for hepatocellular carcinoma?
Ultrasound + increased alpha fetoprotein + biopsy for definitive dx
128
What are the additional clinical manifestation of cirrhosis (not including skin)?
1. hepatic encephalopathy--> confusion and lethargy, increased ammonia levels
2. esophageal varices--> due to portal HTN
3. ascites
4. hepatosplenomegaly
5. gynecomastia--> liver can't metabolize estrogen
129
What are the most common causes of hepatocellular carcinoma?
```
chronic hepatitis (MC)
aflatoxin exposure (from Aspergillus infxn)
```
130
What is primary biliary cirrhosis (PBC)?
idiopathic autoimmune disorder of the intrahepatic small bile ducts causing decreased bile duct excretion, cirrhosis and ESLD.
131
Who does primary biliary cirrhosis commonly effect?
Middle-aged women (40-60 yrs)
132
What is the clinical manifestation of primary biliary cirrhosis (PBC)?
Most asymptomatic! --> incidental finding of high ALP
May have fatigue, pruritus, jaundice, RUQ discomfort, or hepatomegaly
133
What is the hallmark diagnostic for primary biliary cirrhosis (PBC)?
+ anti-mitochondrial antibody!!
134
What liver enzymes will be elevated with PBC?
ALP with GGT
GGT often strikingly elevated --> reflects cholestasis
ALT, AST, and bilirubin will also be elevated
135
What is the first line management for PBC?
Ursodeoxycholic acid --> protects cholangiocytes from toxic effects of bile acids and stabilizes hepatic inflammation
136
What is the management of pruritus for patients with PBC, PSC, or cirrhosis?
Cholestyramine and UV light
binds bile acids in gut, reducing bile salts' irritant effect on skin
137
What is primary sclerosing cholangitis (PSC)?
autoimmune, progressive cholestasis with diffuse fibrosis of intrahepatic and extra hepatic ducts
Is RARE
138
Primary sclerosing cholangitis (PSC) is most commonly associated with what disease?
inflammatory bowel disease
139
90% of people with primary sclerosing cholangitis also have _____
ulcerative colitis
140
What are the clinical manifestations of primary sclerosing cholangitis (PSC)?
1. progressive jaundice
2. pruritus
3. RUQ pain
4. hepatosplenomegaly
141
What is the definitive management of primary sclerosing cholangitis (PSC)?
Liver transplant
| Meds like steroids and immune meds have no significant benefit
142
What is the diagnostic test of choice for primary sclerosing cholangitis (PSC)?
ERCP
143
What liver enzymes will be significantly elevated with primary sclerosing cholangitis (PSC)?
```
Increased ALP (3-5x) + GGT
+ P-ANCA*
```
144
What is Wilson's disease?
free copper accumulation in liver, brain, kidney, and cornea
145
What is the pathophysiology of Wilson's disease?
autosomal recessive disorder with inadequate bile excretion of copper with increased small intestine absorption of copper --> copper deposition in tissues causing cellular damage
146
What are the clinical manifestations of Wilson's disease?
1. Basal ganglia deposition --> Parkinson-like sx (bradykinesia, tremor, rigidity, dementia)
2. Liver disease --> hepatitis, hepatosplenomegaly, cirrhosis, hemolytic anemia
3. Corneal copper deposits --> Kayser-Fleischer rings
147
How does Wilson's disease affect the corneas?
Kayser-Fleischer rings --> brown or green pigment in cornea
148
How is Wilson's disease diagnosed?
increased urinary copper excretion
| decreased ceruloplasmin
149
What is the serum carrier molecule for copper?
ceruloplasmin
150
What is the management for Wilson's disease?
Ammonium tetrathiomolybdate --> increases urinary copper excretion
Pencillamine --> chelates copper
Zinc --> enhances urinary copper excretion and blocks intestinal copper absorption
151
What must also be given in addition to Pencillamine for the treatment of Wilson's disease?
Pyridoxine/vitamin B6 to prevent depletion
