Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

25. GI 2 Exam 2 > 4. GI Tract Liver Path > Flashcards

4. GI Tract Liver Path Flashcards

1
Q

The liver weights around 1500G and has a dual blood supply- 2/3 portal, 1/3 hepatic artery, the liver hilum is called the porta hepatis. What is the name of the functional unit of the liver?

A

Lobule

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

The lobule is the functional unit of the liver, with a terminal hepatic vein in the midle and portal tracts in the periphery. Which zone (1-3) is the farthest away from blood flow and most apt to become necrotic first?

A

Zone 3

Zone 1 is at highest risk for Toxin damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What kind of stain is used to highlight fibrous tissue- coloring it blue?

A

Trichrome Stain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What cells in the liver are mononuclear phagocytes attached to the luminal face of endothelial cells, and fat containing myofibroblastic hepatice stellate cells are found in the space of Disse?

A

Kupffer cells (liver macrophages)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

The main functions of the liver include energy regeneration and substrate interconversion, synthesis and secretion of plasma proteins, solubilizing, transport and storage function and protective and?

A

clearance functions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Hepatic damage may occur secondary to most common disease of illness including heart failure, disseminated cancer and extrahepatic?

A

infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Acute viral hepatitis, ischemic hepatitis and acetaminophen overdose would have a typical range of AST and ALT of over?

A

1000U/L **very high

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Most liver diseases are chronic since its a large organ, less than 26 weeks is classified as a acute liver disease. Liver disease is an insidious process in which clinical detection and symptoms of hepatic decompensation may occur in weeks, months or?

A

years after the onset of injury

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Reversible changes in the hepatocytes include steatosis which is accumulation of fat in the liver, swelling and what, which is accumulation of bilirubin in the liver?

A

Cholestasis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Hepatocyte necrosis is when fluid flows into the cell, the cell swells, and ruptures when osmotic reguation is interrupted, blebs form to carry off intracellular stuff to extracellular, mø cluster at the site of injury, the predominant cuase of death is due to what two things?

A

Ischemic or Hypoxic injury due to oxidative stress

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Hepatocyte apoptosis is the other form of cell death, in which the hepatocyte shrinks, nuclear chromatin condensation (pyknosis), fragmentation (karyorrhexis) and cellular fragmentation into acidophilic apoptotic bodies occurs, what are yellow bodies due to yellow fever?

A

Councilman bodies

apoptosis via DNA damage, accum of misfolded proteins, cetain infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What necrosis is widespread parenchymal loss, severe zonal loss of hepatocytes, may begin around central vein and produces space filled with cellular debris, mø, and remnants of reticular meshwork- seen in acute toxic injury, ischemic injur or viral hepatitis?

A

Confluent Necrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What type of necrosis in the liver is when a zone links central veins to portal tracts or bridges protal tracts, vascular insult leads to parenchymal extinction due to large areas of contiguous hepatocyte death, collapse of supporting framework and cirrhosis may occur?

A

Bridging Necrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Regeneration in the liver occurs as mitotic replication adjacent to those that have died. Stem cell like* hepatocytes can replicate even in the setting of chronic injury, meaning that what is not a significant part of parenchymal repair?

A

Stem cell replenishment (since job is done by the hepatocytes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

The prinicple cell type involved in scar deposition is what cell? Which normally is a lipid (vitA) storing cell, however in several forms of acute and chronic injury, the cell becomes activated and converted to highly fibrinogenic myofibroblasts

A

hepatic stellate cells** = fibrogenic myofibroblasts

Note: activation via increase in PDGFRB/TNF, cytokines from Kupffer cells - TGFB/MMP2/ TIMP1/2 = fibrosis, contraction via endothelin 1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the serum measurements for hepatocyte integrity? 3

A
  • Aspartate aminotransferase (AST)
  • Alanine aminotransferase (ALT)
  • Lactate dehydrogenase (LDH)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What are the tests that look for biliary excretory function? 3

A
  • Serum bilirubin
  • Urine bilirubin
  • Serum bile acids
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What are the tests that look for damage to the bile canaliculus?

A
  • Serum alkaline phosphatase

* Serum gamma-glutamyl transpeptidase (GGT)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What are the tests that look for hepatocyte synthetic function? 3

A
  • Serum *albumin
  • *Coagulation factors: PT, PTT, fibrinogen, prothrombin, factors V, VII, IX, and X
  • Hepatocyte metabolism: serum *ammonia, aminopyrine breath test (hepatic demethylation)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Regeneration of the liver occurs mainly by proliferation of remaining hepatocytes and repopulation from progenitor cells such as the canals of?

A

Hering (intrahepatic biliary tract)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Liver failure occurs when 80-90% of the functional capacity of the liver is lost, 80% mortality without transplant may be due to acute injury, chronic progressive injury or?

A

acute on chronic injury

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What is the first anatomical structure within the liver to dissappear after regeneration occurs?

A

Central vein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Acute liver failure occurs within 26 weeks of initial injury, with an absence of prexisting liver disease, associated with encephalopathy and?

A

coagulopathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Acute liver failure is commonly due to massive hepatic necrosis d/t drugs/toxins… Mnemonic
A: Acetaminophen (**50% of ALF d/t this-ZONE3)
B: Hepatitis B
C: Hepatitis C / Cryptogenic
D: Drugs/Hepatitis D
E: Hepatitis E/ Eosteric causes (wilson)
F:?

A

Fatty change of microvasculature (pregnancy, valporate, tetracycline, Reye syndrome)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Morphologically, acute liver failure has massive hepatic necrosiss leading to parenchymal loss and regeneration, see a small shrunk liver, early scarring in a few weeks, what is deinfed as diffuse poisoning of liver cells without cell death and parenchymal collapse, related to fatty liver of pregnancy?

A

Diffuse Microvesicular Steatosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Clinically with acute liver failure, patient presents with nausea, vomiting, itching, icterus and jaundice progressing to life threatening encephalopathy and coagulation defects, there is a moderate increase in?

A

Liver transaminases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

with ALF there is hepatomegaly due to hepatic swelling, infiltrates and edema, leading to shrunken liver, the following would indicate what? decrease liver enzymes, indicating few remaining hepatocytes confirmed with worsening jaundice, caugulopathy and encephalopathy?

A

POOR prognosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Cholestasis increase the risk of life threatening bacterial infection in acute liver failure. Hepatic encephalopathy d/t increase serum ammonia causing behavioral abnormalities to marked confusion and coma, there is rigidity and hyperreflexia along with a characteristic sign known as what? (nonrhythmic, rapid extension-flexion of the head and wrists)

A

*Asterixis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

with ALF, coagulopathy occurs = impaired clotting due to lack of VitK, factor 10,9,7,2, leading to easy bruising and intracranial bleed, disseminated intravascular coagulation DIC occurs due to failure of liver to remove activated coag factors, and Portal HTN may occurs d/t intrahepatic obstruction causing what two things?

A

Ascites and Hepatic Encephalopathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What is a form of renal failure in indiviuals with liver failure in which their kidneys are morphologically and functionally normal?

A

Hepatorenal syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Chronic liver failure is associated with cirrhosis, chornic hepatitis B and C, non-alcoholic fatty liver disease, and alcoholic fatty liver disease, the ultimate cause of death in chronic is the same in acute, including encephalopathy, bleeding from E varices and?

A

bacterial infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What is diffuse transformation of the entire liver into regenerative parenchymal nodules surrounded by fibrous bands and vairable degrees of vascular (portosystemic) shunting?

A

Cirrhosis- NOT diagnosis

cryptogenic = no clear cause

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Child-Pugh classification of cirrhosis helps monitor the decline of patients on the path to chronic liver failure, Class A is well compensated, B is partially, and C is decompensated. What has an increased incidence in cirrhosis with broad bands of dense scar with dilated lymphatic spaces, less parenchyma, more likely to progress and lead to end stage renal disease?

A

Portal hypertension likelihood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Early stem cell activation is seen in the form of ductular reactions which increase with advancing stage of disease and are usually more prominent in?

A

cirrhosis

**regression of fibrosis does occur- so cant ALWAYS assoc. cirrhosis w end stage liver dz

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Clinical symptoms of chronic liver disease before cirrhosis- 40% are asymptomatic until advanced stages, there is anorexia, weight loss, weakness, jaundice, pruritus, hypoalbuminemia, hyperammonemia, and there is hyperestrogenemia in men d/t impaired metabolism which leads to what? 4

A

Palmer erythema
Spider angiomas
Gynecomastia
Hypogonadism

(sx of acute liver failure + portal HTN)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

The following are what causes of portal HTN?
Obstructive thrombosis, narrowing of the portal vein before entering the liver, massive splenomegaly with icnreased splenic blood flow

A

Prehepatic Causes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

The following are what causes of portal HTN?

severe right heart failure, constrictive pericarditis, hepatic vein outflow obstruction

A

Posthepatic Causes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

The following are what causes of portal HTN?
**Cirrhosis = MCC, schitomiasis, massive fatty change, diffuse fibrosing granulomatous disease (sarcoidosis), dz affecting microcirculation (regeneration), increased resistance to portal flow, increased portal venous flow d/t hyperdynamic circulation

A

Intrahepatic causes of Portal HTN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Increased resistance to portal flow at the level of the sinusoids occurs via decreased NO production, increased endothelin, angiotensin, disruption of BF due to scarring. INcreased portal venous flow is due to NO, prostacyclin and TNF as well as what which is increased effleux into the portal venous system?

A

Splanchnic Arterial vasodilation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

There are four major clinical consequences of portal hypertension, including ascites (85%), portosystemic shunting (esophageal varices, hemorrhoids, caputmedusae), hepatic encephalopathy, and splenomegaly which can lead to?

A

inducing hematologic abnormalities = HYPERsplenism such as thrombocytopenia and pancytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Ascites occurs in 85% people with cirrhosis and occurs if >500mL in stomach with fluid wave, shifting dullness, puddle sign, composed of transudate (serous). Occurs via sinusoidal hypertension, percolation of hepatic lymph into perinoeal cavity and splanchnic?

A

vasodilation and hyperdynamic circulation (causes transudation)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Hepatic sinusoidal HTN drives fluid into space of Disse which is drain by lymphatics, movement also influenced by hypoalbuminemia, percolation of hepatic lymph occurs due to increased amount to 20L (NL: 1000mL), and what cannot keep up?

A

thoracic duct so fluid leaks out

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

In women with portal hypertension, oligomenorrhea, amenorrhea and what may result due to hypogonadism?

A

Sterility

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

What syndrome occurs due to chronic liver failure and is characterized by hypoxia and dyspnea due to ventilation/perfusion mismatch from rapid blood flow through dilated vessels w decreased time for diffusion- exacerbated in upright position *improves when laying down?

A

Hepatopulmonary Syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

What hepatitis virus is benign and self limited, does not cause chronic hepatitis or a carrier state and rarely lethal, ssRNA, spread fecal orally via contaminated water *developing countries?

A

HAV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Hepatitis A Virus has anti-HAV IGM =acute infection- onset of symptoms, IgG appears and IgM declines, but there is no direct serology to check for IgG, so need to take total antiHAV minus antiHAVIgM, IgG persists for years = immunity, it uncommonly (0.1%) causes what?

A

Acute hepatic failure with acetopminophen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

HepB HBV, has 5 different types, acute hepatitis with full recovery, non-progressive chronic hepatitis, progressive w cirrhosis, acute hepatic failure, asymptomatic healthy carrier state… Which type is an important precurosr for hepatocellular carcinoma?

A

Chronic hepatitis B

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Transmission of HepB is highly prevalent in africa and asia via parenteral (childbirth), intermediate prevelance = horizontal transmission in childhood via breaks in skin/mucus membranes w body contact, and low prevelance in what two situations (MC in US)?

A

SEX

IV DRUGS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

What serum marker for HBV appears before the symptoms, peaks during the overt disease and lasts for 12 weeks, donated blood is screen for this?

A

HBsAg

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

What serum marker for HBV doesnt rise until the disease is over, at the same time that the HBsAg goes away, provides immunity, may persist for life, conferring protection = basis for current vaccinations?

A

Anti-HBsAb (HBsAb IgG)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

HBeAg, HBV DNA, and DNA polymerase all appear after HBsAg and indicate that there is ACTIVE viral replication, what specifically can be used to track the disease and antibodies to it indicated disease is about to wane?

A

HBeAg

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Persistent HBeAg is an indicator of continued viral replication, infectivity and probably progression to chronic hepatitis. HBeAb = infection almost over. What appears before the onset of symptoms and shows up w increase AST/ALT**?

A

Anti- HBcAb

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

What is the best predictor of chronicity with HBV?

A

AGE … younger you are the more likely you will have chronic HBV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

The host immune response to the virus determines the outcome of infection; strong response by CD4/8 = acute resolute of infection, HBV does not DIreCTLY cause hepatocyte damage, what does?

A

CD8 T cells attacking the hepatocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

The goal of chronic HBV infection is to slow progression of disease, reduce liver damage and prevent cirrhosis and cancer, there are vaccinations for the dsDNA virus, and in chronic HBV a liver biopsy shows finely granular what?

A

Ground glass hepatocytes PACKED with HBsAg

**Cells with ER swollen by HBsAg = diagnostic hallmark

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

HCV is a ssRNA virus with no vaccination, HCV IgG does not confer immunity and reinfection is possible. It is clinically milder than HBV but 80-90%** develop chronic infection and 20% get?

A

cirrhosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

HCV clinically presents as repeated bouts of hepatic damage, hallmarks being persistent infection and chronic hepatitis, with chronic HCV you seen a persistent elevation in aminotransferases (wax/wane but never normal), what is found in 35% with chronic HCV?

A

Cryoglobulinemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Diagnosis of HCV is detected in blood for 13 weeks during active infection with increase in AST/ALT, associated with metabolic syndrome and can give rise to insulin resistance and nonalcoholic?

A

fatty liver disease NAFLD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

the major risk factors fo HCV include: IV drug abuse, multiple sex partners/ MSM, surgery in last 6 months, contact with HCV person, employment in medical field and most importantly?

A

NEEDLE STICK in 10% (risk 10x greater than HIV needle sticks)

-tattoos, drugs, etc

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

VIRUS FAMILY
HAV: Hepatovirus/Picornavirus
HBV: Hepadnavirus
HCV: Flaviviridae

A

MEOW

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

DIAGNOSIS
HAV: Detect serum IgM abs
HBV: detect HBsAg or ab to HBcAg, PCR for HBV DNA
HCV: 3rd gen ELISA for ab detection, PCR for HCV DNA

A

MEOW

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

What hepatitis is dependent on HBV for its life cycle, vax to HBV prevents it, co-infection with HBV, higher rate of acute hepatic failure in IV drug uses, SUPERINFECTION, usually seen in IV drug users or blood transfusions?

A

Hepatitis D Virus HDV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

What hepatitis has a characteristic of having higher mortality rate among pregnant women, at almost 20%?

A

Hepatitis E

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

What are the following?

1) acute asymptomatic infection w recovery
2) acute symptomatic hepatitis w recovery (anicteric or icteric)
3) chronic hepatitis w or w/o progression to cirrhosis
4) acute liver failure w massive to submassive hepatic necrosis

A

Clinicopathologic syndromes* of viral hepatitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

What is the following syndrome of hepatitis? Worldwide, HAV and HBV infections are frequently subclinical events in childhood verified only in adulthood by the presence of anti-HAV/HBV abs?

A

Acute asymptomatic infection with recovery (serologic evidence only)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

What is the following syndrome of hepatitis?
four phases: incubation period w PEAK infectivity occuring during the last asymptomatic days of the period, symptomatic preicteric phase, symptomatic icteric phase and convalescence

A

Acute symptomatic hepatitis with recovery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

What is the following syndrome of hepatitis?

with progression to cirrhosis or without progression to cirrhosis

A

Chornic hepatitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

What is the following syndrome of hepatitis?

with massive hepatic necrosis, with submassive hepatic necrosis

A

Acute liver failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

What is the following syndrome of hepatitis?
individual with HBsAg, no HBeAg or anti-HBeAg, normal ALT/AST, low serum HBV DNA, liver biopsy showing lack of significant inflammation and necrosis-not in US

A

Carrier state- health carrier-inactive carrier

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

Coinfection with HIV and hepatitis has become common. Chronic HBV and HCV are leading causes of mortality in patients with HIV in patietns who are untreated and progress to ?

A

AIDS - liver disease is the 2nd MCC of death

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

Acute hepatitis morphology includes lymphoplasmacytic (mononuclear) infiltrate (lymphocytes and plasma cells), spotty necrosis or lobular hepatitis is present throughout the lobule, necrosis may be present as empty cytoplasm, cell membrane rupture leading to?

A

hepatocyte dropout/death

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

Acute hepatitis morphology includes collapsed sinusoidal collagen reticulin framework*, lack of portal inflammation and apoptosis may be the other mechanism of death, which can be seen by shrinking of the hepatocyte, esosinophilia, pyknotic and?

A

fragmented

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

In severe acute hepatitis, there is *confluent necrosis of hepatocytes around central veins, cellular debris, collapsed reticulin fibers, congestion +/- hemorrhage w some inflammation, there is central portal what, which leads to parenchymal collapse?

A

Bridging necrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

In severe acute hepatitis, it can lead to massive hepatic necrosis or acute failure, can develop post hepatitis cirrhosis with abundant?

A

SCARRING

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

Chronic hepatitis morphology includes may be mild to severe and varies. MIld will see infiltrates limited to portal tracts, progressive will see extension of chronic inflammation from portal tracts with interface hepatitis, there may also be linking of portal portal central regions known as?

A

bridging necrosis

fibrous septum formation via loss of hepatocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

What is characterized by necrosis of the liver associated with a lymphocytic infiltrated into the adjacent parenchyma (BEYOND the limiting plate) with destruction of individual hepatocytes along the edges of the portal tract?

A

interface hepatitis (seen in viral chronic, autoimmune and steatohepatitis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

In chronic HepB, what is characterized by cells with endoplasmic reticulu, swollen by HBsAg = diagnostic, seen as large pale finely granular pink cytoplasmic inclusions on H&E staining?

A

Ground Glass Hepatocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

Chronic hepatitis C shows lymphoid aggregates or fully formed lymphoid follicles, geneotype 3 shows what kind of change of scattered hepatocytes?

A

FAtty change (causing secondary metabolic syndrome leading to NAFLD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

ACUTE: SCANT mononuclear infiltrate, ballooning degeneration, apoptosis, cholestasis
CHRONIC: DENSE mononuclear infiltrates, bridging necrosis/fibrosis, interface hepatitis, ductular reaction(late stage disease), fatty change (hep C), ground glass cells (hep B),

A

MEOW-review

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

What type of hepatitis is chronic and progressive, with presence of abs, therapeutic response to immunosuppression, trigger is usually viral infection, drug or toxin exposure, white FEMALE predominance, associated with HLA-DRB1**?

A

Autoimmune Hepatitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

What type of autoimmune hepatitis is common in middle aged to older people, with +ANA and ASMA (anti smooth muscle) antibodies?

A

Type 1 Autoimmune hepatitis

Type 2 MC in children,teens with anti-LKM1 = antiliver kidney microsome/ACL1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

With Autoimmune Hepatitis there is an early phase of severe parenchymal destruction followed by rapid scarring, fibrosis takes years to develop, severe necroinflammatory activity, mononuclear infiltration - plasma cells, and can see what in areas of activity?

A

Hepatocyte Rosettes*

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

with Autoimmune Hepatitis there is a progressive or indolent stage with initial signs such as severe hepatocyte injur w necrosis but little scarring and chronically will see burned out cirrhosis with little necroinflammatory activity

A

??? MEOW

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
84
Q

Clinically, Autoimmune Hepatitis has an acute onset with fulminant disease in 8 weeks, encephalopathy, if left untreated, 40% will die in 6 months, 40% survivors have cirrhosis… What are the prominent and characteristic component of the inflammatory infiltrate in biopsy?

A

**Plasma cells = IgG is elevated!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
85
Q

Prognosis for Autoimmune Hepatitis is better in adults than children, 80% respond to immunosuppression for long term survival, there is a 75% survival after 10 years post?

A

liver transplant (20% will recur)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
86
Q

Exposure to what should always be including in the differential diagnosis of any form of liver disease?

A

Toxin (herbal, dietary, tropical, environmental) or therapeutic agent

87
Q

Drug or toxin damage may be immediate or delayed, mild or severe, predictable (dose dependent) or unpredictable (multifactorial), due to direct toxicity, immune mediated toxicity or due to conversion of?

A

XENOBIOTIC to a toxin

88
Q

Acetopminophen is the MCC of acute liver failure necessitating liver transplant in the USA, due to a toxic metabolite produced from the CYP450 breakdown in acinus ZONE 3 hepatocytes. As zone 3 dies, zone 2 takes over metabolic function and becomes injured, in severe overdoses, what occurs?

A

zone of injury extends to zone 1 (periportal hepatocytes) and you get acute hepatic failure

(CYP450 upregulated w alch/codeine = worse damage)

89
Q

Morphologically with acetominophen overdose, one can see ?

A

MASSIVE necrosis

90
Q

Morphologically with anabolic steroid/ oral contraceptives there can be cholestatic injury with bland heptaocellular cholestasis without inflammation. There can also be vascular lesions/injury such as Peliosis hepatitis( blood filled cavities without endothelial lining- more common to anabolic roids) and what - more common to OCP?

A

Budd Chiari syndrome

91
Q

Lastly, Morphologically with anabolic steroid/ oral contraceptives they predispose you to neoplasms, such as ?

A

hepatocellular ADENOMA

angiosarcoma and vinyl chloride

92
Q

Liver failure = acetominophen

Liver disease= alcohol

A

MEOW

93
Q

What drug commonly causes microvesicular steatosis (diffuse small droplet fat)?

A

Aspirin

94
Q

Excessive alcohol consumption is the leading cause of liver disease in the western countries, only 10-15% of alcoholics develop cirrhosis. There are three types, steatosis (fatty change), alcoholic hepatitis and steatofibrosis which includes?

A

Cirrhosis (small fraction)

95
Q

All alcholic changes begin in zone 3. Hepatic steatosis is seen as microvesicular lipid droplets within hepatocytes, chronically accumulates in macrovesicular droplets -displacing the nucleus, there is perivenular fibrosis, liver is large soft yellow and is completely reversible, how large?

A

Hepatomegaly

-reversible if stops drinking

96
Q

Alcoholic (steato-) hepatitis is characterized by 3 things. 1 is hepatocyte swelling and necrosis (ballooning) d/t accum of fat and water, another is what which are present as clumped amorphous eosinophilic material in ballooned hepatocytes?

A

Mallory Denk Bodies

(intracellular eosinophlic aggregates of intermediate filaments (keratin 8/18), seen also in NAFLD,Wilson, Chornic biliary tree dz)

97
Q

A third characteristic finding in alcoholic steato hepatitis is what, in which neutrophils permeate the hepatic lobule and accumulate around degenerating hepatocytes (particullarly ones with mallory denk bodies)?

A

Neutrophilic Reaction

98
Q

Alcoholic steatofibrosis is accompanied by acitvation of sinusoidal stellate cells and portal fibroblasts giving rise to fibrosis, sclerosis of central veins occurs first, perisinusoidal scars then accumulate in space of Disse spreading outward encircling individual or small clusters of hepatocytes in what type of pattern?

A

Chicken wire fence pattern (scarring)

99
Q

Alcoholic steatofibrosis is associated with calssic micronodular cirrhosis first described for end stage alcoholic liver disease knwon as laennec cirrhosis - with continuous use- due to developing nodularity and progressive interwebbing of the scars… so cirrhosis = chronic liver disease while laennec=micronodular=?

A

chicken wire= alcoholic steatofibrosis

100
Q

Risk factors for developing alcoholic cirrhosis - occurs in 15% of alcoholics, *females are more susceptible, *AA > caucasian and have HBV, HCV or what increases the severity?

A

HEMOCHROMATOSIS

101
Q

There are sometimes mutations associated with alcohol intolerance which leads to a higher chance of liver disease, such as homozygous ALDH2 in asians = alch intolerance .. sx include nausea lethargy and?

A

Flushing

102
Q

What is the following describing?
Diffuse nodularity of the surface induced by underlying fibrous scarring, nodule size is usually 3mm, micronodular*, green tint due to cholestasis, on microscope: small nodules entrapped in blue staining fibrous tissue - fat accum no longer seen in this ‘burned out’ stage

A

Alcoholic Cirrhosis

103
Q

Hepatocellular steatosis is due to impaired lipoprotein assembly and secretion, increased release of FFA into the circulation, shunting towards lipid biosynthesis due to increased NADH production… alcohol dehydrogenase and acetaldehyde dehydrogenase increases what production?

A

NADH

104
Q

In alcoholic hepatitis, acetaldehyde induced lipid peroxidation and protein adduct formation (carcinogen) occurs, induced CYP450 may form toxic metabolites, impaired methionin metabolism decreases glutathione levels, alcohol causes release of LPS which is?

A

Proinflammatory

105
Q

Alcoholic liver disease (steatohepatitis) is due to chronic disorder of steatosis, hepatitis, progressive fibrosis and deranged perfusion, due to an agent that was initiaaly only ?

A

marginally harmful

106
Q

Clincally with hepatic steatosis, one can see hepatomegaly*, with mildly elevated bilirubin and ALP levels, severe dysfunction is?

A

rare

107
Q

Clinically with steatohepatitis, there is tender hepatomegaly, +/-cholestasis, hyperbilirubinemia, elevated alk phos, and non specific symptoms, along with what ALT/AST?

A

AST to ALT ratio of 2:1*** (=chornic liver injury)

108
Q

Clinically with steatofibrosis/cirrhosis, labs reveal hepatic dysfunction if enough liver tissue left, hypoproteinemia, anemia, irreversible and what blood abnl?

A

coagualtion abnormalities

109
Q

Outlook for alcoholics with liver disease is variable. 5yr survival is 90% in abstainers who are free of jaundice, ascites or hematemsis, it drops to 50% int hose who?

A

continue to drink

110
Q

In end stage alcoholic liver disease, the MCC of death are hepatic coma, massive GIB, intercurrent infection, hepatorenal syndrome, and what, which develops in 1-6%?

A

Hepatocellular Caricnoma

111
Q

A distinct group of liver diseases is attributatble to disorders of metabolism, acquired or inherited. The MC acquired metabolic DO is nonalcholic fatty liver disease, inherited inlcude hemochromatosis, wilson disease and?

A

alpha1 antitrypsin deficiency

112
Q

Metabolic syndrome is classified as one of the following: DM, impaired glucose tol, impaired fasting glucose, or insulin resistance AND two of the following: high BP, dyslipidemia, central obesity, and microalbuminuria

A

MEOW WHO CRITERIA FOR METABOLIC SYNDROME

113
Q

What is the MCC of chronic liver disease in the USA in patients who consume less than 20g of alcohol in a week (80g/day is threshold for alcoholic), risk factors= obesity/ metabolic syndrome, HISPANICS and AFRICAN AMERICANS MORE THAN CAUCASIANS?

A

Non-alcohol fatty liver disease

114
Q

Path of NAFLD is a two hit model… insulin resistance leads to hepatic steatosis, hepatocellular oxidative injury leads to liver cell necrosis and inflammatory reactions. The former is caused by high calorie food intake, metabolic syndrome (apoptosis), apoptosis also occurs due to oxidative damage to mT and ?

A

plasma membranes

115
Q

Stellate cells also activated through hedgehod signaling pathway– level of hedgehog pathway acitivity correlates with stage of what in NAFLD?

A

Stage of fibrosis = amount SHH activity

116
Q

Morphologically in NAFLD, steatosis is involvement of >5% hepatocytes. NASH overlaps with histology of alcholic steatohepatitis BUT mononuclear cells more prominent, and what is LESS common?

A

Mallory-denk bodies

portal fibrosis is more prominent in NAFLD than steatofibrosis, otherwise looks the same

117
Q

Greater than 90% of cryptogenic cirrhosis is now considered burned out from ?

A

NAFLD

118
Q

IN pediatric NAFLD there is diffuse steatosis and portal fibrosis (rather than central) with portal and parenchymal mononuclear infiltrate rather than parenchymal?

A

neutrophils (as seen in adults)

119
Q

NAFLD clinically is asymptomatic or has nonspecific RUQ pain and hepatomegaly, increased AST/ALT, *dx confirmed by *biopsy, cardiovascular death is the most common(NASH). NASH also increases the chance of cirrhosis, decompensation and?

A

7% over 6.5 years progress to hepatocellular carcinoma

tx: correct obesity, hyperlipidemia, insulin resistance/ metabolic syndrome

120
Q

What is excessive iron absorption depositied in parenchymal organs such as the liver, heart and pancreas, either herditary or d/t excessive intake, hereditary mc in males in 40-50s, iron accum is lifelong, injury caused by excess iron- slow and progressive?

A

Hemochromatosis AR

121
Q

Hemochromatosis (severe) pts exhibit micronodular cirrhosis, DM, and abnormal skin pigmentation, may occur in women (less likely) later due to menstrual bleeding counterbalancing the accumulation, what is the ratio of men to women?

A

5-7:1

122
Q

hereditary Hemochromatosis is MC caused by a mutation in the HFE gene whose product is involved in intestinal iron uptake by its effects on hepcidin levels. Hepcidin is the main regulator of iron absorption (HAMP gene). MC HFE mutation is a POINT mutation at?

A

C282Y = cysteine to tyrosine substituation

123
Q

in Hemochromatosis there is abnormal intestinal uptake of iron, uptaking way more than needed. the disease manifest after how many grams of iron has accumulated?

A

20G (abnormal uptake of 0.5-1G per year = 20-40 year at onset)

124
Q

Hepcidin in Hemochromatosis is the main regulator of iron absorption, it lowers plasma iron by binding ferroportin and degrades the channel, decreasing the amount of dietary iron absorbed… MC caused by mutation at HFE, but also TFR2, TFR1, and HJV which is?

A

hemojuvelin expressed in liver heart and skeletal muscle causes severe juvenile hemochromatosis

125
Q

HFE MCC of adult Hemochromatosis, C282Y substituation on chr6, inactivates HFE = inactivates hepcidin = no degredation of ferroportin =?

A

Excess iron absorption

126
Q

Hemochromatosis morphologically can see hemosiderin deposition, golden-yellow granules leads to small shrunken liver with micronodular cirrhosis, no inflammation, in pancreas, heart skin one can see?

A

Stricking gray-slate-brown color from iron/hemosiderin

127
Q 
Hemosiderin deposition can cause acute synovitis, pseudo gout may form from calcium pyrophosphate deposition. The clinical tetrad of Hemochromatosis includes:
1) cirrhosis w hepatomegaly (liver)
2) abnormal skin pigmentation 
3) DM (pancreas)
4?
A

Cardiac Dysfunction (Dilated cardiomyopathy)

128
Q

in some patients with Hemochromatosis, the presenting complaint it hypogonadism (ammenorrhea in females) - impotence/ loss of libido in males, MC in males, there is a 200X increased risk of?

A

Hepatocellular carcinoma

129
Q

Hemochromatosis can be diagnosed long before irreversible tissue damage occurs, via very high serum elvels of iron and ferratin, liver bx is done to rule out secondary causes, genetic screen is important, especially in?

A

FAMILY members of probands

  • prussian blue stain iron
    tx: regular phlebotomy
130
Q

What is a severe liver disease and exxtrahepatic hemosiderin deposition of unknown origin, NOT inherited, liver injury occurs in utero, may be due to maternal alloimmune injury to the fetal liver, extrahepatic hemosiderin deposition (buccal) has to be documented for proper dx, no tx, supportive care and possible liver transplant?

A

Neonatal (congenital) hemochromatosis

131
Q

What is an autosomal recessive disorder due to mutation of ATP7B, impaired copper excretion into bile and failure to incorporate copper into ceruloplasmin, leading to toxic levels of copper in the liver kidney and eye ?

A

Wilson Disease

132
Q

Wilson Disease is marked by accumulation of toxic copper in mayn tissues and organs, mainly the liver, brain and eye, copper is also toxic to RBC which causes ?

A

hemolytic anemia

133
Q

Wilson Disease causes copper absoprtion and dilvery to the liver but its excretion into bile is reduce and no incorporated into ceruloplasmin (not into blood), so there is accumulation in the liver causing injury via ROS via fenton reaction, binds to sulfhydryl groups of cellular proteins and displaces other metals from hepatic?

A

metalloezymes

134
Q

Morphilogically, Wilson Disease has minor to severe liver damage with fatty change, acute and chronic hepatitis with mallory denk bodies, eventually leading to cirrhosis, there is CNS toxicity in what area? causing atrophy and cavitation?

A

Basal ganglia- Parkinson like

135
Q

Wilson Disease can us rhodamine or orcein stains to look for the copper, what rings are green brown copper deposits in descemets membrane in the eye?

A

Kayser-Fleischer Rings

136
Q

clinically, Wilson Disease has an elevated urinary excretion of copper and low plasma ceruloplasmin, presents with parkinson like spastic dystonia, mask like facies, rigidity, and gait disturbances, psychiatric/behavioral sx, and hemolytic anemia due to copper toxicity in RBC leading to an elevated?

A

direct bilirubin

137
Q

Wilson Disease is diagnosed via decreased serum ceruloplasmin, increased hepatic copper content is most sensitive and accurate and increased urinary Cu (screening), tx with?

A

chelating agent like penicillamine

138
Q

What is an AR disorder of protein folding, PiMM is the wild type, PiZZ is the MC clinically relevant b/c have 10% of normal a1-antitrypsin level- presents with early liver disease, it is the most commonly diagnosed inherited hepatic disorder of infants and children?

A

A1-antitrypsin deficiency

139
Q

A1-antitrypsin inhibits proteases such as neutrophil elastase in the lungs, which when there is a deficiency, it causes pulmonary panacinar emphysema because the proteases are not inhibited, it also leads to hepatic disease because?

A

there is accumulation of the misfolded protein = ER stress = ER stress response = Apoptosis

140
Q

A1-antitrypsin def is associated with a point mutation at Glu342 to Lys342, note that not all PiZZ phenotypes develop liver dz (only 10-25%). Morphologically is it characterized by round to oval cytoplasmic globular inclusions in periportal hepatocytes and is what positive?

A

PAS positive after diastase digestion of liver - highlight characteristic MAGENTA cytoplasmic granules

141
Q

A1-antitrypsin def in neonates presents with hepatitis and cholestatic jaundice (MC dx hepatic DO in infants/children). Adolescents present with hepatitis, cirrhosis and lung disease. Middle to late life may present with?

A

Cirrhosis +/- Hepatocellular carcinoma

142
Q

The functions of bile is to emulsify dietary fat in the lumen of the gut and to eliminate bilirubin, cholesterol and xenobiotics and waste products that are insufficiently water soluble to be excreted in the urine. What form of bilirubin is usually attached to albumin?

A

Unconjugated bilirubin + albumin = blood

143
Q

the ability to conjugate and excrete bilirubin doesnt not occur until 2 weeks, it is normal and expected for newborn to go through stages of neonatal jaundice, which can also be exacerbated by breast feeding due to?

A

B-glucuronidases in milk (bilirubin deconjugating enzyme)

144
Q

What is an AR complete enzyme deficiency with no UGT1A1 activity, fatal in the neonatal period and causes unconjugated hyperbilirubinemia?

A

Crigler-Najjar Syndrome Type 1

145
Q

What is an AD***** with variable penetrance syndrome with decreased UGT1A1 activity, mild clinical course with only occasional **kernicterus (sever neurological damage), and causes unconjugated hyperbilirubinemia?

A

Crigler-Najjar Syndrome Type 2

146
Q

What is an AR syndrome with decreased UGT1A1 activity, it is completely benign and causes unconjugated hyperbilirubinemia?

A

Gilbert Syndrome (Jaundice is only sx) -b9

147
Q

What is an AR syndrome due to mutated canalicular MDR protein (MRP2), inhibiting excretion of bilirubin causing pigmented cytoplasmic globules, a black liver and conjugated hyperbilirubinemia?

A

Dubin-Johnson Syndrome -b9

148
Q

What is an AR syndrome whose mechanism might be due to decreased hepatic uptake/storage or decreased biliary excretion, leading to conjugated hyperbilirubinemia?

A

Rotor Syndrome - b9

149
Q

What is cause by impaired bile formation and bile flow that gives rises to accumulation of bile pgiment in the hepatic parenchyma, can be caused by extra or intrahepatic obstruction of the bile channels or by defects in hepatocyte bile secretion?

A

Cholestasis

150
Q

Cholestasis presents with jaundice, pruritus, skin xanthomas, malabsoprtion and VIT ADEK deficiencies. Which lab test is characteristic and should be elevated to confirm cholestasis is occuring? 2

A

elevated GGT and ALP*

*ALT/AST normal

151
Q

Cholestasis morphologically is associated with accumulation of bile pigment in the hepatic parenchyma, elongated green brown plugs of bile in dilated bile canaliculi, droplets of bile pigment w a fine foamy appearance- which are called what?

A

Feathery degeneration of periportal hepatocytes (zone1?)

152
Q

Large bile duct obstructions (LDO) in adults is commonly due to extrahepatic cholelithiasis or malignancies of the biliary tree, in kids the most common is biliary atresia, CF. Overall, the MCC is?

A

Gall stones and malignancies

153
Q

What is a secondary bacterial infection of the biliary tree that aggravates the inflammatory injury caused by obstruction, due to enteric bugs like Coliforms* or Enterococci*, and lead to charcots triad which is RUQ pain, jaundice, and fever?

A

Ascending cholangitis

*Hallmark:influx of periductular neutrophils directly into the bile duct epithelial cells and lumen

154
Q

What is the MC form of cholestatis of sepsis, characterized by bile plugs within centrilobular canaliculi, associated w activation of Kuppfer cells and portal inflammation, hepatocyte necrosis is absent?

A

Canalicular Cholestasis

155
Q

What is a disorder of intrahepatic gallstone formation that leads to repeated bouts of ascending cholangitis, progressive inflammatory destruction of hepatic parenchyma and predisposes to biliary neoplasia?

A

Primary Hepatolithiasis

156
Q

Primary Hepatolithiasis is most prevalent in east asia, morphologically has pigmented calcium bilirubinate stones in distended intrahepatic bile ducts, ducts show chronic inflammation, mural fibrosis, and peribiliary gland hyperplasia, biliary dysplasia is seen giving them an increased risk of what cancer?

A

Cholangiocarcinoma*

157
Q

Neonatal cholestasis/hepatitis is prolonged conjugated hyperbilirubinemia in the neonate after two weeks, (not physiologic jaundice), due to liver injury/inflammation, leading to jaundice, dark urin, light acholic stools and hepatomegaly, what is seen on histo?***

A

panlobular giant-cell transformation of hepatocytes*

158
Q

What is complete or partial obstruction of the lumen of the extrahepatic biliary tree withint the first 3 months of life, MCC of neonatal cholestasis and death from liver disease in early childhood?

A

Biliary Atresia

159
Q

There are two forms of biliary atresia, the first is associated with other anomalies resulting from ineffective establishment of laterality of thoracic and abdominal organs during development (look for other congen abnl), due to aberrant intrauterine development of the extrahepatic biliary tree?

A

Fetal form = 20%

160
Q

What form of biliary atresia is the MC, due to destruction of the normal biliary tree shortly after birth, roatvirus, CMV, reovirus and AI are all impicated- genetic basis?

A

Perinatla Form = 80%

161
Q

Morphologically, biliary atresia has inflammation and fibrosing stricture of the hepatic or common bile ducts with progressive destruction of intrahepatic biliary tree.. Clinically presents with neonatal cholestasis, normal weight, bilirubin high, jaundice, and normal stools become?

A

clay like or ACHOLIC stools

*direct bilirubin

162
Q

Tx for biliary atresia limited to common duct or R/L hepatic ducts do the kasai procedure (type 1& 2), obstruction above the porta hepatis (type III) is not correctable and requires?

A

Liver transplant!

163
Q

What is a non-suppurative, inflammatory destruction of small/medium sized intrahepatic bile ducts, most cases do not progress to cirrhosis, with florid duct lesions, dz most common in women 40-50?

A

Primary Biliary Cirrhosis (PBC)

164
Q

Primary Biliary Cirrhosis (PBC) has anti-mitochondrial antibodies, and on histo has florid duct lesions where interlobular ducts are actively destroyed by lymphoplasmacytic inflamm with/without granulomas, there is patchy loss of ducts, *feathery degeneration and ballooned bile stained hepatocytes with?

A

Mallory Denk Bodies

eventually widespread leading to cirrhosis and portal HTN w nodularity

165
Q

Primary Biliary Cirrhosis (PBC) clinically will see ONLY elevated serum ALP, and GGT in the asymptomatic patient, dx via biopsy, patients may have fatigue and pruritius, osetoporosis, hyperpigmentation, xanthelasmas, steatorrhea and other autoimmune disorders, treat with?

A

Ursodeoxycholic Acid

166
Q

What is the following describing? a portal tract markedly expanded by an infiltrate of lymphocytes and plasma cells surrounding a destructive granulomatous reaction centered on a bile duct

A

florid duct lesion in PBC

167
Q

what is inflammation and fibrosis of intrahepatic and extrahepatic bile ducts with dilation of preserved segments, causing beading seen on radiographs, MC affects males 20-40s, genetic influences, UC increases risk, and inc risk for cholangiocarcinoma?

A

Primary Sclerosing Cholangitis PSC

168
Q

Primary Sclerosing Cholangitis PSC commonly has pANCA (like UC), there is neutrophilic infiltration of the epithelium, inflammation and edema leading to obstruction and scarring that narrows large ducts, in small ducts there is little inflammation and a degerating bile duct is entrapped in a dense?

A

Onion skin concentric scar (tombstone scar)

169
Q

Clinically Primary Sclerosing Cholangitis PSC there is a persistent elevation of serum alk phos, progressive pruritus and jaundice, bouts of ascending cholangitis, dx via radiology of larger ducts showing strictures and beading******* with pruning of smaller ducts, tx?

A

no treatment - transplant

170
Q 
Disease:?
50 years
Female
Progressive
Assoc:Sjogrens, Scleroderma, Thyroid dz
95% AMA +, 50% ANA+
Normal Radiology
Bx shows florid duct lesion and loss of small ducts ONLY
A

Primary biliary cirrhosis

171
Q 
Disease: ?
30 years
Males
Progressive
Assoc: Ulceratice colitis, Pancreatitis, retroperitoneal fibrosis
65% +pANCA (0-5% AMA+)
Radiology shows beading/ beads on string
Duct: inflamm destruction of extra and intrahepatic LARGE ducts, fibrotic obliteration of smaller ducts (onion skinning)
A

Primary Sclerosing Cholangitis

172
Q

Choledochal cysts are congenital dilations of the common bile duct (kids before 10- jaundice/abd pain-MC in females), what disease of the liver is a heterogenous group of lesions in which the primary abnormalities are congenital malformations of the biliary tree?

A

Fibropolycystic Disease

173
Q

What type of fibropolycystic disease are small bile duct hamartomas that are normal if limited, but indicative of FPD if they are diffuse?

A

Von Meyenburg Complexes (always associated with portal tracts- dilated and irregulary shaped bile ducts)

174
Q

What type of fibropolycystic disease has simple multiple biliary cysts in isolation that lead to clinical symptoms, in syndrome form biliary cysts occur with congenital hepatic fibrosis?

A

Caroli disease

Caroli syndrome if simple multiple biliary cysts + congenital hepatic fibrosis

175
Q

Impaired BF into the liver occurs via what artery compromise, leading to rare liver infarcts d/t dual blood supply, localized pale or hemorrhagic infarct, transplanted livers more apt to become infarcts?

A

Hepatic Artery Compromise

176
Q

manifestations of Hepatic Artery Compromise cuasing impaired blood flow into the liver include esophageal varices, intestinal congestion and?

A

Splenomegaly

177
Q

Impaired blood flow through the liver is MCC by what, along with sinusoidal occlusions like sickle cell disease, DIC, eclampsia, and diffuse intrasinusoidal metastatic tumor?

A

CIRRHOSIS

178
Q

What is a cause of hepatic vein thrombosis which obstructs two or more major hepatic veins that leads to hepatomegaly, pain and ascites, associated with high mortality, liver is usually swollen red-purple with a tense capsule?

A

Budd-Chiari Syndrome

179
Q

Budd-Chiari Syndrome causes severe centrilobular congestion and necrosis, or fiborisis if slowly developing, affect veins can thrombi. What causes obliteration of the terminal hepatic venules by subendothelial swelling and collagen deposition, commonly caused by jamaican bush tea, cancer, or stem cell transplant?

A

Sinusoidal Obstruction Syndrome

180
Q

Sinusoidal Obstruction Syndrome and Budd-Chiari syndrome usually present with tender hepatomegaly, ascites, weight gain, jaundice, and elevated?

A

aminotransferases

181
Q

Impaired intrahepatic blood flow due to cirrhosis or sinusoid occlusion commonly presents with ascites, esophageal varices, hepatomegaly, and elevated?

A

amintransferases

182
Q

Liver infarcts are rare due to dual blood supply with an exception being hepatic artery thrombosis in liver transplant as major biles ducts only have ?

A

one blood supply

183
Q

Portal vein occlusion causes esophageal varices, mCC of intrahepatic blood flow obstruction is?

A

Cirrhosis

184
Q

MCC of SMALL portal vein branch obstruction is Schistosomiasis, obliterative portal venopathy is caused mainly by?

A

HIV

185
Q

Peliosis hepatis = sinusoidal dilation “blood lakes” (sex hormones/infections?), hepatic vein obstruction/thrombosis is MCC by?

A

Budd Chiari Syndrome

186
Q

Veno-occlusive disease )sinusoidal obstruction syndrome) is caused by jamaican bush tea, cancer, and 3 weeks after stem cell transplant, passive congestion of liver is caused by?

A

right sided heart disease

187
Q

GVHD - acute = 10-50 days and chronic is 100+ days… transplant rejection- acute (acellular), chronic is vascular (chronic seen with vanishing bile duct syndrome), cardiac sclerosis = chornic congestive heart failure, what is the presentation in the liver of hypoperfusion and retrograde congestion?

A

NUTMEG LIVER D/T R SIDE HEART FAILURE

188
Q

Abnormal liver function tests in 3-5% of pregnant women, viral hepatitis is the MCC of jaundice in pregnancy, HEV during pregnancy has a more severe course and 20% mortality,hepatic complications that develop that are DIRECTLY attributable to pregnancy is?

A

0.1% - not common usually something else

189
Q

Preeclampsia is maternal HTN, proteinuria, peripheral edema and coagulation problems which becomes eclampsia when hyperreflexia and convulsions occur. there may also be subclinical manifestion of preeclampsia known as HELLP syndrome which stands for?

A

Hemolysis
Elevated Liver enzymes
Low Platelets (thrombocytopenia)

*Modest to severe elevation of ALT/AST and mild elevation of bilirubin

190
Q

Acute fatty liver of pregnancy presents in latter half of pregnancy (3rd trimester), dysfunction varies, leading to failure, coma or death, elevated AST/ALT, sx include bleeding nausea, vomiting, jaundice, and coma, diagnosed via characteristic diffuse what on bx?

A

Diffuse microvesicular steatosis of hepatocytes

tx w death of bb :/

191
Q

What can occur during pregnancy with an onset of pruritus, followed by darkening of urine and light acholic stools with jaundice, bilirubin/ALP is slight elevated, bile salt levels are GREATLY increased, b9 to mom but distress/stillborn is increased?

A

Intrahepatic cholestasis of pregnancy

192
Q

What hepatic disease with pregnancy causes periportal sinusoids containing fibrin deposits associated with hemorrhage into the space of Disse?

A

Preeclampsia (leads to coagulative necrosis)

193
Q

Nodular hyperplasia of the liver is not a true neoplasm, develop in noncirrhotic liver from focal nodular hyperplasia of regenerative hyperplasia, due to focal or diffuse alterations in hepatic blood flow from obliteration of portal vein radicles and compensatory flow of arterial blood supply……

A

(HUH?)

194
Q

What hyperplasia is single benign, well demarcated but poorly encapsulated lesion with a central scar, in young to middle age adults, spontaneous mass in otherwise normal liver?

A

Focal Nodular Hyperplasia (FNH)

195
Q

What hyperplasia is when the entire liver is transformed into nodules with NO FIBROSIS or fibrous septa, can develop portal HTN, associated with HIV, rheumatologic dz, asymptomatic, histo shows plump hepatocytes surrounded by rims of atrophic hepatocytes?

A

Nodular Regenerative Hyperplasia (NRH)

196
Q

What is a benign neoplasm of blood vessels and is the MC b9 liver tumor, seen as red-blue nodules under the capsule, vascular channels in a bed of fibrous connective tissue, MC in females, may incidentally hemorrhage?

A

Cavernous Hemangiomas

197
Q

Hepatocellular adenomas are benign neoplasms developing from hepatocytes, rupture may lead to intraabdominal bleeding which is a surgical emergency, hepatocellular adenomas were unknown until the advent of what was used?

A

Oral contraceptives and anabolic steroids (Main cause)

198
Q

HNF1-a inactivated adenomas are associated with MODY-3, fatty with no atypia, look for NO staining with liver fatty acid binding protein (LFABP), what is the risk for malignant transformation?

A

NO RISK - completely B9

199
Q

B-Catenin activated adenomas are associated with OCP and anabolic steroids, high degree of dysplasia, look for glutamine synthetase diffusely and B-catenin in the nucleus, what is the risk for malignant transformation?

A

Very high risk**

200
Q

Inflammatory adenomas are associated with NAFLD, mutations in GP130, have areas of fibrotic stroma, mononuclear inflam, ductular reactions, dilated sinusoids and telangiectatic vessesl, over express CRP and serum amyloid, malignant transformation?

A

Small but definite risk of malignant transformation

201
Q

What is the MC malignant liver tumor of early childhood, associated with fetal cells or embryonal cells or primitive mesenchyme, frequently activates WNT pathway (APC mutation)- so pts with FAP develop these?

A

Hepatoblastomas-fatal or 80% 5 yr survival

associated with FAP and beckwith wiedemann syndromes

202
Q

What carcinoma is the MC primary malignancy of hepatocytes, risk factors include HBV in asian countries, HCV in USE, alcohol, aflatoxin, a1AT, hemochromatosis, NAFLD, Wilson disease, adenomas, genetic factors include B-catenin activation and p53 inactivation (aflatoxin) are the two MC?

A

Hepatocellular Carcinoma (HCC)

203
Q

Hepatocellular Carcinoma (HCC) has precursor lesions of high-grade dysplastic nodules- sign of atypia with high risk of becoming HCC, which is the most important pathways for emergence of HCC in what two diseases?

A

Viral hepatitis and alcoholic liver disease (high grade dysplasia)

204
Q

What is a rare, distinctive variant of HCC mostly occuring in people <35 years old, presents as a single large hard scirrhous tumor with fibrous bands coursing through it, composed of well differentiated cells rich in MITOCHONDRIA (oncocytes) growing in nests of cords separated by parallel lamellae of dense collagen bundles?

A

Fibrolamellar Variant (NOT associated with liver disease)

205
Q

What malignant tumor of the liver has associations with poly vinyl chloride, arsenic and thorotrast?

A

Angiosarcoma

206
Q

What malignant tumor of the liver are primarily diseases of middle aged men and are seen in associated with HBV,HCV, HIV and PBC, most are diffuse large B-cells and then MALTomas?

A

Lymphomas

207
Q

Involvement of the liver in metastatic malignancy is far more common than primary hepatic neoplasia, any cancer site may metastasize here but the MC are colon breast lung and ?

A

Pancreas

208
Q

HCV is more common related to HCC in the US, while HBV is more common related to HCC in asia and africa

A

MEOW

209
Q

What is the second MC primary malignant tumor of the liver, with risk factors including chronic inflammation and cholestasis and in the US: PSC, gallstones, HBV, HCV, NAFLD, in Asia: PSC or Liver flukes (MC:Clonorchis Sinensis)?

A

Cholangiocarcinoma

210
Q

Cholangiocarcinoma is most commonly extrahepatic perihilar tumors (Klatskin tumors) located at the junction of the hepatic ducts = Hepatic HILUM- very hard to repair, it is a cancer of the ?

A

biliary tree coming from bile ducts within and outside of the liver- adenocarcinoma

211
Q

Cholangiocarcinoma has precursor lesions called biliary intraepithelial neoplasia 1-3, 3 being the highest grade and risk of malignant transformation, MC extrahepatic at hilum look like grey nodules in the bild duct wall that can be diffuse to papillary, 2yr survival is?

A

15% - Not good

212
Q

Clinically an intrahepatic Cholangiocarcinoma presents with obstruction to bile flow or symptomatic liver mass, extrahepatic presents as biliary obstruction, cholagitis and ?

A

RUQ pain

213
Q

Hydatid cysts are usually caused by what infection, which often have calcifications in the cyst walls which may aid radiologic diagnosis - undeeveloped countries? (immigrant)

A

Echinococcus

  • liver fluke fasciola hepatic, opisthorchis, clonorchis sinensis inc risk of cholangiocarcinoma
  • Anchovie paste material in cyst in liver = entomeba histolytica

25. GI 2 Exam 2 (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions