Hepatic Pathophysiology

Question 1

what effects does hepatic dysfunction have on the digestive system?

Answer 1

impaired metabolism (carbs, cholesterol, bile salts)
esophageal varices
GI bleeding

Question 2

what effects does hepatic dysfunction have on the cardiovascular system

Answer 2

portal hypertension = RHF
ascites = reduced cardiac function and cardiomyopathy
blood pressure disorders (hypotension)

Question 3

what effects does hepatic dysfunction cause on the respiratory system?

Answer 3

ascites = diaphragm pressure causing alveolar dysfunction

portal hypertension = porto-pulmonary hypertension = aspiration risk

Question 4

what effects does hepatic dysfunction cause for hematology?

Answer 4

anemia - d/t GI bleed, hypersplenism, hemolysis
malnutrition
disorders of coag and homeostasis - d/t decreased protein production in liver

Question 5

what effects does hepatic dysfunction cause on the adrenal system?

Answer 5

pre-renal azotemia (elevated urea and nitrogen in blood)
severe electrolyte disorder
- hyponatremia: parallels ascites formation and triggers encephalopathy
- hypokalemia: common in setting of cirrhosis
- acid-base disturbances: range from classic resp alkalosis to high anion gap met acidosis

Question 6

what effects does hepatic dysfunction have on the central nervous system?

Answer 6

hepatic encephalopathy

Question 7

cholelithiasis - definition, etiology, and risk factors

Answer 7

definition: hardened deposits of digestive fluid that form in your gallbladder (stones)
etiology: cholesterol over-secretion, excess bilirubin, gallbladder hypomotility
risk factors: obesity, hyperlipidemia, diabetes, pregnancy, family history, female

Question 8

cholelithiasis signs and symptoms

Answer 8

RUQ pain - or referred pain to shoulder
N/V
indigestion

Question 9

acute cholecystitis

Answer 9

occurs when a gallstone obstructs the cystic duct causing gallbladder to become distended and inflamed

patients will present with fever, RUQ pain, tenderness over gallbladder

Question 10

management of cholelithiasis

Answer 10

initial - fluids, abx, pain
stabilized? take them for lap chole
septic shock? percutaneous chole

Question 11

cholecystectomy anesthetic considerations

Answer 11

risk of opioid-induced sphinter of Oddi spasm

- treat this with glucagon, narcan, or nitrate

Question 12

choledocholithaisis - definition and S&S

Answer 12

definition: complication of cholelithiasis in which a gallstone obstructs the common bile duct impeding the flow of bile from the liver to the duodenum

S&S: biliary colic (N/V/Cramp RUQ intermittent) and cholangitis (fever, rigors, jaundice)

Question 13

management of choledocholithaisis

Answer 13

surgical or endoscopic removal of the obstruction

• preop ERCP to ID stone
• followed by endoscopic sphincterotomy to remove obstruction

Question 14

hyperbilirubinemia definition and types

Answer 14

build up of bilirubin in blood

• conjugated (direct): follows biliary obstruction
• unconjugated (indirect): imbalance between synthesis and catabolism of bilirubin

Question 15

conjugated/direct hyperbilirubinemia

Answer 15

remember this type is more related to biliary obstruction 
****obstructive jaundice***
intrehepatic cholestasis
hepatocellular injury
congenital infections
benign post-op jaundice
dubin-johnson
biliary atresia

Question 16

unconjugated/indirect hyperbilirubinemia

Answer 16

remember this type is more to do with synthesis/catabolism
***hemolytic anemia***
physiologic jaundice of the newborn
jaundice of prematurity
hgb disorder (sickle cell, thalassemia)
hereditary defects in conjugation
red blood cell enzyme disorders
drug inducted sepsis

Question 17

Obstructive Jaundice

Answer 17

form of conjugated/direct hyperbilirubinemia
most commonly d/t obstruction of common bile duct
damage to hepatocytes = hepatitis
conjugated bili is unable to pass from liver to intestines
- enters the blood (likely through rupture of bile canaliculi
- direct emptying of bile into lymph system

MOST BILI IN PLASMA IS CONJUGATED

Question 18

hemolytic jaundice

Answer 18

type of unconjugated/indirect hyperbilirubinemia
increased production of RBCs = increased production of bilirubin by macrophages = increased unconjugated bilirubin in blood
liver cannot conjugate all of the bilirubin so you get a primary risk in unconjugated bilirubin and a secondary rise in conjugated bilirubin
****excretory function of liver is not the problem - its the overproduction of bili
- there is in an increased urobilinogen production and excretion rate

** most bilirubin in plasma is unconjugated

Question 19

Gilbert Syndrome

Answer 19

• benign autosomal dominant inherited disorder that results in unconjugated hyperbilirubinemia
• etiology = decreased activity of the uridine diphosphoclucuronate glucuronyltransferase
• S&S = jaundice, fatigue, or abdominal discomfort that is precipitated by dehydration, exercise, fasting, or stress

Question 20

Crigler-Najarr

Answer 20

one of the most severe forms of inherited unconjucated hyperbilirubinemia - autosomal recessive - diagnosed by lab (high serum concentration of unconjucgated bilirubin)

no or very little expression of the enzyme UGT1A1

severe jaundice, fever, and vomiting - can result in severe brain damage if left untreated

Question 21

treatment for crigler-najjar

Answer 21

daily exchange transfusion
12 hours/day phototherapy
heme oxygenase inhibitors
oral calcium phosphate is often used to bind bili in the gut

curative - liver transplant before the onset of brain damage

Question 22

benign postop intrahepatic cholestasis

Answer 22

definition - postop jaundice in which there is no hepatic inflammation or cell necrosis
etiology - multifactorial and associated with hypotension, significant blood loss, multiple transfusions or hypoxemia

Question 23

S&S of benign postoperative intrahepatic cholestasis

Answer 23

bilirubin and alk phos levels can increase two-to-fourfold within the first 7-10 days postop

Question 24

hepatitis

Answer 24

inflammation of the liver

mostly do not have symptoms - clinical manifestations depend on severity of the inflammatory reaction and amount of cellular necrosis

Question 25

symptoms of chronic viral hepatisis

Answer 25

fever, fatigue, loss of appetite, N&V, abd pain, dark urine, light-color stools, joint pain, jaundice

Question 26

Hep B Virus

Answer 26

can cause both acute and chronic disease transmitted by exposure to infected blood, semen, and other body fluids high risk of death from cirrhosis and liver cancer Vaccine offers 98-100% protection

Question 27

Hep C Virus

Answer 27

most common viral hepatitis leading to liver transplantation in the US antiviral medications can cure > 95% of persons affected no effective vaccine

Question 28

Hep A Virus

Answer 28

lacks chronic stage and contributes only to acute infection present in feces of infected persons transmission - consumption of water food that is contaminated

Question 29

hep D virus

Answer 29

does not produce hepatitis by itself - occurs as a coinfection with acute HBV or as a super infection with chronic HBV - Hep B vaccines provide protection from Hep D

Question 30

hep E virus

Answer 30

rarely results in chronic liver disease | transmitted through consumption of contaminated water/food

Question 31

autoimmune hepatitis

Answer 31

inflammatory disease of the liver characterized by autoantibodies and hypergammaglobulinemia S&S - can present with minimal/no symptoms, as an acute disease, or chronic disease diagnosed by exclusion

Question 32

management of autoimmune hepatitis

Answer 32

prednisone alone or in combo with azathioprine refractory disease therapy - immunosuppression with mycophenolate, cyclosprine A, or tacro failed therapy = liver transplant

Question 33

covid 19 liver injury

Answer 33

etiology - unknown, but the theory is viral-induced injury, cytokine storm, sepsis, drug-induced injury diagnosis - abnormal liver chemistries with elevated aminotransferases treatment - ICU, vent support, CRRT, maintain hemodynamics and treat MODS

Question 34

alcohol-associated liver disease definition and diagnosis

Answer 34

a spectrum of liver injury resulting from alcohol use, ranging from hepatic steatosis to more advanced forms including alcoholic hepatitis, alcohol-associated cirrhosis, and acute AH presenting as acute-on-chronic liver failure diagnoses with alcohol-specific biomarkers such as ethyl glucuronide and ethyl sulfate, which are detected in the urine, blood, and hair can be used to narrow the diagnosis

Question 35

signs and symptoms of ETOH liver disease

Answer 35

none with early ALD or with compensated cirrhosis patient may present with clinical manifestations of alcohol abuse such as malnutrition, muscle wasting, or parotid gland hypertrophy evidence of jaundice, ascites, hepatosplenomegaly, or pedal edema

Question 36

management of ETOH liver disease

Answer 36

abstain from alcohol and manage liver failure | liver transplant is the cure

Question 37

nonETOH fatty liver disease

Answer 37

excessive fat accumulation in the liver without any clear cause, associated with obesity, insulin resistance, type 2 DM, and metabolic syndrome

Question 38

signs and symptoms of NASH

Answer 38

hallmark feature is hepatic steatosis which occurs when there is more than 5% of fat in hepatocytes progression will happen in these fatty hepatocytes are exposed to insults or stress which can then cause NASH diagnosed via liver biopsy

Question 39

drug-induced liver injury

Answer 39

liver injury resulting from direct toxicity of an administered drug or from an immune-mediated mechanism signs and symptoms are the same as all the other ones plus some systemic hypersensitivity (rash, fever, eosinophilia) diagnosed by exclusion and knowledge of med list management - cessation of the inciting drug, supportive care, and evaluation for liver transplantation

Question 40

inborn errors of metabolism

Answer 40

a group of rare genetic or inherited disorders resulting from an enzyme defect that affects the breakdown or storage of carbs, fats, and proteins inherited as autosomal recessive, can rarely be autosomal dominant or x-linked the diseases are wilson, AAT, hemochromatosis

Question 41

Wilson Disease

Answer 41

hepatolenticular degradation characterized by impaired copper metabolism, excess copper levels lead to increased oxidative stress in the cells of the liver, basal ganglia of the brain, and the cornea autosomal recessive managed by copper chelation therapy oral zinc can also be used to stimulate binding of copper in the gut, preventing absorption and transport of copper to the liver

Question 42

S&S&diagnosis of wilson disease

Answer 42

asymptomatic or present with fulminant hepatic failure, alone with neuro or psych manifestations other findings = hemolytic anemia, thrombocytopenia, renal failure, cardiomyopathy, hyperpigmentation

Question 43

alpha 1 antitrypsin (AAT) deficiency

Answer 43

a genetic disorder that results in defective production or AAT protein which is a protein that protects the liver and lungs from neutrophil elastase autosomal dominant diagnoses with elevated transaminase levels and may present with signs of liver dysfunction - confirmed with AAT phenotyping

Question 44

AAT signs and symptoms and management

Answer 44

transplant early onset panlobular emphysema and symptoms of COPD

Question 45

hemochromatosis

Answer 45

disorder assoc with excess iron in the body that can lead to MODS autosomal recessive presents with cirrhosis, heart failure, diabetes, adrenal insufficiency, or polyarthropathy fatigue and malaise management - phlebotomy, iron chelating drugs, liver transplant

Question 46

diagnosis of hemochromatosis

Answer 46

serum transferrin, transferrin saturation, ferritin levels genetic testing for the HFE mutation or other common mutations will confirm the diagnosis echo and MRI can be used to ID cardiomyopathy and assess liver biopsy can be used to quantify iron in liver and assess liver damage

Question 47

primary biliary cholangitis

Answer 47

autoimmune disorder that leads to progressive destruction of intrhepatic bile ducts in addition to periportal inflammation and cholestasis trigger - environmental like cigarettes and hair dye

Question 48

primary sclerosing cholangitis

Answer 48

chronic inflammatory autoimmune disease affecting the bile ducts (primarily the large ducts) S&S - fibrosis of biliary tree leading to cirrhosis and ESLD 40yo male

Question 49

cardiogenic ischemic hepatitis

Answer 49

insufficient perfusion of liver secondary to cardiogenic shock (caused by hypotension)

Question 50

congestive hepatopathy

Answer 50

passive hepatic congestion caused by elevated right sided heart pressures = perisinusoidal edema and impairment of the diffusion of oxygen and nutrients to hepatocytes

Question 51

portal hypertension

Answer 51

high BP in splanchnic venous system d/t high vasc resistance in portal vein from fibrosis and regenerative nodules within the liver diagnosed via hepatic venous pressure gradient measurement treat with intrahepatic portosystemic shunt

Question 52

prehepatic portal hypertension

Answer 52

increased blood flow or obstruction within portal vein or splenic vein - thrombosis - compression - splenomegaly, AVM, fistula

Question 53

intrahepatic portal hypertension

Answer 53

pre-sinusoidal - early primary biliary cholangitis, chronic active hepatitis sinusoidal - cirrhosis, hepatitis, cytotoxicity post-sinusoidal - sinusoidal obstruction, veno-occlusive disease

Question 54

post hepatic portal hypertension

Answer 54

heart - right heart fail, constrictive pericard hepatic vein - budd chiari syndrome IVC - stenosis, thrombosis, tumor invasion

Question 55

hepatic venous pressure gradient

Answer 55

1-5: normal >10: clinical significant portal hypertension >12: variceal rupture

Question 56

ascites

Answer 56

accumulation of fluid in peritoneal cavity causing abdominal swelling usually caused by cirrhosis extensive liver fibrosis blocks blood flow from portal vein, blood backs up causing portal hypertension, fluid leaks out of portal vein into abdomen diagnosed by paracentesis

Question 57

management of ascites

Answer 57

mild - low salt diet, diuretics, albumin replacement, avoid NSAIDs and alcohol more severe ceases - paracentesis refractory cases - TIPS, transplant

Question 58

varices

Answer 58

enlarged veins in lining of esophagus caused by portal hypertension diagnosed by endoscopy asymptomatic until varices begin to bleed - hematemsis black, tarry stools

Question 59

management of varices

Answer 59

prevent further liver damage prevent varices from bleeding - nonselective beat blockers or variceal ligation control bleeding if it occurs - volume resuscitation - endoscopy for ligation - TIPS for refractory bleeding

Question 60

variceal bleeding

Answer 60

high mortality: 40-70% for cirrhotic patient and 5-10% for non-cirrhotic 30% of initial bleeding episodes are fatal treatment - TIPS, shunt, balloon tamponade, banding, sclerosant injection, pharm

Question 61

hepatic encephalopathy

Answer 61

brain injury d/t accumulation of ammonia can be triggered by infection, electrolyte, metabolic disturbance, or meds (benzos and antipsychotics) s&S = confusion, poor concentration, stupor, coma, asterixis, rigidity, hyperreflexia diagnosed with serum ammonia levels

Question 62

management of hep enceph

Answer 62

treat underlying cause lactulose - decrease absorption of ammonia rifaximin- kills bowel flora that produce ammonia

Question 63

hepatorenal syndrome

Answer 63

development of renal failure in patient with advanced chronic liver disease - significant circulatory dysfunction secondary to locally acting vasodilators such as NO and prostaglandins S&S are increased serum creat, normal urine sediment, minimal proteinuria, decreased urine sodium, oliguria diagnosis is presence of cirrhosis and acute renal failure and exclusion of other causes of renal failure managed by midodrine, octriotide, and albumin, hemodialysis, TIPS, liver transplant

Question 64

hepatopulmonary syndrome

Answer 64

hypoxemia due to dilated intrapulm vasc in the presence of liver disease or portal hypertension platypnea - SOB that worsens in upright position and improves when lying supine diagnosed via detection of an intrapulmonary right to left shunt by bubble study manage with liver transplant

Question 65

portopulmonary hypertension

Answer 65

pulmonary arterial hypertension in the setting of portal hypertension with or without liver disease etiology - systemic vasodilation with local pulm production of vasoconstrictors S&S - dyspnea, weakness, systolic murmur, JVD, edema, ascites, hypoxemia, increased A-a gradient mPAP > 25 at rest, screen with TEE and diagnosis with heart cath