Bilirubin and hepatic disease

Question 1

What is gilbert disease?

Answer 1

autosomal recessive or dominant mild deficiency of glcuronosyl transferase and problems with bilirubin uptake that leads to mild jaundice after exercise, stress, or fasting. it causes an increased indirect bilirubin; less than 5 mg/dL

Question 2

What is the tx for crigler najjar type I?

Answer 2

remember, this is severe glucuronosyltranserase deficiency that can cause severe jaundice and permanent brain damange due to kernicterus
-tx: phototherapy, plasmaphoresis, calcium phosphate combined with orlistat, liver transplant

Question 3

What is the treatment for criggler najjar type II?

Answer 3

this is a milder form of criggler najjar that responds to treatment with phenobarbital, which stimulates hepatic production of glucuronyltransferase

Question 4

What is dubin Johnson syndrome?

Answer 4

conjugated hyperbilirubinemia due to defective liver excretion that leads to a black liver. benign. Rotor syndrome is basically the same thing but even more mild in that the liver doesn’t turn black.

Question 5

What are the findings from paracentesis of ascites fluid due to liver cirrohsis?

Question 6

What are the findings of spontaneous bacterial peritonitis on paracentesis?

Answer 6

> 250 PMN/uL, total protienin >1 g/dL, glucose normal serum LDH

Question 7

What are causes of portal HTN?

Answer 7

1. Prehepatic: portal vein thrombosis
2. intrahepatic: cirrhosis, schistosomiasis, parenchymal disease, granulomatous disease
3. Posthepatic: right sided heart failure, hepatic vein thrombosis/Budd-Chiari sydnrome

Question 8

What are the findings on paracentesis for portal HTN?

Answer 8

serum-ascities albumin gradient greater than or equal to 1.1

Question 9

What findings on paracentesis may be suggestive of CA?

Answer 9

high albumin and LDH equal to 60% serum LDH

Question 10

What are the treatments for portal HTN?

Answer 10

1. Salt restriction and diuretics (furosemide and spironolactone)
2. IV abx for bacterial peritonitis if suspected; IV abx for variceal hemorrhage
3. dialysis if in renal failure
4. lactulose/rifamixin for hepatic encephalopathy
5. vasopressin or sclerotherapy for bleeding varicies
6. hepatic shunting and/or liver transplant

Question 11

What are the most affected areas of the body in hemochromatosis? What are potential complications?

Answer 11

• Heart, liver, pancreas, and pituitary; thyroid. these patients may present with high iron levels, increased % saturation of iron, increased ferritin, increased transferrin saturation.
• May also have bone/joint disease (early presentation is often impotence, arthralgias, weakness, fatigue)
• Increased risk of HCC

Question 12

What is the tx for hemochromatosis?

Answer 12

weekly or biweekly phlebotomy, deferoxamine for iron chelation

Question 13

What are the labs that suggest Wilson’s disease?

Answer 13

decreased serum ceruloplasmin, incr. urinary copper. mild increases in AST and ALT possible

Question 14

What is the treatment for Wilson’s disease?

Answer 14

trientine (bolded) or penicillamine for copper chelation, lifelong zinc, B6 replacement, dietary copper restriction,,

Question 15

Which organs commonly send mets to the liver?

Answer 15

lung, colon, breast

Question 16

What infections/infectious causes can predispose to HCC?

Answer 16

HCV, HBC, schistosomiasis, aflatoxin from aspergillis infected food

Question 17

What are some paraneoplastic syndromes associated with HCC?

Answer 17

hypoglycemia, excessive RBC production, watery diarrhea, hypercalcemia, skin lesions