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58 Cards in this Set

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Autosomal recessive condition involving a mutation on Chromosome 6; associated with HLA-A3
Hereditary Hemochromatosis
Describe Hereditary Hemochromatosis
What are the causes of Secondary Hemochromatosis?
Iron Overload
-repeated transfusions
-ineffective Erythropoiesis (thalassemia, sideroblastic anemia)
-increased dietary iron uptake (Bantu in S. Africa)

Chronic Liver disease in which iron can't be metabolized
What is the classic triad associated with Hereditary Hemochromatosis?
1. Cirrhosis w/ Hemosiderosis

2. "Bronze Diabetes"

3. Skin Hyperpigmentation

*also Cardiomyopathy, Arthritis, & Hypogonadism
What is the treatment for Hereditary Hemochromatosis?
Phlebotomy + Deferoxamine
What are the lab values in Hereditary Hemochromatosis?
1. increased Ferritin
2. increased Iron
3. decreased TIBC
4. increased Transferrin saturation
Hereditary Hemochromatosis
-hepatocytes are filled with blue iron granules
What is seen here?
What is the microscopic difference between Hemochromatosis & Hemosiderosis?
in Hemosiderosis, iron deposits are more prevalent in Kupffer cells than in parenchymal tissue (hepatocytes)
Disease in which the gene for ATP-dependent metal ion transporter (ATP7B) on Chromosome 13 is mutated; results in defective excretion of Copper into Bile -> Copper spills into blood
Wilson Disease
List the pathologies seen in Wilson Disease
1. Fatty liver -> Acute & Chronic Hepatitis -> Cirrhosis

2. Degeneration of Basal Ganglia in the brain = asterixis, Parkinsonian symptoms, choreiform movements, dementia

3. Kayser-Fleischer ring in Cornea

4. Renal tubular damage = aminoaciduria & glycosuria
What are the lab findings are associated with Wilson Disease?
1. low Serum Ceruloplasmin
2. high tissue Copperl levels
3. increased urinary copper excretion
Kayser-Fleischer ring

Wilson's disease = increased Copper accumulation
What is this lesion called?
What disease?
Autosomal recessive mutation in which a small protease inhibitor cannot be secreted & remains in liver cells
Alpha-1-Antitrypsin deficiency
What percent of homozygotes with Alpha-1-Antitrypsin disease actually develop liver disease?
Explain Alpha-1-Antitrypsin deficiency
PiZZ genotype results in 90% inhibition of hepatic secretion of AAT which results in its accumulation in the liver
Alpha-1-antitrypsin deficiency
-PAS with AAT granule accumulation
What is seen here?
What are the possible pathologies seen in AAT deficiency?
1. Micronodular Cirrhosis & increased risk of HCC

2. Panacinar Emphysema
Chronic Hepatitis that occurs in the association with other autoimmune disorders (Thyroiditis, Sjogren, SLE)
Autoimmune Hepatitis
What is the gender preference for Autoimmune Hepatitis? What HLA's are associated with it?
F:M = 7:3

What autoantibodies are associated with Autoimmune Hepatitis?
1. Anti-nuclear Antibody (ANA)

2. Anti-Smooth Muscle Antibody (ASM)
What is the treatment for Autoimmune Hepatitis?
Corticosteroid therapy
-decreases the incidence of cirrhosis to only 5%
Chronic autoimmune progressive disease due to destruction of the Bile Ducts from non-suppurative Granulomas in Portal Triads
Primary Biliary Cirrhosis
Autoimmune destruction of Bile Ducts in Portal Triads
Primary Biliary Cirrhosis
What is the gender preference for Primary Biliary Cirrhosis?

What autoantibodies are present 90% of the time?
F:M = 10:1

Anti-mitochondrial (Mitochondrial Pyruvate Kinase)
What are the clinical findings in Primary Biliary Cirrhosis?
1. Middle-aged women with other autoimmune diseases
2. Progressive Jaundice
3. Skin itching = due to bile salts in skin
4. Xanthomas = cholesterol uptaken by macrophages
What lab findings are associated with Primary Biliary Cirrhosis?
1. increased Bilirubin
2. increased Alk. Phos.
3. increased Cholesterol
4. Anti-mitochondrial Ab
What would a biopsy show in Primary Biliary Cirrhosis?
Granulomatous destruction of Bile ducts in portal triads
Primary Biliary Cirrhosis
-destruction of Bile Ducts by periportal granuloma formation
-lymphocyte infiltration into portal tract
What is this showing?
Chronic progressive pericholangitis of intrahepatic & extrahepatic Bile Ducts with concentric fibrosis -> obstruction of bile flow -> cirrhosis
Primary Sclerosing Cholangitis
What is the gender preference in Primary Sclerosing Cholangitis? What is it associated with?
M:F = 2:1

Ulcerative Colitis
What autoantibody is present in 80% of cases with Primary Sclerosing Cholangitis?
Cholangiography shows "Beading Strictures" in this Liver disease
Primary Sclerosing Cholangitis
Liver disease associated with Ulcerative Colitis
Primary Sclerosing Cholangitis
Primary Sclerosing Cholangitis
What schematic does this represent?
Primary Sclerosing Cholangitis = fibrosing destruction of INTRAHEPATIC BILE DUCTS
Males = 2:1
Ulcerative Colitis
What liver disease is this?
Gender preference?
Associated disease?
Occlusion of the Hepatin Vein by a thrombus, often resulting in death
Budd-Chiari Syndrome
What is Primary Sclerosing Cholangitis associated with an increased incidence of ?
What possible etiologies could cause Budd-Chiari Syndrome?
1. Polycythemia vera
2. Pregnancy
3. Oral Contraceptives
4. Paroxysmal Nocturnal Hemoglobinuria
5. Hepatocellular CA
6. Idiopathic
Budd-Chiari Syndrome
-Centrilobular congestion & necrosis with sinusoidal dilation
What is this showing?
List the 3 Benign Tumors of the liver
1. Hemangioma

2. Liver Cell Adenoma

3. Focal Nodular Hyperplasia
Benign tumor of the liver associated with oral contraceptive use & can rupture causing Hematoperitoneum
Liver Cell Adenoma = Hepatic Adenoma
Most common Benign tumor of the Liver
Circumscribed nodule found in an otherwise normal liver; represents a hamartoma composed of liver cells arranged around a central fibrotic scar containing thick-walled vessels
Focal Nodular Hyperplasia
Liver Cell Adenoma
What is seen here?
Liver Cell Adenoma
-resembles normal liver except for the lack of Portal Tracts
What is seen here?
Focal Nodular Hyperplasia
What Benign Liver tumor is seen here?
List the Malignant tumors of the liver
1. Hepatocellular Carcinoma
2. Cholangiocarcinoma
3. Angiosarcoma (rare)
4. Metastases to the Liver
What is Hepatocellular Carcinoma associated with?
Alcohol consumption
Aflatoxin B1
What is the gender ratio for Hepatocellular Carcinoma?
Male:Female = 4:1

also more common in African-Americans
What lab finding is associated with Hepatocellular Carcinoma?
serum Alpha-Fetoprotein (AFP)
What is the most common malignant tumor of the liver?
Metasteses to the liver
What are the most common primary sites of Metastases of the liver?
1. Lung (most common)

2. GI tract

3. Breast
What are Angiosarcomas associated with?
Vinyl chloride, throtrast, arsenic exposures
Hepatocellular Carcinoma
What is seen here?
Hepatocellular Carcinoma
What is seen here?
Cholangiocellular Carcinoma = adenocarcinoma arising from bile duct epithelium

Clonorchis Sinensis
What is shown here? What is an associated cause?
Def: Adenocarcinoma arising from bile duct epithelium
Metastatic Carcinoma
What is seen here?