End stage liver disease

Question 1

What might you find in a patient with compensated liver cirrhosis?

Answer 1

evidence of liver damage like nodular liver but no change in the function of the liver, less likely to see signs of portal HTN

Question 2

Describe the syndrome of decompensated cirrhosis (= end stage liver disease)

Answer 2

hepatocellular dysfunction, often complications of portal HTN including:
jaundice, cholestasis, fat soluble vitamin deficiency, coagulopathy (mucosal bleeding, bruising), hepatic encephalopathy, hypoalbulinemia, changes in hormonal levels

Question 3

What are the complications portal hypertension?

Answer 3

ascites, spontaneous bacterial peritonitis, hepatorenal syndrome, vatical hemorrhage, hepatic encephalopathy, pulmonary complications (hepatic hydrothorax, portopulmonary HTN

Question 4

What are the two main contributors to increasing portal HTN?

Answer 4

increased resistance due to fibrosis and splanchnic vasodialtion causing increased portal flow

Question 5

List causes of pre sinusoidal, sinusoidal and post sinusoidal portal HTN.

Answer 5

pre: portal vein occlusion, splenic vein occlusion, schistosomiasis
sinusoidal: cirrhosis
post: pericardial constriction, IVC occlusion (Budd Chiari), vent occlusive disease

Question 6

What qualities of a vatical hemorrhage make it a risk to bleed?

Answer 6

portal pressure gradient >10-12mmHg
large vary, with surface marks (red wales)
decompensated liver

(based on LaPlace’s Law taking into account wall tension, pressure difference, radius, wall thickness )

Question 7

What are methods to treat acute vatical hemorrhage?

Answer 7

early endoscopy: with vatical band ligation, gastric glue possible, sclerosing agents

reduce pressure gradient by portosystemic shunts with meds (octreotide, vasopressin) or chronically with TIPS

Question 8

What are ways to assess the portal pressure?

Answer 8

direct measurement of hepatic venous pressure gradient

indirect indicators include portosystemic collaterals, splenomegaly, thrombocytopenia

Question 9

Chronic portal HTN causes initiation of what cascade of events?

Answer 9

portal HTN, endogenous vasodilators (NO, glucagon, endocannibanoids) leads to splanchnic and peripheral vasodilation

splanchnic dilation leads to decreased arterial pressure, decreased SVR and increased HR to maintain CO and ultimately ineffective arterial filling

ineffective arterial filling (decreased effective circulating volume-like) leads to stimulation of RAAS, SNS and ADH causing sodium and water retention, renal vasoconstriction and hepatorenal syndrome

Question 10

T/F Ascites is due to portal HTN.

Answer 10

sometimes but not always true, can be due to other causes

Question 11

How would you determine if ascites is due to portal HTN or non-portal HTN causes? How do you calculate SAAG

Answer 11

Portal HTN: SAAG> 1.1
Non-portal HTN: <1.1 (malignancy, infection, pancreatic/biliary leaks

calculate SAAG= (serum albumin- ascites)
Serum-ascites albumin gradient

Question 12

Describe treatments available for ascites therapy.

Answer 12

salt restriction diet
diuretics (aldosterone antagonists and loop diuretics)
paracentesis (when removing large amounts add albumin)
portosystemic shunt
liver transplantation

Question 13

What is spontaneous bacterial peritonitis and how is it dx?

Answer 13

usually occurring in those with cirrhosis (causes the ascites) and then is infected with colonic bacteria, commonly E. coli, K pneumonia and S. pneumonia; if patient has poor hepatic reserve there is a poor prognosis

dx: with parencentesis > 250 PMN per mm3, although there is no organism found in 30-50% of people
tx. antibiotics and albumin

Question 14

What is Hepato-renal syndrome?

Answer 14

renal failure failure occur in cirrhosis which the kidney acts as if the patient were in shock (portal HTN is a key factor)- poor prognosis

asscociated with splachnic/peripheral vasodilation and internal vasoconstriction and retention of sodium

Question 15

How might you treat hepatorenal syndrome?

Answer 15

maintain intravascular volume and avoid nephrotoxins (NSAIDs)

octreotide, midodrine and albumin may help to reduce the portal HTN and ascites

occasionally transjugular intravascular portosystemic shunt or liver transplantation may be use

Question 16

How does hepatic encephalopathy present?

Answer 16

diurnal sleep pattern reversal (insomnia, daytime somnolence)
poor short term memory
can be acutely progressive to confusion, agitation, somnolence and comma

includes motor abnormalities like slurred and slow speech, asterixis, hyperactive deep tendon reflexes, transient decerebrate posturing, seizures

Question 17

What is the mechanism of hepatic encephalopathy?

Answer 17

nitrogenous substance from the gut causes altered cerebral neurotransmission and ammonia which is detoxified in the brain contributes to astrocyte swelling

precipitating causes include: GI bleed, hyypovolemia, infection, hypokalemia, alkalosis, hypoxia, toxins/meds or portosytemic shunting

Question 18

What types of therapies are available for hepatic encephalopathy?

Answer 18

lactulose (non-absorbable disaccharide) works by trapping NH3 int he gut as NH4+ with its production of H+

antibiotics can be used for gut decontamination, but do not restrict protein

Question 19

What is hepatopulmonary syndrome?

Answer 19

circulating vasodilators in portal HTN are thought to cause intrapulmonary vascular dilatations and shunts and increased alveolar-arterial gradient while breathing room air

note excess production of prostacyclin and thromboxane by Kupffer cells can lead to portopulmonary HTN (confirmed by echocardiography and R heart cath)

Question 20

Which side would you most commonly find a hydrothorax

Answer 20

pleural effusion in a patient with cirrhosis and no evidence of cardiopulamonary dz is caused by movement of ascitic fluid into the pleural space through defects in the diaphragm (usually right sided)

Question 21

What is the probability of HCC after cirrhosis?

Answer 21

1-5% each year

Question 22

Give an example of something that may obstruct outflow or inflow of portal blood?

Answer 22

inflow: obstruction at birth due to infection
outflow: Schistomiasis eggs getting logged int eh portal veins