Chemical Pathology - Liver disease CPC

Question 1

What is the inheritance pattern of Gilbert’s?

Answer 1

Autosomal recessive

Question 2

What test measures bilirubin, and how does it work?

Answer 2

van den Bergh test
Fractionation
A direct reaction shows the conjugated bilirubin
Additional of methanol shows total bilirubin
Therefore you can calculate uncojugated bilirubin

Question 3

How can normal paediatric jaundice be distinguished from pathological paediatric jaundice?

Answer 3

physiological jauncide in neonates- the bilirubin will be unconjugated and it is just due to liver immaturity

if not or doesnt go away - look for rare causes like congenital hypothyroidism

Question 4

How can normal paediatric jaundice be treated?

Answer 4

phototherapy

Question 5

What would make jaundice worse in Gilbert’s?

Answer 5

Fasting

Question 6

What is the abnormally-functioning protein that causes Gilbert’s?

Answer 6

UDP glucoronyl transferase - it is reduced to 30% activity

Question 7

What increases in Gilbert’s - conjugated bilirubin, unconjugated bilirubin or both?

Answer 7

ONLY unconjugated bilirubin

Question 8

What is the best marker of liver function in acute liver injury?

Answer 8

Prothrombin time

Question 9

How can you tell that a paracetamol OD is bad enough to need transplant?

Answer 9

PT in seconds is > hours since OD

Question 10

How does acute alcoholic hepatitis present?

Answer 10

Nausea, abdominal pain and jaundice
Pain is due to inflammation

Question 11

What are 3 key histological findings that is pathognemonic for alcoholic hepatitis?

Answer 11

Mallory denk bodies
Megamitochondria
Fibrosis

Question 12

Which antibody to hep A will be the first to be produced, and for what time period post-exposure to the virus will it be present in serum?

Answer 12

IgM
Initial production at 3 weeks
Peaks at 5 weeks
Gone by 13 weeks

note in hep A virus will be in faecaes from 2-4 weeks

Question 13

Which immunoglobulin class provides long-term hep A immunity, and how soon after exposure is it produced?

Answer 13

IgG
From 5 weeks

Question 14

From when, and for how long, does hep A cause jaundice in an infected person?

Answer 14

From 4 weeks, potentially until 8 weeks, post-exposure

Question 15

What are the 2 markers of hep B infection that can first be identified in an infected person,

Answer 15

HbS antigen - infectivity or vaccinatin

and HbE antigen- infectivity

Question 16

Which hepatitis antigen is most infectious?

Answer 16

HbE antigen

Question 17

In a patient who successfully fights off Hep B, for how long are HbS and HbE present in serum?

Answer 17

HbS = 4 months 
HbE = 2 months

Question 18

what does anti-HBc or HBcAb mean in hep B

Answer 18

past or current hepatitis B infection.

core antibody does not provide any protection against the hepatitis B virus unlike the surface antibody

Question 19

What are the 3 key signs of portal hypertension?

Answer 19

Caput medusae
Splenomegaly
Ascites

Question 20

What sign may be indicative of liver failure?

Answer 20

Asterixis - flapping tremor

Question 21

If a patient has scratch marks, what does this tell you about their jaundice?

Answer 21

It must be post-hepatic, as itching is called by bile salts and bile acids

Question 22

What is courvoisier’s law?

Answer 22

If gallbladder is palpable but painless, it is almost always pancreatic cancer causing the jaundice

Question 23

give 5 genetic causes of cirrhosis

Answer 23

haemochromastosis
wilsons
A1AT def
galactosemia
glycogen storage disease

Question 24

what drug can cause cirrhosis

Answer 24

methotrexate

Question 25

the 3 main alcoholic liver diseases that cause cirrhosis

Answer 25

hepatic steatosis (fatty liver) alcoholic hepaitits alcoholic cirrhosis

Question 26

micronodular cirrhosis is seen in

Answer 26

alcoholic cirrhosis

Question 27

steatosis is seen in

Answer 27

fatty liver | (fully reversible if alcohol avoided)

Question 28

who is autoimmune hep more common in? what is the associated HLA

Answer 28

78% female HLA DR3 linked to the other autoimmune's

Question 29

2 types of autoimmune hep and the antibodies

Answer 29

type 1 = ANA (antinuclear Ig), anti SMA etc type 2 = anti LKM (liver, kidney, microsomal) Ig

Question 30

2 biliary causes of cirrhosis

Answer 30

PBC AND PSC

Question 31

outline the main differences between PBC & PSC

Answer 31

PBC = inflammation of intrahepatic bile ducts, PSC = intra & extra hepatic bile ducts, structure formation PBC = 10X more in F, PSC = M\> F PBC scan = no bile duct dilation, PSC = bile duct dilation PBC = treat w ursodeoxycholic acid, PSC = linked to IBD (UC) PSC - linked to chalngiocarcinoma, histology shows onion skinning fibrosis

Question 32

PSC increases risk of what cancer

Answer 32

cholangiocarcinoma

Question 33

histology of PBC vs PSC

Answer 33

``` PBC = bile duct loss w granulomas PSC = onion skinning (concentric) fibrosis ```

Question 34

are strictures are formed in PBS or PSC

Answer 34

strictures are in PSC, giving ending appearance of bile ducts

Question 35

outline 3 zones of the liver

Answer 35

zone 1 - closest to portal triad - peri portal hepatocytes zone 2 - mid zone zone 3- close to terminal hepatic vein, perivenular hepatocytes are most mature and metabolically enzyme. most liver enzymes.

Question 36

spotty necrosis on histopathology signifies

Answer 36

acute hepatitis (due to viruses a to e or drugs)

Question 37

piecemeal necrosis on histology indicates

Answer 37

chronic hepatitis

Question 38

is alcoholic hepatitis macro or micro nodular

Answer 38

micronodular

Question 39

give 3 macronodular causes of hepatitis

Answer 39

viral hepatitis Wilsons A1AT

Question 40

outline the 3 alcoholic liver diseases

Answer 40

1. hepatic steatosis - fat droplets in hepatocytes 2. alcoholic hepatitis - ballooning, mallory denk bodies 3. alcoholic cirrhosis - micro nodular fibrosis can reverse steatosis if alcohol avoided

Question 41

which hepatitis virus tends to cause chronic infection more commonly

Answer 41

HCV\> HBV

Question 42

4 histopathological signs of cirrhotic liver

Answer 42

hepatocyte necrosis fibrosis nodules of regenerating hepaatocytes disturbance of vascular architecture

Question 43

what score indicated prognosis in liver cirrhosis

Answer 43

modified Childs Pugh score higher score = worse under 7 = child Pugh A = HALF SURVIVE 5 YEARS 7-9 = child Pugh B = 20% SURVIVE 5 YEARS 10+ = child Pugh C = UNDER 20% SURVIVE 5 YEARS total out of 15, 3 points for each indicator

Question 44

what are the 5 components of the child Pugh score

Answer 44

albumin bilirubin PT ascites encephalopathy

Question 45

what are 3 genetic causes of cirrhosis & inheritance

Answer 45

haemachromatosis - AR Wilsons - AR A1AT def - AD

Question 46

what gene is affected in haemachromatosis

Answer 46

HFE gene

Question 47

what gene is affected in Wilsons

Answer 47

ATP7B on chromosome 13

Question 48

histolgoical finding of haemochromatosis

Answer 48

iron deposits in liver stain w Prussian blue

Question 49

main histo finding of Wilsons

Answer 49

copper stains w rhodaNine

Question 50

histolgoical findings of A1AT def & what stain used

Answer 50

intracytopalmsic inclusions of A1AT which stain with periodic acid Schiff

Question 51

3 main signs in haemochromatosis

Answer 51

skin bronzing (melanin deposition) diabetes hepatomegaly

Question 52

triad of Wilsons signs

Answer 52

liver disease neuro disease (basal ganglia involvement) Kayser flesicher rings

Question 53

presentation of A1AT in children vs adults

Answer 53

``` children = neonatal jaundice adults = emphysema and chronic liver disease ```

Question 54

3 iron results in haemochromatosis

Answer 54

iron and ferritin increased transferrin saturation over 45% TIBC decreased

Question 55

3 blood results in Wilsons

Answer 55

serum careuloplasmin decerased serum copper decreased urinary copper Increased

Question 56

2 results of investigations in A1AT def

Answer 56

A1AT decreased absent alpha globulin band on electrophoresis

Question 57

2 treatments for haemochromatosis

Answer 57

venesection desferrioxamine

Question 58

drug for Wilsons

Answer 58

life long penicillamine

Question 59

state 2 benign liver tumours? which is most common

Answer 59

``` haaemangioma (most common, no treatment needed) hepatic adenoma (linked to COCP) ```

Question 60

state 4 malignant liver tumours

Answer 60

hepatocellular carcinoma cholangiocarcinoma haemangiosarcoma hepatoblastoma

Question 61

what is deposited in the space of disse in liver injury

Answer 61

collagen

Question 62

what are liver macrophages called

Answer 62

kupffer cells

Question 63

biomarker for HCC?

Answer 63

alpha fetoprotein

Question 64

which malignant tumour tends to arise in those w chronic liver disease

Answer 64

HCC

Question 65

which malignant tumour presents as an abdominal mass, originating from immature liver precursor cells

Answer 65

hepatoblastma

Question 66

which malignant liver tumour is highly invasive

Answer 66

haemoangiosarcoma cancer of vascular epithelium

Question 67

what is the most common cause of malignant liver lesions

Answer 67

usually multiple secondary tumours from GIT, breast or bronchus

Question 68

transmission of hep A & hep E plus type of infection

Answer 68

BOTH = acute fAeco-oral - food (shellfish) or men or men sex hep A - contaminated water hep B- also uncooked pork

Question 69

treatment of hep A

Answer 69

after jaundice and fever symptoms IgG is produced so cured and immune

Question 70

hepatitis D requires co infection with

Answer 70

hepatitis B

Question 71

what antigens are typically measured in hep B? what do they signify

Answer 71

HBsAg - Hepatitis B surface antigen = infected HBeAg = infected & can spread HBsAb - indicates that a person is protected/immune against the hepatitis B virus througb vaccination or naturally. if vaccinated you'll have anti HBs but NOT HBe

Question 72

what % of hep B go on to be chronic compared to hep C

Answer 72

hep B only 10% go on to be chronic hep C up to 80% go on to be chronic

Question 73

HBV & HCV are associated w what liver cancer

Answer 73

HCC

Question 74

give a risk factor for HCV

Answer 74

thalsscemia due to recurrent transfusions transfusions/blood product spread are a MAJOR risk factor for HCV (much less so in HBV)

Question 75

what types of viruses are hep B & C

Answer 75

hep B - DNA hep C - RNA

Question 76

is hep B or hep C more commonly transmitted through unprotected sex

Answer 76

hep B more so

Question 77

give a drug that can cause chronic hep

Answer 77

methotrexate

Question 78

3 signs of portal hypertension

Answer 78

caput medusae ascites spelnomegaly

Question 79

4 signs of chronic stable (alcoholic) liver disease

Answer 79

palmar erythema gynaecomastia (liver can't break oestrdiol down) spider nave \> 5 duputryens contracture

Question 80

3 vitamin to give in alcoholic hep

Answer 80

B1 - thiamine - beri beri B3 -niacin - pellagra B12 - cobalamin - B12 def/SCDC

Question 81

what does courvoiseirs law state

Answer 81

if gallbladder is palpable in jaundiced patient, unlikely to be gallstones (ie probs pancreatic cancer which foot mets to liver)

Question 82

itching is a sign of what type of jaundice

Answer 82

obstructive