Usera: Liver 2 Flashcards by Megan Foggia

Idiopathic, chronic progressive hepatitis
Female preponderance
Defective t-cell regulation
May be triggered by infection, acute illness, drugs
a/w other Autoimmune disease
Portal plasma cell infiltrate
Elevated serum IgG and γ-globulin levels

autoimmune hepatitis

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Which antibodies are associated with type 1 autoimmune hepatitis? What serotype is type 1 associated with?

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

associated with HLA-DR3 serotype

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Which antibodies are associated with type 2 autoimmune hepatitis?

Anti-liver kidney microsome-1 antibodies (ALKM-1)
Directed against CYP2D6
Anti-liver cytosol-1 antibodies (ACL-1)

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(blank) is the most common cause of fulminant hepatitis

toxicity

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What are three mechanisms of toxin damage in the liver?

direct injury
injury due to toxic metabolites (ex: reactive intermediate of acetaminophen)
immunogenic

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What can acetaminophen toxicity due to the liver?

causes perivenular necrosis

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What kind of drugs can cause cholestasis?

Contraceptives, anabolic steroids, estrogen replacement therapy

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What kinds of drugs can cause cholestatic hepatitis?

antibiotics, phenothiazines

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Rare and potentially fatal syndrome of mitochondrial dysfunction in liver and brain
Characterized by extensive microvesicular steatosis
Associated with administration of acetylsalicyclic acid (aspirin)
Avoid use of aspirin in children

Reye syndrome

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It is important to avoid the use of (blank) in children to avoid Reye’s syndrome (mitochondrial dysfunction in liver & brain)

aspirin

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Appears acutely after heavy drinking episode
Lab findings may range from minimal to fulminant hepatitis
Anorexia
Weight loss
Upper abdominal discomfort
Tender hepatomegaly

alcoholic steatohepatitis

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Final and irreversible form of alcoholic liver disease

alcoholic cirrhosis

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T/F: Only 10-15% of patients with alcoholic liver disease develop cirrhosis

True

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What causes fatty changes of microvesicular & macrovesicular steatosis?

alcohol!

**fatty change is reversible with abstention from alcohol

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What are the clinical features of fatty liver?

mild elevation of serum bilirubin

mild elevation of alkaline phosphatase

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What are some histologic features of alcoholic steatohepatitis?

hepatocytes swelling (ballooning degeneration)
mallory bodies
lymphocyte & neutrophilic inflammation
perisinusoidal fibrosis

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This is an acquired disorder of hepatic metabolism

non-alcoholic fatty liver disease

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These are inherited disorders of hepatic metabolism

hemochromatosis
Wilson disease
alpha-1 anti-trypsin deficiency

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Most common cause of chronic liver disease in the US

70% of obese individuals have some form

non-alcoholic fatty liver disease

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These are liver conditions seen in people who do not consume much alcohol

Hepatic steatosis
Steatosis with minor inflammation
Non-alcoholic steatohepatitis (NASH)

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T/F: Hepatic steatosis with or without inflammation is a stable condition without significant clinical problems

True

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Non-alcoholic fatty liver disease is strongly associated with (blank) and (blank)

obesity

metabolic syndrome

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Homozygous recessive inherited disorder of excessive body iron absorption

primary hemochromatosis

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Iron accumulation (hemosiderosis) is due to acquired causes

secondary hemochromatosis

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Hereditary hemochromatosis can be associated with mutations in the following...

HFE Transferrin receptor 2 Hepcidin genes HJV gene

What is the normal total body iron? How much iron must have accumulated before disease will manifest (hemochromatosis)?

total body iron: 2-6GM Disease will manifest after 20 gm stored have accumulated

How does iron damage liver tissues?

it is directly toxic to the tissues lipid peroxidation thru Fe-catalyzed free radical production Stimulation of collagen formation through activation of hepatic stellate cells interaction of ROS & Fe with DNA leads to lethal cell injury

Are the toxic effects of iron to liver cells reversible?

yes, if the cells are not fatally injured

Main regulator of Fe absorption by lowering plasma Fe levels Functions through an efflux channel ferroportin that prevents release of Fe from intestinal cells and macrophages A deficiency causes iron overload

Hepcidin

Iron overload can be due to deficiency in this iron regulator

hepcidin **hepcidin keeps iron inside of cells

Regulates hepcidin levels | Mutations cause severe juvenile hemochromatosis

HJV (hemojuvelin)

Regulates hepcidin levels | Mutations cause classic adult hemochromatosis

transferrin receptor 2 or HFE (hemochromatosis gene)

Who gets hemochromatosis? What are the symptoms?

``` male predominance hepatosplenomegaly abdominal pain skin pigmentation diabetes mellitus (due to pancreatic fibrosis) cardiac dysfunction arthritis hypogonadism ```

Disease of unknown origin manifested by severe liver disease and extrahepatic hemosiderin deposition Not an inherited disease Liver injury occurs in utero possibly due to maternal alloimmune injury to fetal liver

neonatal hemochromatosis

Autosomal recessive disorder caused by a mutation in the ATP7B gene There is impaired copper excretion into bile Failure to incorporate copper into ceruloplasmin

Wilson disease

Where does copper accumulate in Wilson disease? What are the manifestations?

liver --> steatosis, hepatitis, cirrhosis brain --> atrophy of basal ganglia & putamen eye --> Kayser-Fleischer rings

What does a mutation in ATP7B cause?

decreased copper transport into bile impaired copper incorporation into ceruloplasmin inhibition of ceruloplasmin secretion into blood

How can you diagnose Wilson disease?

Serum ceruloplasmin levels Increase in hepatic copper content Increased urinary excretion of copper **Do not use serum copper levels

What are the clinical features of Wilson disease?

chronic liver disease | neuropsych manifestations

Autosomal recessive disorder that is characterized by decreased inhibition of proteases Manifests as Pulmonary emphysema Liver disease

Alpha-1 antitrypsin deficiency

Synthesized in the liver A serine protease inhibitor Polymorphic with at least 75 forms identified

alpha-1 antitrypsin

What are the common genotypes of alpha-1 antitrypsin

PIMM (normal) PIZZ (clinically significant mutation) PIMZ (decreased alpha-1 antitrypsin)

In what disease state will you see PASD+ intracellular accumulations?

alpha-1 antitrypsin deficiency

Due to uncorrected obstruction of the extrahepatic biliary tree Extrahepatic cholelithiasis Malignancy of the biliary tree or head the pancreas Strictures from previous surgical procedures Biliary atresia Cystic fibrosis Choledochal cysts Paucity of bile duct syndromes

Secondary biliary cirrhosis

What happens in secondary biliary cirrhosis?

initially, there is cholestasis which is reversible with the correction of the obstruction then, secondary inflammation initiates periportal fibrosis --> leads to hepatic scarring & nodule formation

Inflammatory autoimmune disease affecting the intrahepatic bile ducts Primary feature is a nonsuppurative inflammatory destruction of medium- and small sized intrahepatic bile ducts Antimitochondrial antibodies are characteristic and essential for the diagnosis

primary biliary cirrhosis

What is the primary feature of primary biliary cirrhosis?

inflammatory destruction of medium & small sized intrahepatic bile ducts

What is essential for the diagnosis of primary biliary cirrhosis?

antimitochondrial antibodies

What are the clinical features of primary biliary cirrhosis?

usu in middle aged women insidious onset fatigue & abdominal discomfort

Chronic cholestatic disorder characterized by non-specific inflammation, sclerosing fibrosis and strictures of the large intra and extra hepatic bile ducts May result from immunologically mediated injury

primary sclerosing cholangitis

Primary sclerosing cholangitis involves sclerosing fibrosis & strictures of the (blank) and is associated with (blank)

large intra/extra hepatic bile ducts; ulcerative colitis

Small clusters of modestly dilated bile ducts embedded in fibrous stroma AKA “bile duct hamartomas” Common and clinically insignificant Associated with PCKD

Von Meyenberg complexes

Multiple diffuse cysts in the liver | Associated with PCKD

Polycystic liver disease

``` Arises due to a persistence of the embryonic form of the biliary tree Portal tracts show fibrosis Normally no cirrhosis Increased risk of cholangiocarcinoma Associated with PCKD ```

Congenital hepatic fibrosis – a/w Caroli’s disease

Larger ducts of the intrahepatic biliary tree are segmentally dilated Associated with congenital hepatic fibrosis Increased risk of cholangiocarcinoma Associated with PCKD

Caroli disease

Congenital absence/dearth of bile ducts | Rare autosomal dominant multi-organ disorder

Alagille syndrome

What are 5 major clinical features of congenital absence of the bile ducts (Alagille syndrome)?

``` Chronic cholestasis Peripheral stenosis of the pulmonary artery Butterfly-like vertebral arch defects Eye defects Peculiar hypertelic facies ```

Pseudo-mass lesion in an otherwise normal liver Central stellate scar from which fibrous septa radiate Results from congenital vascular malformation

Focal nodular hyperplasia

What is the major notable feature in a focal nodular hyperplasia?

central stellate scar from which fibrous septa radiate

Most common benign liver tumor Tumor of vascular channels in a bed of fibrous connective tissue Should not be mistaken for metastatic tumors Blind percutaneous biopsies should not be performed

cavernous hemangioma

Is a cavernous hemangioma benign or malignant?

benign!

Benign tumor arising from hepatocytes Also called liver cell adenoma Strongly associated with oral contraceptives or anabolic steroid use Subcapsular adenomas may rupture causing severe intra-abdominal hemorrhage

hepatic adenoma

Hepatic adenomas are highly associated with use of ?

oral contraceptives | anabolic steroids

What can happen to subcapsular adenomas in the liver?

they can rupture leading to severe intra-abdominal hemorrhage

Malignant tumor of young children, usually fatal within a few years Two types Epithelial type that recapitulates the developing liver Mixed epithelial and mesenchymal types that shows areas of primitive mesenchyme May be associated with familial adenomatous polyposis syndrome of Beckwith-Wiedmann syndrome

hepatoblastoma

Two types of hepatoblastoma?

epithelial type: looks like the developing liver | mixed epithelial & mesenchymal type: shows areas of primitive mesenchyme

Who get hepatoblastomas? Benign or malignant?

malignant tumor of young children

Accounts for more than 90% of primary liver cancers

hepatocellular carcinoma

List four things that can cause hepatocellular carcinoma

chronic viral infection chronic alcoholism non-alcoholic steatohepatitis food contaminants

What are the three types of hepatocellular carcinoma?

uninodular multinodular diffusely infiltrative

Hepatocellular carcinomas have a strong propensity to invade (blank)

vascular structures, like the portal vein & IVC

This is a variant of hepatocellular carcinoma that occurs in young males & females with NO underlying chronic liver disease or cirrhosis Serum shows elevated AFP levels

fibrolamellar variant

Malignancy of the biliary tree arising from bile ducts within and outside the liver

cholangiocarcinoma

What are risk factors for cholangiocarcinoma?

primary sclerosing cholangitis congential fibropolycystic disease HCV infection thorotrast

Extrahepatic perihilar tumors (near the formation of the common hepatic duct) are known as (blank) tumors

Klatskin

Most common sarcoma arising in the liver Very aggressive malignant tumor with widespread metastasis Thorotrast, vinyl chloride and arsenic exposure have been implicated as causative

angiosarcoma

What causes angiosarcomas?

thorotrast vinyl chloride arsenic exposure

Mets are far more common than primary hepatic neoplasia. Most common primary sites for mets?

colon breast lung pancreas

What are the 3 major groupings of circulatory disorders?

Impaired blood into the liver Impaired blood flow through the liver Hepatic venous outflow obstruction

What can cause impaired blood inflow into the liver?

hepatic artery compromise - due to embolism, neoplasia, polyarteritis nodosa or sepsis portal vein obstruction & thrombosis

What are some symptoms of portal vein obstruction & thrombosis?

abdominal pain portal hypertension ascites

What kinds of things can cause impaired blood flow through the liver?

``` Cirrhosis Sickle cell disease DIC Metastatic tumor Eclampsia Right and left sided heart failure Peliosis hepatis ```

``` Thrombosis of 2 or more major hepatic veins Produces liver enlargement, pain, ascites ¾ of patients have predisposing factors Hypercoagulable state Polycythemia vera Factor V Leiden mutation Contraceptive use Pregnancy ```

Budd-Chiari syndrome

Wide range of presenting symptoms (hepatic dysfunction, hepatic failure, coma, death) Mitochondrial dysfunction implicated as the cause Primary treatment is termination of pregnancy

acute fatty liver of pregnancy

Altered hormonal state of pregnancy with biliary defects in secretion creates cholestasis Benign clinical course

intrahepatic cholestasis of pregnancy

What is cholelithiasis?

gallstones

What are the two types of gallstones?

cholesterol stones | pigment stones - composed of bilirubin calcium salts

What is cholecystitis?

inflammation of the gallbladder

Calculous acute cholecystitis is due to (blank) from obstruction of the neck or cystic duct; acalculous is due to (blank)

chemical irritation & inflammation; ischemia

Clinical features of acute cholecystitis?

``` RUQ or epigastric pain fever anorexia tachycardia nausea vomiting ```

Clinical features of chronic cholecystitis?

Recurrent attacks of steady or colicky epigastric or RUQ pain Nausea Vomiting Intolerance for fatty foods

Complete or partial obstruction of the lumen of the extrahepatic biliary tree within the first 3 months of life

Biliary atresia

What happens in biliary atresia

complete or partial obstruction of the biliary tree causes progressive inflammation and fibrosis of intrahepatic and extrahepatic bile ducts

Two forms of biliary atreisa

Fetal: aberrant intrauterine development of the extrahepatic biliary tree Perinatal: normally formed biliary tree is destroyed following birth

Congenital dilations of the common bile duct Patients present with jaundice or biliary colic Predisposed to stone formation, stenosis and stricture, pancreatitis and obstructive biliary complications

choledochal cysts

Usera: Liver 2 Flashcards

(95 cards)