GI: Pancreas, Liver, Gallbladder Flashcards
(49 cards)
1
Q
- Developmental malformation in which the Pancreas forms a Ring around the Duodenum
- Risk of Duodenal obstruction
- Embryology tie-in w/ Head of Pancreas
A
Annular Pancreas
2
Q
- Inflammation and Hemorrhage of the Pancreas
- Autodigestion of Pancreatic Parenchyma by Enzymes
- Trypsin activates other Pancreatic enzymes
- Liquefactive Hemorrhagic Necrosis and Fat Necrosis
- A/w Alcohol (Sphincter of Oddi) and Gallstones
- Trauma, Hypercalemia, Hyperlipidemia, Drugs, Scorpion Stings, Mumps, Rupture of a Posterior Duodenal Ulcer (Head of Pancreas sits posterior)
- Pain radiates to the Back a ‘Boring’
- Nausea and Vomiting
- Periumbilical and Flank Hemorrhage (Periumbilical soft tissue and Retroperitoneum)
- Elevated Serum Lipase and Amylase
- Phospholipase –> ARDS
- Hypocalcemia w/ Saponification of Fat Necrosis
A
Acute Pancreatitis
3
Q
- Fibrosis of Pancreatic Parenchyma
- 2nd to Recurrent Acute Pancreatitis
-
Alcohol (Adults) and Cystic fibrosis (Children);
Most –> Idiopathic - Epigastric abdominal pain –> Radiates to the Back
- Pancreatic insufficiency –> Results in Malabsorption w/ Steatorrhea and Fat-soluble Vit. Def. (ADEK)
- Amylase and Lipase are not useful Serologic markers –> Destroyed
- Dystrophic Calcification of Pancreatic Parenchyma on Imaging
- ‘Chain of Lakes’ pattern due to dilation of Pancreatic Ducts
- 2nd to Diabetes Mellitus –> Late complication due to Destruction of Islets
- Increased risk Pancreatic Carcinoma (Adenocarcinoma of Pancreatic ducts)
A
Chronic Pancreatitis
4
Q
- Adenocarcinoma arising from the Pancreatic Ducts
- Elderly (avg. 70 y.o.), < 5% 5-year survival
- Smoking and Chronic Pancreatitis
- Epigastric Abdominal pain and Weight loss
- Obstructive Jaundice w/ Pale stools and Palpable gallbladder, a/w Tumors that arise in the Head of the Pancreas
- 2nd Diabetes Mellitus; a/w Tumors in Body or Tail of Pancreas
- Pancreatitis
- Migratory thrombophlebitis (Trousseau sign); Swelling, Erythema, Tenderness in extremities (10%)
- Serum Tumor marker CA 19-9
- Surgical en bloc of Head and Neck, proximal Dudenum, and Gallbladder –> Whipple procedure
- -> 1 yr. survival < 10%
A
Pancreatic Carcinoma
5
Q
- Failure to form or early Destruction of Exrahepatic Biliary Tree –> No lumen
- Leads to Biliary Obstruction w/in first 2 months of life
- Jaundice and progresses to Cirrhosis –> Pale stool
A
Biliary Atresia
6
Q
- Solid, Round stones in Gallbladder
- Precipitation of Cholesterol or Bilirubin in Bile
- Supersaturation of Cholesterol or Bilirubin
- Decreased Phospholipids (Solubilize cholesterol)(lecithin) or Bile acids
- Stasis
- RUQ pain, Nausea, Vomiting, Low-grade Fever, Leukocytosis
- Gangrene of the Gallbladder, Peforation, Fistula, Bowel obstruction, etc…
- Clofibrate – Lipid lowering agent used to control High Cholesterol
A
Cholelithiasis (Gallstones)
7
Q
- Radiolucent (10% are opaque), Cholesterol monohydrate
- Age (40s), Estrogen (Female gender, Obesity, Multiple pregnancies, Oral contraceptives, Hormone replacement therapy
- Fat, Female, Forty, Fertile, and Fucks (5 –F’s)
- Clofibrate – lipid lowering agent used to control for High Cholesterol (Hmg)
- Native American Pima and Navajo Indian ethnicity
- Crohn disease – dmg to terminal ilieum –> decreased Bile reuptake –> increased cholesterol precipitation
- Cirrhosis – decreased productionof Bile salts
A
Cholesterol Stones
8
Q
- Black pigmented Stones composed of Calcium salts and Unconjugated Bilirubin (UCB)
- Radiopaque
- A/w Chronic hemolytis anemias, Cirrhosis, Bacterial infection, and Parasites
- A/w Extravascular Hemolysis (Reticular endothelial
–> Splenic Macrophages –> Unconjugated bilirubin)(increased Bilirubin in bile) and Biliary tract infection
(E coli, Ascaris lumbricoides, and Clonorchis sinensis)
A
Bilirubin Stones
9
Q
- Common Roundworm that infects 25% of the Worlds population, especially in areas w/ Poor sanitation (Fecal-oral transmission)
- Infects the Biliary tract
- Increases risk for Gallstones
A
Ascaris lumbricoides
10
Q
- Endemic in China, Korea, and Vietnam (Chinese Liver flukes)
- Infects the Biliary tract
- Increases the risk of Gallstones
- Cholangitis
- Cholangiocarcinoma
A
Clonorchis sinensis
11
Q
- Waxing and Waning RUQ Pain
- Gallbladder contracting against a Stone lodged in the Cystic duct
- Symptoms relived when Stone passes
- Common Bile duct obstruction may result in
- *Acute Pancreatitis** or Obstructive Jaundice
A
Biliary Colic
12
Q
- Acute Inflammation of the Gallbladder wall
- -> squeezes blood vessels –> Ischemia
- Impacted stone in Cystic duct
- -> Dilation w/ Pressure ischemia
- Bacterial overgrowth (E coli)
- Inflammation and Bloating
- RUQ Pain –> Radiating to Right Scapula, Fever w/ increased WBC count, Nausea, Vomiting, and increased Serum Alkaline Phosphatase (Duct dmg)
- Risk of rupture if left untreated
A
Acute Cholecystitis
13
Q
- Chronic inflammation of Gallbladder
- Chemical irritation from Longstanding cholelithiasis w/ or w/out Superimposed bouts of Acute cholecystitis
- A/w Herniation of Gallbladder mucosa into Muscular Wall (Rokitansky-Aschoff sinuses)
- Vague RUQ Pain, Postprandyl pain
- Porcelain Gallbladder is a late complication – Shrunken, Hard Gallbladder due to Chronic Inflammation, Fibrosis, and Dystrophic Calcification
- Increased risk for Carcinoma
- Tx: Coholecystectomy, esp. w/ Porcelain Gallbladder
A
Chronic Cholecystitis
14
Q
- Bacterial infection of the Bile ducts
- A/w Ascending infection w/ Enteric Gram-Negative bacteria
- Presents as Sepsis (High Fever and Chills), Jaundice, and Abdominal Pain
- Increased incidence w/ Choledocholithiasis (stone in Biliary duct –> decreases flow washout)
- Triad: Epigastric / RUQ pain, Fever, and Jaundice
A
Ascending Cholangitis
15
Q
- Gallstone enters and Obstructs the Small Bowel
- Due to Cholecystitis w/ Fistula formation between the Gallbladder and Small Bowel
A
Gallstone Ileus
16
Q
- Adenocarcinoma arising from the Glandular epithelium that lines the Gallbladder-wall
- Gallstones are a Major risk factor, esp. w/ Porcelain Gallbladder
- Clonorchis sinensis (Liver Flukes)
- Cholecystitis in an Elderly Woman (40 – 70 y.o.)
- -> Gallbladder Carcinoma –> Poor prognosis
- Klatskin tumor - carcinoma of the Bifurcation of the Right and Left Hepatic Bile ducts
A
Gallbladder Carcinoma
17
Q
- Yellow discoloration of the Skin and Scleral Icterus
- Increased Serum Bilirubin > 2.5 mg/dL
- A/w Bilirubin metabolism disturbance
A
Jaundice
18
Q
- High lvls of UCB overwhelm the conjugating ability of the Liver
- Increased UCB (not water soluble)
- Dark urine due to increased urine Urobilinogen, increased Conjugated-UCB
- Increased Risk for Pigmented Bilirubin Gallstones
A
Extravascular Hemolysis
or
Ineffective Erythropoiesis
19
Q
- Newborn Liver has transiently low UGT activity (UGT1A1 activity, decreased excretion)
- Increased UCB –> Fat soluble –> Depositions in Basal Ganglia of the Brain (Kernicterus) –> Neurological deficits and Death
- Tx: Phototherapy (makes UCB water soluble) –> urinate out Bilirubin
A
Physiologic Jaundice of the Newborn
20
Q
- Mildly low UGT activity (UGT1A1)
(UDP-glucuronosyltransferase deficiency) - Autosomal recessive
- Increased UCB
- Diffuse Hepatocellular disease
- Jaundice during stress (e.g. Fasting, Severe Infection); otherwise not clinically significant
A
Gilbert Syndrome
21
Q
- Absence of UGT (severe)
(Deficiency of UGT1A1 activity)
–> Unconjugated hyperbilirubinemia - Increased UCB
- Type I - Kernicterus - Usually fatal - Autosomal recessive - Fatal in Neonatal period
- Type II - Jaundice - Autosomal Dominant w/ variable penetrance - Generally mild, occasional Kernicterus
A
Crigler-Najjar Syndrome
22
Q
- Deficiency of Bilirubin Canalicular ABC transport protein (cMOAT)
- Impaired biliary excretion of bilirubin glucouroides due to Mutation in Multiple Drug-resistant Protein 2 (MRP2) –> Pigmented Cytoplasmic Globules ‘BLACK’
- Benign Autosomal recessive
- Increased CB (Conjugated Hyperbilirubinemia)
- Normal AST and ALT
- No Clinical Consequences
- The Liver is dark; otherwise, not clinically significant –> ‘Pitch Black Liver’ ‘Pitch Dark’ – Gallbladder cannot be visualized
- Similar to Rotor Syndrome –-> Normal appearance, Gallbladder can be visualized
A
Dubin-Johnson Syndrome
23
Q
- A/w Gallstones, Pancreatic Carcinoma, Cholangiocarcinoma, Parasites, and Liver Fluke (Clonorchis sinensis)
- Increased CB
- Decreased Urine Urobilinogen
- Increased Alkaline phosphatase
- Dark urine (Bilirubinuria) and Pale stool
- Pruritus due to increased Plasma bile acids
- Hypercholesterolemia w/ Xanthomas
- Steatorrhea w/ Malabsorption of Fat-soluble vit. (ADEK)
A
Biliary Tract Obstruction
(Obstructive Jaundice)
24
Q
- Inflammation disrupts Hepatocytes and Small Bile Ductules
- Increased in Both CB and UCB
- Increased AST and ALT
- Dark urine due to increased Urine Bilirubine
- Urine Urobilinogen is normal or decreased
A
Viral Hepatitis
25
* **Fecal-oral transmission**
* Herpesvirus naked capsid RNA
* HAV is commonly acquired by **Travelers - 'Infectious'**
* HEV is commonly acquired from **Contaminated water or Undercooked seafood - 'Enteric'**
* Acute Hepatitis; No Chronic state
* Anti-virus IgM marks active Infection
* Anti-virus IgG is protective, and its presence indicates prior infection or immunization (HAV only)
* HEV infection in **Pregnant Women** is a/w **Fulminant Hepatitis** (Liver Failure w/ Massive Liver Necrosis)
Hepatitis A (HAV) and Hepatitis E (HEV)
26
* '**Serum'**
* **Hepadnavirus enveloped DNA**
* **Parenteral transmission** (Childbirth, Unprotected intercourse, Intravenous Drug Abuse [IVDA], and Needle stick)
* **'Ground glass'** Hepatocytes (due to cytoplasmic HBsAg)
* Results in Acute Hepatitis
* Chronic disease occurs in 20% of cases --\> a long-term risk of Hepatocellular carcinoma in pts. infected w/ HBV.
* Vaccine
Hepatitis B (HBV)
27
HBV Acute Stage
* *Increase* HBsAG (Hepatitis B surface marker antigen)
* *Increase* HBeAG and HBV DNA (envelope Antigen Transmission)
* IgM – HBcAB – core Acute Battle
28
HBV Window Stage
* IgM – HBcAB – core Acute Battle
29
HBV Resolved Stage
* IgG – HBcAB – core Acute Battle
* IgG – HBsAB – surface Acute Battle (protective)
30
HBV Chronic Stage
* *Increase* HBsAG (\> 6 months)
* *+/-* HBeAG and HBV DNA indicates infectivity
* IgG – HBcAB – core Acute Battle
31
HBV Immunization Stage
* IgG – HBsAB – surface Acute Battle (protective)
32
* **'Post-transfusion'** and **'non-A, non-B'**
* **Flavivirus enveloped RNA**
* **Parenteral transmission** (IVDA, Unprotected intercourse)
* Risk from transfusion is almost nonexistent due to screening of the blood supply
* Results in Acute hepatitis; Chronic disease occurs in most cases --\> 'Necrosis w/ portal bridging'
* HCV-RNA test confirms infection ELISA
* *Decreased* RNA levels indicate recovery
* Persistence indicates Chronic disease
Hepatitis C (HCV)
33
* **'Delta'**
* Defective enveloped circular RNA
* Dependent on HBV for infection
* Superinfection upon existing HBV is more severe than Coinfection (Infection w/ HBV and HDV at the same time)
* Dx: Anti-HDV ELISA
Hepatitis D (HDV)
34
* **End-stage liver damage**
- -\> Disruption of Normal Hepatic parenchyma
* Bands of Fibrosis and Regenerative nodules of Hepatocytes
* Prothrombin time (PT) assess coagulopathy due to Liver disease
* Fibrosis is mediated by **TGF-β**; from **Stellate cells** which lie beneath the endothelial cells that line the Sinusoids
* Portal Hypertension leads to Ascites (fluid), Congestive Splenomegaly / Hypersplenism (consume RBCs and Platelets) and Portosystemic shunts (esophageal varices, hemorrhoids, and caput medusae) and Hepatorenal syndrome (rapidly developing Renal failure secondary to Cirrhosis)
* Decreased detoxification results in Mental status change, Asterixis, Coma
* Gynocomastia, Spider angiomata and Palmar erythema due to Hyperestrinism
* Jaundice
* Decreased Protein Synthesis leads to Hypoalbuminea w/ Edema and Coagulopathy due to *decreased* clotting factors
Cirrhosis
35
* **Dmg to Hepatic Parenchyma**
* **Fatty Liver** – accumulation of Fat in Hepatocytes (Steatosis)
* Heavy and Greasy Liver
* Alcoholic Hepatitis results from Chemical injury to Hepatocytes (Binge)
* **Acetaldehyde mediates dmg**
* Swelling of Hepatocytes w/ formation of **Mallory bodies** (dmg to **_cytokeratin intermediate filaments_**), Neutrophils, Fatty change, necrosis and acute inflammation
* Fibrosis around the Central vein
* Painful Hepatomegaly w/ (AST \> ALT)
* May result in Death
Alcohol-Related Liver Disease
36
* **Fatty change**, Hepatitis, and/or Cirrhosis that develop **w/out Exposure to Alcohol** or other known insult
* A/w Obesity, Hyperinsulinemia, Insulin resistance, Type 2 Diabetes mellitus
* Lipid accumulation in Hepatocytes
- -\> Steatohepatitis --\> Cirrhosis
* M = F
* Diagnosis of exclusion; ALT \> AST
Nonalcoholic Fatty Liver Disease
37
* **Excess body Iron (Fe)** leading to deposition in tissues and Organ dmg, **HFE, HJV, TFR1, TFR2**
* Tissue dmg is mediated by Generation of Free Radicals
- -\> **Fenton Rxn --\> Heart, Pancreas, and Liver**
* _Autosomal Recessive_ defect in Iron absorption (primary) or Chronic transfusions (secondary)
* Primary Hemochromatosis mutations in **HFE, gene**, usually **Chrom 6p21.3 -** **C282Y --\> Increases small intestine absorption of Fe**
* Cysteine replaced by Tyrosine at AA 282
* **Triad: Cirrhosis, 2nd Diabetes Mellitus, Bronze skin**
* Dilated Cardiobyopathy, Cardiac Arrhythmias, Gonadal dysfunction (atrophy)
* 5x M \> F, Common among Northern European descent
* Brown pigment in Hepatocytes
* *Increased* risk of Hepatocellular carcinoma
- -\> Free radicals --\> Dmg DNA
* Tx: Phlebotomy and Chelating agents (Deferoxamine)
Hemochromatosis
* Hemosiderosis = Acquired disorder of Fe overload due to repeated blood transfusions in pts. w/ Thalassemia
* Hemochromatosis = Inherited disorder
38
* Hepatolenticular degeneration --\> genetic disorder
* _Autosomal recessive_ defect (**chrom 13q14.3** **ATP7B gene**)
- -\> **P-type ATPase -** ATP-mediated hepatocyte Copper **transport into Bile and Ceruloplasmin**
* Lack of Copper transport into Bile
* Lack of Copper incorporation into Ceruloplasmin – carries copper in Blood
* Copper builds up in Hepatocytes, Leaks into Serum, Deposits in Tissues
* Copper-production of Hydroxyl Free Radicals
- -\> Tissue Damage
* Childhood or Adolescence cirrhosis (Liver disease)
* Neurologic manifistations (Behavior, Dementia, Chorea, Parkinsonian)
* **Kayser-Fleisher rings** in the Cornea
* *Increased* Urinary Copper
* *Increased* Hepatocellular carcinoma
* Tx: **D-penicillamine** (chelates copper)
Wilson Disease
39
* **Autoimmune Granulomatous destruction of Intrahepatic Bile Ducts**
* Women (avg. age is 40 y.o., 35 - 65 y.o's)
* 10x Females \> Males
* A/w other Autoimmune diseases (Sjogren Syndrome (70%), Scleroderma (5%), Typhoid disease (20%) Rheumatoid arthritis or SLE)
* Unknown etiology
* **Antimitochondrial autoantibodies (AMA) (90%)** are present - **AMA + ANA + ANCA**
* Obstructive jaundice - **_Florid duct lesions_ and Loss of Small ducts**
* Pruritus, Xanthomas, Xanthelasmas, Serum Cholesterol, Fatigue
* Cirrhosis is a late complication
Primary Biliary Cirrhosis
40
* **Segmental Inflammation and Fibrosis of Intrahepatic and Extrahepatic Bile Ducts**
* Periductal Fibrosis w/ ‘**onion-skin**’ appearance: Concentric Fibrosis around Bile ducts and Segmental stenosis of Bile ducts w/ **_Bending and Strictures_**
* Uninvolved regions are dilated resulting in a
‘**Beaded appearance'** of Bile duct
* **A/w Ulcerative colitis, IBD (70%), Pancreatitis, Fibrosing disease (Retroperitoneal diseases)**
* Males (70%) w/ 20 - 40 y.o's
* Unknown etiology; a/w Ulcerative Colitis and **p-ANCA**
* Obstructive jaundice, and late Cirrhosis
* ***Increased*** risk for **Cholangiocarcinoma**
Primary Sclerosing Cholangitis
41
* **Fulminant Liver failure** and **Encephalopathy** in **Children** w/ **Viral illness w/ take Aspirin**
* Varicella or Influenza
* Likely related to Mitochondrial dmg of Hepatocytes
* Hypoglycemia
* Elevated Liver enzymes
* Causes Hepatic Fatty change (microvesicular steatosis) and Cerebral edema / Encephalopathy
* Nausea and Vomiting --\> Coma and Death
Reye Syndrome
42
* **Benign Tumor of Hepatocytes (Liver cell adenoma)**
* A/w **Oral contraceptive** / anabolic steroids and Type I glycogen storage disease, in **Young Women**
* Regress upon cessation of Oral contraceptive drugs
* Microscopically tissue resembles normal Liver but lack of Portal tracts **No Kupffer cells**
* Risk of Rupture and Intraperitoneal bleeding, especially during Pregnancy
* Tumors are Subcapsular and **Grow w/ Exposure to Estrogen**
Hepatic Adenoma
43
* **Malignant tumor of Hepatocytes (Asia, Japan, and sub-Saharan Africa)**
* **"Eosinophilic Hepatocytes" w/ non-cirrhotic liver and fibrous collagen bundles**
* Chronic Hepatitis (**HBV** and **HCV**), and 4x Male \> Female
* **Cirrhosis** (Alcohol, Nonalcoholic Fatty Liver disease, Hemochromatosis, Wilson disease, and **A1AT** deficiency)
* **Aflatoxin B1** derived from Aspergillus (induced **p53** mutations) - **β-Catenin** - a/w APC
* *Increased* risk for **Budd-Chiari syndrome**
* Liver infarction secondary to Hepatic vein obstruction
* Painful Hepatomegaly and Ascites **RUQ**
* Tumors often detected Late --\> masked by Cirrhosis; poor prognosis, **Increased** **AST and ALT**
* Serum Tumor marker is **_α-fetoprotein_**
Hepatocellular Carcinoma
44
* More common than primary liver tumors
* Multiple well circumscribed masses
* Detected as Hepatomegaly w/ nodular Free edge of the Liver
* Colon
* Stomach
* Pancreas
* Breast
* Lung
* Metastatic Melanoma
Metastasis to Liver
45
* Accumulation of Cholesterol-laden Macrophages w/in the Mucosa of the Gallbladder wall
* Yellow speckling of the Red-tan mucosa - "**Strawberry gallbladder"**
* **Liped-laden Macrophages** w/in **Lamina Propria**
Cholesterolosis
46
* Recent immigrants from Mexico, South America, India, etc.
* *Entamoeba histolytica*
* Necrotic abscess filled w/ Brown Pastelike Material **'Anchovy paste'**
* Tx: ABX w/ or w/out Surgical drainage
Amebic Liver Abscess
47
* Most common genetic disease requiring Liver transplantation in Children
* _Autosomal recessive_ **disorder of Protein folding**
* Production of **α-1-AT** produced by **Pi gene** (chrom 14) accumulates in Hepatocytes --\> Liver damage
* Inhibition of Proteases; Neutrophil elastase, Cathepsin G, and Proteinase 3 --\> **Pulmonary Emphysema**
* PiS (mild varient)
* PiZ (markedly reduced levels)
* PiZZ (severly reduced levels)
* **Micronodular cirrhosis** w/ *increased* risk of **Hepatocellular carcinoma** and **Panacinar emphysema** (lungs)
* PAS positive, Eosinophilic cytoplasmic globules w/in Hepatocytes - **Round - Oval Cytoplasmic Globular inclusions in Hepatocytes**
* Tx: Smoking cessation and Liver Transplantation
α-1-Antitrypsin Deficiency
48
* **Hepatic vein Thrombosis** - occlusion of Two or more Hepatic veins by a Thrombus --\> Hemodynamic Liver Disease --\> Pain and Ascities
* A/w **predisposing for Thrombosis**; **_Polycythemia vera_**, Pregnancy, Oral contraceptives, Paroxysmal Nocturnal hemoglobinuria, or Hepatocellular carcinoma
* Abdominal pain, Hepatomegaly, Ascites, and Death
* **Microscopic Centrilobular Congestion and Necrosis**
Budd-Chiari Syndrome
49
* Asymptomatic
* Solid tumor in the Right lobe of the Liver
* Consiting of a Fibrous core w/ Stellate projections
* NOT a/w use of Oral contraceptives
* Contains **Biliary epithelium** and **Kupffer cells** (Technetium scans show uptake)
Focal Nodular Hyperplasia