Liver II-Usera Flashcards by Danielle Hayes

What is this:
idiopathic, chronic progressive hepatitis.
What gender is it more common in?
What is the defect?
What can trigger it?

Autoimmune hepatitis
females
defective T-Cell regulation
Infection, acute illness, drugs

How well did you know this?

Not at all

Perfectly

Autoimmune hepatitis is associated with other (blank) disease

autoimmune

How well did you know this?

Not at all

Perfectly

In autoimmune hepatitis, what will the cell infiltrate look like?
What will the labs look like?

portal plasma

Elevated serum IGG and T-globulin levels

How well did you know this?

Not at all

Perfectly

What are the drugs that can cause autoimmune hepatitis?

Minocycline
Statins
Methyldopa
Interferons
Nitrofurantoin
Herbal products

How well did you know this?

Not at all

Perfectly

What antibodies will be present in autoimmune hepatitis?

anti-nuclear antibodies (ANA)
anti-smooth muscle antibodies (SMA)
anti-actin antibodies (AAA)
anti-soluble liver antigen/liver pancreas antigen antbodies (SLA/LP)

How well did you know this?

Not at all

Perfectly

What is type 1 autoimmune hepatitis associated with?

HLA-DR3 serotype

How well did you know this?

Not at all

Perfectly

What are the antibodies to type 2 autoimmune hepatitis?

anti-liver kidney microsome-1
antibodies (ALKM-1) (directed against CYP2D6)
anti-liver cytosol-1 antibodies (ACL-1)

How well did you know this?

Not at all

Perfectly

What is the most common cause of fulminant hepatitis?

(blank) percent of adverse drug reaxns result in toxicity

toxicity

10%

How well did you know this?

Not at all

Perfectly

Hepatic injury because of toxicity is due to one of three mechanisms, what are they?

direct injury
injury due to toxic metabolites
immunogenic

How well did you know this?

Not at all

Perfectly

Why is acetaminophen the worst?

it binds to liver and idney causing toxicity

How well did you know this?

Not at all

Perfectly

How long does it take for hepatic toxicity to occur?

weeks to motnhs

How well did you know this?

Not at all

Perfectly

What injury is associated with this:

contraceptives, anabolic steroids, estrogen replacement therapy

cholestatic

How well did you know this?

Not at all

Perfectly

What injury is this associated with:

antibiotics and phenothiazines

cholestatic hepatitis

How well did you know this?

Not at all

Perfectly

What injury is this associated with:

methyldopa, phenytoin, acetaminophen, halothane, isoniazid, phenytoin.

Hepatocellular necrosis

How well did you know this?

Not at all

Perfectly

What inury is this associated with:

ethanol, methotrexacte, corticosteroids, total parental nutrition

Steatosis

How well did you know this?

Not at all

Perfectly

What injury is associated with this:

amiodarone, ethanol

steatohepatitis

How well did you know this?

Not at all

Perfectly

What injury is associated with this:

methotrexate, isoniazid, enalapril

Fibrosis and cirrhosis

How well did you know this?

Not at all

Perfectly

What injury is associated with this:

sulfonamides

granulomas

How well did you know this?

Not at all

Perfectly

What injury is associated with this:
High-dose chemotherapy, bush teas
Oral contraceptives
Oral contraceptives
Anabolic steroids, tamoxifen

Veno-occlusive disease, obliteration of central veins
Budd-Chiari syndrome
Sinusoidal dilation
Peliosis hepatis

How well did you know this?

Not at all

Perfectly

What injury is associated with this:
Oral contraceptives, anabolic steroids
Thorotrast
Thorotrast
Thorotrast, vinyl chloride

Hepatic adenoma
HCC
Cholangiocarcinoma
Angiosarcoma

How well did you know this?

Not at all

Perfectly

What kind of necrosis occurs with acetaminophen toxicity?

perivenular necrosis

How well did you know this?

Not at all

Perfectly

What kind of fibrosis do you get with ethanol toxicity?

perivenular fibrosis with perisinusoidal extension

How well did you know this?

Not at all

Perfectly

What are the histological findings of drug and toxins on hepatocytes?

cholestasis
cholestatic hepatitis
ballooning degeneration
hepatocyte necrosis

How well did you know this?

Not at all

Perfectly

What is this:
Rare and potentially fatal syndrome of mitochondrial dysfunction in liver and brain

What is it characterized?

What is it associated with?

Reye syndrome

extensive microvesicular stetosis

Administration of aspirin (acetylsalicyclic acid) i.e you should avoid use of aspirin in children

How well did you know this?

Not at all

Perfectly

What are the clinical features of alcoholic liver disease?

appears acutely after heavy drinking episode - anorexia - weight loss - upper abdominal discomfort - tender hepatomegaly

(blank) is a type of liver disease, characterized by inflammation of the liver with concurrent fat accumulation in liver.

Steatohepatitis (also known as fatty liver disease)

What is the final stage of alcoholic liver disease? Is this reversible? What percent of patients develop cirrhosis?

alcoholic cirrhosis no 10-15%

What is hepatic steatosis? | Is it reversible?

microvesicular and macrovesicular steatosis | Fatty change is reversible with abstention from alcohol

What are the clinical features of hepatic steatosis?

- mild elevation of serum bilirubin and alkaline phosphatase | - severe hepatic dysfunction is unusual

What is indicative of alcoholic steatohepatitis?

``` hepatocyte swelling (ballooning degeneration) mallory bodies ```

What does hepatocyte swelling look like?

swollen cells, feathery cytoplasm, reactive, not pathological

(bank) fibrosis is specific for alcoholic fibrosis/ethanol toxicity, Where does the fibrosis start? What does the necrosis look like in fatty liver disease? What is it characterized by?

perisinusoidal, central vein spotty necrosis throughout the lobular pain, anorexia, lethargy

What kind of inflammation do you have in alcoholic hepatitis? What kind of inflammation do you have in viral and autoimmune hepatitis?

lobular inflammation | Periportal inflammation

What are the 2 types of metabolic liver disease?

Inherited or acquired disorders of metabolism

What is the acquired metabolic liver disease?

non-alcoholic fatty liver disease

What is the inherited metabolic liver disease?

- hemochromatosis - wilson disease (build up of copper) - A1-anti-trypsin deficiency

What is the most common cause of chronic liver disease in the US? What type of person does this typically present in?

Nonalcoholic fatty liver disease | 70% of obese people

What are the group of conditions associated with nonalcoholic fatty liver disease?

- hepatic steatosis - steatosis with minor inflammation - non-alcoholic steatohepatitis (NASH)

What are the clinical findings of nonalcoholic fatty liver disease?

``` Asymptomatic (steatosis) Elevated AST/ ALT Fatigue Malaise Right sided abdominal pain ```

In nonalcoholic fatty liver disease, you will get hepatic steatosis (with/without) inflammation and is a (unstable/stable) condition without significant clinical problems

with or without | stable

In non alcoholic fatty liver disease (NASH), there will be hepatocyte injury with progression to (Blank) in 10-20% of cases. It is strongly asociated with (blank) and (Blank).

cirrhosis | Obesity and metabolic syndrome

NASH (nonalcoholic fatty liver disease) is the most common cause of (Blank)

cryptogenic cirrhosis

What is this: | primary and secondary disorder of excessive body iron accumulation

Hemochromatosis

(blank) is a homozygous recessive inherited disorder of excessive body iron absorption

Primary hemochromatosis

Secondary iron accumulation (hemosiderosis) is due to (blank)

acquired causes

In hereditary hemochromatosis what are the mutations?

- HFE - Transferrin receptor 2 - Hepcidine genes - HJV gene

What causes hemosiderosis?

``` Parenteral iron overload Ineffective erythropoiesis with increased erythroid activity Increased oral iron intake Congenital atransferrinemia Chronic liver disease Neonatal hemochromatosis ```

In hemochromatosis, normal total body iron is (blank) GM. Regulation of intestinal absorption of dietary iron is (blank). Iron accumulates at a rate of (blank) /year The disease will manifest after (blank) GM have accumulated. Iron levels may reach (blank) with >30% accumulated in the liver

``` 2-6 GM abnormal 0.5-1.0 GM/year 20 GM 50 GM ```

Hemochromatosis presents early in life or late in life? If you suspect this, what are the first things you should look at? When should you send for genetics?

late transferrin and iron sat (first thing that goes up) -When iron sat gets greater than 50%

(blank) is directly toxic to tissues | Why?

Iron - lipid peroxidation through Fe-catalyzed free radical production - stimulation of collagen formation through activation of hepatic stellate cells - interaxn of ROS and Fe w/ DNA leading to lethal cell injury

The effects of Fe are (reversible/irreversible) in cells that are not fatally injured

reversible

What is the main regulator of Fe absorption by lowering plasma Fe levels? How does it work?

Hepcidin | an efflux channel ferroportin that prevents release of Fe from intestinal cells and macrophages

What does a deficiency in hepcidin result in?

iron overload

What does HJV do? | What can mutations in this cause?

regulates hepcidin levels | severe juvenile hemochromatosis

What does transferrin receptor 2 do? | What will mutations in this cause?

regulates hepcidin levels | classic adult hemochromatosis

What does HFE do? | What would a mutation in this cause?

Regulates hepcidin leels | calssic adult hemochromatosis

What regulates hepcidin? What will cause classic adut hemochromatosis if there is a mutation in it? What will cause severe juvenile hemochromatosis?

HFE Transferrin receptor 2 HJV Classic Adult: transferrin receptor 2 and JFE -HJV

What all does hemochromatosis affect?

- liver - pancreas - myocardium - pituitary - adrenal - thyroid - parathyroid - joints - skin

What gender is hemochromatosis more common in? What age do symptoms appear? What are physical signs you will see? What are other diseases that result form this?

men 50-60s hepatosplenomegaly skin pigmentation abdominal pain DM Arthritis hypogonadism cardiac dysfunction

Where is iron located in hemosiderosis?

in the macrophages (kupffer cells) and if prolonged, goes to hepatocytes

What is this: Disease of unknown origin manifested by severe liver disease and extrahepatic hemosiderin deposition Not an inherited disease What do people speculate this is due to?

Neonatal Hemochromatosis liver damage happens in utero due to maternal alloimmune injury to fetal liver

Wilison disease is a (blank) disorder caused by a mutation in the (Blank) gene.

AR | ATP7B

What is the pathology of Wilsons diseae?

- Impaired copper excretion into bile - failure to incorporate copper into ceruloplasmin - inhibition of ceruloplasmin secretion into blood

How will Wilson's disease clinically manifest?

Liver-steatosis, hepatitis, cirrhosis Brain-atrophy of the basal ganglia and putamen Eye- Kayser-Fleischer Rings

What is the most common way to get Wilson disease and what is the gene that is mutated?

Compound heterozygotes | ATP7B

What are the really sad consequences of Wilson disease?

Chronic liver disease | Neuropsychiatric manifestations

How do you diagnose Wilson disease? WHAT LEVELS SHOULD YOU NOT GET!!?!?!?

- Serum ceruloplasmin levels - Increase in hepatic copper content - Increased urinary excretion of copper -Do not use SERUM copper levels

Alpha 1 antitrypsin is an (blank) disorder that is characterized by decreased inhibition of (blank).

proteases (i.e increased proteases)

A1 antitrypsin deficiency manifests as (blank) and (blank)

pulmonary emphysema | Liver disease

``` Where is A1 antitrypsin synthesized? What kind of protease inhibitor is it? It is (blank)morphic with at least (bank) forms identified ```

liver Serine protease inhibitor poly 75

What are the common genotypes for a1 antitrypsin deficiency?

PiMM (90% of people) PiZZ (clinically significant mutation) PiMZ