Liver conditions

Question 1

Non-alcoholic fatty liver disease risk factors

Answer 1

Obesity
HTN
T2DM
Hyperlipidaemia

Question 2

Metabolic syndrome Dx

Answer 2

Central obesity/increased waist circumference + 2 of:

• Raised triglycerides
• Lowered HDL
• HTN
• Hyperglycaemia

Question 3

Rx metabolic syndrome

Answer 3

Lose weight, control HTN, DM and lipids

Question 4

Causes of Budd-chiari syndrome

Answer 4

Hypercoaglable state - myeloproliferative disorder, anti-phospholipid, OCP
Local tumour - HCC
Congenital - membranous obstruction of IVC

Question 5

Ix in Budd chiari

Answer 5

Bloods, US+hepatic vein doppler

Ascitis tap - raised protein (>2.5g/dl)

Question 6

Rx Budd chiari

Answer 6

Anticoagulate, treat ascites, thrombolysis

Question 7

Hereditary haemochromatosis prevalence, age of onset, generics, pathophys

Answer 7

1/3000
40-60yrs, women later due to menses
Autosomal recessive
Increasing intestinal Fe absorption

Question 8

Clinical features in HH

Answer 8

MEALS:
Myocardial - dilated cardiomyopathy, arrhythmia
Endo - DM, pituitary (hypogonadism, amenorrhea, infertility), parathyroid (hypocalcaemia, osteoporosis), hyporenaemic hypoaldosteronism
Arthritis - 2/3rd MCP, knees + shoulders
Liver - cirrhosis, hepatomegaly
Skin - Slate grey discolourisation

Question 9

Ix in HH

Answer 9

Bloods - raised LFTS, ferritin, Fe, reduced TIBC

Liver biopsy - Pearl’s stain (quantifies iron loading and disease severity)

Question 10

Rx HH

Answer 10

Iron removal: 1. Vensection. 2. Desferrioxamine

Question 11

a1 antitrypsin deficiency prevalence, genetics and pathophys

Answer 11

1/4000
Autosomal recessive
a1AT inhibits neutrophil elastase

Question 12

Presentation of a1AT

Answer 12

hepatitis, cirrhosis, emphysema

Question 13

Ix in a1AT

Answer 13

Blood - reduced a1AT
Liver biopsy - PAS+ve
CXR + spirometry - emphysema + obstructive defect

Question 14

Mx a1AT

Answer 14

Support pulmonary and hepatic complications

Question 15

Wilson disease prevalence, presentation age, genetics, pathophys

Answer 15

3/100,000
Present between childhood - 30, psychotic teenager
Autosomal recessive
Impaired hepatocyte incorporation of Cu into caeruloplasmin + excretion into bile, accumulates in liver and late other organs

Question 16

Clinical features in Wilson

Answer 16

CLANK AH
Cornea - Kayser-Fleischer rings
Liver disease - Acute hepatitis, cirrhosis
Arthritis -chondrocalcinosis, osteoporosis
Neurology - PD, ataxia, depression, dementia, psychosis, spasticity
Kidney - Fanconi’s syndrome –> osteomalacia
Abortions
Haemolytic anaemia - Coom’s negative

Question 17

Ix in Wilson

Answer 17

Blood - low Cu and caerulopasmin

Question 18

Rx Wilson

Answer 18

Diet - avoid high Cu foods (liver, chocolate, nuts)
Penicillamine lifelong (Cu chelator) - SE: nausea, rash, low WCC/Hb/platelets, nephrosis, lupus

Question 19

Autoimmune hepatitis target group and classification

Answer 19

Young/middle aged women
T1 - Adult, SMA (80%), ANA+, raised IgG
T2 - young, LKM+
T3 - adult, SLA+

Question 20

Presentation of autoimmune hepatitis

Answer 20

Teens/early 20’s (25%):
Constituitional - fatigue, fever, malaise
Cushingoid - hirsuite, acne, striae
Hepatitis
Hepatosplenomegaly, amenorrhoea, polyarthritis, pleurisy
Post/peri menopausal
Chronic liver disease

Question 21

Associations to autoimmune hepatitis

Answer 21

Autoimmune conditions
PSC, UC
GN, AIHA

Question 22

Ix in autoimmune hepatitis

Answer 22

Liver biopsy

Question 23

Mx autoimmune hepatitis

Answer 23

Immunosuppression - pred, azathioprine

Liver transplant

Question 24

Primary biliary cirrhosis prevalence, sex, age, pathophys

Answer 24

4/100,000
F more than M = 9:1
Aged 50
Intrahepatic bile duct destruction 
M rule - IgM, Middle aged females, anti-Mitochondrial antibody

Question 25

Presentation in PBC

Answer 25

Jaundice late, pruritis, fatigue Face pigmentation Bones - osteoporosis, osteomalacia Hepatosplenomegaly Cirrhosis and coagulopathy (reduced vit K) Cholesterol raised - xanthelasma, xanthomata Steatorrhoea

Question 26

PBC associated diseases

Answer 26

Sjogrens (80%), RA, sclerderma | Thyroid disease, coeliac disease, RTA, membranous GN

Question 27

Ix in PBC biopsy, LFTs, US

Answer 27

LFTS - obstructive + liver function deranged AMA US - exclude extrhepatic cholestasis Liver biopsy - non-caseating granulomatous inflammation

Question 28

Rx PBC

Answer 28

``` Symptoms -Pruritis - colestyramine, naltrexone -Diarrhoea -codeine -Osteoporosis - bisphosphonates Specific - ADEK vitamins, urseodeoxycholic acid ```

Question 29

Primary sclerosing cholangitis pathophys, age and sex

Answer 29

Inflammation, fibrosis, strictures of intra+extra hepatic ducts Age 30-50 M more than F 2:1

Question 30

Symptoms and signs of PSC + complications

Answer 30

Jaundice, pruritis, fatigue Abdo pain Hepatosplenomegaly Complications - bacterial cholangitis, increased cholangiocarcinoma, colorectal carcinoma

Question 31

Associated disease to PSC

Answer 31

UC, AIH, HIV

Question 32

Ix in PSC

Answer 32

pANCA (80%) ERCP - beaded appearance of ducts Biopsy - fibrous, obliterative cholangitis

Question 33

Rx PSC

Answer 33

Symptoms: pruritis (cholestyramine), diarrhoea (codeine) | ADEK vitamins, ursedeoxycholic acid

Question 34

Liver tumour location of primary in men and women

Answer 34

Men - Stomach, lung, colon | Women - Stomach, breast, colon, uterus

Question 35

Benign liver tumours

Answer 35

Haemangioma- most common Adenoma Cyst

Question 36

Ix in liver tumours

Answer 36

US OR CT/MRI biopsy - diagnose | AFP raised in HCC

Question 37

HCC demographic and causes

Answer 37

China + Sub-saharan Africa | Viral hep, cirrhosis (alcohol, HH, PBC), aflatoxins (aspergillus)

Question 38

Cholangiocarinoma prevalence

Answer 38

10% of primary liver tumours | 90% of liver tumours are 2nd mets

Question 39

Causes of cholangiocarcinoma

Answer 39

Flukes PSC, UC Congenital biliary cysts

Question 40

Mx HCC and cholangiocarcinoma

Answer 40

Resect