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LiverPancreasBilliary

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eesohbel's version from 2015-08-01 17:00

Basics

Question Answer
What germ layer is the liver derived from?Endoderm
What grows off off anterior distal foregutHepatic diverticulum
Hepatic diverticulum becomesLiver
Biliary system
Head of pancreas
Where does hematopoisis take place in the fetus? Liver: mesodermal cells from mesonephros migrate at week 3
Which way does blood flow in the liver?Towards central veins zone 1 to zone 3
Portal triadProper Hepatic artery
Portal vein
Common bile duct
What does portal vein bring into liver?Nutrients
Drugs
Toxins
hepatic artery bring?Oxygenated blood supply to liver
Zone 1 HepatocytesHepatocytes closest to Portal triad
- Closest to oxygenated blood
- Most affected by viral hepatitis
Zone 3 HepatocytesHepatocytes closest to central vein
- Most susceptible to ischemia and toxins
Highest concentration of P450 enzymesZone 3
Which way does bile flow?Toward portal triad: Zone 3 to Zone 1
- Dumps into bile canaliculi
What proteins do hepatocytes make?Coagulation factors
Complement
Albumin
Apolipoprotein
CRP
Angiotensinogen
Transferrin
Ceruloplasmin
What do hepatocytes metabolizeCytochrome P450
UDP-glucuronyl transferase
Alt and AST
Inactivate steroid hormones
What do hepatocytes store?Glucose
Cholesterol(VLDL)
Minerals
Fat soluble vitamins
Liver synthesizes:Bile: Break down fat and excrete bilirubin
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Serum markers

Question Answer
ALT>ASTViral hepatitis
AST>ALTAlcohol (2:1)
GGTLiver pathology
Excessive alcohol
Biliary tract
ALPBiliary obstruction (Gallstones, Cancer, PBC or PSC)
Bone formation (Children, Paget, Bone cancer)
↓ CeruloplasminWilson's
LipaseAcute pancreatitis
AmylaseAcute pancreatitis
Mumps
↑AFPHCC
↑FerritinHemochromatosis
BilirubinuriaHepatitis
High bilirubinViral hepatitis
ANA, anti-SM antibodyAutoimmune hepatitis Type 1
Anti-liver-kidney microsomal antibody, Anti-liver cytosol AntibodyAutoimmune hepatitis Type 2
prothrombin, albumin, and bilirubinmarkers of hepatocyte function
markers hepatocyte integrityAST, ALT
markers cholestasisbilirubin, ALP, GGT
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Hepatic Blood Flow Obstruction

Question Answer
pre-hepatic obstructionvessel thrombosis either in hepatic artery or portal vein
intrahepatic obstructioncentrilobubular necrosis, cirrhosis clonorchis
post hepatic obstructionthrombosis of hepatic vein Budd Chiari
no hepatomegaly with what type of obstructionpre-hepatic
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Neonatal Hyperbilitubinemia

Question Answer
Signs of KernicterusChorea
Cerebral palsy
Hearing loss
Gaze abnormalities
What causes KernicterusNeonatal hyperbilirubinemia
How do you treat kernicterus?UV light phototherapy
Indirect bilirubinUnconjugated bilirubin
Direct bilirubinConjugated bilirubin
Gilbert SyndromeAR: mutation in promotor region of UDP glucoronosyltransferase
Benign slight elevation of indirect bilirubin
Crigler Najjar type IComplete absence of UDP GT
Jaundice and HIgh indirect bilirubinCrigler Najjar
Treatment for Crigler Najjar I?1. Phototherapy 2. Plasmapheresis 3. Transplant
Crigler Najjar type IIMutated UDP-GT
Less severe hyperbilirubinemia
How do you tell differentiate Crigler Najjar from I and IIGive phenobarbital
Type I: no change
Type II Decrease bilirubin
Dubin Johnson Benign defect in MRP2 transport protein (Can't excrete conjugated bilirubin)
Bilirubin gets trapped in hepatocytes
Black liverDubin Johnson
High serum Conjugated BilirubinDubin Johnson
Conjugated bilirubinemia? Dubin Johnson and Rotor Syndrome
Unconjugated bilirubinemia? Crigler Najjar
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Rapid fire

Question Answer
Severe Hyperbilirubinemia with high unconjugated bilirubinCrigler Najjar I
Completely absent UDP-GT?Crigler Najjar I
Treatment of Crigler Najjar IPlasmapheresis, Phototherapy
Mildly decreased UDP-GTCrigler Najjar II
Responds to PhenobarbitolCrigler Najjar II (and Gilbert)
Grossly black liverDubin johnson
Asymptomatic unless stressedGilbert
Benign hyperbilirubinemiaGilbert, Crigler Najjar II, Dubin Johnson, rotor syndrome
Conjugated hyperbilirubinemiaDubin Johnson
Rotor
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Liver Disease

Question Answer
Budd-Chiari SyndromeOcclusion of IVC or Hepatic vein →Congestive liver disease
Nutmeg liverBudd-Chiari Syndrome
Symptoms of Budd-ChiariLiver failure: Hepatomegaly, Ascites, Abdominal pain, Portal HTN
No JVD
Associated with Budd-ChiariHypercoagulable states
Polycythemia vera
Postpartum
HCC
Reye syndromeRare childhood hepatic encephalopathy often seen with children who take aspirin
Damage to mitochondriaReye syndrome
Wilson diseaseAR inability to excrete hepatic copper
Defect in Wilson?ATP7B: Excretes bile
Impaired conversion of copper →Ceruloplasmin decreased
Golden brown corneal ringKayser-Fleischer ring
Where does copper accumulate in Wilson's disease?Liver
Brain
Cornea
Kidneys
Joints
Symptoms of Wilson'sCirrhosis, Corneal deposits, Carcinoma
Hemolytic anemia
Fanconi syndrome
Basal ganglia degeneration
Asterixis
Dementia, Dyskinesia, Dysarthria
[Copper is Hella Fuckin BAD]
Treatment of Wilson'sPenicillamine
HemochromatosisDeposition of hemosiderin
Triad of HemochromatosisCirrhosis
DM
Bronze skin pigmentation
[Bronze Diabetes]
CHF, Testicular atrophyHemochromatosis
Causes of HemochromatosisPrimary: AR disease
Secondary: Excessive transfusions
Lab findings in Hemochromatosis↑Ferritin
↑Serum Fe+
↑Transferrin saturation
↓Total iron binding capacity
Treatment of Hemochromatosis1. Phlebotomy
2. Deferoxamine (sub-Q)
alpha-antitrypsin deficiency in LiverDefective A1-AT polymerizes and accumulates in liver cells
- Autosomal Dominant
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Rapid Fire

Question Answer
Cirrhosis + Pan-acinar emphysemaAlpha-1-Antitrypsin
Kids with virus+aspirinReye syndrome
Ascites without JVDBudd Chiari
↑CopperWilson
↓CeruloplasminWilson
↑Fe+ ↑Ferritin ↑Transferrin saturation ↓TIBCHemochromatosis
Bronze skinHemochromatosis
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Cirrhosis

Question Answer
granulomatous bile duct obstructionPBC
Macrovesicular fatty changeHepatic steatosis
Reversible alcoholic liver damageHepatic steatosis
Alcoholic hepatitis
Swollen and necrotic hepatocytes with neutrophil infiltrationAlcoholic hepatitis (note inflammation!)
Eosinophilic inclusions in cytoplasm of hepatocytesMallory bodies: Alcoholic hepatitis
Liver enzymes in alcoholic hepatitis?AST>ALT by 2x
Symptoms of Alcoholic hepatitis?Painful RUQ with hepatomegaly
Hepatomegaly?Alcoholic hepatitis
Shrunken liverAlcoholic Cirrhosis
Liver enzymes in Alcoholic cirrrhosis?Decreased
Sclerosis around the central veinAlcoholic sclerosis
Which cell mediates cirrhosis?Stellate cells! Cause fibrosis and nodular regeneration that disrupts the architecture of the liver
MC cause of Cirrhosis?Alcohol
TIPSShunt between Portal circulation and systemic circulation to relieve portal hypertension
Downside: ↑ risk of encephalopathy
Effects of liver failureHepatic encephalopathy
Jaundice
Coagulopathy
Edema/ascites
CNS effects of liver failureHepatic encephalopathy and Asterixis
Due to inability to metabolize NH3
Hormonal effects of liver failure↑Estradiol:
-Gynecomastia
Spider angioma
Testicular atrophy
Palmar erythema
Hematologic effects of liver failure↓Clotting factors causes Coagulopathy: ↑PT ↑PTT
Effects of Portal hypertension?Splenomegaly
Esophageal varices (Melena, hematochezia)
Ascites
Spontaneous Bacterial Peritonitis
Treatment of hepatic encephalopathyLactulose/ neomycin
Treatment of Portal hypertension?Diuretic
Beta blocker
Vitamin K
Lactulose
Esophageal varices is caused by shunting between which veins?L. gastric (portal) to Esophageal (systemic)
Caput medusae is caused by shunting between which veins?Paraumbilical (portal) and Sup/inf Epigastric veins
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Cancer

Question Answer
Hepatic adenomaRare, benign liver tumor
Who is susceptible to Hepatic adenomaFemales 20-44, OCP's, Steroids, Glycogen storage disease
Hepatic angiosarcomaMalignant endothelial liver tumor
Hepatic angiosarcoma is associated withArsenic
Vinyl Chloride
MC Primary Liver cancerHepatocellular Carcinoma (HCC)
Risk of HCCHep B
Hepatitis C
Wilson disease
Hemochromatosis
alpha-1-AT deficiency
Alcoholic cirrhosis
Aflatoxin from aspergillus
Symptoms of HCCJaundice
Tender hepatomegaly
Ascites
Polycythemia
Hypoglycemia
Serum marker for HCCAFP
cavernous hemangiomabiopsy contraindicated
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Hepatitis

Question Answer
Symptoms of Hepatitis, when presentMalaise, Arthralgia, Fatigue, N/V, RUQ pain, Jaundice, Scleral Icterus, Hepatosplenomegaly
ssRNAHepatitis A and Hepatitis E
Picorna virusHepatitis A
Fecal-Oral transmissionHepatits A and E [Ends]
Acute onlyHepatitis A and E
IgMHepatitis A
only Hep that is DNA virusHep B
RNA hepevirusHepatitis E [Enteric, Expectant, Epidemic]
Fecal oral, especially waterborne seafoodHepatitis E
Which population is most susceptible to Hepatitis E?Pregnant women have high mortality
enveloped dsDNAHBV
HepadnavirusHBV
Incubation of HBVLong (months)
How many HBV become chronic?10%
Parenteral transmissionHBV and HDV and HCV
Maternal-fetal TransplacentalHBV and HDV and HCV
HDVUninfectious on its own but can cause superinfection with HBV
Circular incomplete ssRNAHDV
RNA delta virusHDV
How many HCV become chronic?80-90%
Sexual transmissionHBV
HCV
Carrier stateHBV, HCV, HDV
HCC riskHBV, HCV, HDV
Vaccine for Hepatitis C?None
Treatment for Hepatitis CInterferon, Ribavirin
Autoimmune hepatitis Type 1ANA, anti-SM antibody
Autoimmune hepatitis Type 2Anti-liver-kidney microsomal antibody, Anti-liver cytosol Antibody
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Hepatitis serology

Question Answer
Acute infectionHBsAg (active infection)
HBcAb IgM (acute)
HBeAg (infectious)
Window periodAnti-HBc IgM
HBeAb (Anti-HBe) (not infectious)
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Liver histology

Question Answer
lymphoid aggregates within the portal tracts and focal areas of macrovesicular steatosisHep C
accumulation of large and small vesicles of fat within hepatocytessteatosis
portal inflammation and eventual fibrosis and destruction of bile ducts, portal tracts and liver parenchymaPBC
deposition of hemosiderin in the liverhemochromatosis
ground glass hepatocytesHepatitis B infection
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Gallbladder pathology

Question Answer
GallstonesCholelithiasis
Gall stones in the bile ductsCholedocholithiasis
Concentric fibrosis of bile ducts (onion skin)PSC (Primary Sclerosing Cholangitis)
"Beads on string" (Strictures + Dilation) on ERCPPSC (Primary Sclerosing Cholangitis)
Men ~40 y.o.PSC (Primary Sclerosing Cholangitis)
Ulcerative colitisPSC (Primary Sclerosing Cholangitis)
+ p-ANCA (MPO-ANCA)PSC (Primary Sclerosing Cholangitis)
Prognosis of PSCCan lead to Cholangiocarcinoma or Secondary biliary sclerosis
Granulomas and lymphocyte infiltrate in biliary tractPBC (Primary Biliary Cirrhosis)
Middle aged women PBC (Primary Biliary Cirrhosis)
What is the mechanism of PBC?Autoimmune destruction of intralobar bile ducts
Mediated by T cell infiltrate
AMAPBC (Primary Biliary Cirrhosis)
Associated with CREST, Sjogren, RA and celiac PBC (Primary Biliary Cirrhosis)
How does biliary tract disease present?Jaundice
Pruritis
Dark pee
Light poo
↑ALP
↑Direct bilirubin
Who is at risk for gallstonesFat
Female
Fertile
Forty (>40 y.o)
Radiolucent gall stonesCholesterol stones (15% are opaque from calcification)
Which population is at risk for Cholesterol stones?Native americans and Crohn's
Black, radiopaque gall stonesPIgment stones
Due to hemolysis
Which population gets pigment stones?Chronic hemolysis, alcoholic cirrohosis, biliary infections
MC gall stonesCholesterol stone
Wax and waning RUQ painBiliary colic caused by contraction of the GB against stone (by CCK)
Fistula between GB and small intestinePneumobilia (complication of gall stones)
Diagnose Gall stoneUS or HIDA scan
What drug can cause gall stones?Cholestyramine (lipid lowering agent that binds bile acid)
Infection of the Biliary treeCholangitis (usually ascending g- infection)
Fever+Jaundice+RUQ painCharcot's triad of Cholangitis
Fever+Jaundice+RUQ pain +Hypotension+Altered MSReynold's pentad of Cholangitis
Inflammation of the gall bladderCholecystitis
Sharp inhale on RUQ palpation from pain+ Murphy's sign for Cholecystitis
Causes of CholecystitisBlocked cystic duct
Ischemia
CMV
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Pancreas

Question Answer
Which enzymes are secreted by the Pancreas?Alpha amylase
Lipase
Protease
Acute pancreatitisAutodigestion of pancreas by pancreatic enzymea
Lab findings in Pancreatitis?Elevated Lipase
Elevated amylase
Complications of Acute Pancreatitis?DIC
ARDS
Fat necrosis
Hypocalcemia with saponification
Pseudocyst
What is a pancreatic pseudocyst?Cyst lined by granulaton scar tissue
Filled with Pancreatic juice
Presentation of Pancreatic adenocarcinoma?Presents late: aggressive and usually already metastasized
Abdominal pain radiating to back
Weight loss
Trousseau syndrome
Obstructive Jaundice
Trousseau syndromeMigratory thrombophlebitis
Hypercoagulable state
Venous thrombosis
Who is at risk for Pancreatic adenocarcinoma?Jewish and African american males >50 y.o.
Risk factors for Pancreatic adenocarcinoma?Tobacco, Chronic pancreatitis, Diabetes
Causes of acute pancreatitisGET SMASHED
Gallstones
Ethanol
Trauma
Steroids
Mumps
Autoimmune
Scorpion sting
Hypercalcemia/Hypertriglyceridemia (>1000)
ERCP - Endoscopic retrograde cholangiopancreatography
Drugs (sulfa drugs)
alcohol and gallstones mc causes
Acute pancreatitis can lead to...DIC
ARDS
Diffuse fat necrosis
hypocalcemia
pseudocyst formation (lined by granulation tissue, not epithelium)
hemorrhage
infxn
multiorgan failure
Acute pancreatitis and labsEpigastric abdominal pain radiating to back
Anorexia
Nausea
Labs: ↑ amylase, lipase (higher specificity)
Pancreatic adenocarcinoma findingsCA-19-9 and CEA
Pancreatic adenocarcinoma presentationAbdominal pain raidiation to back
weight loss
migratory thrombophlebitis - redness/tenderness on palpation of extremities (Trousseau's syndrome)
Obstructive jaundice with palpable,non-tender gallbladder (Courvoisier's sign)
Pancreatic adenocarcinoma txWhipple procedure, chemo, radiation
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