Kas 6-20C: Primární biliární cirhóza

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Course 6, case 20
Revised 2009
Kas 6-20E: Itching and hepatomegaly
A so far healthy 34-year-old woman is consulting her general practitioner because of skin
itching lasting two months and getting worse. Itching is most intensive on forearms, hands
and lower extremities, it sometimes disappears during the day but returns in the evening and
is most troubling during the night. The patient was taking Dithiaden and Claritin which
somewhat alleviated the itching. The patient has no other complaints.
On physical examination the GP found a mild symmetric goiter, skin bruises due to
scratching, and a slight hepatomegaly without other abnormalities. There was no icterus and
no other skin changes.
Basic laboratory tests were as follows:
ESR 13/25, serum concentrations: creatinin 76 μmol/l, bilirubin total 17 μmol/l, conjugated 6
μmol/l, cholesterol and glycemia normal. Activity of ALT 1.07 μcat/l, AST 0.92 μcat/l, GMT
4.72 μcat/l, ALP 7.34 μcat/l. The blood count and serum protein electrophoresis were normal,
the thrombocyte count was 156x109/l. There was a trace of urobilinogen in the urine with no
other abnormalitites. The prothrombin time - INR 1.1, APTT 37 s (control 35 s).
Sonography of the abdomen: liver without focal changes, signs of a diffuse liver lesion, a
small amount of biliary sludge in the gallbladder, the biliary tree was narrow, normal findings
on the pancreas and the spleen.
Questions:
1. Which syndrome correspond the symptoms of the patient to?
2. Which further examinations will you recommend and in which order?
3. Might autoantibody assessment contribute to diagnosis?
4. Would you recommend liver biopsy?
5. Which drug might be of therapeutic benefit?
6. Would pruritus disappear following cholecystectomy?
7. Might be the goiter related to liver disease in this patient and is the thyroid function
likely to be changed?
8. Primary sclerosing cholangitis and primary biliary cirrhosis
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