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Hepatobiliary and pancreatic disorders > Goljan lab eval of liver cell injury, bilirubin metab, LFTs, labs > Flashcards

Goljan lab eval of liver cell injury, bilirubin metab, LFTs, labs Flashcards

1
Q

What are the 5 major steps in bilirubin metabolism?

A

unconjugated bilirubin (UCB);
UCB combines w/ albumin in blood;
Conjugated bilirubin (CB) secreted into intrahepatic bile ducts;
intestinal bacteria convert CB to urobilinogen (UBG)
20% UBG recycled to liver (90%) & kidneys (10%)

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2
Q

How is UCB formed?

A

senescent RBCs phagocytosed by splenic macs=> UCB is end product of heme degradation => UCB is lipid soluble (indirect bilirubin)

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3
Q

What is the purpose of UCB combining w/ albumin in blood?

A

taken up by hepatocytes then conjugated to glucuronic acid to produce CB => water soluble (direct bilirubin)

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4
Q

what occurs to CB after being secreted into intrahepatic bile ducts?

A

temporarily stored in gallbladder;

enters duodenum via common bile duct

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5
Q

what occurs after CB is converted to UBG?

A

UBG is spontaneously oxidized to urobilin which produces brown color of stool

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6
Q

What is jaundice due to?

A

increase in UCB and/or CB

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7
Q

Where and why is jaundice first noticed?

A

sclera => has high affinity for bilirubin

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8
Q

How is jaundice classified?

A

%CB = CB / total bilirubin

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9
Q

What are the types of hyperbilirubinemia assoc w/ CB < 20%?

A

increased production of UCB;

decreased uptake or conjugation of UCB

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10
Q

What is the urine bilirubin w/ CB < 20%?

A

absent in increased production and decreased uptake/conjugation

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11
Q

What is the urine UBG w/ CB <20%?

A

w/ increased UCB production=> increase urine UBG

decreased uptake / conjugation of UCB => normal urine UBG

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12
Q

What are disorders assoc w/ hyperbilirubinemia of CB <20% and increased production of UCB?

A

extravascular hemolytic anemia => spherocytosis, Rh and ABO HCN, warm AIHA

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13
Q

What are disorders in hyperbilirubinemia w/ CB <20% and decreased uptake or conjugation of UCB?

A

Gilbert’s syndrome;
Crigler-Najjar syndrome;
physiologic jaundice of newborn;
breast milk jaundice

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14
Q

Epidemiology of Gilbert’s syndrome

A 
familial nonhemolytic jaundice;
common AR or AD (mutation dependent);
>5% of population
2nd most common cause of jaundice;
most common hereditary cause of jaundice;
males > females
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15
Q

Pathogenesis of Gilbert’s syndrome

A

impaired glucuronyl transferase activity (70-75% decrease in activity)

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16
Q

When does jaundice occur in Gilbert’s syndrome?

A

w/ fasting or increase in alcohol or phenobarbital intake

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17
Q

Labs and LFTs w/ Gilbert’s syndrome?

A

serum UCB rarely > 5mg/dL;

other LFTs normal;

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18
Q

Bx and Tx for Gilbert’s syndrome

A

liver Bx not necessary;

no Tx

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19
Q

Define Crigler-Najjar syndrome

A

genetic disorder w/ decreased to absent glucuronyl transferase enzyme

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20
Q

If person is develops Crigler-Najjar syndrome w/ absent glucuronyl transferase enzyme, what must be done?

A

liver transplant is required bc it is incompatible w/ life

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21
Q

When and why does physiologic jaundice of newborn occur?

A

day 3 and caused by normal mac destruction of fetal RBCs and inability of newborn liver to handle excess load

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22
Q

What is the cause of breast milk jaundice? Tx?

A

due to pregnane-3a,2oa-diol;

no Tx

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23
Q

What is the CB in mixed type hyperbilirubinemia?

A

CB 20-50%

24
Q

what is the urine bilirubin and UBG in mixed hyperbilirubinemia?

A

increased both bilirubin and UBG

25
Q

disorders of mixed hyperbilirubinemia w/ CB 20-50%

A

viral hepatitis

26
Q

How does viral hepatitis lead to CB 20-50%?

A

defect in uptake, conjugation of UCB and secretion of CB

27
Q

What is the CB, urine bilirubin and urine UBG in obstructive hyperbilirubinemia?

A

CB >50%;
increase in urine bilirubin;
absent urine UBG

28
Q

Main causes of obstructive hyperbilirubinemia w/ CB>50%?

A

decreased intrahepatic bile flow;

decreased extrahepatic bile flow

29
Q

What are disorders associated w/ decreased intrahepatic bile flow?

A

drug induced (OCP);
primary biliary cirrhosis;
Dubin-Johnson syndrome;
Rotor’s syndrome

30
Q

define Dubin Johnson syndrome in obstructive hyperbilirubinemia. How does it appear grossly?

A

AR disorder in secretion into intrahepatic bile ducts;

black pigment in hepatocytes

31
Q

Define Rotor’s syndrome and its association w/ obstructive hyperbilirubinemia

A

AR disorder in secretion into intrahepatic bile ducts but NO BLACK pigment in hepatocytes

32
Q

What are disorders w/ decreased extrahepatic bile flow leading to obstructive hyperbilirubinemia?

A

gallstone in common bile duct;

carcinoma of head of pancreas

33
Q

LFTs for liver cell necrosis

A

ALT;

AST

34
Q

characteristics of ALT test

A

specific enzyme fo rliver clel necrosis;
present in cytosol;
ALT > AST => viral hepatitis

35
Q

characteristics of AST test

A

present in mitochondria;
alcohol damages mitochondria
AST > ALT => alcoholic hepatitis

36
Q

LFTs for cholestasis

A

GGT;

ALP

37
Q

characteristics of GGT test

A

intra- or extra hepatic obstruction to bile flow;
induction of p450 system (alcohol) =>
increases GGT

38
Q

characteristics of ALP test

A

normal GGT and increased ALP => not liver (may be osteoblastic bone activity);
increased GGT and increased ALP=> liver cholestasis

39
Q

LFTs of hepatocyte function

A

serum albumin;
prothrombin time (PT);
BUN;
serum ammonia

40
Q

characteristics of serum albumin test

A

albumin synthesized in liver;

hypoalbuminemia => severe liver disease (cirrhosis)

41
Q

characteristics of PT test

A

majority of coag factors synthesized in liver;

increased PT => severe liver disease

42
Q

characteristics of BUN test

A

urea cycle is present in liver;

decreased BUN=> cirrhosis

43
Q

characteristics of serum ammonia test

A

ammonia is metabolized in urea cycle in liver => derived from large bowel and AA degradation;
increased serum ammonia => cirrhosis, Reye’s

44
Q

LFTs for immune function

A

serum IgM;
antimitochondrial Ab;
Anti-smooth muscle Ab;
ANA

45
Q

increased IgM wrt liver disease

A

primary biliary cirrhosis

46
Q

presence of antimitochondrial Ab

A

primary biliary cirrhosis

47
Q

presence of Anti-smooth muscle Ab

A

autoimmune hepatitis

48
Q

presence of ANA

A

autoimmune hepatitis

49
Q

LFT tumor markers

A

AFP

50
Q

presence of AFP

A

hepatocellular CA

51
Q

give lab findings for normal liver function

A

absent UB;

increased urine UBG

52
Q

lab findings for viral hepatitis

A 
20-50% CB;
very high AST; very very high ALT;
slightly high ALP;
slightly high GGT;
high UB and high urine UBG
53
Q

lab findings for alcoholic hepatitis

A 
20-50% CB;
high AST; slightly elevated ALT;
slightly elevated ALP;
very high GGT;
high UB and urine UBG
54
Q

lab findings for cholestasis

A 
>50% CB;
slightly elevated AST; high ALT;
very high ALP;
very high GGT;
very high UB;
absent UBG
55
Q

lab findings for hemolysis

A 
<20% CB; 
high AST RBCs;
No change in ALT, ALP, GGT;
absent UB;
high urine UBG

Hepatobiliary and pancreatic disorders (7 decks)

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