Other liver/biliary disease

1.Primary biliary cirrhosis(PBC)

Prevalence- 90% female, 40~60 yrs

Presentation- Jaundice, pruritus, xanthelasma, cognitive dysfunction, steatorrhea, autoimmune disorder

Lab- Cholestatic pattern, Ig M, cholesterol, AMA, Alk-p

Treatment- UDCA(13~15 mg/kg/day)(bile acid move from hepatocyte into bile canaliculus), cholestyramine(2~4 hrs after UDCA)(for pruritus), colchicine, MTX, budesonide

*Not used prednisolone!!



2.Primary sclerosing cholangitis(PSC)

Gene- HLA-B8, HLA-DR3, HLA-DR4

Prevalence– 20~50 y/o male

Association- UC, p-ANCA

Histology- Onion skin

Management- Colonscopy for IBD survey, UDCA(20~30mg/kg/day)

Complication- Cholangiocarcinoma, cholangitis


3.Wilson disease

Gene- AR chromosome 13

Presentation- Jaundice, Kayser-Fleischer ring, Neurological form

Lab- Serum Cu↓, Serum free Cu> 25 ug/dL, 24 hrs urine Cu> 100ug, Ceruloplasmin< 20 mg/dL, liver copper>250 mg/g dry weight liver

Treatment- D-penicillamine(1.5g/day)(bone marrow suppression, liver, MG), Zinc 50mg tid


4.Hemachromatosis

Gene- AR chromosome 6

Presentation- Pancreas damage(DM), melanin content over skin, anterior pituitary gland, liver cirrhosis, cardiomyopathy, arthritis

Diagnosis- Serum iron>220 ug/dL, Transferrin saturation(male>55%, female>45%), serum ferritin> 500x, HFE gene mutation

Hepatic Iron index- >1.9→ Hereditary hemochromatosis

Treatment- Phlebotomy(500ml) weekly, avoid vitamin C, deferoxamine(20~40mg/kg/day)

Published by Steve Johnson

I am a doctor who like to organise knowledges and share ideas with others. As a doctor, a lot of knowledges influx everyday and need some patient to organise and absorb. Here was my notes to share with you and hope to save your time to know it.

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