Cirrhosis Flashcards
What factors can precipitate hepatic encephalopathy in cirrhotic pts?
drugs (sedatives)
hypovolumia (diarrhea, vomiting, diuretic use, excessive paracentesis),
excessive nitrogen (GI bleeding, constipation, high protein diet)
hypokalemia and metabolic alkalosis hypoxia and hypoglycemia
infection (pneumonia, UTI, SBP)
portosystemic shunting (surgical and radiographical placed shunts)
Subtle signs of hepatic encephalopathy:
reversal of sleep wake cycle, progressing to confusion. Worse can progress to coma and death if untreated.
What are the lab values that patients can safely do paracentesis?
INR of 9 or PLT of 19.
1st line treatment of hepatic encephalopathy
lactulose (oral or rectal) and titrate to two to three bowel movements per day.
what is hepatopulmonary syndrome?
seen in cirrhosis who develop dypsnea on exertion or rest, platiypnea, orthodeoxia, chronic hypoxemia from portal hypertension and presents years after onset of chronic lifer disease
classic triad of hepatopulmonary syndrome:
liver disease, hypoxemia, and intrapulmonary vascular dilations (IPVDs)
IPVD or intrapulmonary vascular dilations cause what
right to left shunting which results in cyanosis and hypoxemia in hepatopulmonary syndrome
diagnosis of hepatopulmonary syndrome
liver disease, decreased oxygen and intrapulmonary vascular dilations
Diagnosis of hepatopulmonary syndrome
contrast echocardiogram (bubble study) showing right to left intracardiac or intrapulmonary shunt also clinical triad of liver disease and decreased oxygenation and IPVDs
Pulmonary symptoms on presentation and manifestations of hepatopulmonary syndrome
dypsnea on rest or exertion platypnea (increased dyspnea with sitting upright or relieved when supine) orthodeoxia (decreased areterial saturation by 5% from supine to upright, chronic hypoxemia with peripheral cyanosis
Hepatic manifestations of hepatopulmonary syndrome
stigmata of cirrhosis (spider nevi, splenomegaly, palmar erythema, ascites, clubbing, and hyperdynamic circulation and elevated cardiac output and decreased systemic and pulmonary vascular resistance)
what do you see on contrast echocardiography (bubble study) with HPS (hepatopulmonary syndrome)?
see left heart opacification 3-6 heart beats after right heart opacification which confirms intrapulmonary right o left shunt if microbubbles pass through dilated pulmonary vessels
Treatment of hepatopulmonary syndrome
no good treatment other than could consider liver transplant with severe hypoxemia.
New diagnosis of cirrhosis. what do you warn them to not do:
no eating rawshell fish (vibro vulnificus exposure) and no NSAIDS, no over the counter herbal supplements without talking to doctor 1st.
hx of cirrhosis varices and continued ETOH with new hematemesis and hypotension
variceal hemorrhage
management of variceal hemorrhage
HDS resuscitation, prevention and treatment of complications and stop bleeding
how to treat variceal hemorrhage
Need volume resuscitation, octreotide (constrict splanchnic circulation and reduce blood flow to portal vein) and needs urgent EGD. Also need abx to prevent SBP and UTI. Give prior to EGD and continue even if cultures are negative
SBP prophylaxis
norfloxacin, ciprofloxacin, or ceftriaxone for 7 days even if cultures are negative. Do this in any GI bleed and cirrhosis pt
SBP is defined by:
PMN >250 cell/mm3
SAAG or serum ascites albumin gradient is
>1.1 g/dl have ascites due to portal hypertension
greater gradient means liver is not making albumin like in cirrhosis
>2.5 total protein points to heart failure
<2.5 total protein points to cirrhosis
SAAG<1.1 and total protein <2.5 points to nephrotic syndrome
portal hypertension management:
managed initially with complete alcohol abstinence and 2 g/day sodium diet
Pts who don’t have improvement (remain symptomatic and have serum sodium >120) with ascites and portal hypertension despite stopping EOTH and sodium restriction should have:
combination oral high dose spironolactone and low dose lasix to reduce fluid retention and avoid hyperkalemia from spironolactone spironolactone 100 mg and lasix 40 mg ratio.
Can spironolactone be used as a single agent for treatment of ascites and portal HTN?
yes only if there’s profound hypokalemia.
why do we avoid use of IV lasix with ascites and portal HTN
risk for acute pre renal azotemia which can be confused with hepatorenal syndrome
what do we do for pts who have refractory ascites?
need large volume paracentesis midodrine transjugular intrahepatic portosystemic stent shunt (TIPS) liver transplant
what medications should be avoided in cirrhosis?
NSAIDs, ACEi and ARBs because they can reduce GFR and precipitate renal failure.
BP drops with increased nitric oxide release and worsened portal HTN and so the body naturally increases vasopressins (aldosterone and vasopressin and anigotensin) to keep BP up and renal perfusion up. These meds impair the body’s and can worsen renal blood flow and prevent excretion of salt and worsen ascites due to portal HTN.
what do most cirrhotics die from?
renal failure, infection, GI bleeds.
when to do a therapeutic paracentesis?
to reduce intraabdominal pressure in symptomatic pts (SOB, abdominal pain, early satiety)
Management of portal HTN and ascites algorithm
evaluation of ascites based on diagnostic paracentesis
ascites fluid quality based on etiology
ascities fluid leukocytes <500 and neutrophils <250 argue against
infection or significant inflammaotry process (malignancy or pancreatitis
Symptoms of Budd Chiari syndrome?
right upper quadrant pain and history of thrombophilia and decompensated heart failure as a result of ascites
Diagnosis and management of Hepatorenal syndrome
what is the pathophysiology that causes hepatorenal syndrome?
increased activity of circulating vasodilators (nitric oxide) and see arterial vasodilation in the splanchnic and systemic circulation and so see less renal perfusion pressure which causes compensatory renal vasoconstriction and reduces GFR and UOP.
how to diagnose Hepatorenal syndrome
diagnosis of exclusion and other things that are suggestive are:
evidence of liver dx (cirrhosis) with portal HTN
AKI without other causes (shock nephrotoxic agents)
urine red blood cells <50 /hpf and urine protein <500 mg/day
Lack of improvement in AKI after 48 hrs of diuretic cessation and volume expansion with albumin
if suspected Hepatorenal syndrome and AKI with Cr elevation, what is the next step?
stop diuretics. trial albumin and if no improvement in kidney function in 48 hrs then hepatorenal syndrome is diagnosed.
Need to start midodrine (selective alpha 1 adrenergic agent) and octreotide (somatostatin analog to reduce splanchnic vasodilation)
what does MELD score do?
helps predict mortality post TIPS
>18 predicts poor 3 month survival .
MELD score <15 needs referral for liver transplant.
common acid base disturbance with cirrhotics
see repiratory alkalosis due to increased minute ventilation and may be related to increased progesterone or estradiol which stimulate ventilation.
Hepatopulmonary syndrome = can also cause increased minute ventilation due to hypoxemia increasing respiratory rate.
Guidelines for variceal prophylaxis in portal HTN chart
for decompensated cirrhosis need to get a
EGD at time of cirrhosis diagnosis to rule out or document presence of varices.
why do we do variceal prophylaxis?
what is it based off?
reduce risk for variceal hemorrhage
based on varix size, presence of decompensated cirrhosis and signs indicating high risk bleed.
1st line tx for variceal prophylaxis in cirrhotic pt:
non selective beta blockers propranolol and nadolol.
they do not prevent formation of varices and should not be started prior to EGD.
SBP prophylaxis is indicated for:
active GI bleeed and ascites
>1 or more episodes of SBP
ascities protein<1g/dl and have impaired renal function (Cr>1.2) or Child Pugh Class C with bilirubin >3
are hospitalized.
Clinical presentation of Wilson’s dx
what are the hepatic manifestations of Wilson’s dx?
asymptomatic liver function abnormalities,
chronic hepatitis
acute liver failure
what are the neuropsychiatric manifestations of Wilson’s dx?
Parkinsonian like tremor
rigidity and clumsiness of gait
subtle personality changes to overt depression, paranoia, and catatonia
What are the hematology condition and renal conditions associated with Wilson’s dx?
Hemolytic anemia
fanconi syndrome
recurrent nephrolithiasis
What are some rheumatological manifestations of Wilson’s dx?
premature arthropathy and chondrocalcinosis (common in knees)
pt presents with psychosis, liver disease that is not releated to hepatitis B or C. has tremor and unsteady gait and liver abnormalities
Wilson’s dx (hepatolenticular degeneration)
Wilson’s is a
autosomal recessive disorder that causes defective copper transport leading to copper acummulation in the brain and liver.
35% of pts present with neurological symptoms (Parkinsonian life tremor, rigidity, clumsy gait, and slurred speech) before age of 30.
can have facial grimacing due to spasms or see “sarcastic smile” or riscus sardonicus
Kayser Fleischer rings
copper deposition in the eye (seen in 50-60% of Wilson’s dx pts) with isolated hepatitis and seen in 90% with pts who had neurological symptoms
Lack of Kayser Fleischer rings doesn’t rule out diagnosis.
Diagnosis of Wilson’s dx
what tests to order?
everyone needs slit lamp examiniation
Diagnosis for Wilsons is: decreased serum ceruloplasmin levels (screening test) and confirmation tests increased 24 hr urine copper levels >40 mcg (high specificity)
high urine copper levels are not unique to Wilson’s seen in autoimmune hepatitis and cholestasis - any dx that obstructs flow of bile from liver to small bowel.
serum copper levels are decreased (paradoxically)
Early diagnosis and medical treatment prevents permanent sequalae.
Tx with penicilliamine and trientine
how to treat Wilson’s dx?
Tx with penicilliamine and trientine
autoimmune hepatitis antibodies
seen with anti smooth muscle antibodies
also see arthralgias, fatigue and weight loss
associated with immune thrombocytopenia, DM1, and thyroiditis and celiac dx and ulcerative colitis.
Alpha1 antitrypsin deficiency is a common
genetic disorder that affects lungs, liver and skin (panniculitis)
lacks AAT protease inhibitor that protects lungs from neutrophil elastases that can lead to emphysematous changes if unchecked.
what worsens and accelerates lung dx in Alpha 1 antitrypsin deficiency pts?
when do you start to see liver dx?
smoking and infections
see hepatitis and cirrhosis in the first 4 decades of live and pulmonary dx (pneumothrax, emphysema, bronchiectasis) happens in older ages
see bibasilar emphysema on imaging
when getting diagnosed with COPD what must be checked?
AAT (alpha antitrypsin) should be checked.
Especially in pts who have early onset emphysema <45 yrs old and emphysema with minimal smoking history
how does early identification of alpha 1 antitrypsin deficiency help?
it allows for early management via smoking cessation, inhaled bronchodilators, corticosteroids, supplemental oxygen for affected pts.
IV augmentation therapy for pooled human plasma AAT helps increase levels and can slow emphysema progression and decline of lung function.
What medications should be discontinued with development of ascites in cirrhosis pts?
stop any NSAIDS, ACEi or ARBs.
These decrease kidney perfusion. BP falls with worsening cirrhosis and reduces renal blood flow and glomerular filtration and the body increases levels of vasoconstrictors including vasopressin, angiotensin and aldosterone and support systemic blood pressure and kidney function due to falling BP. ACEi and NSAIDs impair that and worsen kidney function and prevent excretion of excess sodium and water and affect survival
Tx ascites with sodium restriction to 2g daily. If this doesn’t improve ascites, then start diuretic therapy.
Fre water restriction is only if there’s dilutional hyponatremia.
Anyone <40 yrs old with liver dx must be tested for
Wilson’s dx.
Does propranolol help with management of ascites?
no it doesn’t and can worsen clinical outcomes in pts who have ascites that is refractory to medications (diuretics)
non specific beta blockers are only for prophylactic management and prevention of variceal hemorrhage.
Hepatic encephlopathy
hemolytic anemia
LOW alkaline phosphatase level and unconjugated bilirubinemia
consider Wilson dx - accumulation of copper in the liver, CNS, cornea, kidney, joints, cardiac muscle that results in end organ damage.
sudden release of copper from liver cells can induce hemolytic anemia.
hepatorenal labs
bland urine sediment (no protein, no blood), urine sodium <10, ascites and edema. normal U/S of renal
severe portal hypertension + AKI
Type 1 - triggered but acute AKI and dysfunction like infection, SBP, GI or surgical procedure. Renal function doesn’t improve with holding diuretics.
Type 2 is progressive kidney dysfunction with refactory ascites
Stage 3 and 4 fibrosis pts who have Hep C need to get:
after treatment or with treatment need to get screened for HCC every 6 months with abd U/S
standard interval for following varices without being on a beta blocker
small varices in cirrhosis should get screening EGD q2years
REFERRAL TO LIVER TRANSPLANT
MELD >15 or decompensated cirrhosis
what foods and supplements should people with chronic LIVER dsease avoid?
no iron, no vitamin C -iron storage is impaired in chronic liver dx and vitamin C can increase absorption of iron.
no excessive ETOH - >4 drinks /day
no raw seafood or mullusks - avoid vibrio vulnificus
