non viral chronic liver diseases

Question 1

definition of hepatocellular diseases? 6 examples? cholestatic diseases and 2 examples?

Answer 1

liver cell injury, elevated AST/ALT. alcoholic liver disease. NASH. hemochromatosis. wilson’s disease. alpha 1 antitrypsin def. autoimmune hepatitis. bile duct injury = primary biliary cholangitis, primary sclerosing cholangitis

Question 2

chronic liver disease definition? pts have?

Answer 2

> 6 months. abnormal liver tests + clinical symptoms/signs of liver disease

Question 3

alcoholic liver disease is __ common cause of liver disease? how much alc can be safely consumed?

Answer 3

second. men <15/week, women <10/week, max 3/day

Question 4

3 main types of alc. LD?

Answer 4

fatty liver = metabolic. alcoholic hepatitis = inflammatory. alc. cirrhosis = fibrotic

Question 5

presentation of fatty liver vs. alc hepatitis vs. cirrhosis?

Answer 5

FL = asymptomatic but elevated liver tests (AST>ALT ratio of 2). AH = nausea, vomiting, RUQ pain, AST>ALT, elevated bili and PT. C = can be asymp or in liver failure

Question 6

management of of fatty liver vs. alc hepatitis vs. cirrhosis?

Answer 6

for all: stop alcohol. for AH = also optimize nutrition, watch for alc withdrawal, steroids if severe. C = treat complications: varices, ascites, encephalopathy

Question 7

NASH stands for? 2 forms?

Answer 7

non alcoholic steatohepatitis. 1 = simple fatty liver, common, no inflammation. 2 = NASH = has inflammation, resembles alc liver disease and can progress to cirrhosis/liver failure

Question 8

cause of fatty liver/NASH?

Answer 8

metabolic syndrome - insulin resistance (liver is an innocent bystander) - more fat goes into hepatocytes.

Question 9

definition of met. syndrome

Answer 9

at least 3 of: central obesity. dyslipidemia (high TG, low HDL). high BP. high fasting plasma glucose

Question 10

presentation of fatty liver/NASH?

Answer 10

usually asymptomatic. raised LFTs esp GGT/ALT. fatty liver on ultrasound. could also present with advanced liver disease

Question 11

management of fatty liver/NASH

Answer 11

weight control, diabetes control, treat hyperlipidemia, exercise, vit E

Question 12

hemochromatosis: def? inheritance? common in?

Answer 12

autosomal recessive. abnormal retention of body iron = organ damage = cirrhosis. common in whites.

Question 13

hemochromatosis: mutation? result?

Answer 13

excessive absoprtion of iron because of gene defect on chromosome 6: HFE.

Question 14

presentation of hemochromatosis

Answer 14

skin bronzing, cardiomyopathy, cirrhosis, HCC, diabetes, arthropathy (MCP joint in hand), pituitary problems, testicular atrophy –> but usually presentation isn’t until later decades

Question 15

indications ofr hemochromatosis testing?

Answer 15

liver disease, abnormal liver tests, DM, arthropathy, heart disease, bronzed skin, impotence, FDRs

Question 16

hemochromatosis diagnostic algorithm

Answer 16

Fe/TIBC = % Sat elevated above 50. also high ferritin (but remember it’s elevated with inflammation). then do genetic testing for C282Y. then liver biopsy. treat with phlebotomy which will prevent clinical manifestations, reverse some complications (irreversible: cirrhosis, arthropathy, hypogonads, HCC)

Question 17

wilson disease: excess? what do you measure?rare or common? inheritance?

Answer 17

excess copper. ceruloplasmin will be decreased = binding protein for copper. more rare than hemochromatosis. aut recessive.

Question 18

clinical manifestations of wilson’s disease?

Answer 18

kayser fleischer rings in eyes. neuropsych. hepatitis, cirrhosis. fanconi (renal). hemolysis. osteopenia. arthropathy.

Question 19

indications for wilson’s testing

Answer 19

unexplained liver disease in children, adolescents, young adults. any clinical manifestations such as those eye rings, neuro diseases, hemolysis. also family history - siblings.

Question 20

wilson’s diag. algorithm

Answer 20

ceruloplasmin low, slit lamp shows KF rings, urine copper high. then do biopsy. genetic testing not as useful.

Question 21

management of wilson’s?

Answer 21

chelation, zinc, avoid high copper, maybe transplant. monitor KF rings, urine copper, liver enzymes. results: prevents disease if treated early. can actually cure, improve liver/CNS disease, prolong life.

Question 22

alpha 1 antitrypsin def: 2 consequences?

Answer 22

insuff A1AT = unopposed elastase = emphysema in lung. accumulation in liver = liver disease.

Question 23

A1AT: inheritance? common in?

Answer 23

common in caucasians. codominant: M = normal, Z = disease, null = no A1AT so emphysema but not liver disease

Question 24

indications of A1AT testing

Answer 24

neonatal hepatitis, chronic hepatitis, cryptogenic cirrhosis, HCC, early emphysema w/o smoking, FDRs

Question 25

A1AT def diagnostic algorithm

Answer 25

serum AAT low, Pi phenotype abnormal (Z abnormal, M normal). might do liver biopsy.

Question 26

management of A1AT def

Answer 26

treat complications, HCC surveillance: U/S and a-feto protein every 3 months. liver transplant. AA1 transfusion for lung disease. screen family.

Question 27

autoimmune hep: strong association with? often progresses to? liver biopsy? presentation can be?

Answer 27

autoantibodies + other autoimmune diseases. cirrhosis + liver failure. periportal inflammation with prominent plasma cells.. 2 presentations: CLD or acute liver failure

Question 28

presenting symptoms of AIH?

Answer 28

non specific and can be asymptomatic - fatigue, rash, abdo pain, nausea

Question 29

diagnosis of AIH: +/- criteria?

Answer 29

young female, high total IgG, autoantibodies esp. anti-actin, liver biopsy findings, response to steroids. -: AMA neg, neg viral hep, no biliary disease, no drug/alc history

Question 30

treatment of AIH? make sure to exclude?

Answer 30

anti-inflammatory drugs: corticosteroids initially then azathioprine for maintenance. make sure to exclude other liver disease like viral hep, alcohol, NASH, drug hepatotoxicity.

Question 31

primary biliary cholangitis: definition? results in? affects predominantly?

Answer 31

chronic liver disease characterized by inflamm destruction of interlobar bile ducts (microscopic) = chronic cholestasis. predominantly middle aged women.

Question 32

presentation of PBC + lab findings. liver biopsy?

Answer 32

fatigue, pruritis, sicca, high ALP + GGT. positive mitochondrial antibody. elevated IgM. mononuclear inflamm infiltrate centred on bile ducts. granulomas

Question 33

management of PBC

Answer 33

improve bile flow with ursodeoxycholic acid. artificial tears/lozenges for sicca. ca/vit D, biphosphonates, fat soluble vitamins. cholestryamine treats pruritis.

Question 34

Primary sclerosing cholangitis - what? presentation - mostly who?

Answer 34

inflamm and fibrosing condition of MICRO AND MACROscopic bile ducts - involve intra and extra hepatic ducts. 70% males, most have IBD - UC.

Question 35

PSC presentation/lab findings?

Answer 35

fatigue/RUQ pain/pruritis/jaundice + high ALP/GGT + positive auto antibodies: p ANCA + ANA but not very specific so not helpful for diagnosis

Question 36

diagnosis of PSC?

Answer 36

cholangiogram --> via MRCP or ERCP = will see abnormal biliary tree, ducts don't fully fill. biopsy not helpful.

Question 37

PSC - management?

Answer 37

no specific treatment. manage cholestasis as per PBC + vitamin def +bones. majority will need liver transplants around 12 years