Liver, Gallbladder, Pancreas 2 - Dr. Dobson Flashcards
(162 cards)
1
Q
Choledochal cysts
A
congenital dilation of the common bile duct
before age 10yo, jaundice + biliary colic = recurrent abd pain
2
Q
Choledochal cysts risk of getting what
A
- stones
- stenosis + strictures
- pancreatits
- obstructive biliary complications in liver
3
Q
Fibropolycystic disease
A
group of lesions causing congenital malformation of the biliary tree
4
Q
Fibropolycystic disease lesions happen how
A
persistence of fetal periportal ductal plates
5
Q
Fibropolycystic disease associated with what
A
- polycystic renal disease
- cholangiocarcinoma
- Von Meyenburg complexes (Hamartomas)
6
Q
Fibropolycystic disease histology
A
Von Meyenburg complexes (Hamartomas)
7
Q
Caroli disease is what
A
multifocal cystic dilation of large intrahepatic bile ducts
8
Q
Caroli syndrome is what
A
Caroli disease + congenital hepatic fibrosis
9
Q
impaired BF to portal vein or extrahepatic veins = inflow (obstruction or thrombosis) SX
A
esophageal varices, splenomegally, GI congestion
10
Q
Impaired BF in intrahepatic vessels (cirrhosis, sinusoid occlusion) SX
A
Ascities
varices
hepatomegaly
high AST/ ALP
11
Q
Impaired hepatic vein (outflow) destruction thrombosis = Budd-chiari syndrome, sinusoidal obstructive syndrome SX
A
Ascities hepatomegaly ABD pain jaundice high AST /ALP
12
Q
Hepatic Artery occlusion causes
A
local infarct (pale or hemorhagic)
13
Q
Hepatic Artery occlusion common causes
A
- neoplasm
- polyarteritis nodosa (vasculitis)
- sepsis
14
Q
portal vein obstruction causes what
A
well tolerated to catastrophic sx
= ABD pain, portal HTN (varices that can rupture)
15
Q
extrahepatic portal vein obstruction happens how often and can be caused by what in neonatal
A
- 1/3 of cases
2. neonatal umbilical sepsis, umbilical vein catheterization (varices bleeding and ascites years later)
16
Q
Hepatic Artery occlusion caused by what in adults 6
A
- acute diverticulitits ABD infection —-> pyelophlebitis in spleen
- hypercoag. state (thrombosis)
- trauma
- pancreatitis or pancreatic cancer causing splenic vein thrombosis
- portal vein invades into it from HCC
- cirrhosis
17
Q
small portal vein obstruction cause
A
schistosomiasis **
prothrombotic state, virus, drugs, chronic biliary obstr., autoimmune
18
Q
small portal vein obstruction SX
A
upper GI bleed
splenomegaly
19
Q
most common cause of impaired BF to liver
A
cirrhosis
20
Q
sinusoidal BF occlusion caused by what 4
A
- Sickle cell
- DIC
- Eclampsia
- Diffuse intrasinusoidal tumor
21
Q
sinusoidal BF occlusion can lead to what
A
massive necrosis, acute liver failure
22
Q
Peliosis hepatis is what
A
- type of sinusoidal dilation, CAUSING —-I EFFLUX of hepatic blood
- blood filled cystic spaces in liver
23
Q
Peliosis hepatis can cause what complication
A
ABD hemorrhage
24
Q
Hepatic Vein thrombosis is what
A
Budd - Chiari Syndrome
= deadly if not tx
25
Budd - Chiari Syndrome TRIAD
1. hepatomegaly
2. ABD pain
3. ascities
26
Budd - Chiari Syndrome associated conditions : 5
1. myeloproliferative neoplasms
2. inherited hypercoag.
3. anti-phospholipid AB syndrome
4. PNH (proxysmal nocturnal hemoglobinuria)
5. HCC
27
Sinusoidal obstruction syndrome is what
pyrrolizidine alkaloid in bush tea from Jamaica = veno-occlusive disease*
28
Sinusoidal obstruction syndrome caused by what + mortality rate
1. post hematopoietic stem cell transplant*
2. chemo
= 80% mortality
= jamaican tea
= vano-occlusive disease
29
Sinusoidal obstruction syndrome DX
1. biposy gold standard only risky
2. hepatomegaly, jaundice, ascitis, WG,
3. attenuated hepatic venous flow (Doppler US)**
30
which organ is effected most after an organ transplant
liver
31
jaundice in preg caused by
Viral hep (20% fatal if HEV)
32
acute liver failure in preg also caused by
HSV
33
liver abscesses in preg caused by
Listeria
34
3 serious liver complications in preg.
1. eclampsia / preeclampsia
2. acute fatty liver
3. intrahepatic cholestasis
35
4 sx of eclampsia / preeclampsia that happen
1. maternal HTN
2. proteinuria
3. peripheral edema
4. coagulation abnormal
36
eclampsia / preeclampsia what you see in pt with this (acronym)
HELLP
1. Hemolysis
2. Elevated Liver enzymes (AST /ALP)
3. Low Plts
37
preeclampsia becomes eclampsia when
when hyperreflexia and convulsions happen
38
eclampsia / preeclampsia most dangerous in who
women with acute fatty liver of preg
39
acute fatty liver of preg sx and tx and complications
1. bleeding, N/V, jaundice, coma
2. termination of preg
3. hepatic failure and death
40
intrahepatic cholestasis of preg sx
pruritus in 2nd or 3rd trimester, darkening urine, light color stools
41
intrahepatic cholestasis of preg labs, histology
1. low bilirubin,
| 2. canicular cholestasis
42
intrahepatic cholestasis of preg tx and associated with what risk
1. resolved 2-3 weeks after delivery
| 2. fetal loss, and recur in future preg
43
liver infarction risk
RARE (dual BS, 1/3 hepatic A, 2/3 portal vein)
| = bile ducts can die if hepatic A obstructed since they get O2 from artery only)
44
most common cause of varices
portal vein occlusion
45
obliterative portal venopathy caused by
HIV
46
sinusoidal dilations caused by
peliosis hepatis
47
passive liver congestion caused by
right heart failure
48
chronic vs acute transplant rejection
acute : cellular
| chronic : vascular
49
nutmug liver formed from
hypoperfusion, retrograde congestion
50
Focal Nodular Hepatis (FNH) : what and looks like
altered BF causing hyperplastic changes, and a mass development in liver
= single well marked lesion, central scar with no capsule (less then 5cm)
51
Focal Nodular Hepatis (FNH) : histology
"map like pattern"
52
Cavernous hemangioma of liver : prevalence and location and risk
1. most common benign liver neoplasm, F
2. subcapsular location
3. hemorrhage
53
Hepatocellular adenoma is what , who, from what (Strong link or association)
benign neoplsm ,
young women,
oral contraceptives + anabolic steroids, obesity
54
Hepatocellular adenoma risk
rupture hemorrhage
55
Hepatocellular adenoma 3 subtypes
1. HNF1-a adenoma: F, HCC can happen , fatty looking
2. Inflammatory adenoma: F, obesity, malignant risk if from B-catenin, amyloid
3. B-Catenin Adenoma : malignant risk to HCC, oral contraceptives, anabolic steroids
56
2 most common primary epithelial tumors of liver
1. HCC
| 2. cholangiocarcinoma
57
pediatric liver tumor most common
hepatoblastoma
58
nonepithelial tumor of liver
angiosarcoma
59
rare primary hepatic lymphoma
Diffuse Large B-cell lymphoma (DLBCL)
60
hepatoblastoma associated with 2 diseases
1. Familial Adenomatous polyposis
| 2. Beckwith-Wiedemann syndrome
61
hepatoblastoma SX
abnormal ABD swelling (can see jaundice and pruritus)
62
hepatoblastoma complication
20% of times tumor has gone to lungs by the time of DX
63
Hepatocellular Carcinoma (HCC)
prevalence
what has made this increase in rate in western countries
1. most common primary malignancy to hepatocytes (esp. chronic liver disease with cirrhosis)
2. HCV and Metabolic syndrome + alcolol****
64
Aflatoxin is what and from what
fungi toxin in crops = aspergillus flavus and aspergillus parasiticus
65
noncirrhotic livers that can cause HCC
aflatoxin, adenomas
66
fibrolamellar carcinoma is what and histology
type of HCC (more survivor rate)
| cirrhosis tumor with fibrous band (large polygonal cells + granular cytoplasm, dense collagen bundles
67
unresectable tumors tx
image guided tumor ablation with alcohol or radiofequency waves
68
intrahepatic cholangiocarcinoma is what
adenocarcinoma
69
extrahepatic cholangiocarcinoma
biliary adenocarcinoma
70
intrahepatic cholangiocarcinoma most common locations and reason
= SE asia (thiland, Laos, Camboidia)
| = liver fluke infection high
71
intrahepatic cholangiocarcinoma sx and spreads
spreads to perineural and LN
| none usually accidental, or cholastasis, feel liver mass
72
extrahepatic cholangiocarcinoma sx
biliary tree obstruction sx
73
angiosarcoma hepatic, associated with what
vinyl chloride, arsenic, thorotrast exposure
74
Lymphoma in liver usually associated with
DLBCL mostly and then
| MALToma
75
Metastatic liver associated with
primary hepatic neoplasia (however if from some other place mostly from colon, breast, lung, pancreas)
76
liver secretes how much bile per day and GB can store how much
1L per day
| and 50mL storage in GB
77
GB formed from what embryonically
superior bud of the hepatic diverticulum
78
CCK function and secreted by
1. SM in GB to contract and release bile, relax muscular sphincter of Oddi
2. from duodenal cells
79
most common anomaly in gallbladder congenital defect
fundus folding = phrygian cap
80
most common disease causing biliary tract disease
Cholelithiasis
81
2 types of gallstones
1. cholesterol stones (developed countries, high in native Americans)
2. pigment stones (developing countries, bacterial infection, parasitic infection, chronic red cell hemolysis)
82
cholesterol gallstones contain what and from where
from gallbladder, 100% to 50% cholesterol + calcium carbonate, phosphates, bilirubin
83
cholesterol gallstones with a lot of cholesterol look like :
| cholesterol gallstones with a lot of calcium carbonate look like :
1. radiolucent
| 2. radiopaque
84
black stones have what + from where + look like
```
= oxidized calcium salts (unconjugated bilirubin )
= ca carbonate
= ca phosphate
cholesterol a little
FROM : sterile GB bile
LOOK : radiopaque
```
85
brown stones have what and from where + look like
= same as black + more cholesterol + calcium salts
FROM : infected large bile ducts
LOOK : radiolucent
86
risk factors of cholesterol cholelithiasis
- F
- Fertile
- Fat
- Forty
- Fair skinned
- FH
87
risk factors of pigment cholelithiasis
- clinorchia sinensis
- Ascaris Lumbricoides
- E. Coli
88
types of stones that are more dangerous
smaller ones, they can travel and obstruct `
89
consequences of cholelithiasis
- cholecystitis
- cholangitis
- cholestasis
- pancreatits
- GB carcinoma
90
Gallstone ileus other name and what
Bouvert syndrome = large stone eroding into SI causing obstruction
91
cholecystitis is what
inflammation of the gallbaldder
92
acute cholecystitis : what + SX
obstruction in neck of GB or cystic duct by stone
= RUQ/ epi pain, fever, tachy, N/V,
= no jaundice, can have elevated alk phosphatase
93
acute calculous cholecystitis : what is it and tx
FROM GALLSTONES
| resolve on its own or emergency surgery
94
acute acalculous cholecystitis : dx and sx
NOT FROM GALLSTONES
hard to dx since pt have sx not referrable to GB, failure to recognize early can lead to death = gangrene and perforation can happen
95
acute acalculous cholecystitis : how pt can get this
= rare : S. Typhi and staph,
= severe atherosclerotic ischemic disease
= ascending biliary tree infection
96
chronic cholecystitis happens usually from
no other previous cholecystitis usually, however can be from repeated mild to severe acute cholecystitis
97
carcinoma of GB prevalence and risks
= most common extrahepatic biliary tract malignancy , Female
= gallstones (95% of cases), chronic bacterial or parasitic infections have been risks in Asia
98
most carcinomas in GB are located where and what type and common sites of spread
1. fundus
2. adenocarcinomas
3. lungs, liver, cystic duct, bile ducts, GI, peritoneum, porta-hepatic LNs
99
sterile perintonitis
leakage of bile or pancreatic enzymes into peritoneum
100
bacteria superinfection to the peritoneum happens when
perforation or rupture of biliary tree system
101
granulomatous reaction to the peritoneum happens how
ruptured dermoid cysts releasing keratins
102
spontaneous bacterial peritonitis happens how
from ascites and cirrhosis
103
Idiopathic Retroperitoneal Fibrosis is what and happens how + SX
= fibroinflammatory process surrounding the abd aorta and ureters, higher in M
= IgG4 related disease
= back + ABD pain
104
Idiopathic Retroperitoneal Fibrosis DX and labs
= radiologic imaging, bilateral impingement on ureters, hydronephrosis
= soft tissue in retroperitoneum,
= ureter obstruction ----> acute renal failure \
= high C-reactive P, HIGH erythrocyte sedimentation rates
105
Idiopathic Retroperitoneal Fibrosis can happen from what
= IgG4 related disease
| = radiation therapy from tumors or lymphoma
106
Idiopathic Retroperitoneal Fibrosis histology
= dense fibrosis and chronic inflammation (small lymphocytes)
= high IgG4/IgG plasma cell ratio
107
primary tumor of the Peritoneum
Mesotheliomas : desmoplastic small round cell tumor localized at some place= can metastasize into peritoneal carcinomatosis
108
location of the pancreas
from C-loop of duodenum to the hilum of the spleen
109
2 ducts from pancreas emptying into the duodenum
1. pancreatic duct ----> Major duodenal papilla (sphincter of oddi, connection to the bile duct)
2. accessory pancreatic duct -----> minor duodenal papilla
110
uncinate process
ventral pancreas that rotates during development with bile duct attached to it
= fuses with the dorsal pancreas (tail and body)
111
exocrine vs endocrine pancreas
1. exocrine : 80%, zymogens
| 2. islets of Langerhans cells clusters, secreting insulin and glucagon
112
congenital anomalie of pancreas most common
1. Pancreas divisum : ventral and dorsal bud dont fuse, so the majority of tail and body of pancreas only drain from the minor papilla
= can cause chronic pancreatitis
113
annular pancreas
= many also have pancreas divisum
= bilobed ventral lobe each going around the foregut endoderm in opposite directions to the dorsal lobe -----> duodenal obstruction can happen
114
ectopic pancreas
area not supposed to be in, stomach, duodenum,
= can cause pain if local inflammation
= rare to cause mucosal bleeding
115
most enzymes are packed how in the pancreas
into secretory granules as inactive proenzymes = zymogens
116
proenzymes are activated how and by what
1. enteropeptidase (enterokinase) = activates Trypsin in the DUODENUM
2. Trypsin activates proenzymes
117
acinar and ductal cells of the pancreas secrete what
trypsin inhibitors like SPINK1 (Serine proteas inhibitor Kazal type 1
118
severe acute pancreatitis causes what SX and things to happen
1. enzymes and cytokines released into circulation
= systemic inflammation, leukocytosis, DIC, edema, acute respiratory distress
= acute renal necrosis
119
severe acute pancreatitis can present how and tx
1. severe sudden epigastric pain, high serum lipase
| 2. fluid hyrdation, pain meds, bowel rest, emergency surgery
120
PE sx on severe acute pancreatitis
1. Cullens sign : periumbilical ecchymosis
2. Grey Turners sign : flank ecchymosis
3. jaundice, abd distension
121
ecchymosis from pancreatitis happen how
from free pancreatic enzymes causing diffusion of fat necrosis and retroperitoneal / intraabdominal bleeding
122
diffusion of fat necrosis and inflammation in Cullens sign happens how
from retroperitoneum to umbilicus (Through the round ligament)
123
diffusion of fat necrosis and inflammation in Grey Turners sign happens how
from retroperitoneum to the subQ tissues of flanks
124
sx of high mortality in sever acute pancreatitis
eccymosis signs
125
radiation of pain in acute pancreatitis + 2 other labs that are elevated besides lipase * and amylase (10 % of pts and if fat necrosis)
1. upper / mid back + left shoulder
2. Glycosuria (10% of pts)
Hypocalcemia (if saponification of necrotic fat)
126
trypsin activated in the pancreas can do what
1. activate proenzymes = autodigestion
2. blood components : coag, complement, fibrinolytic pathways = inflammation + small vessels thrombosis , causing more duct cell destruction and trypsin activation
127
CFTR and acute pancreatitis
X bicarbonate secretion + obstruct ducts with mucus = duct destruction and trypsin activation
128
PRSS1 and acute pancreatitis
cationic trypsin (mutated serine protease 1), = X self inactivation of trypsin
129
SPINK1 and acute pancreatitis
serine peptidase inhibitor kazal type 1 : INHIBITS trypsin activation (mutated)
130
CASR and acute pancreatitis
ca- sensing R in ECF : X not working so alters Ca concentrations in ducts + trypsin gets activated
131
CTRC and acute pancreatitis
Chymotrypsin C (caldecrin) : degrades trypsin , protects pancreas from destruction from trypsin (mutated)
132
CPA1 and acute pancreatitis
Carboxypeptidase A1 : exopeptidase regulates zymogen activation by trypsin (mutated)
133
stages of acute pancreatitis in sx
1. microvascular leak and edema
2. fat necrosis
3. acute inflammation
4. damage + autodigestion (pancreatic parenchyma)
5. BV destruction + interstitial hemorrhage
134
brown fluid in peritoneum
fat cells digested from lipase
135
acute pancreatitis tx
bowel rest : NPO (IV fluids, analgesia)
| most recover
136
40% to 60% acute necrotizing pancreatitis pts get what
acellular debri gets infected (gram- from gI) = further complicating disease
137
lungs and acute pancreatitis
ARDS
138
most common cause of chronic pancreatitis and what is it
1. long term alcohol use
| 2. irreversible destruction of exocrine parenchyma, fibrosis + later loss of endocrine parenchyma
139
chronic pancreatitis cytokine involved in fibrosis
TGF-B causing fibrosis after injury from inflammation, activated by M,
140
autoimmune pancreatitis type 1 and type 2
TYPE 1 : IgG4 plasma cells in pancreas (IgG related disese)
| TYPE 2: mass in head on imaging = looks like carcinoma (pts with UC)
141
risks of chronic pancreatitis
1. repeated acute pancreatitis with persistent back / abd pain
2. calcifications
3. exocrine insufficiency
4. DM
142
hereditary pancreatitis with high risk of becoming cancer
PRSS-1 mutation (40% chance)
143
most common cysts on pancreas
nonneoplastic pseudocysts
144
Congenital cysts in pancreas
rare, thin walled cyst from anomaly in pancreatic duct development
= serous fluid filled fibrous capsule
145
congenital cycts can be associated with what 2 conditions
1. Polycystic Kidney Disease
| 2. von Hippel -Lindau disease
146
nonneoplastic pseudocysts lining and from what and tx
no epithelial lining
from acute pancreatitis, esp from chronic alcoholic pancreatitis, trauma
= spontaneously resolve (can compress adjacent structures, or get infected)
147
serous cystic neoplasm what, prevalence, location
benign, Female, 6th-7th decade, pancreas tail
148
Mucinous cystic neoplasms what, prevalence, location
carcinomas, 95% Female, tail
149
Solid-pseudopapillary neoplasms what, prevalence, location
young women, tail, some can be locally aggressive
150
Intraductal papillary mucinous neoplasms (IPMNs) what, prevalence, location
connected to duct system in pancreas,
Men
Head of pancreas
can become cancer
151
pancreatic cancer 5yr survival rate
10%
152
pancreatic cancer high risk groups
AA, Japanese, Native Americans, Hawaiians, Jews
153
strongest risk of pancreatic cancer + other risks
smoking *
- chronic pancreatitis
- visceral obesity, high BMI
- DM
154
invasice pancreatic cancers come from what
noninvasive precursor lesions (Pancreatic intraepithelial neoplasms (PanIN)
155
PanIN stages
1. telomere shortening, KRAS activated
2. CDKN2A inactivated
3. TP53, SMAD4, BRCA2 inactivated
4. invasive carcinoma
156
Pancreatic ductal adenocarcinomas 4 genes involved
KRAS*, TP53*, CDKN2A*, SMAD4* (C/G to T/A or CpG>TpG mutations) = from methylation
157
Pancreas cancers 1st sx and when and most common location
pain, when it has invaded into adjacent structures, so beyond cure at that point, head
158
head pancreatic cancer sx and what you see in imaging
distal common bile duct obstruction, = obstructive jaundice, Courvoiser sign* = palpable mass felt above GB nontender
159
pancreatic cancer speads most common to
lung and liver
| grow along nerves and invade into BVs + retroperitoneum
160
pancreatic cancer type
adenocarcinoma with dense desmoplastic reaction
161
Migratory thrombophlebitis
Trousseau sign** = 10% pts with pancreatic cancer , tumor associated inflammation and coag factors = high risk of thromboembolism in BVs
162
some serum markers for pancreatic cancer
CA19-9 Ag + Carcinoembryonic Ag not specific enough, Early detection is key for survival and tx with resection
