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HE, A1AT defect, NAFLD clinical
GI
37
Medical
Graduate
09/23/2010

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Term
what does the advent of hepatic encephalopathy indicate in a cirrotic pt? what is the prognosis for pts who develop this?
Definition
severe liver damage. there is a 64% one year mortality rate for these pts.
Term
what is hepatic encephalopathy characterized by?
Definition
confusion, diminishing of cognitive function and asterixis
Term
what are the precipitating causes of hepatic encephalopathy?
Definition
electrolyte imbalance: diuretics, vomiting (lose H+/Cl-), diarrhea (lose K+/bicarb). GI bleeding: esophageal/gastric varices (lose protein/gain ammonia) and gastroduodenal erosions. medications: alcohol, benzodiazepines and other sedatives, analgesics (take pts off all if possible). infection: spontaneous bacterial pertitonitis, UTI, chest infection (viral/bacterial - prophylactic antibx). diet: decrease protein overload, worsens ammonia accumulation.
Term
how is hepatic encephalopathy treated?
Definition
ID precipitating factors, stop diuretics, check Na+/K+/BUN/creatinine, eliminate nitrogen containing compounds, control bleeding, lactulose, neomycin, rifaximin, and w/recovery - increase dietary protein.
Term
how does lactulose help with hepatic encephalopathy?
Definition
this dissacharide is barely absorbed in the stomach/small intestine, and once it gets to the large intestine, it is acted on by bacteria. these bacteria break lactulose down into lactic acid/formic acid/acetic acid, which changes the large intestine pH from alkaline to acidic. ammonia circulating through the vascular tree crosses into the bowel, binds with H+ - which brings pH back up and gives the pt diarrhea to get rid of the ammonia.
Term
how does neomycin help with hepatic encephalopathy? ADRs?
Definition
neomycin destroys ammonia-forming bacteria. ADRs include: loss of auditory/renal failure
Term
how does rifaximin help with hepatic encephalopathy?
Definition
rifaximin doesn't have the ADRs associated with other antibx and it also has a low rate of absorption - allowing it to remain in the GI tract. it can cause some complications: diarrhea/flatulence/abdominal pain and is expensive. it is better than lactulose.
Term
what are the main developing problems with alpha1 antitrypsin deficicency? when do they arise?
Definition
COPD and liver disease - which usually don't arise until after age 30
Term
what are the genetics of alpha1 antitrypsin deficiency?
Definition
autosomal recessive. PiZZ is the worst. caucasians of european descent are most often affected.
Term
what is the function of alpha1 antitrypsin?
Definition
neutrophil elastase circulates through pts systems, which alpha1 antitrypsin inhibits. in pts with the Z protein, A1-AT gets stuck in the RER of hepatocytes and the neutrophil elastase circulates unregulated.
Term
what is the most common cause of liver disease in children?
Definition
A1-AT deficiency
Term
what are the clinical manifestations of A1-AT?
Definition
most commonly: emphysema/liver disease. early childhood: jaundice/ascites/poor nutrition. adolescent or adulthood: fatigue, anorexia, ascites, peripheral edema
Term
how is A1-AT diagnosed?
Definition
1) serum A1-AT level. 2) PCR to detect PiZZ (severe) or PiSZ/PiZ null (mild) genes
Term
how is A1-AT treated?
Definition
no cure, just symptomatic tx. can give A1-AT, but it is expensive. for jaundice/pruritis: phenobarbital (enzyme induction to reduce bilirubin) or cholestyramine (binds bilirubin, prevents absorption). for ascites/peripheral edema: diuretics (spironolactone). vitamin therapy.
Term
what is the prognosis for A1-AT pts?
Definition
eventually liver disease, but not usually during childhood. liver transplantation is the best tx if cirrhosis occurs.
Term
what is primary biliary cirrhosis?
Definition
a chronic progressive cholestatic liver disease of unknown cause - characterized by *destruction of intrahepatic bile ducts
Term
who does primary biliary cirrhosis generally happen to? is there a genetic component?
Definition
white females. there is an association with HLA-DRw8.
Term
what is the clinical presentation of primary biliary cirrhosis?
Definition
pruritis w/o jaundice, fatigue, unexplained wt loss, RUQ discomfort, liver problems
Term
what may be seen on a physical exam for primary biliary cirrhosis pts?
Definition
hepatomegaly, splenomegaly, jaundice, xanthomas (eyes, elbows, knees), spider nevi (capillaries dilate on skin - estrogen not metabolized = cherry red angiomas), proximal muscle weakness, ascites, and edema
Term
what are lab findings in primary biliary cirrhosis pts?
Definition
elevated serum alk phos (do 5' nucleotidase, GGTP to confirm liver), AST/ALT maybe slightly high, serum bilirubin rises later in disease, antimitochondrial antibodies, raised ESR, serum lipids raised in later disease (high LDL, low HDL), elevated ceruloplasmin (opposite of wilsons), and elevated serum bile acids.
Term
what should be in the ddx for primary biliary cirrhosis?
Definition
drug induced cholestasis, incomplete bile duct obstruction, primary sclerosing cholangitis, autoimmunie chronic hepatitis, sarcoidosis, and granulomatous hepatitis
Term
what complications are associated with primary biliary cirrhosis?
Definition
portal HTN, pruritis, steatorrhea, osteopenia, xanthomas, and gallstones (due to high conc of bile salts in biliary tree)
Term
how is primary biliary cirrhosis treated?
Definition
colchicine, ursodeoxycholic acid (helps dissolve cholesterol), liver transplant, corticosteroids, azathiaprine, and methotrexate.
Term
what is non-alcoholic fatty liver disease?
Definition
an increasingly recognized condition (half of obese pts between 16-20 have it) that may progress to end stage liver disease. it resembles alcoholic liver injury in pts who don't abuse alcohol and refers to a wide spectrum of liver disease.
Term
what are risk factors for non-alcoholic fatty liver disease?
Definition
obesity, DM type II, hyperlipidemia, and hypertriglyceridemia
Term
what are causes of non-alcoholic fatty liver disease?
Definition
malnutrition (bypass sx, starvation diet), medications, and IBD
Term
what is the prevalence of non-alcoholic fatty liver disease?
Definition
up to 25% of the general pop, and a majority of obese adults. 90% of these obese adults will have steatosis and 19% will have steatohepatitis.
Term
what is the most common cause of abnormal LFTs in adults?
Definition
non-alcoholic fatty liver disease
Term
how does non-alcoholic fatty liver disease appear histologically?
Definition
TGs w/in hepatocytes push the nuclei to one side and fibrous tissue/collagen is laid down between hepatocytes - chicken wire appearance.
Term
what are the clinical manifestations of non-alcoholic fatty liver disease?
Definition
generally asymptomatic livers. some fatigue, malaise, RUQ discomfort, hepatomegaly, acanthosis nigricans
Term
what is seen on labs for non alcoholic fatty liver disease pts?
Definition
mild-moderate AST, ALT, alk phos elevations
Term
what is the pathogenesis of non alcoholic fatty liver disease?
Definition
poorly understood. retention of lipids (TGs) w/in hepatocytes, insulin resistance, and mitochondrial ROS (lipid peroxidation, cytokine induction/Fas ligand induction) are involved.
Term
how is non alcoholic fatty liver disease diagnosed?
Definition
suspicion, based on AST/ALT, radiographic findings (fatty liver), bx and r/o ETOH abuse
Term
what is enough ETOH to induce liver disease?
Definition
M: as low as 30g/day (3 drinks)
F: as low as 20g/day (2 drinks)
Term
how is non alcoholic fatty liver disease treated?
Definition
no good tx plan. slow wt loss. 500 g/wk with kids and 1600 g/wk with adults. drug therapy hasn't helped so far.
Term
non alcoholic fatty liver disease conclusions:
Definition
affects large population of the world. insulin resistance/oxidative stress play critical role. liver bx provides prognosis. no medical therapy currently available other than gradual wt reduction. liver transplant is an option.
Term
what are the risk factors for non alcoholic liver disease?
Definition
> 45 yrs old: 5x. obese: 4x. AST/ALT >1: 4x. DM type II: 3x
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