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70 Cards in this Set
- Front
- Back
GENETIC LIVER DISEASES
- list 5 |
(G WASH)
- Gilbert's Dz - Wilson's Dz - AAT-1 Deficiency - Storage Dz - Hereditary Hemochromatosis |
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STORAGE DISEASES
- what are the 2 types? - give examples of each type. |
Glycogen Storage Dz (GGG)
- Galactosemia - von Gierke's Lysosomal Storage Dz (TNG) - Tay-Sachs - Nieman Pick - Gaucher's |
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STORAGE DISEASES
- Glycogen Storage Dz would show up how on histo? - what stain would you use? |
- Plant-like appearance
- Swollen hepatocyte - Rigid cell wall - PAS positive - Diastase |
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STORAGE DISEASES
- in Glycogen storage Dz, the storage in where? - how would you use staining to demonstrate that it is a Glycogen Storage Dz |
- Hepatocytes
- PAS would be positive - then a Diastase would make it gone (b/c Diastase digests glycogen) |
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STORAGE DISEASES
- in Lysosomal Storage Dz, the "storage" of material is where? |
- Kupffer cells (macrophages)
(not in hepatocytes) |
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HEREDITARY HYPERBILIRUBINEMIA
- List 2 diseases associated with UNConjugated bilirubin - List 2 diseases associated with Conjugated bilirubin |
- Gilbert Dz
- Crigler-Najjar Syndrome - Dubin-Johnson - Rotor |
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HEREDITARY HYPERBILIRUBINEMIA
- name a familial disorder - is this heterogenous or homogenous? |
- Inherited Colestatic Disorder
- Heterogenous |
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IRON OVERLOAD SYNDROME
- give 2 examples - which one is hereditary? |
- Hereditary Hemochromatosis
(hereditary) - Secondary Iron Overload |
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IRON OVERLOAD SYNDROME
- what 5 conditions can cause Secondary Iron Overload? |
(SS CMT)
- Chronic Liver Dz - Multiple Blood Transfusion - Thalassemia - Sickle cell - Sideroblastic anemia |
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IRON OVERLOAD SYNDROME
- Hereditary Hemochromatosis has iron deposits where? - Secondary Overload has iron deposits where? |
- in hepatocytes
- early Kuppfer cells |
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HEREDITARY HEMOCHROMATOSIS
- inheritance? - defect in? |
- AR
- Defect in Iron Absorption |
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HEREDITARY HEMOCHROMATOSIS
- excessive Iron accumulation in what organs? |
- Liver
- Pancreas - Heart |
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HEREDITARY HEMOCHROMATOSIS
- Homozygote ratio? - Clinical Symptom ratio? |
- 1:220
- 1:400 |
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HEREDITARY HEMOCHROMATOSIS
- where is the defect gene located? - 70% of patients have a locus where? |
- Chromosome 6
- Next to HLA locus - HLA A3 |
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HEREDITARY HEMOCHROMATOSIS
- associated with what term? - above term refers to what? |
- Bronze Diabetes
Combo of: - Increased Skin pigmentation - DM |
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HEREDITARY HEMOCHROMATOSIS
- what is the change in Total Body Iron? |
- 10 times normal (20 to 40 grams)
|
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HEREDITARY HEMOCHROMATOSIS
- excess iron in liver causes what injury? - above will predispose to what condition? |
- Hepatocyte injury
- Cirrhosis |
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HEREDITARY HEMOCHROMATOSIS
- what is the stain used? - Diagnosis is based on what 3 criteria? |
- Prussion Blue
(iron cross of prussia) (BLT) - high Blood iron - high Liver Deposits of iron - high Transferrin saturation |
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HEREDITARY HEMOCHROMATOSIS
- when you get a cirrhotic liver, what does it look like? - common cause of death in hereditary hemochromatosis |
- enlarged
(unlike most cirrhosis) - CHF |
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HEREDITARY HEMOCHROMATOSIS
- besides an enlarged cirrhotic liver, what other gross observation can be made? |
- irregularly nodular
|
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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? |
(PHD)
- Parenchymal cells - Hepatocytes - Duct cells - everywhere |
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HEREDITARY HEMOCHROMATOSIS
- describe the type of nodular cirrhosis associated |
- Micronodular Cirrhosis
|
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WILSON'S DISEASE
- inheritance? - defect? - defective gene called? - defective gene on what chromosome? |
- AR
- Defective copper metabolism - ATP 7B gene - chromosome 13 (unlucky wilson) |
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WILSON'S DISEASE
- describe the defect causing Cu accumulation - Cu accumulation in where? - Cu accumulation in excess of? |
- Defective Cu EXCRETION
- Hepatocytes - Ceruloplasmin-bound Cu |
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WILSON'S DISEASE
- Excess copper accumulates in what 2 places? |
- Liver
- Eye |
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WILSON'S DISEASE
- what 2 things are decreased in Wilson's Dz? |
- Bilirubin Excretion
- Ceruloplasmin in serum |
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WILSON'S DISEASE
- can complicate into? - TX for Wilson's Dz? (give example) |
- Cirrhosis
- Chelating Agents (D-penicillamine) |
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WILSON'S DISEASE
- copper staining with? - copper accumulation predominantly in which hepatocytes? |
- Rhodanine stain
- Periportal (Zone 1) hepatocytes |
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ALPHA 1 ANTITRYPSIN DEFICIENCY
- inheritance? - Homozygous ratio? - Homozygous mutants develop what 2 symptoms? |
- AR
- 1:2000 - Emphysema - Cirrhosis |
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ALPHA 1 ANTITRYPSIN DEFICIENCY
- AAT1 deficiency is one of the MCC of? |
- Childhood cirrhosis
|
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ALPHA 1 ANTITRYPSIN DEFICIENCY
- what condition is seen with Neonates? |
- Neonatal Hepatitis
with Intracanalicular Cholestasis |
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ALPHA 1 ANTITRYPSIN DEFICIENCY
- Accumulation of what is seen? - what other 2 histological characters are seen? |
- Intracytoplasmic PAS positive Periportal Globules
- Bile Duct Proliferation - Bile Duct Fibrosis |
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ALPHA 1 ANTITRYPSIN DEFICIENCY
- possible complications? x3 |
- HCC
- Chronic ACTIVE Hepatitis - Cirrhosis |
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ALPHA 1 ANTITRYPSIN DEFICIENCY
- what staining would you use? - above stain for what? - what stain is AAT-1 resistant to? |
- PAS
- IntraCytoplasmic Periportal Globules - Diastase resistant |
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CIRRHOSIS
- Cirrhosis is also known as what Dz? |
- End Stage Liver Dz
|
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CIRRHOSIS
- what are 4 characters of Cirrhosis in terms of histology? |
(H - FAR)
- Hepatocellular damage - Fibrosis - Architectural destruction - Regeneration (micro-,macro- nodules) |
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CIRRHOSIS
- since Cirrhosis has architectural destruction, there will also be what? |
- Abnormal Network of Vasculature
|
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CIRRHOSIS
- Cirrhosis is usually associated with what nodular cirrhosis character? - when is this not the case? |
- Macronodular cirrhosis
- Hereditary Hemochromatosis (irregular nodules) |
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CIRRHOSIS
- describe the typical size of Cirrhotic Livers - when is this not the case? |
- Enlarged
- Hereditary Hemochromatosis |
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CIRRHOSIS
- Micronodular Cirrhosis can be seen with what conditions? x3 |
(BAH)
- Biliary system associated cirrhosis - Alcohol Liver Dz - Hereditary Hemochromatosis |
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CIRRHOSIS
- HBV & HCV tend to give rise to what type of nodular cirrhosis? |
- Macronodular cirrhosis
|
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CIRRHOSIS
- with any kind of cirrhosis, if there is Fibrosis, then what reactive process occurs as a result? |
- Proliferation of Portal Bile Duct
|
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CIRRHOSIS
- clinical manifestations of Cirrhosis x9 |
(PEACH OHHH)
- Portal HTN - Endocrine Sx (AGI) - Anemia - CardioPulmonary Failure & PE - HepatoRenal Syndrome - Osteodystrophy - Hypoalbuminemia - Hypothyroidism - Hepatic encephalopathy |
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CIRRHOSIS MANIFESTATIONS
- the manifest of Hypoalbuminemia is associated with what SX? - the manifest of Hypothyroidism is assicated with what Signs? x2 |
- Fluid Retention
- Abnormal Thyroglobulin metabolism - Deficiency of Thyroid Binding Protein |
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CIRRHOSIS MANIFESTATIONS
- the manifest of Osteodystrophy is associated with what other 2 abnormalities? |
Abnormal:
- Vitamin D metabolism - Ca2+ homeostasis |
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CIRRHOSIS MANIFESTATIONS
- the manifest of HepatoRenal Syndrome involves what 2 conditions? |
- renal HYPOPerfusion
- Retention of Na+ |
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CIRRHOSIS MANIFESTATIONS
- manifest of Endocrine Sx involves what 3 symptoms? |
- Anovulation cycles
- Gynecomastia - Impotence |
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CIRRHOSIS
- increased risk factor for? - Prognosis? - Liver Failure unless? |
- HCC
- Bad prognosis ==> Liver failure - unless Transplant |
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CIRRHOSIS
- cirrhosis can also be associated with an enlargement of what other organ? |
- Splenomegaly
|
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LIVER NEOPLASIA
- give 3 examples of Benign liver neoplasia |
(FAB)
- Focal Nodular Hyperplasia - Adenoma of liver - Bile duct Hamartoma & Cysts |
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LIVER NEOPLASIA
- what benign liver neoplasia is usually incidental in finding? - 7% of them are found when? |
- Hemangioma
- Autopsy |
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FOCAL NODULAR HYPERPLASIA
- predominant in what gender group? - predominant in what age groups? x2 |
- Female
- Young - Middle aged |
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FOCAL NODULAR HYPERPLASIA
- histological character? x2 |
- Central Scar in lesion
- Prominant Vascular channels |
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LIVER ADENOMA
- common in what population group? - usually located where? |
- Young Females
- SubCapsular |
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LIVER ADENOMA
- associated with use of what? - tendency for what complication? |
- oral contraceptives
- Rupture & Bleed into Peritoneum |
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LIVER ADENOMA
- histological characters? x3 |
- Hepatocyte proliferation
- LACK of PORTAL TRACT!!!! - Fatty changes |
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LIVER MALIGNANT NEOPLASMS
- MCC? |
- Secondary Metastasis
|
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LIVER MALIGNANT NEOPLASMS
- List 4 Primary Malignant neoplasms |
(FACH!!!)
- Fibrolamellar subtype HCC - Angiosarcoma - CholangioCarcinoma - HepatoBlastoma (Epithelial type) (Mixed Epithelial & Mesenchymal type) |
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LIVER MALIGNANT NEOPLASMS
- which Primary Malignant Neoplasm is seen in childhood? |
- HepatoBlastoma
(epithelial type) (mixed epithelial & mesenchymal type) |
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HEPATOCELLULAR CARCINOMA
- common worldwide especially in regions with high infections of what? x2 - geographic variability increases in what 2 places? |
- HBV
- HCV - Asia - Africa |
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HEPATOCELLULAR CARCINOMA
- most of the HCC types are associated with Cirrhosis except? |
- Fibrolamellar subtype HCC
|
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HEPATOCELLULAR CARCINOMA
- Fibrolamellar HCC seen in what population group? |
- younger people of Asia & Africa
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HEPATOCELLULAR CARCINOMA
- besides Cirrhosis (except for Fibrolamellar subtype) what other associations are there? x3 |
- AAT-1
- Hemochromatosis - Aflotoxin B1 |
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HEPATOCELLULAR CARCINOMA
- what type of genetic mutation is often associated? |
- p53 mutation
|
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HEPATOCELLULAR CARCINOMA
- Histological features for the Fibrolamellar subtype HCC. x4 |
(FILE)
- Fibrosis - Intravascular invasion - Large hepatocytes - Eosinophilic hepatocytes |
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HEPATOCELLULAR CARCINOMA
- possible gross features of HCC. x3 |
- Focal single Nodule
- Multi-nodular pattern - Diffuse, Infiltrative pattern (w/ no nodules) |
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HEPATOCELLULAR CARCINOMA
- corroborate DX by checking what levels? |
- Alpha-Feto Protein (AFP)
|
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HEPATOCELLULAR CARCINOMA
- associated with what 4 signs and symptoms? |
(HAMP)
- Hepatic failure - Associated Paraneoplastic syndrome - Massive bleed - Portal/Hepatic Vein Thrombosis |
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LIVER METASTASIS
- usually NOT associated with what? |
- Cirrhosis
|
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LIVER METASTASIS
- histological character x2 |
- Multiple Nodules
- Central necrosis |