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73 Cards in this Set
- Front
- Back
Unconjugated Bilirubin
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Insoluble
not excreted tight complex with Albumin |
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Conjugated Bilirubin
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Water
Excreted in Urine loosely bound in Albumin |
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Conjugated HyperBilirubinemia
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1. impaired transport into CANALICULUS
2. Canalicular CHOLESTASIS |
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Unconjugated HyperBilirubinemia
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1. impaired bilirubin UPTAKE
2. altered glucoronyl TRANSFERASE activity |
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Alcoholic Liver Disease
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excessive ethanol consumption
1. hepatic steatosis (fatty liver disease) 2. Alcoholic Hepatitis 3. Cirrhosis |
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Alcoholic Consumption
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12 beers / day
2 glasses of 80-proof liquor/day for 10-20 years |
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Hepatic Steatosis w/ Alcohol
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ethanol increases Lipolysis & delivery of Free Fatty Acids to the liver
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Hepatic Steatosis
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↑ fatty acid synthesis
↑ production of Triglycerides ↑impairs release of Lipoproteins ↓mitochondrial oxidation of Fatty Acids |
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Hepatic Steatosis
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lipid lobules displace nucleus of hepatocytes
"signet ring appearance" Gross enlargement & yellow-color |
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Alcoholic Hepatitis
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Hepatocyte swelling & necrosis
Mallory Bodies Neutrophilic reaction Fibrosis -- activates stellate cells & portal fibroblasts |
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Mallory Bodies
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red cytoplasmic inclusions of cytokeratin filaments
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Alcoholic Cirrhosis
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End-stage liver disease
Anorexia + Weight Loss + Weakness |
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Alcoholic Cirrhosis -- death
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progressive Liver Failure
complications of Portal HTN development of Hepatocellular Carcinoma |
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Alcoholic Cirrhosis---appearance
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mis-shaped w/ surface Nodules
bridging fibrous septa + Parenchymal Nodules diffuse disruption of architecture |
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Portal Hypertension
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increase resistance to portal blood flow
1. ↑resistance @ sinusoids 2. ↑ portal venous flow b/c hyperdynamic circulation |
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Post Hepatic -- portal HTN
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1. Inferior Vena Cava Thrombosis/clot
2. Hepatic Vein Thrombosis/clot 3. Veno-occlusive disease |
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Intrahepatic ---portal HTN
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1. Cirrhosis
2. Sinusoidal Occlusion |
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Pre-hepatic -- portal HTN
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1. Hepatic Artery Compromise
2. Portal Vein Onstruction/clot |
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Circulatory Disorders
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the main category Portal HTN causes
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Cirrhosis
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most common cause of Portal Hypertension
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Circulatory Disorders of Portal HTN
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1. Impaired blood flow thru LIver
2. Hepatic Venous Outflow Obstruction |
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Physiologic Jaundice -- Newborn
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after first 24-hours within first 2 weeks
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Physiologic Jaundice
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immature transhepatic clearance of bilirubin
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Premature Infants --Jaundice
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immaturity and Increased turnover of RBC
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Breastfed Babies --Jaundice
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presence of bilirubin deconjugating enzymes in breast milk
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Physiologic Newborn Jaundice -- treatment
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phototherapy -- absorption of light by unconjugated bilirubin generates water soluble isomers
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Hereditary UNCONjugated Hyperbilirubemias
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1. Gilbert syndrome
2. Crigler Najjar type I 3. Crigler Najjar type II |
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Hereditary CONJUGATED Hyperbilirubemias
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1. Dubin Johnson syndrome
2. Rotor syndrome |
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Gilbert Syndrome
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Autosomal recessive
Benign, common disorder Mild fluctuating Hyperbilirubinemia w/ STRESS |
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Crigler Najjar Type II
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Autosomal Dominant
generally mild VERY Yellow skin **Phenobarbital = (+) Hypertrophy of ER In hepatocytes |
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Crigler Najjar Type I
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Autosomal Recessive
Fatal in neonatal period Death due to Kernicterus in 18mos (if no transplant) |
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Hereditary UNCONjugated Hyperbilirubemia
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due to mutations in UGT1A1
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Gilbert syndrome & Crigler Najjar Type II
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mutated UGT1A1 causes decrease activity of glucoronyl TRANSFERASE
decreased ability to conjugate bilirubin |
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Crigler Najjar Type I
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mutated UGT1A1 results in lack of activity
no ability to conjugate bilirubin |
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Dubin Johnson Syndrome
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Autosomal Recessive
lack of MDR2 canalicular protein Chronic, recurrent Jaundice of varying intensity Asymptomatic & normal lifespan |
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Rotor Syndrome
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impaired bile formation/flow
normal liver Jaundice w/ normal lives |
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Cholestasis
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impaired bile formation and flow
due to obstruction of Bile channels & defects in hepatocyte bile secretion |
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Cholestasis
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Jaundice + Xanthomas + Pruritis
deficiencies of Fat Soluble Vitamins A, D, E, K ↑ alkaline phosphatase & gamma glutamyl transferase |
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Cholestasis
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Parenchyma
swollen hepatocytes, bile pigments in cytoplasm dilated canaculi apoptic cells kupffer cells w/ Bile Portal Tracts Bile ductular proliferation Edema Bile pigment retention Neutrophilic infiltration |
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Intrahepatic Biliary Tract Disease
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Primary Biliary Cirrhosis
Secondary Biliary CIrrhosis Primary Sclerosing Cholangitis |
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Primary Biliary Cirrhosis
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Females
↑ serum IgM autoantibodies dense lymphocytic infiltrate Granulomatous destruction |
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Primary Sclerosing Cholangitis
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Males
atrophy of bile duct epithelium concentric peri-ductal fibrosis "onion-skin" Lymphocytic infiltrate |
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Intrahepatic Biliary Tract disease
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Pruritus + Jaundice + Malaise
Dark Urine + Light Stools + Hepatosplenomegaly Conjugated Hyperbilirubinemia ↑alkaline phosphatase, bile acids, cholesterol |
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Hepatic Disease & Pregnancy
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1. Pre-eclampsia / Eclampsia
2. Acute Fatty Liver of Pregnancy 3. Intrahepatic Cholestasis of Pregnancy |
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Pre-eclampsia
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1. Maternal Hypertension
2. Proteinuria 3. Peripheral Edema 4. Coagulation abnormality & DIC HELLP syndrome |
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HELLP syndrome of Hepatic Disease (pregnancy)
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Hemolysis
Elevated Liver enzymes Low Platelets |
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Eclampsia
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HTN + Protein + Edema + Hyper-reflexia + Convulsion
hemolysis & elevated liver enzymes = Hepatic disease |
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Acute Fatty Liver of Pregnancy
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3rd trimester presentation
Bleeding + Nausea/Vomiting + Jaundice + coma Microvesicular Steatosis |
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Microvesicular Steatosis
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confirms Acute Fatty Liver in pregnancy
Oil Red / Sudan Black stain on frozen tissue |
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Intrahepatic Cholestasis of Pregnancy
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3rd Trimester (BENIGN condition)
Pruritus + Dark Urine + light stool + Jaundice ↑ risk Fetal Distress / Stillbirth / premature birth |
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Jaundice
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yellowing of skin
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Icterus
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yellowing of sclerae (white part of eyes)
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Kernicterus
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rare type of brain damage associated w/ jaundice
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Cholestasis
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systemic retention of bilirubin and other solutes normally eliminated in Bile
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Pruritus
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itching of skin due to deposition of bile salts
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Bile
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thick, greenish yellow fluid made of . . .
1. bilirubin 2. Bile salts 3. Cholesterol 4. Electrolytes |
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Function of Bile
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1. Emulsification of dietary Fat in lumen of gut
2. Elimination of Bilirubin, excess Cholesterol, Xenobiotics & waste products |
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Emulsification of Dietary Fat
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in lumen of gut thru detergent action of bile salts
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Elimination via Bile
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removes Bilirubin, excess Cholesterol, xenobiotics, other waste products that are not water-soluble enough for urinary excretion
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Bilirubin
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end product of heme degradation
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Heme oxygenase
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converts Heme into Bilverdin
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Biliverdin Reductase
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converts Bilverdin in RBC into circulating "unconjugated" bilirubin
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Ascites
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accumulation of excess serous fluid
Cirrhosis most common cause > 500 ml = detectable |
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Portal Hypertension -- Clinical consequences
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1. Hepatic Encephalopathy
2. Esophageal Varices 3. Congestive Splenomegaly 4. Ascites |
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Sinusoidal Obstruction Syndrome
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3 weeks after allogenic Bone Marrow Transplant
toxic injury = slough epithelium @ sinusoids Sloughed off epithelium = obstruct sinusoidal bloodflow |
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Sinusoidal Obstruction
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Ascites + Weight Gain + Jaundice
Tender Hepatosplenomegaly |
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Budd-Chiari syndrome
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Liver Enlargement + Pain + Ascites
due to one of the following.... 1. obstruction of 2 or more Hepatic veins 2. Inferior Vena Cava obstruction **Needs SURGERY ASAP |
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Peliosis Hepatis
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any condition in which Hepatic EFFLUX blood is obstructed
Sinusoidal Dilation is primary ** |
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Peliosis Hepatis -- complications
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potentially Fatal Intra-abdominal hemorrhage
assoc. with CA, TB, AIDS, Post-transplant immunodeficiency anabolic steroids |
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Liver Infarct
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rarely occurs due to its dual blood supply
distal hepatic tissue is pale tan |
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Dual Blood supply to Liver
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Portal Veins & Hepatic Arteries
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Paasive Congestion
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Right-sided cardiac decompensation
Centrilobular Atrophy |
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Centrilobular Necrosis
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Left-sided heart failure
hypoxia = ischemic coagulative necrosis |