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 |
|
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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. |
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Term
| how is hepatic encephalopathy treated? |
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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. |
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Term
| how does lactulose help with hepatic encephalopathy? |
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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. |
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Term
| how does neomycin help with hepatic encephalopathy? ADRs? |
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Definition
| neomycin destroys ammonia-forming bacteria. ADRs include: loss of auditory/renal failure |
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|
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. |
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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. |
|
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Term
| what is the most common cause of liver disease in children? |
|
Definition
|
|
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 |
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Term
|
Definition
| 1) serum A1-AT level. 2) PCR to detect PiZZ (severe) or PiSZ/PiZ null (mild) genes |
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|
Term
|
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 |
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|
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 |
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|
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. |
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|
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. |
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|
Term
| what are the clinical manifestations of non-alcoholic fatty liver disease? |
|
Definition
| generally asymptomatic livers. some fatigue, malaise, RUQ discomfort, hepatomegaly, acanthosis nigricans |
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|
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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