Usera > Liver 3 (or 2 cont'd) Flashcards
(115 cards)
1
Q
what are the 3 major intrahepatic biliary tract disorders?
A
- primary biliary cirrhosis
- secondary biliary cirrhosis
- primary sclerosing cholangitis
2
Q
how do you get secondary biliary cirrhosis?
A
uncorrected obstruction of the EXTRAhepatic biliary tree
3
Q
what are the clinical features of secondary biliary cirrhosis?
A
pruritis jaundice malaise dark urine light stools HSM
4
Q
what are the 4 lab findings of secondary biliary cirrhosis?
A
- conjugated hyperbilirubinemia
- inc serum alk phos
- inc bile acids
- inc cholesterol
5
Q
what can obstruct the extrahepatic biliary tree & cause secondary biliary cirrhosis (7 things)
A
- extrahepatic cholelithiasis
- malignancy of biliary tree or head of pancreas
- strictures d/t previous surgery
- biliary atresia
- CF
- choledochal cysts
- paucity of bile duct syndromes
6
Q
what happens initially with secondary biliary cirrhosis?
A
cholestasis
it’s reversible if you fix the obstruction
7
Q
what happens when you get secondary inflammation in secondary biliary cirrhosis?
A
initiates periportal fibrosis > hepatic scarring + nodule formation
8
Q
what is primary biliary cirrhosis?
A
inflammatory autoimmune dz affecting the INTRAhepatic bile ducts
9
Q
what is the primary feature of primary biliary cirrhosis?
A
nonsuppurative inflammatory destruction of MEDIUM & SMALL sized intrahepatic bile ducts
10
Q
what is characteristic of primary biliary cirrhosis & essential for the dx?
A
ANTI-MITOCHONDRIAL ABS!
11
Q
what are the 4 clinical features of primary biliary cirrhosis?
A
- middle aged woman
- insidious onset
- fatigue
- abd discomfort
12
Q
what is primary sclerosing cholangitis?
A
chronic cholestatic disorder
13
Q
what 3 things characterize primary sclerosing cholangitis?
A
- non-specific inflammation
- sclerosing fibrosis
- strictures of the LARGE INTRA & EXTRA HEPATIC bile ducts
14
Q
what might cause primary sclerosing cholangitis, broadly speaking?
A
immunologically-mediated injury
15
Q
what is the most important association of primary sclerosing cholangitis?
A
ulcerative colitis
16
Q
what are the top 5 immunologically-mediated injuries that can cause primary sclerosing cholangitis?
A
- ULCERATIVE COLITIS
- anti-smooth muscle ab
- ANA
- rheumatoid factor
- atypical P-ANCA
17
Q
what are the clinical features of primary sclerosing cholangitis?
A
fatigue pruritis jaundice INC alk phos chronic pancreatitis
18
Q
what can primary sclerosing cholangitis predispose you to?
A
cholangiocarcinoma
19
Q
what happens to contrast media when you do radiography in a pt w/ primary sclerosing cholangitis?
A
it beads
in the intra & extra hepatic biliary tree
20
Q
what can you see on histology of primary sclerosing cholangitis?
A
onion-skin sclerosing pattern surrounding large intra (or extra) hepatic bile ducts
21
Q
what are the 4 main anomalies of the biliary tree?
A
- von meyenberg complexes
- polycystic liver dz
- congenital hepatic fibrosis/caroli dz
- alagille syndrome
22
Q
what are von meyenberg complexes?
A
small clusters of modestly dilated bile ducts embedded in fibrous stroma
23
Q
what is another name for von meyenberg complexes?
A
bile duct hamartomas
24
Q
T/F: von meyenberg complexes are rare and dangerous
A
FALSE
common!
clinically insignificant!
25
what are von meyenberg complexes a/w?
PCKD
26
what is polycystic liver dz?
multiple diffuse cysts in the liver
27
what is polycystic liver dz a/w?
PCKD
28
why does congenital hepatic fibrosis a/w caroli's dz arise?
d/t persistance of the embryonic form of the biliary tree
29
what happens to the portal tracts in congenital hepatic fibrosis a/w caroli's dz?
fibrosis
30
do you get cirrhosis w/ congenital hepatic fibrosis a/w caroli's dz?
NOPE
31
what does congenital hepatic fibrosis a/w caroli's dz increase your risk for?
cholangiocarcinoma
32
what is congenital hepatic fibrosis a/w caroli's dz a/w?
PCKD
33
what is caroli dz?
segmental dilation of the larger ducts of the intrahepatic biliary tree
34
what is caroli dz a/w?
congenital hepatic fibrosis
&
PCKD
35
what does caroli dz inc your risk for?
cholangiocarcinoma
36
what is alagille syndrome?
congenital absence/dearth of bile ducts
37
how do you inherit alagille syndrome?
autosomal dominant
38
what are the 5 major clinical features of alagille syndrome?
1. chronic cholestasis
2. peripheral stenosis of pulmonary artery
3. butterfly-like vertebral arch defects
4. eye defects
5. peculiar hypertelic facies
39
T/F: focal nodular hyperplasia is a neoplasm
FALSE
| "not a true neoplasm"
40
what are the 2 major types of hepatic "neoplasms"?
1. focal nodular hyperplasia
| 2. nodular regenerative hyperplasia
41
what is focal nodular hyperplasia?
pseudo-mass lesion in an otherwise normal liver
42
what does focal nodular hyperplasia look like grossly?
central stellate scar w/ radiations of fibrous septae
43
what does focal nodular hyperplasia result from?
congenital vascular malformation
44
what is the most common benign liver tumor?
cavernous hemangioma
45
what is a cavernous hemangioma?
tumor of vascular channels in a bed of fibrous connective tissue
46
what can you confuse w/ a cavernous hemangioma?
metastatic tumors
| but don't confuse them!
47
what should you NOT perform on a cavernous hemangioma?
blind percutaneous biopsy!
48
what is a hepatic adenoma?
a benign tumor arising from hepatocytes
49
what is another name for hepatic adenoma?
liver cell adenoma
50
what is hepatic adenoma strongly a/w?
ORAL CONTRACEPTIVES
&
ANABOLIC STEROIDS
51
what bad thing can happen w/ hepatic adenoma?
subcapsular adenomas can RUPTURE > severe intra-abd hemorrhage
52
what is a hepatoblastoma?
MALIGNANT tumor of young children
53
is hepatoblastoma fatal?
YES, usu w/i a few years
54
what are the 2 types of hepatoblastoma?
1. epithelial type
| 2. mixed epithelial & mesenchymal type
55
what does an epithelial type hepatoblastoma recapitulate?
the developing liver
56
what does a mixed type hepatoblastoma show?
areas of primitive mesenchyme
57
what is hepatoblastoma a/w?
familial adenomatous polyposis syndrome
OR
beckwith-wiedmann syndrome
58
what hepatic neoplasm accounts for >90% of primary liver cancers?
hepatocellular carcinoma
59
what are the 4 important etiologic factors for hepatocellular carcinoma?
1. chronic viral infxn
2. chronic alcoholism
3. non-alcoholic steatohepatitis
4. food contaminants (aflatoxins)
60
what are the 3 morphological types of hepatocellular carcinoma?
1. uninodular
2. multinodular
3. diffusely infiltrative
61
what does hepatocellular carcinoma have a strong propensity for?
invasion of vascular structures
62
what vascular structures does hepatocellular carcinoma like to invade?
portal vein & IVC
63
what are satellite nodules?
intrahepatic metastases of hepatocellular carcinoma
64
what type of variant are 5% of hepatocellular carcinomas?
fibrolamellar variant
65
who gets fibrolamellar variant HCC?
young M & F w/o underlying chronic liver dz or cirrhosis
66
what lab value increases in fibrolamellar variant HCC?
serum AFP
67
what is cholangiocarcinoma?
malignancy of the biliary tree arising from bile ducts w/i & outside the liver
68
what are the 4 risk factors for cholangiocarcinoma?
1. primary sclerosing cholangitis
2. congenital fibropolycystic dz
3. HCV infxn
4. thorotrast
69
what is the distribution of cholangiocarcinomas?
60% perihilar
20-30% distal
10% intrahepatic
70
what are Klatskin tumors?
extrahepatic perihilar tumors near the formation of the hepatic duct
(cholangiocarcinoma)
71
what is notable about cholangiocarcinoma on histo?
"brisk desmoplastic response"
72
what is the most common sarcoma arising in the liver?
angiosarcoma
73
what is the behavior of angiosarcoma?
VERY AGGRESSIVE
malignant
widespread mets
74
what 3 things can cause angiosarcoma?
1. thorotrast
2. vinyl chloride
3. arsenic exposure
75
which is more common: metastatic tumors or primary hepatic neoplasia?
metastatic tumors
76
what are the 4 most common sites for metastasis of liver cancer?
1. colon
2. breast
3. lung
4. pancreas
77
what are the 3 major groupings of circulatory disorders involving the liver?
1. impaired blood into the liver
2. impaired blood flow thru the liver
3. hepatic venous outflow obstruction
(in, thru, out)
78
what 2 things can cause impaired blood INFLOW to the liver?
1. hepatic artery compromise
| 2. portal vein obstruction & thrombosis
79
what causes hepatic artery compromise?
embolism
neoplasia
polyarteritis nodosa
sepsis
80
what can hepatic artery compromise result in?
liver infarct
| not often though, bc the liver has a dual blood supply
81
what are the sx of portal vein obstruction & thrombosis?
abd pain
portal HTN
ascites
82
what 7 things can cause EXTRAhepatic obstruction?
1. subclinical occlusion from neonatal sepsis or umbilical vein cath
2. intrabd sepsis
3. hypercoagulable disorders
4. trauma
5. pancreatitis or pancreatic cancer
6. invasion by HCC
7. cirrhosis
83
what can cause INTRAhepatic obstruction?
acute thrombosis
84
what 7 things can cause impaired blood flow THROUGH the liver?
1. R & L sided HEART FAILURE
2. cirrhosis
3. DIC
4. mets
5. eclampsia
6. peliosis hepatis
7. sickle cell
85
what is budd-chiari syndrome?
thrombosis of 2 or more hepatic veins
86
what does budd-chiari syndrome produce?
liver enlargement
pain
ascites
87
what are predisposing factors for budd-chiari syndrome that 3/4 of pts have?
1. hypercoagulable state
2. polycythemia vera
3. factor V leiden mutation
4. contraceptive use
5. pregnancy
88
what is the cause of acute fatty liver of pregnancy?
mitochondrial dysfxn
89
what is the primary treatment for acute fatty liver of pregnancy?
terminate the pregnancy
90
what does the range of sx for acute fatty liver of pregnancy include?
hepatic dysfxn, failure, coma, death
91
what is intrahepatic cholestasis of pregnancy?
altered hormonal state of pregnancy w/ biliary defects in secretion >>> cholestasis
92
T/F: acute fatty liver of pregnancy & intrahepatic cholestasis of pregnancy are both malignant
false
| intrahepatic cholestasis is benign
93
what % of the population is affected by cholelithiasis?
10-20%
94
what is responsible for >95% of biliary tract dz?
cholelithiasis
95
what are the 2 types of cholelithiasis?
1. cholesterol stones
| 2. pigment stones
96
what are cholesterol stones composed of?
cholesterol
97
what are pigment stones composed of?
bilirubin calcium salts
98
what is cholecystitis?
inflammation of the gallbladder
| can be acute, chronic, or acute superimposed on chronic
99
what are the 2 types of acute cholecystitis?
calculous or acalculous
100
what is acute calculous cholecystitis d/t?
chemical irritation & inflammation from obstruction of the neck or cystic duct
101
what is acute acalculous cholecystitis d/t?
ischemia
102
what is weird about chronic cholecystitis?
evolution is obscure
| can have calculous or acalculous forms
103
what are the clinical features of acute cholecystitis?
```
RUQ or epigastric pain
fever
anorexia
tachycardia
N/V
```
104
what are the clinical features of chronic cholecystitis?
recurrent attacks of steady or colicky RUQ or epigastric pain
N/V
fatty food intolerance
105
what are the 6 complications of cholecystitis (ugh sorry)?
1. bacterial superinfection, cholangitis, sepsis
2. gallbladder perforation + abscess
3. gallbladder rupture + peritonitis
4. biliary enteric fistula
5. porcelain gallbladder
6. aggravation of preexisting medical illness
106
what is biliary atresia?
complete or partial obstruction of the lumen of the extrahepatic biliary tree w/i the first 3 mos of life
107
what characterizes biliary atresia?
progressive inflammation & fibrosis of intra & extra hepatic ducts
108
what are the 2 forms of biliary atresia we need to know?
1. fetal
| 2. perinatal
109
what is fetal biliary atresia?
aberrant intrauterine dvlpmnt of the extra hepatic biliary tree
110
what is perinatal biliary atresia?
normally-formed biliary tree is destroyed following birth
111
how do infants w/ biliary atresia present?
persistent neonatal cholestasis
112
what type are most cases of biliary atresia?
type 4
113
what are choledochal cysts?
congenital dilations of the common bile duct
114
how do pts w/ choledochal cysts present?
jaundice or biliary colic
115
what are choledochal cyst pts predisposed to?
```
stone formation
stenosis
stricture
pancreatitis
obstructive biliary complications
```
