M2-Cx10-L18B --> Liver Path Iib

Front 1

GENETIC LIVER DISEASES

- list 5

Back 1

(G WASH)

- Gilbert's Dz
- Wilson's Dz
- AAT-1 Deficiency
- Storage Dz
- Hereditary Hemochromatosis

Front 2

STORAGE DISEASES

- what are the 2 types?

- give examples of each type.

Back 2

Glycogen Storage Dz (GGG)
- Galactosemia
- von Gierke's

Lysosomal Storage Dz (TNG)
- Tay-Sachs
- Nieman Pick
- Gaucher's

Front 3

STORAGE DISEASES

- Glycogen Storage Dz would show up how on histo?

- what stain would you use?

Back 3

- Plant-like appearance
- Swollen hepatocyte
- Rigid cell wall

- PAS positive
- Diastase

Front 4

STORAGE DISEASES

- in Glycogen storage Dz, the storage in where?

- how would you use staining to demonstrate that it is a Glycogen Storage Dz

Back 4

- Hepatocytes

- PAS would be positive
- then a Diastase would make it gone
(b/c Diastase digests glycogen)

Front 5

STORAGE DISEASES

- in Lysosomal Storage Dz, the "storage" of material is where?

Back 5

- Kupffer cells (macrophages)
(not in hepatocytes)

Front 6

HEREDITARY HYPERBILIRUBINEMIA

- List 2 diseases associated with UNConjugated bilirubin

- List 2 diseases associated with Conjugated bilirubin

Back 6

- Gilbert Dz
- Crigler-Najjar Syndrome

- Dubin-Johnson
- Rotor

Front 7

HEREDITARY HYPERBILIRUBINEMIA

- name a familial disorder

- is this heterogenous or homogenous?

Back 7

- Inherited Colestatic Disorder

- Heterogenous

Front 8

IRON OVERLOAD SYNDROME

- give 2 examples

- which one is hereditary?

Back 8

- Hereditary Hemochromatosis
(hereditary)

- Secondary Iron Overload

Front 9

IRON OVERLOAD SYNDROME

- what 5 conditions can cause Secondary Iron Overload?

Back 9

(SS CMT)

- Chronic Liver Dz
- Multiple Blood Transfusion
- Thalassemia

- Sickle cell
- Sideroblastic anemia

Front 10

IRON OVERLOAD SYNDROME

- Hereditary Hemochromatosis has iron deposits where?

- Secondary Overload has iron deposits where?

Back 10

- in hepatocytes

- early Kuppfer cells

Front 11

HEREDITARY HEMOCHROMATOSIS

- inheritance?

- defect in?

Back 11

- AR

- Defect in Iron Absorption

Front 12

HEREDITARY HEMOCHROMATOSIS

- excessive Iron accumulation in what organs?

Back 12

- Liver

- Pancreas

- Heart

Front 13

HEREDITARY HEMOCHROMATOSIS

- Homozygote ratio?

- Clinical Symptom ratio?

Back 13

- 1:220

- 1:400

Front 14

HEREDITARY HEMOCHROMATOSIS

- where is the defect gene located?

- 70% of patients have a locus where?

Back 14

- Chromosome 6
- Next to HLA locus

- HLA A3

Front 15

HEREDITARY HEMOCHROMATOSIS

- associated with what term?

- above term refers to what?

Back 15

- Bronze Diabetes

Combo of:
- Increased Skin pigmentation
- DM

Front 16

HEREDITARY HEMOCHROMATOSIS

- what is the change in Total Body Iron?

Back 16

- 10 times normal (20 to 40 grams)

Front 17

HEREDITARY HEMOCHROMATOSIS

- excess iron in liver causes what injury?

- above will predispose to what condition?

Back 17

- Hepatocyte injury

- Cirrhosis

Front 18

HEREDITARY HEMOCHROMATOSIS

- what is the stain used?

- Diagnosis is based on what 3 criteria?

Back 18

- Prussion Blue
(iron cross of prussia)

(BLT)
- high Blood iron
- high Liver Deposits of iron
- high Transferrin saturation

Front 19

HEREDITARY HEMOCHROMATOSIS

- when you get a cirrhotic liver, what does it look like?

- common cause of death in hereditary hemochromatosis

Back 19

- enlarged
(unlike most cirrhosis)

- CHF

Front 20

HEREDITARY HEMOCHROMATOSIS

- besides an enlarged cirrhotic liver, what other gross observation can be made?

Back 20

- irregularly nodular

Front 21

HEREDITARY HEMOCHROMATOSIS

- besides inside the hepatocytes, iron accumulation can also be found where in early stages? x2

- with Secondary Hemochromatosis, in later phases, iron accumulation can be found where?

Back 21

(PHD)
- Parenchymal cells
- Hepatocytes
- Duct cells

- everywhere

Front 22

HEREDITARY HEMOCHROMATOSIS

- describe the type of nodular cirrhosis associated

Back 22

- Micronodular Cirrhosis

Front 23

WILSON'S DISEASE

- inheritance?

- defect?

- defective gene called?

- defective gene on what chromosome?

Back 23

- AR

- Defective copper metabolism

- ATP 7B gene

- chromosome 13
(unlucky wilson)

Front 24

WILSON'S DISEASE

- describe the defect causing Cu accumulation

- Cu accumulation in where?

- Cu accumulation in excess of?

Back 24

- Defective Cu EXCRETION

- Hepatocytes

- Ceruloplasmin-bound Cu

Front 25

WILSON'S DISEASE

- Excess copper accumulates in what 2 places?

Back 25

- Liver

- Eye

Front 26

WILSON'S DISEASE

- what 2 things are decreased in Wilson's Dz?

Back 26

- Bilirubin Excretion

- Ceruloplasmin in serum

Front 27

WILSON'S DISEASE

- can complicate into?

- TX for Wilson's Dz? (give example)

Back 27

- Cirrhosis

- Chelating Agents
(D-penicillamine)

Front 28

WILSON'S DISEASE

- copper staining with?

- copper accumulation predominantly in which hepatocytes?

Back 28

- Rhodanine stain

- Periportal (Zone 1) hepatocytes

Front 29

ALPHA 1 ANTITRYPSIN DEFICIENCY

- inheritance?

- Homozygous ratio?

- Homozygous mutants develop what 2 symptoms?

Back 29

- AR

- 1:2000

- Emphysema
- Cirrhosis

Front 30

ALPHA 1 ANTITRYPSIN DEFICIENCY

- AAT1 deficiency is one of the MCC of?

Back 30

- Childhood cirrhosis

Front 31

ALPHA 1 ANTITRYPSIN DEFICIENCY

- what condition is seen with Neonates?

Back 31

- Neonatal Hepatitis
with
Intracanalicular Cholestasis

Front 32

ALPHA 1 ANTITRYPSIN DEFICIENCY

- Accumulation of what is seen?

- what other 2 histological characters are seen?

Back 32

- Intracytoplasmic PAS positive Periportal Globules

- Bile Duct Proliferation
- Bile Duct Fibrosis

Front 33

ALPHA 1 ANTITRYPSIN DEFICIENCY

- possible complications? x3

Back 33

- HCC

- Chronic ACTIVE Hepatitis

- Cirrhosis

Front 34

ALPHA 1 ANTITRYPSIN DEFICIENCY

- what staining would you use?

- above stain for what?

- what stain is AAT-1 resistant to?

Back 34

- PAS

- IntraCytoplasmic Periportal Globules

- Diastase resistant

Front 35

CIRRHOSIS

- Cirrhosis is also known as what Dz?

Back 35

- End Stage Liver Dz

Front 36

CIRRHOSIS

- what are 4 characters of Cirrhosis in terms of histology?

Back 36

(H - FAR)

- Hepatocellular damage

- Fibrosis
- Architectural destruction
- Regeneration
(micro-,macro- nodules)

Front 37

CIRRHOSIS

- since Cirrhosis has architectural destruction, there will also be what?

Back 37

- Abnormal Network of Vasculature

Front 38

CIRRHOSIS

- Cirrhosis is usually associated with what nodular cirrhosis character?

- when is this not the case?

Back 38

- Macronodular cirrhosis

- Hereditary Hemochromatosis
(irregular nodules)

Front 39

CIRRHOSIS

- describe the typical size of Cirrhotic Livers

- when is this not the case?

Back 39

- Enlarged

- Hereditary Hemochromatosis

Front 40

CIRRHOSIS

- Micronodular Cirrhosis can be seen with what conditions? x3

Back 40

(BAH)

- Biliary system associated cirrhosis

- Alcohol Liver Dz

- Hereditary Hemochromatosis

Front 41

CIRRHOSIS

- HBV & HCV tend to give rise to what type of nodular cirrhosis?

Back 41

- Macronodular cirrhosis

Front 42

CIRRHOSIS

- with any kind of cirrhosis, if there is Fibrosis, then what reactive process occurs as a result?

Back 42

- Proliferation of Portal Bile Duct

Front 43

CIRRHOSIS

- clinical manifestations of Cirrhosis x9

Back 43

(PEACH OHHH)

- Portal HTN
- Endocrine Sx (AGI)
- Anemia
- CardioPulmonary Failure & PE
- HepatoRenal Syndrome

- Osteodystrophy
- Hypoalbuminemia
- Hypothyroidism
- Hepatic encephalopathy

Front 44

CIRRHOSIS MANIFESTATIONS

- the manifest of Hypoalbuminemia is associated with what SX?

- the manifest of Hypothyroidism is assicated with what Signs? x2

Back 44

- Fluid Retention

- Abnormal Thyroglobulin metabolism
- Deficiency of Thyroid Binding Protein

Front 45

CIRRHOSIS MANIFESTATIONS

- the manifest of Osteodystrophy is associated with what other 2 abnormalities?

Back 45

Abnormal:
- Vitamin D metabolism
- Ca2+ homeostasis

Front 46

CIRRHOSIS MANIFESTATIONS

- the manifest of HepatoRenal Syndrome involves what 2 conditions?

Back 46

- renal HYPOPerfusion

- Retention of Na+

Front 47

CIRRHOSIS MANIFESTATIONS

- manifest of Endocrine Sx involves what 3 symptoms?

Back 47

- Anovulation cycles

- Gynecomastia

- Impotence

Front 48

CIRRHOSIS

- increased risk factor for?

- Prognosis?

- Liver Failure unless?

Back 48

- HCC

- Bad prognosis ==> Liver failure

- unless Transplant

Front 49

CIRRHOSIS

- cirrhosis can also be associated with an enlargement of what other organ?

Back 49

- Splenomegaly

Front 50

LIVER NEOPLASIA

- give 3 examples of Benign liver neoplasia

Back 50

(FAB)

- Focal Nodular Hyperplasia
- Adenoma of liver
- Bile duct Hamartoma & Cysts

Front 51

LIVER NEOPLASIA

- what benign liver neoplasia is usually incidental in finding?

- 7% of them are found when?

Back 51

- Hemangioma

- Autopsy

Front 52

FOCAL NODULAR HYPERPLASIA

- predominant in what gender group?

- predominant in what age groups? x2

Back 52

- Female

- Young
- Middle aged

Front 53

FOCAL NODULAR HYPERPLASIA

- histological character? x2

Back 53

- Central Scar in lesion

- Prominant Vascular channels

Front 54

LIVER ADENOMA

- common in what population group?

- usually located where?

Back 54

- Young Females

- SubCapsular

Front 55

LIVER ADENOMA

- associated with use of what?

- tendency for what complication?

Back 55

- oral contraceptives

- Rupture & Bleed into Peritoneum

Front 56

LIVER ADENOMA

- histological characters? x3

Back 56

- Hepatocyte proliferation

- LACK of PORTAL TRACT!!!!

- Fatty changes

Front 57

LIVER MALIGNANT NEOPLASMS

- MCC?

Back 57

- Secondary Metastasis

Front 58

LIVER MALIGNANT NEOPLASMS

- List 4 Primary Malignant neoplasms

Back 58

(FACH!!!)

- Fibrolamellar subtype HCC

- Angiosarcoma

- CholangioCarcinoma

- HepatoBlastoma
(Epithelial type)
(Mixed Epithelial & Mesenchymal type)

Front 59

LIVER MALIGNANT NEOPLASMS

- which Primary Malignant Neoplasm is seen in childhood?

Back 59

- HepatoBlastoma
(epithelial type)
(mixed epithelial & mesenchymal type)

Front 60

HEPATOCELLULAR CARCINOMA

- common worldwide especially in regions with high infections of what? x2

- geographic variability increases in what 2 places?

Back 60

- HBV
- HCV

- Asia
- Africa

Front 61

HEPATOCELLULAR CARCINOMA

- most of the HCC types are associated with Cirrhosis except?

Back 61

- Fibrolamellar subtype HCC

Front 62

HEPATOCELLULAR CARCINOMA

- Fibrolamellar HCC seen in what population group?

Back 62

- younger people of Asia & Africa

Front 63

HEPATOCELLULAR CARCINOMA

- besides Cirrhosis (except for Fibrolamellar subtype) what other associations are there? x3

Back 63

- AAT-1

- Hemochromatosis

- Aflotoxin B1

Front 64

HEPATOCELLULAR CARCINOMA

- what type of genetic mutation is often associated?

Back 64

- p53 mutation

Front 65

HEPATOCELLULAR CARCINOMA

- Histological features for the Fibrolamellar subtype HCC. x4

Back 65

(FILE)

- Fibrosis
- Intravascular invasion
- Large hepatocytes
- Eosinophilic hepatocytes

Front 66

HEPATOCELLULAR CARCINOMA

- possible gross features of HCC. x3

Back 66

- Focal single Nodule

- Multi-nodular pattern

- Diffuse, Infiltrative pattern
(w/ no nodules)

Front 67

HEPATOCELLULAR CARCINOMA

- corroborate DX by checking what levels?

Back 67

- Alpha-Feto Protein (AFP)

Front 68

HEPATOCELLULAR CARCINOMA

- associated with what 4 signs and symptoms?

Back 68

(HAMP)

- Hepatic failure
- Associated Paraneoplastic syndrome
- Massive bleed
- Portal/Hepatic Vein Thrombosis

Front 69

LIVER METASTASIS

- usually NOT associated with what?

Back 69

- Cirrhosis

Front 70

LIVER METASTASIS

- histological character x2

Back 70

- Multiple Nodules

- Central necrosis