Jaundice Flashcards
(230 cards)
1
Q
What is cholelithiasis?
A
Hard deposits/stones developing in gallbladder if bile has high levels of cholesterol/bilirubin or low levels of bile salts; gallbladder dysfunction or release of bile impaired
2
Q
What are the two main types of gallstones and how are they categorised?
A
1) Pigment (bilirubin) stones: Gallstones made of bilirubin
2) Cholesterol stones: Gallstones made of cholesterol
3
Q
In which condition are you most likely to see cholesterol stones?
A. Haemolytic disorders
B. Cirrhosis
C. Dyslipidemia
D. Down’s Syndrome
A
C. Dyslipidemia
4
Q
In which condition are you most likely to see cholesterol stones?
A. Haemolytic disorders
B. Cirrhosis
C. Insulin Resistance
D. Down’s Syndrome
A
C. Insulin Resistance
5
Q
In which condition are you most likely to see cholesterol stones?
A. Haemolytic disorders
B. Metabolic Syndrome
C. Cirrhosis
D. Down’s Syndrome
A
B. Metabolic Syndrome
6
Q
In which condition are you most likely to see pigment stones?
A. Metabolic Syndrome
B. Obesity
C. Haemolytic disorders
D. Dyslipidemia
A
C. Haemolytic disorders
7
Q
In which condition are you most likely to see pigment stones?
A. Metabolic Syndrome
B. Obesity
C. Cirrhosis
D. Dyslipidemia
A
C. Cirrhosis
8
Q
List the lifestyle + nutrition advice is there for prevention and reduction of gallstones.
A
Good diet: Regular meals/Micronutrient intake/ Fruits and vegetables
Physical activity: Glucose utilisation/HDL:LDL ratio/HDL increase
Maintain body weight: Stable weight/Progressive weight loss/Weight diet/High BMI/Rapid weight loss
9
Q
Outline 3 dietary habits which can help prevent gallstones.
A
1) Regular eating patterns
- Increase gallbladder emptying
2) Fibre + Calcium
- Reduce biliary hydrophobic bile acids
3) PUFA/MUFA (and nuts)
- May protect
4) Fruit and Vegetables
- May protect
5) Micronutrients: Vitamin C + Calcium
- Conversion of cholesterol to bile acids
10
Q
Outline the stages of Liver disease.
A
Hepatitis: Inflammation
Fibrosis (scarring)
Cirrhosis (spread + fibrosis)
Hepatocellular carcinoma
11
Q
List 3 common causes of Liver Disease.
A
Viral Hepatitis (B+C; A+E)
Chronic alcoholism
Obesity
Dyslipidemia
Genetics: LDL-/-; ApoE-/-
12
Q
Outline the lifestyle changes which can be made in NAFLD.
A
Reduce dietary intake 500-1000kcal/day
Micronutrient composition of diet: Vitamin A, B, C, D, E etc
Physical Activity: 150CME with x2 resistance
Drinks: Caffeine
13
Q
List 3 ways alcohol may increase the risk of malnutrition in an alcoholic patient.
A
Reduced food intake: Replacement + Altered appetite + Change behaviour (risky)
Digestion: Reduced secretion of pancreatic enzymes + bile
Absorption: Damaged mucosal lining + inhibited by nutritional deficiencies e.g. folate
Nutrient transport + assimilation: Reduced liver stores of vitamin A + increased excretion (fat stores)
Secondary malnutrition: Chronic pancreatitis + liver disease; infection and injury; ascites reduces appetite; REE increased in alcoholic hepatitis
14
Q
Why might sarcopenia occur in a patient with liver disease?
A
Lipid oxidation + reduced protein synthesis ≈ muscle atrophy ≈ susceptible to complications (ascites, infections, falls etc)
15
Q
How can you detect malnutrition in liver disease?
A
Malnutrition Universal Screening Tool (MUST) is a validated screening tool comprising of 5 steps which is scored from 0-6.
1) BMI Calculation
> 20 = 0
18.5-20 = 1
< 18.5 = 2
2) Unwanted weight loss in 3-6 months
5% = 0
5-10% = 1
> 10% = 2
3) Disease state
Acutely ill > 5 days = 2
4) Calculate Score
5) Stratification
0 = low risk
1 = medium risk
2 = high risk
16
Q
A patient comes and requires a MUST score generated. They are 184cm and weigh 80kg. In the last 4 month they have lost 5kg however they were on a weight-loss regime. In the last 4 days they have been acutely ill with D+V.
What is their score?
A
BMI > 20 = 0
Weight loss ≈ 6% however intended so scores 0
Acutely ill = 2
MUST score = 2
17
Q
A patient comes and requires a MUST score generated. They are 173cm and weigh 90kg. In the last 4 month they have lost 5kg however they were on a weight-loss regime. In the last 2 days they have been acutely ill with D+V.
What is their score?
A
BMI > 20 = 0
Weight loss ≈ 5% however intended so scores 0
Acutely ill = 2
MUST score = 2
18
Q
A patient comes and requires a MUST score generated. They are 173cm and weigh 90kg. In the last 4 month they have lost 5kg. In the last 2 days they have been acutely ill with D+V.
What is their score?
A
BMI > 20 = 0
Weight loss ≈ 5% thus scores 1
Acutely ill = 2
MUST score = 3
19
Q
List 3 common potential deficiencies in Alcoholic Liver Disease.
A
1) Vitamin B1 (Thiamine): Poor intake, Reduced conversion to co-enzyme, Reduced storage in fatty liver; Inhibited intestinal absorption; Increased metabolic demand (used in ethanol metabolism)
2) Folate and B12: Metabolism impaired ≈ megaloblastic anaemia
3) Niacin: Poor intake, Reduced conversion to co-enzyme, Reduced storage in fatty liver; Inhibited intestinal absorption
- Pellagra
4) Vitamin C: Poor intake, Reduced conversion to co-enzyme, Reduced storage in fatty liver; Inhibited intestinal absorption
- Scurvy
5) Vitamin A: Poor intake, Reduced conversion to co-enzyme, Reduced storage in fatty liver; Inhibited intestinal absorption
- Night blindness due to reduced levels of plasma retinol
6) Vitamin D: Poor intake, Reduced conversion to co-enzyme, Reduced storage in fatty liver; Inhibited intestinal absorption
- Rickets/Osteomalacia
- Osteoporosis
7) Calcium: Increased urinary excretion + Poor intake, Reduced conversion to co-enzyme, Reduced storage in fatty liver; Inhibited intestinal absorption
- Osteoporosis
- Hypocalcemia
8) Zinc: Poor intake, Reduced conversion to co-enzyme, Reduced storage in fatty liver; Inhibited intestinal absorption
- Impaired healing
- Liver regeneration retardation
- Mental status ∆
- Immune function
20
Q
What are the general functions of vitamin B1?
What conditions may be caused by deficiency of vitamin B1 (thiamine)?
A
Vitamin B1 is a water-soluble micronutrient which is phosphorylated in the gut to active coenzyme form
Vitamin B uses: - Cofactor: Pyruvate dehydrogenate, Alpha-ketoglutaric acid dehydrogenase - ATP production - Nerve conduction - Maintenance of neural membranes
Vitamin B1 deficiency = Beriberi Disease
21
Q
What is the difference between Dry and Wet Beriberi Disease?
A
Dry affects the Nerves cf Wet affects the Cardiovascular System
22
Q
List 5 potential causes of Beriberi disease.
A
Alcoholism Malnutrition Malabsorption Malignancy Diarrhoea Prolonged vomiting Diuretics
23
Q
What are the clinical features of Dry Beriberi syndrome?
A
Dry Beriberi
Symmetrical peripheral neuropathy (sensory + motor)
Progressive muscle wasting + paralysis
Confusion
24
Q
What are the clinical features of Wet Beriberi syndrome?
A
Wet Beriberi
High-output cardiac failure (dilated cardiomyopathy)
Oedema
Cardiomegaly
25
List 5 causes of Wernicke Encephalopathy.
```
Alcoholism
Malnutrition
Malabsorption
Malignancy
Diarrhoea
Prolonged vomiting
Diuretics
```
26
Which of the following is not a feature of Wernicke Encephalopathy.
A. Confusion
B. Ataxia
C. Nystagmus
D. Korsakoff amnestic state
D. Korsakoff amnestic state
27
Which of the following is not a feature of Wernicke Encephalopathy.
A. Confusion
B. Ataxia
C. Nystagmus
D. Confabulation
D. Confabulation
28
What are the clinical features of Wernicke-Korsakoff Syndrome?
```
Confusion
Ataxia
Nystagmus
Blunted or apathetic affect
Confabulation
Korsakoff amnestic state
```
29
How may alcohol cause cancer (mechanisms)?
- Occupy enzymes of metabolic pathway (ADH or LADH) elevating oestrogen
- Carcinogenic breakdown products (e.g. acetate)
- Oxidative stress: genotoxic reactive oxygen species
- Solvent: Easier for carcinogens to enter cells
- Deficiencies in essential nutrients: tissues susceptible to carcinogenic effect
30
List 3 ways to reduce calories when consuming alcohol.
- Non-alcoholic: Alcoholic Drink in 1:1 ratio
- Lighter versions of drinks
- Non-alcoholic drinks
- Drink water
- Sip
- Stealth drinking
31
Describe the weight gain paradox.
Weight Gain Paradox regarding Alcoholism where predicted would evoke weight gain but the opposite happens with chronic alcoholism
32
Which of the following is a phase I reaction in drug metabolism?
A. Glucuronidation
B. Sulfation
C. Methylation
D. Oxidation
D. Oxidation
33
Which of the following is a phase I reaction in drug metabolism?
A. Glucuronidation
B. Sulfation
C. Methylation
D. Reduction
D. Reduction
34
Which of the following is a phase I reaction in drug metabolism?
A. Glucuronidation
B. Sulfation
C. Methylation
D. Hydrolysis
D. Hydrolysis
35
Which of the following is a phase I reaction in drug metabolism?
A. Glucuronidation
B. Sulfation
C. Methylation
D. Isomerisation
D. Isomerisation
36
Which of the following is a phase I reaction in drug metabolism?
A. Glucuronidation
B. Sulfation
C. Hydrolysis
D. Methylation
C. Hydrolysis
37
Which of the following is a phase II reaction in drug metabolism?
A. Reduction
B. Sulfation
C. Hydrolysis
D. Oxidation
B. Sulfation
38
Which of the following is a phase II reaction in drug metabolism?
A. Reduction
B. Methylation
C. Hydrolysis
D. Oxidation
B. Methylation
39
Which of the following is a phase II reaction in drug metabolism?
A. Reduction
B. Acetylation
C. Hydrolysis
D. Oxidation
B. Acetylation
40
Which of the following is a phase II reaction in drug metabolism?
A. Reduction
B. Glucuronidation
C. Hydrolysis
D. Oxidation
B. Glucuronidation
41
Which of the following is a phase II reaction in drug metabolism?
A. Reduction
B. Glycosidation
C. Hydrolysis
D. Oxidation
B. Glycosidation
42
Which of the following best describes a phase I reaction in hepatic metabolism of drugs?
A. Functionalisation of the drug to create a less reactive product
B. Functionalisation of the drug to create a more stable product
C. Functionalisation of the drug to create a more reactive product
D. Conjugation of the drug to create a more reactive product
C. Functionalisation of the drug to create a more reactive product
43
Which of the following best describes a phase II reaction in hepatic metabolism of drugs?
A. Conjugation of the primary metabolite to create a stable product
B. Conjugation of the primary metabolite to create a more stable, less reactive product
C. Functionalisation of the drug to create a more reactive product
D. Conjugation of the drug to create a more reactive product
B. Conjugation of the primary metabolite to create a more stable, less reactive product
44
List 5 routes of drug elimination.
* Breath
* Saliva
* Hair
* Perspiration
* Bile
* Milk
* Urine
* Faeces
45
Which of the following is true?
A. The liver is a retroperitoneal organ
B. The liver is in the LUQ
C. The liver's quadrate lobe is on the lower aspect of the visceral surface
D. The liver's caudate lobe is on the lower aspect of the visceral surface
C. The liver's quadrate lobe is on the lower aspect of the visceral surface
46
Which of the following is true?
A. The liver is a retroperitoneal organ
B. The liver is in the LUQ
C. The liver spans ribs 6-11
D. The liver's caudate lobe is on the lower aspect of the visceral surface
C. The liver spans ribs 6-11
47
Which of the following is true?
A. The liver is a retroperitoneal organ
B. The liver is in the LUQ
C. The liver spans ribs 6-12
D. The liver's caudate lobe is on the upper aspect of the visceral surface
D. The liver's caudate lobe is on the upper aspect of the visceral surface
48
What is the correct order of blood flow through the liver?
A. Pericentral -> Midcentral -> Periportal
B. Midcentral -> Pericentral -> Periportal
C. Periportal -> Midcentral -> Pericentral
D. Periportal -> Pericentral -> Midcentral
C. Periportal -> Midcentral -> Pericentral
49
Which artery predominantly perfuses the liver?
A. Hepatic Artery Proper
B. Hepatic Portal Vein
C. Common Hepatic Artery
D. Gastroduodenal Artery
B. Hepatic Portal Vein
Hepatic portal vein (75%) ≈ supplies the liver with partially deoxygenated blood, carriage nutrients absorbed from the small intestine. This is the dominant blood supply to the liver parenchyma and allows the liver to perform its functions e.g. detoxifications
50
The hepatic artery branches from an anterior division of the abdominal aorta which is at the vertebral level...
A. T10
B. T12
C. L1
D. L3
B. T12
51
What is the correct order of venous drainage from the liver?
A. Portal venules -> Central Vein -> Hepatic vein -> Interlobular vein -> IVC
B. Portal venules -> Central Vein -> Interlobular vein -> Hepatic vein -> IVC
C. Portal venules -> Hepatic vein -> Interlobular vein -> Central Vein -> IVC
D. Central Vein -> Portal venules -> Hepatic vein -> Interlobular vein -> IVC
B. Portal venules -> Central Vein -> Interlobular vein -> Hepatic vein -> IVC
52
Which of following liver cells is predominantly responsible for protein production and metabolism?
A. Progenitor Cells
B. Liver Sinusoidal Endothelial Cells
C. Hepatic Stellate Cells
D. Hepatocytes
D. Hepatocytes
53
Which of following liver cells is predominantly responsible for liver regeneration?
A. Progenitor Cells
B. Liver Sinusoidal Endothelial Cells
C. Hepatic Stellate Cells
D. Hepatocytes
A. Progenitor Cells
54
Which of following liver cells is predominantly responsible for liver regeneration by production of HGF?
A. Progenitor Cells
B. Liver Sinusoidal Endothelial Cells
C. Hepatic Stellate Cells
D. Hepatocytes
A. Progenitor Cells
55
Which of following liver cells is predominantly responsible for liver regeneration by differentiation into LSECs?
A. Progenitor Cells
B. Liver Sinusoidal Endothelial Cells
C. Hepatic Stellate Cells
D. Hepatocytes
A. Progenitor Cells
56
Which of following liver cells is predominantly responsible for liver regeneration by regeneration of cells composing liver parenchyma?
A. Progenitor Cells
B. Liver Sinusoidal Endothelial Cells
C. Hepatic Stellate Cells
D. Hepatocytes
D. Hepatocytes
57
Which of following liver cells is predominantly responsible for pathogen detection and filtration in the space of Disse?
A. Progenitor Cells
B. Liver Sinusoidal Endothelial Cells
C. Hepatic Stellate Cells
D. Hepatocytes
B. Liver Sinusoidal Endothelial Cells
58
Which of following liver cells is predominantly responsible for pathogen detection and phagocytosis?
A. Kupffer Cells
B. Liver Sinusoidal Endothelial Cells
C. Hepatic Stellate Cells
D. Hepatocytes
A. Kupffer Cells
59
Which of following liver cells is predominantly responsible for immune surveillance and cytotoxic killing of viral and IC bacterial infections?
A. Kupffer Cells
B. Liver Sinusoidal Endothelial Cells
C. Natural Killer Cells
D. Hepatocytes
C. Natural Killer Cells
60
Give 5 functions of the Liver.
1. Protein Metabolism: Albumin/Clotting Factors/ Complement/a1-antitrypsin/thrombopoeitin/Protein C/Protein S/IGF-1
2. Fat Metabolism: Cl/FA/LDL/HDL/Ketogenesis/Steroid hormones
3. Carbohydrate Metabolism: Fed state (glycolysis/glycogen synthesis) vs Fasting state (glycogenolysis/gluconeogenesis/ß-oxidation of FAs)
4. Bile secretion: Production of bile salts + Bilirubin elimination
5. Storage: Glycogen/Vitamin A/Vitamin B12
6. Biotransformation and detoxification: Drugs (I + II)/Gonadal hormones/Aldosterone/Glucocorticoids/Nitrogenous gut toxins
7. Protection (immune-surveillance): Kuppfer cells
8. Haematopoiesis: Foetal liver
9. Natural regeneration of liver tissue: Injury VEGF production + BM-SPCs HGF ≈ Hepatocyte and SPCs replace LSECs in canal of Heron
61
Which of the following processes is most responsible for mediating the mechanism of liver regeneration?
A. Injury
B. VEGF production by hepatocytes
C. Production of HGF
D. LSEC replacement
C. Production of HGF
62
Outline the process of bile release.
• Bile released (bile salts/PL/Cl) at hepatocyte apical membrane into bile canaliculi -> R+L hepatic duct -> Common Hepatic Duct -> GB for storage -> Bile Duct -> HPAV -> Duodenum
63
Outline the process of endogenous lipid metabolism in the Liver.
Endogenous pathway: Ingestion -> Digestion + Absorption -> Insulin (AKT pathway ≈ SREBP-1c upregulated) -> Lipogenesis
64
Which of the following is false?
A. Dietary fats are absorbed with pancreatic cholesteryl ester hydrolase producing free Cholesterol
B. Chylomicrons are hydrolysed at the brush border by LPL to produce a CM remnant with a B100 apolipoprotein
C. VLDL remnants are hydrolysed by LPL to produce IDL
D. LDL is an end-stage product produced by hydrolysis of IDL by LPL
B. Chylomicrons are hydrolysed at the brush border by LPL to produce a CM remnant with a B100 apolipoprotein
65
Which of the following is false?
A. Bilirubin is directly produced from the reduction of biliverdin by biliverdin reductase
B. Haem is a breakdown product of Haemoglobin
C. Bilirubin monoglucuronide is produced by the conjugation of glucuronic acid via Glucuronysyltransferase
D. Bilirubin is predominantly absorbed (50%)
D. Bilirubin is predominantly absorbed (50%)
False, Bilirubin is predominantly excreted as stercobilin and urobilin
66
Which of the following is false?
A. Haem is metabolised by haem oxygenase to biliverdin
B. Unconjugated bilirubin is soluble thus can travel freely in the plasma
C. Conjugated bilirubin is insoluble thus must be bound to albumin
D. Bilirubin monoglucuronide is degraded by instestinal bacteria to urobilinogen
C. Conjugated bilirubin is insoluble thus must be bound to albumin
67
Which of the following is false?
A. Unconjugated bilirubin is insoluble thus travels in the plasma bound to albumin
B. Unconjugated bilirubin is conjugated with glucuronic acid via UDPGT in the liver
C. Urobilinogen may be absorbed across the enterocyte brush border into the bloodstream
D. Urobilin is responsible for making faeces brown
D. Urobilin is responsible for making faeces brown
68
Which of the following is false?
A. Bilirubin is recirculated via enterohepatic circulation
B. Urobilinogen is present in the LI
C. Urobilin gives urine a yellow pigment
D. Stercobilinogen is the brown pigment in faeces
D. Stercobilinogen is the brown pigment in faeces
False, Stercobilin is the brown pigment in faeces
69
Which of the following is false?
A. Post-hepatic jaundice leads to a reduction in colour of the stools (acholic stools)
B. Pre-hepatic jaundice leads to changes in urine colour
C. Intra-hepatic jaundice may involve dark urine (haemoglobinuria)
D. Urobilin is excreted in urine
B. Pre-hepatic jaundice leads to changes in urine colour
70
Mr. Jonnes, a 45 year old middle aged, Caucasian male, presented with fatigue. He says he has been tired for the past 4 months but didn't want to bother the busy, hard working NHS doctors. He says he has had some joint pain but other than that he is well. He mentions he has had some relationship difficulties due to his loss of libido and impotence.
O/E you notice mild hepatomegaly and slate-grey patches on his face and neck.
i) What are the notable pieces of information from Mr. Jonnes' history?
ii) What would you want to know?
iii) What investigations may you run?
His tests come back and you identify an elevated serum transferrin and raised ferritin.
iv) What test may you want to consider now?
v) What is your DDx should the test in iv) come back positive?
vi) Outline your treatment for this patient.
i)
- 45 (advanced age/ middle aged)
- Caucasian
- Male
- 4 months (longer than 3)
- Lack of desire to see a doctor, serious?
- Arthralgia
- Impotence
- Loss of libido
- Hepatomegaly
- Slate-Grey patches (Haemochromatosis)
ii)
- PMHx
- DHx: OTC/Recreational
- FHx
- SHx: Alcohol/Drugs/Risky behaviour/Travel to endemic areas
- SE: General health?
iii) Blood tests
- LFTs: Aminotransferases raised
- Serum transferrin: Raised
- Serum ferritin: Raised
iv) Genotype for HFE mutation (C282Y ∆)
v) Haemochromatosis (1º)
vi)
• Observe
• Lifestyle: Avoid Iron supplements/Avoid Vitamin C supplements/Avoid excess alcohol
• Phlebotomy
• Desferrioxamine: 20-60mg/kg/day (iron-chelation)
71
What is the pharmacological treatment for Haemochromatosis?
A. Ferritin replacement
B. Desferrioxamine
C. Phlebotomy
D. None of the above
B. Desferrioxamine
72
What is the best way to determine 1º Haemochromatosis? For the correct choice, what would you expect to see (most likely)?
A. Serum transferrin
B. Serum ferritin
C. HFE mutation analysis
D. LFTs
C. HFE mutation analysis for C282Y
For C282Y
73
Mr. Cassan, a 45 year old Middle Eastern male, presents with sudden pallor, jaundice and fatigue. He says his urine has gone dark and the yellowing gets worse in the cold. He says this has never happened but since he had an infection of his kidneys, he has experienced this. He doesn't know why and wonders if it could have spread elsewhere? He says he took a course of antibiotics which helped the kidney infection resolve. He eats a well balanced diet but has recently enjoyed snacking on a variety of green bean, he likens to peas but says they are native to his homeland.
O/E you observe icterus in the cornea, mucosal linings and skin as well as splenomegaly.
i) What are the notable pieces of information from Mr. Cassan's history?
ii) What would you want to know?
iii) What investigations may you run?
His tests come back and you identify an elevated LDH, Unconjugated bilirubin, and MCHC raised with Low Hb.
iv) What is your DDx?
vi) Outline your treatment for this patient.
i)
- Middle aged
- Ethnic: Middle Eastern
- Male
- Cephalosporins? - Treatment for Pyelonephritis
- Fava Beans
- Recent infection - autoimmune?
- Hepatomegaly/Splenomegaly
- Jaundice
- Pallor
ii)
- PMHx
- DHx: OTC/Recreational
- FHx
- SHx: Alcohol/Drugs/Stress
- SE: General health?
iii)
1. Bloods:
• FBC: Low Hb -> Anaemia
• MCHC: Increased (spherocytes/reticulocytes)
• Reticulocyte count: Increased
• Peripheral blood smear: Schistocytes/Spherocytes/Elliptocytes/Spur cells/ Blister cells/ Bite cells/ Tear drops
• LDH: Raised
2. LFTs:
• Unconjugated (indirect) bilirubin: Elevated
iv) Haemolytic Anaemia
v)
• Supportive care + Disease-specific specialist care
• Remove offending agent/Corticosteroid/Splenectomy
74
Which of the following is the most accurate indicator of haemolytic anaemia?
A. Low Hb
B. Low Hb and Schistocytes/Elliptocytes
C. Unconjugated bilirubin raised
D. Low haptoglobin
B. Low Hb and Schistocytes/Elliptocytes
75
Which of the following is not a risk factor for Haemolytic Anaemia?
A. Middle-Eastern origin
B. Sickle-Cell Anaemia
C. Autoimmune Disease
D. Scandanavian Origin
D. Scandanavian Origin
76
Which of the following is not a risk factor for Haemolytic Anaemia?
A. Advanced age
B. Cephalosporin use
C. Fava Beans
D. Chinese Origin
A. Advanced age
77
Which of the following diseases results form a mutation to ∆AAT resulting in reduced protease activity with implications on Pulmonary, Hepatic and GI function?
A. Gilbert Syndrome
B. Crigler-Najjar Syndrome
C. Alpha-1 Antitrypsin Deficiency
D. Dubin-Johnson Syndrome
C. Alpha-1 Antitrypsin Deficiency
78
Which of the following is not a feature of AAT deficiency?
A. Productive cough
B. SOBE
C. Hepatomegaly
D. Increased breath sounds
D. Increased breath sounds
79
Which investigation would reveal AAT1 deficiency best?
A. CXR: Hyperinflation
B. CT-Chest: Panacinar emphysema
C. Plasma AAT1 level: Reduced
D. PFT: FEV1 reduced and FEV1/FEVC reduced
C. Plasma AAT1 level: Reduced
80
Which of the following is not a risk factor for NAFLD?
A. Obesity
B. Metabolic Syndrome
C. Hypertension
D. Physical Inactivity
D. Physical Inactivity
81
Which of the following pathological changes involves lipid droplets and hepatocyte depletion?
A. Cirrhosis
B. Fibrosis
C. Steatosis
D. Steatohepatitis
C. Steatosis
82
Which of the following pathological changes involves lipid droplets and collagen deposition without spreading?
A. Cirrhosis
B. Fibrosis
C. Steatosis
D. Steatohepatitis
D. Steatohepatitis
83
Which of the following pathological changes involves increased collagen deposition without regenerative liver nodules?
A. Cirrhosis
B. Fibrosis
C. Steatosis
D. Steatohepatitis
B. Fibrosis
84
Which of the following pathological changes involves increased collagen deposition with regenerative liver nodules?
A. Cirrhosis
B. Fibrosis
C. Steatosis
D. Steatohepatitis
A. Cirrhosis
85
Which stain can be used to detect collagen deposition in a liver biopsy of a patient with NAFLD?
A. H+E
B. Oil Red O
C. PAS
D. Masson's Trichrome
D. Masson's Trichrome
86
Which stain can be used to detect fat deposition in a liver biopsy of a patient with NAFLD?
A. H+E
B. Oil Red O
C. PAS
D. Masson's Trichrome
B. Oil Red O
87
Which stain can be used to detect cell morphology in a liver biopsy of a patient with NAFLD?
A. H+E
B. Oil Red O
C. PAS
D. Masson's Trichrome
A. H+E
88
Which are the main clinical features, specific to NAFLD?
A. Fatigue/Obesity/HSM/RUQ discomfort/Jaundice
B. Obesity/HSM/RUQ discomfort
C. Obesity/Jaundice/RUQ discomfort/Pruritus
D. Obesity/Fatigue/Palmar Erythema/Ascites/RUQ discomfort
B. Obesity/HSM/RUQ discomfort
89
List the investigations you may wish to conduct in a patient with suspected NAFLD. For each, say what you may expect to see.
* LFTs: AST/ALT/ALP/GGT raised*
* Bilirubin: Elevated
* FBC: Anaemia/Thrombocytopenia
* U+E: Hyponatremia (in Cirrhosis)
* Lipid panel: LDL raised/Elevated Cl/Low HDL*
* PT time and INR: Elevated
* Serum albumin: Decreased
* US-Liver: Abnormal echotexture*
* MRI-Abdomen: Increased liver fat content
* CT-Abdomen: Low attenuation liver
* Liver Biopsy: Steatohepatitis/Fibrosis/Cirrhosis/HCC
* Elastography: increased stiffness
90
Which management plan is best for moderate NAFLD?
A. Lifestyle change + Vitamin E + Weight loss pharmacotherapy + Metformin + Statins
B. Liver transplantation
C. Vitamin E + Weight loss pharmacotherapy + Metformin + Statins
D. Lifestyle change + Vitamin E + Metformin + Statins
A. Lifestyle change + Vitamin E + Weight loss pharmacotherapy + Metformin + Statins
91
A patient presents with abdominal distension, fibrosis, jaundice, haematemesis and xanthelasma. He has previously had recurring bouts of NAFLD. In his recent investigations, he had deranged LFTs, he was thrombocytopenic and had HBV+ Abs. Additionally, the US-Abdomen showed nodularity.
What is the most likely diagnosis?
A. Steatohepatitis
B. Hepatocellular carcinoma
C. Liver fibrosis
D. Liver Cirrhosis
D. Liver Cirrhosis
92
What is the primary therapeutic treatment for a patient with Liver Cirrhosis?
A. Pentoxylphelline
B. Plasmapheresis
C. Liver transplant
D. Liver resection
C. Liver transplant
93
What is the pathophysiology of Wilson's Disease?
A. ∆ATP7A gene causing ∆ copper-transporting ATPase ≈ ∆ ATP7A protein ≈ reduced copper export ≈ accumulation
B. ∆ATP7B gene causing ∆ copper-transporting ATPase ≈ ∆ ATP7B protein ≈ reduced copper export ≈ accumulation
C. ∆ATP7B gene causing ∆ copper-transporting ATPase ≈ ∆ ATP7B protein ≈ increased copper export ≈ reduction
D. ∆ATP7A gene causing ∆ copper-transporting ATPase ≈ ∆ ATP7A protein ≈ increased copper export ≈ reduction
B. ∆ATP7B gene causing ∆ copper-transporting ATPase ≈ ∆ ATP7B protein ≈ reduced copper export ≈ accumulation
94
List 5 clinical features of Wilson's Disease.
* Tremor: Fine/Intentional/Wing-Beating
* Dysarthria: Slurred or hypokinetic
* Dystonia: upper extremities
* Dyspraxia
* Ataxia
* Dystonic syndrome
* Micrographia
* Dysdiadochokinesis
* Abnormal extraocular movements: Esotropia/Saccadic/Diplopia
* Keyser-Fleischer rings: Copper deposits in Descemet’s membrane
* Sunflower cataracts: Copper deposits affecting lens
* Behavioural change: Impulsive/Sexual exhibitionism/Decline in academic perofrmace
* Psychosis
* Depression
* Jaundice
* Ascites
* Liver tenderness
* Spider naevus
* Peripheral oedema
* Bruising
* Encephalopathy
95
Which ophthalmological sign is indicative of Wilson's Disease?
A. Conjunctival palor
B. Corneal arcus
C. Keyser-Fleischer rings
D. Glaucoma
C. Keyser-Fleischer rings
96
Which ophthalmological sign is indicative of Wilson's Disease?
A. Conjunctival palor
B. Corneal arcus
C. Sunflower cataracts
D. Glaucoma
C. Sunflower cataracts
97
Which test result is most indicative of Wilson's Disease?
A. Serum caeruloplasmin decreased and Copper Raised
B. Copper Raised
C. Copper reduced and Serum Caeruloplasmin stable
D. None of the above
A. Serum caeruloplasmin decreased and Copper Raised
98
What is the pharmacological management of Wilson's Disease?
A. Desferrioxamine
B. Zinc supplements
C. Liver transplant
D. Pentoxylphelline
B. Zinc supplements
99
Which of the following most strongly suggests Hepatocellular Carcinoma?
A. FBC: Microcytic Anaemia
B. U+E: Hyponatremia
C. Viral hepatitis panel: Positive
D. Alpha Fetoprotein (AFP)
D. Alpha Fetoprotein (AFP)
100
What is the transmission of HAV?
A. Percutaneous
B. Droplet
C. Faeco-oral
D. Cutaneous
C. Faeco-oral
101
List 5 clinical features of Hepatitis A Virus.
* Fever
* Malaise
* Nausea and Vomiting
* Jaundice
* Acholic stools
* Hepatomegaly
* RUQ pain
* Splenomegaly
102
List the investigations you would order and expected results for a patient with HAV.
* LFTs: ALT/ALP/AST/GGT raised
* Bilirubin: Elevated
* Blood urea: Elevated in fulminant hepatitis
* Serum creatinine: Elevated above 177micromol/L
* PT time: Prolonged
* Serology: IgM anti-hepatitis A virus positive
* NAAT: Hepatitis A virus RNA detection
103
Which investigation and result will most likely determine a patient's HAV status?
A. LFTs: ALT/AST/ALP/GGT
B. Serum creatinine: Elevated
C. PT: Prolonged
D. NAAT: Hepatitis A Virus RNA Detection
D. NAAT: Hepatitis A Virus RNA Detection
104
Which investigation and result will most likely determine a patient's HAV status?
A. LFTs: ALT/AST/ALP/GGT
B. Serum creatinine: Elevated
C. PT: Prolonged
D. Serology: IgM anti-hepatitis A virus positive
D. Serology: IgM anti-hepatitis A virus positive
105
How would you manage a patient with a positive case of HAV?
A. Lifestyle change: Dont go to endemic areas
B. Supportive care
C. HAV vaccine: 0.02ml/kg IM
D. HAV vaccine: 0.2mk/kg IM
C. HAV vaccine: 0.02ml/kg IM
106
What is the transmission of HBV?
A. Parenteral
B. Sexual
C. Faeco-oral
D. Vertical
C. Faeco-oral
107
What is the transmission of HBV?
A. Vertical
B. Droplet
C. Faeco-oral
D. Cutaneous
A. Vertical
108
List 3 risk factors for HBV.
* Perinatal exposure
* High-risk sexual behaviours
* IVDU
* Male sex
109
List 5 clinical features of HBV.
• Asymptomatic
* RUQ pain
* Jaundice
* Hepatomegaly
* Ascites
* Fever/chills
* Malaise
* Maculopapular/urticarial rash (Sickness-like syndrome)
* Nausea and vomiting
* Arthralgia/arthritis
* Palmar erythema
* Spider naevi
* Splenomegaly
* Asterixis
110
List the investigations for a suspected case of HBV and what you might expect to see.
* LFTs: Elevated ALT/elevated AST/elevated ALP/ elevated bilirubin/Low albumin
* FBC: Microcytic anaemia ± thrombocytopenia
* U+E: Hyponatremia/Uremia
* Coagulation: Normal/Elevated
* Serology: HBV antibody
* PCR amplification assay: HBV DNA
* US-Abdomen: Poorly defined margins and coarse, irregular internal echoes
* CT/MRI-Abdomen: Hypervascular pattern (CT); High intensity pattern on T2; low-intensity on T1
* Liver Biopsy: Inflammation Fibrosis Cirrhosis
111
Which investigation and result will most likely determine a patient's HBV status?
A. LFTs: ALT/AST/ALP/GGT
B. Serum creatinine: Elevated
C. PT: Prolonged
D. Serology: IgM anti-hepatitis B virus positive
D. Serology: IgM anti-hepatitis B virus positive
112
Which investigation and result will most likely determine a patient's HBV status?
A. LFTs: ALT/AST/ALP/GGT
B. Serum creatinine: Elevated
C. PT: Prolonged
D. PCR amplification assay HBV
D. PCR amplification assay HBV
113
What is the most accurate management for HBV?
A. Supportive care
B. Entecavir + Supportive care
C. Entecavir + HBV Vaccine
D. Supportive care + Entercavir + HBV Vaccine
D. Supportive care + Entercavir + HBV Vaccine
114
List 3 risk factors for Hepatitis C.
* Unsafe medical practices
* IV
* Intranasal drug use
* History of blood transfusion or organ transplant
115
What is the transmission of HCV?
A. Parenteral
B. Droplet
C. Faeco-oral
D. Cutaneous
A. Parenteral
116
Outline the clinical features of Hepatitis C.
* Fatigue
* Myalgia
* Jaundice
* Ascites
* Hepatic encephalopathy confusion/ altered consciousness/coma
* Extrahepatic complications: Vasculitis/Skin manifestations
117
Which investigation most correctly identifies the likelihood of HCV in a patient?
A. LFTs
B. Liver biopsy
C. US
D. Serology
D. Serology
118
What is the management for HCV?
A. Entecavir
B. Vaccine
C. Elbasvir/Grazoprevir
D. Education
C. Elbasvir/Grazoprevir
119
What is the transmission of HDV?
A. Parenteral
B. Droplet
C. Faeco-oral
D. Cutaneous
A. Parenteral
120
With which other hepatitis virus is HDV always present?
A. HEV
B. HCV
C. HAV
D. HBV
D. HBV
121
Give 3 risk factors of HDV.
* Unsafe medical practices
* IV
* Intranasal drug use
* History of blood transfusion or organ transplant
* Current HBV infection
122
List the clinical features of HDV.
* Abdominal pain/swelling
* Jaundice
* Pruritus
* Haematemesis
* Melaena
* Confusion
* Lethargy
* Weight loss
* Weakness
* Bruising
123
Which investigation is most likely to show a positive case of HDV?
A. LFTs
B. FBC
C. US-Abdomen
D. Serology: HDAg-IgM
D. Serology: HDAg-IgM
124
What is the management of HDV?
A. HBV vaccine
B. Tenofovir/Entecavir
C. Entecavir + HBV vaccine
D. Tenofovir/Entecavir + HBV vaccine
D. Tenofovir/Entecavir + HBV vaccine
125
What is the transmission of HEV?
A. Vertical
B. Droplet
C. Faeco-oral
D. Cutaneous
C. Faeco-oral
126
List 3 risk factors of HEV.
* Never contracted HEV
* Poor sanitation
* Ingestion of undercooked meat and shellfish
* Contamination of water
* Travel to an endemic area
127
Outline the clinical features of HEV.
* Abdominal pain
* Tender
* Hepatosplenomegaly
* Lymphadenopathy
* Jaundice
* Ascites
* Encephalopathy (memory/attention/confusion/Asterixis/nystagmus/clonus/rigidity/coma)
128
Which investigation is most likely to show HEV?
A. LFTs: Elevated AST/ALT/Bilirubin elevated
B. PT time: Increased possibly
C. US-abdomen: Hepatitis observed
D. Serology: Anti-HEV IgM (active) or Anti-HEV IgG (past) + PCR
D. Serology: Anti-HEV IgM (active) or Anti-HEV IgG (past) + PCR
129
What i the best treatment for HEV?
A. Vaccine
B. Entecavir
C. Elbasavir + Grazoprevir
D. Supportive
D. Supportive
There is no Vaccine for Hepatitis E, it usually self-resolves
130
What is HAV present in?
A. Blood
B. Stool
C. Air
D. Animals
B. Stool
Contaminated food, water or other transmission of faeco-oral matter
131
What is HBV present in?
A. Blood
B. Stool
C. Air
D. Animals
A. Blood
132
What is HCV present in?
A. Blood
B. Stool
C. Air
D. Animals
A. Blood
133
What is HDV present in?
A. Blood
B. Stool
C. Air
D. Animals
A. Blood
134
What is HEV present in?
A. Blood
B. Stool
C. Air
D. Animals
B. Stool
135
Which virus is most likely to give chronic infection on its own?
A. HAV
B. HBV
C. HCV
D. HDV
C. HCV
80%
136
Which virus is most likely to give chronic infection?
A. HAV
B. HBV
C. HCV
D. HDV
D. HDV
85% (if superinfection together with hepatitis B virus)
137
Which virus is most likely transmitted by sexual contact?
A. HAV
B. HBV
C. HCV
D. HDV
B. HBV
138
Which virus is most likely transmitted by vertical transmission?
A. HAV
B. HBV
C. HCV
D. HDV
B. HBV
139
Which virus is can be, but is unlikely, transmitted by vertical transmission?
A. HAV
B. HBV
C. HCV
D. HDV
C. HCV
140
Which hepatitis virus causes deranged LFTs?
A. HBV
B. HAV
C. None
D. All
D. All
141
Which of the following viruses cannot be prevented by vaccine?
A. HAV
B. HBV
C. HCV
D. HDV
C. HCV
142
Which of the following viruses is a DNA virus?
A. HAV
B. HBV
C. HCV
D. HDV
B. HBV
143
Which of the following is true for type 1 Autoimmune Hepatitis?
A. Anti-LKM-1 antibodies are present
B. Anti-LC1 antibodies are present
C. AMA antibodies are present
D. It happens following an exposure to environmental triggering agents
C. AMA antibodies are present
144
Which of the following is true for type 1 Autoimmune Hepatitis?
A. Anti-LKM-1 antibodies are present
B. Anti-LC1 antibodies are present
C. SMA antibodies are present
D. It happens following an exposure to environmental triggering agents
C. SMA antibodies are present
145
Which of the following is true for type 2 Autoimmune Hepatitis?
A. You may have a genetic predisposition to it
B. Anti-LC1 antibodies are present
C. SMA antibodies are present
D. It happens following an exposure to environmental triggering agents
B. Anti-LC1 antibodies are present
146
Which of the following is true for type 2 Autoimmune Hepatitis?
A. You may have a genetic predisposition to it
B. Anti-LKM antibodies are present
C. SMA antibodies are present
D. It happens following an exposure to environmental triggering agents
B. Anti-LKM antibodies are present
147
List the key clinical features of autoimmune hepatitis.
* Fatigue
* Malaise
* Nausea
* Fever
* Anorexia
* Abdominal discomfort
* Hepatomegaly*
* Jaundice*
* Pruritus
* Spider naevi*
148
Which would be best for the management of autoimmune hepatitis without primary biliary cirrhosis?
A. Entacevir
B. Elbasavir/Grazoprevir
C. Prednisolone ± Azathioprine
D. Prednisolone
D. Prednisolone
149
Which would be best for the management of autoimmune hepatitis with primary biliary cirrhosis?
A. Entacevir
B. Elbasavir/Grazoprevir
C. Prednisolone ± Azathioprine
D. Prednisolone
C. Prednisolone ± Azathioprine
150
Of the following risk factors, which is the primary driver for alcoholic liver disease?
A. Female sex
B. Hepatitis C
C. Prolonged, heavy alcohol consumption
C. Prolonged, heavy alcohol consumption
151
List the clinical features of Alcoholic Liver Disease.
* Abdominal pain
* Hepatomegaly
* Ascites
* Weight loss
* Anorexia
* Fatigue
* Weight gain
* Malnutrition/wasting
* Thenar eminence atrophy
* Jaundice
* Palmar erythema
* Spider naevi
* Cutaneous telangiectasia
* Asterixis
* Hematemesis
152
Outline the management for treatment of alcoholic liver disease if a patient was dependent on alcohol,
```
• Alcohol abstinence and withdrawal: Diazepam 10mg IV/Oxazepam: 15-30mg PO TDS-QDS
+
• Weight reduction
+
• Smoking cessation
+
• Immunisation: HBV and HAV
+
• Prednisolone: 40mg PO OD
+
• Sodium restriction + Furosemide: 40-160mg PO OD and Spironolactone: 100-400mg PO OD
+
• Pentoxifylline (haemorrheologic): 400mg PO TDS
```
153
Outline the risk factors for a patient developing gallstones in choledocholithiasis.
```
RF: 6Fs
• Female
• Fat
• Fair
• Fourty
• Fertile
• FH
```
154
Outline the pathophysiology of Choledocholithiasis.
Increased bile salts + Ca++ + Cholesterol -> formation of gallstones passing into and occluding CBD --> reduced excretion of bilirubin (= jaundice) + distension of CBD (= biliary colic)
155
Outline the clinical features of Choledocholithiasis.
* Nausea
* Vomiting
* RUQ pain
* Fever (cholecystitis)
* Jaundice
* Pruritus
* Dark urine (haemoglobinuria)
* Acholic stools
156
Which of the following is not a direct clinical feature of choledocholithiasis?
A. RUQ pain
B. Jaundice
C. Acholic stools
D. Fever
D. Fever (cholecystitis)
157
Which of the following deranged LFT gives the indication that the stone is affecting the bile duct?
A. AST
B. ASP
C. Clotting factors
D. GGT
D. GGT
158
Which of the following deranged LFT gives the indication that the stone is affecting the bile duct?
A. AST
B. ASP
C. Clotting factors
D. ALP
D. ALP
159
Outline the investigations used in suspected choledocholithiasis.
* LFTs: High direct bilirubin/GGT raised/ALP raised
* PT time: Normal
* FBC: Leukocytosis
* US-Abdominal: Intra and extrahepatic biliary tree dilation ± stones in CBD
* CT-Abdomen: Stones in bile duct
* ERCP/MRCP
160
Which of the following should not be used as an investigation for choledocholithiasis?
A. LFTs
B. US-Abdomen
C. MRCP
D. ERCP
D. ERCP
Interventional procedure
161
Outline the management of a patient with choledocholithiasis,
• ERCP
+
• Papillary balloon dilation
• Biliary stenting
162
What is cholelithiasis?
Bile calculi in the gallbladder
163
Outline the risk factors for cholelithiasis.
```
RF: 6Fs
• Female
• Fat
• Fair
• Fourty
• Fertile
• FH
```
164
What is the management of a patient with cholelithiasis?
A. ERCP
B. Chemotherapy ± Radiotherapy
C. Cholecystectomy
D. Morphine only
C. Cholecystectomy
165
What is a cholangiocarcinoma?
Cancer of the bile duct epithelium which can occur anywhere within the biliary system (intra- and extra-hepatic)
166
List 3 risk factors of cholangiocarcinoma?
* Advanced age > 50 years
* Previous biliary tree disease: Cholangitis/Choledocholithiasis/Cholelithiasis
* Ulcerative colitis
* Liver disease
* Exposure to thorium dioxide
167
List the clinical features of a cholangiocarcinoma.
* Painless jaundice
* Weight loss
* Abdominal pain
* Pruritus
* Palpable gallbladder
* Dark urine
* Pale stools
168
Which investigation would most clearly identify a cholangiocarcinoma?
A. LFTs:
B. PT time
C. Serum CEA
D. CT/MRI-Abdomen: Intrahepatic mass lesion/Dilated intrahepatic ducts
D. CT/MRI-Abdomen: Intrahepatic mass lesion/Dilated intrahepatic ducts
Note: Serum CEA (Carcinoembryonic Antigen) is specific for cancer but not to cholangiocarcinoma
169
Which is the best management for a cholangiocarcinoma?
A. Partial Liver Resection
B. Chemotherapy ± Radiotherapy
C. Partial Liver Resection + Chemotherapy ± Radiotherapy
D. Watch and wait
C. Partial Liver Resection + Chemotherapy ± Radiotherapy
170
Describe Primary Biliary Cholangitis.
Autoimmune-mediated inflammation and destruction of small/medium sized intrahepatic bile ducts causing ductopenia and secondary hepatocyte damage due to cirrhosis
171
List 3 risk factors for Primary Biliary Cholangitis.
* Female
* Middle aged: 45-60
* FHx
* Smoking
172
Outline the pathophysiology of Primary Biliary Cholangitis.
Pathophysiology: Autoimmune destruction (AMA, IgM) of bile duct epithelium (lymphocyte infiltration + granulomas) —> progressive ductopenia —> secondary hepatocyte damage —> architectural damage: bridging fibrosis + cirrhosis
173
Mrs. Jones, a 47 year old woman presents with severe itch. She says she has been experiencing this for the past few months but thought it was hay fever, although when Winter came, she wasn't so sure. She also mentions she has had dry eyes and mouth.
O/E you notice she has hepatomegaly, yellowing of the conjunctiva, mucosa and skin. Additionally, you notice yellow deposits under her eyelids.
i) Which risk factors does Mrs. Jones have?
ii) What clinical features can you identify in Mrs. Jones?
iii) Give the differentials which you may consider.
iv) What investigations would you order and what might you expect to see?
Results come back:
The LFTs: ALP raised/GGT raised/AST raised/ ALT raised
Albumin low
Serology: ANA/ IgM raised markedly
US-Abdomen: No obstruction
v) What is your DDx?
vi) Outline your management plan.
```
i)
• Female
• Middle aged: 45-60
• FHx
• Smoking
```
```
ii)
• Itch
• Fatigue
• Dry Eyes and Mouth
• Sleep disturbance
```
* Hepatomegaly
* Jaundice
* Ascites
* Xanthelasmata
iii)
Cholelithiasis
Choledocholelithiasis
NOT Cholecystitis
PSC
PBC
Cholangiocarcinoma? - Unlikely as no weight loss, cachexia, or painless abdominal mass (Courvoursier's Law)
iv)
• LFTs: ALP raised/ GGT raised/ ALT raised/ AST raised
• Albumin: Low
• Serology: ANA/Antipyruvate dehydrogenase/AMA/Anti-Sp100/ IgM raised
• US-Abdomen: Exclude obstruction
• MRCP: Exclude obstruction
v) Primary Biliary Cholangitis (Cirrhosis)
- LFT: Raised
- Albumin: Low
- Serology: ANA and Marked IgM
- US-Abdomen: Exclude obstruction
- MRCP: Exclude obstruction
vi)
• Bile acid analogue: Ursodeoxycholic acid: 12-16mg/kg/day
• Liver transplantation
174
Mrs. Jones, a 47 year old woman presents with severe itch. She says she has been experiencing this for the past few months but thought it was hay fever, although when Winter came, she wasn't so sure. She also mentions she has had dry eyes and mouth.
O/E you notice she has hepatomegaly, yellowing of the conjunctiva, mucosa and ski. Additionally, you notice yellow deposits under her eyelids.
You order investigations, the results come back:
The LFTs: ALP raised/GGT raised/AST raised/ ALT raised
Albumin low
Serology: ANA/ IgM raised markedly
US-Abdomen: No obstruction
What is the most likely differential?
A. Cholangiocarcinoma
B. Cholecystitis
C. Primary Biliary Sclerosis
D. Primary Biliary Cholangitis/Cirrhosis
D. Primary Biliary Cirrhosis
175
Outline the pathophysiology of Primary Sclerosing Cholangitis.
Inflammation of medium/large-sized bile ducts -> fibrosis and multi-focal stricturing -> thickened fibrotic duct wall/ductopenia/cholestasis -> Jaundice/Pruritus/Bacterial cholangitis/Biliary cirrhosis
176
Mr. Altringo, a 24 year old, presents with abdominal pain, fatigue and itching. He mentions he has recently lost weight. Mr. Altringo has no PMHx other than IBD which he manages.
O/E you notice yellowing of the mucosal linings, skin and conjunctiva. You also notice an enlarged spleen, and ascites.
i) What are his risk factors?
ii) What are his clinical features?
iii) What investigations would you order?
The results come back:
LFTs: ALP raised/ GGT raised/ Bilirubin raised
Serum albumin: Reduced
FBC: Normal or thrombocytopenia
PT time: Normal or prolonged
Serology: ANCA/IgG elevated
US-Abdominal: Abnormal bile ducts
CT-Abdomen: Bile duct thickening/Focal or saccular intrahepatic duct dilatation
iv) What is your DDx?
v) Outline your managements.
i)
• Male
• IBD
```
ii)
• Abdominal pain
• Pruritus
• Fatigue
• Jaundice
• Weight loss
```
* Splenomegaly
* Ascites
```
iii)
• LFTs: ALP raised/ GGT raised/ Bilirubin raised
• Serum albumin: Reduced
• FBC: Normal or thrombocytopenia
• PT time: Normal or prolonged
• Serology: ANCA/IgG elevated
```
* US-Abdominal: Abnormal bile ducts
* CT-Abdomen: Bile duct thickening/Focal or saccular intrahepatic duct dilatation
iv) Primary Biliary Sclerosis
```
v)
• Observation
• Lifestyle: Healthy diet + Weight + Reduce alcohol
(+ Pruritus)
• Colestyramine (pruritus relief)
(+ Autoimmune hepatitis)
• Immunosuppressants
```
177
Mr. Altringo, a 24 year old, presents with abdominal pain, fatigue and itching. He mentions he has recently lost weight. Mr. Altringo has no PMHx other than IBD which he manages.
O/E you notice yellowing of the mucosal linings, skin and conjunctiva. You also notice an enlarged spleen, and ascites.
The results come back:
LFTs: ALP raised/ GGT raised/ Bilirubin raised
Serum albumin: Reduced
FBC: Normal or thrombocytopenia
PT time: Normal or prolonged
Serology: ANCA/IgG elevated
US-Abdominal: Abnormal bile ducts
CT-Abdomen: Bile duct thickening/Focal or saccular intrahepatic duct dilatation/ Stricturing
What is your DDx?
A. Cholangiocarcinoma
B. Cholecystitis
C. Primary Biliary Sclerosis
D. Primary Biliary Cholangitis/Cirrhosis
C. Primary Biliary Sclerosis
- Male
- IBD
- M > F
- No significant increase Immunoglobulins
- Large ducts
- Extrahepatic + Intrahepatic
178
Mrs. Johansson, a 55 year old female presents with acute pain she describes to be in the RUQ/RH. She says she has had pale stools (acholic) and experienced itching. Mrs. Johansson has previously had biliary surgery. She has a BMI of 32, has had previous episodes of choledocholithiasis.
O/E you notice she has RUQ with a Positive Murphy's Sign, Jaundice and Fever. However, you note her cognition and blood pressure to remain normal and stable.
i) What are her risk factors?
ii) What are the clinical features?
iii) What is your DDx so far?
iv) What investigations would you order?
Your tests come back as:
• FBC: Leukocytosis • LFTs: Hyperbilirubinemia/Raised ALT/Raised ALP/ Raised AST/ Raised GGT
• CRP: Raised
• Blood culture: Bacteria usually gram negative
• US-Abdomen: Dilated CBD/Gallstones
v) What is your DDx?
vi) What is your management plan?
i)
Fat
Forty <
Female
Biliary surgery
Biliary tree disease
```
ii)
• RUQ pain
• Positive Murphy’s sign
• Jaundice
• Fever
• Acholic stools
• Pruritus
```
iii) Ascending Cholangitis (Charcot's Triad)
OR
Choledolithiasis
Choledocholithiasis
iv)
• FBC: Leukocytosis
• U+E: Uremia/Hypercreatinemia/Hypokalemia/Hypomagnesemia
• LFTs: Hyperbilirubinemia/Raised ALT/Raised ALP/ Raised AST/ Raised GGT
• CRP: Raised
• Blood culture: Bacteria usually gram negative
• PT: Raised (with sepsis)
• US-Abdomen: Dilated CBD/Gallstones
v) Ascending Cholangitis
vi)
• IV ABX: Piperacillin/Tazobactam
• Morphine sulfate: 2.5mg to 10mg every 2-6 hours
* Surgical biliary decompression
* ERCP/Cholecystectomy
179
Mrs. Johansson, a 55 year old female presents with acute pain she describes to be in the RUQ/RH. She says she has had pale stools (acholic) and experienced itching. Mrs. Johansson has previously had biliary surgery. She has a BMI of 32, has had previous episodes of choledocholithiasis.
O/E you notice she has RUQ with a Positive Murphy's Sign, Jaundice and Fever. However, you note her cognition and blood pressure to remain normal and stable.
Your tests come back as:
• FBC: Leukocytosis • LFTs: Hyperbilirubinemia/Raised ALT/Raised ALP/ Raised AST/ Raised GGT
• CRP: Raised
• Blood culture: Bacteria usually gram negative
• US-Abdomen: Dilated CBD/Gallstones
What is your DDx?
A. Cholangiocarcinoma
B. Cholecystitis
C. Ascending Cholangitis
D. Primary Biliary Cholangitis/Cirrhosis
C. Ascending Cholangitis
180
List the aetiology of Acute Pancreatitis.
```
I-GET-SMASHED
• Idiopathic
• Gallstones
• Ethanol
• Trauma
• Steroids
• Mumps
• Autoimmune
• Scorpion Stings
• Hypertriglyceridemia
• ERCP
• Drugs: Azathioprine/Tetracycline/Sulindac/Sulfonamides/Oestrogens/DPP4i/L-asparaginase/Valproic acid
```
181
Which of the following is not a risk factor of Acute Pancreatitis?
A. Middle-aged women
B. Young-middle aged Men
C. Gallstones
D. Drugs: Metformin
D. Drugs: Metformin
182
Which of the following is not a risk factor of Acute Pancreatitis?
A. Middle-aged women
B. Young-middle aged Men
C. Low protein
D. ERCP
C. Low protein
183
List the clinical features of Acute Pancreatitis.
* Upper abdominal pain (epigastric)
* Nausea and vomiting
```
Hypovolemia signs: SHODS
• Hypotension
• Oliguria
• Dry mucous membranes
• Decreased skin turgor
• Sweating
• Tachycardia
• Tachypnoea
```
Signs of pleural effusion:
• Reduced air entry
• Dullness to percussion
• Anorexia
184
What investigations would you order to investigate a potential acute pancreatitis?
* Serum lipase: Elevated* > 3 times
* Serum amylase: Elevated* > 3 times
* FBC: Leukocytosis; Hct (> 44%)*
* CRP: Elevated
* Urea/Creatinine: Elevated in severe
* Pulse oximetry: Hypoxaemia
* LFTs: ALT markedly raised > 3x ***
* CXR: Atelectasis ± pleural effusion ***
* US-Transabdominal: Gallstones/Pancreatic inflammation/Peri-pancreatic stranding/Calcifications/Fluid collections ***
* Serum Calcium: Hypercalcaemia ***
Consider
• CT-Abdomen: Diffuse segmental enlargement of pancreas/irregular contour/obliteration of peri-pancreatic fat/necrosis or pseudocysts
• Urinary trypsinogen-2: Elevated **
185
Outline the treatment of a patient with Gallstone Pancreatitis and Cholangitis
```
• Fluid resuscitation: Crystalloid or Saline
+
• Analgesia: Ibuprofen/Codeine phosphate
+
• Oxygen
+
• ABX: Ciprofloxacin
+
• Anti-emetic: Ondansetron
+
• Nutritional support
+
• ERCP
```
186
Mr. John, a 24 year old DJ presents with upper abdominal pain. He says the pain is central but radiates to his back at times. He has been nauseous and worries he may vomit when performing a wicked set in Ibiza with the lads. He has previously had gallstones which were managed with ERCP. Additionally, he is an avid drinker and has taken steroids previously to look big at Ocean Beach.
O/E you notice signs of sweating, hypotension, oliguria, dry mucous membranes and reduced skin turgor.
i) What are his risk factors?
ii) What clinical features are present?
iii) What is your DDx potentially?
iv) What investigations might you order and what would you expect to see?
```
The investigations come back as:
Elevated serum lipase
Elevated serum amylase
Leukocytosis
CRP elevated
LFTs raised
US-Transabdominal: Fluid collections/Pancreatic Inflammation
Serum calcium: Hypercalcemia
Urinary trypsinogen-2 elevated
```
v) What is your DDx?
vi) What is your management for this?
```
i)
Male young-middle aged
Gallstones
Alcohol
Drugs?
Steroids
```
```
ii)
Abdominal pain
Nausea and vomiting
Hypovolemia signs: SHODS
Sweating
Hypotension
Oliguria
Decreased skin turgor
Dry mucous membranes
```
iii)
Acute Pancreatitis
Aortic Dissection
Heart (Atypical Angina)
Dehydration
Pre-Renal AKI
iv)
• Upper abdominal pain (epigastric)
• Nausea and vomiting
```
Hypovolemia signs: SHODS
• Hypotension
• Oliguria
• Dry mucous membranes
• Decreased skin turgor
• Sweating
• Tachycardia
• Tachypnoea
```
Signs of pleural effusion:
• Reduced air entry
• Dullness to percussion
• Anorexia
v) Acute Pancreatitis
```
vi) Gallstone pancreatitis + Cholangitis
• Fluid resuscitation: Crystalloid or Saline
+
• Analgesia: Ibuprofen/Codeine phosphate
+
• Oxygen
+
• ABX: Ciprofloxacin
+
• Anti-emetic: Ondansetron
+
• Nutritional support
+
• ERCP
```
```
Gallstone pancreatitis
• Fluid resuscitation: Crystalloid or Saline
+
• Analgesia: Ibuprofen/Codeine phosphate
+
• Oxygen
+
• ABX: Ciprofloxacin
+
• Anti-emetic: Ondansetron
+
• Nutritional support
+
• Cholecystectomy
```
187
What is the main difference in management between gallstone pancreatitis with cholangitis and gallstone pancreatitis without cholangitis?
A. With cholangitis you do not do an ERCP
B. With cholangitis you do a cholecystectomy
C. Without cholangitis you do not do an ERCP
D. Without cholangitis you do a cholecystectomy
D. Without cholangitis you do a cholecystectomy
188
Mrs. Jones, a 73 year old female presents with weight loss and anorexia. She mentions she has had pale stools and dark urine. She has a PMHx of MI, DM, chronic pancreatitis and has a BMI of 32.
O/E you notice yellowing of the sclera and mucosal lining s but an absence of Abdominal pain and positive Courvoisier's sign.
i) What are Mrs. Jones' risk factors?
ii) What are Mrs. Jones' clinical features?
iii) What investigations would you order?
The results come back as deranged LFTs, a pancreatic mass seen on US with dilated bile ducts.
iv) What additional investigation may you order?
v) What is your DDx?
vi) What is your management for Mrs. Jones?
i)
Advanced age
PMHx of DM and Chronic Pancreatitis
ii)
Jaundice (icterus)
Courvoisier's Sign
Jaundice (obstructive?)
Weight loss + anorexia
Pale stools
Dark urine
iii)
• US-Abdomen: Pancreatic mass; Dilated bile ducts; Liver metastases
• CT-Abdomen: Mass in pancreas (head of pancreas); Spread or focal
• LFTs: Bilirubinemia/ALT/ALP/GGT/AST
Consider:
• CA19-9 Biomarker: Elevated
• PT time: Prolonged (derangement of Vitamin-K dependent clotting factors)
• Biopsy: Confirm pancreatic ductal adenocarcinoma
iv)
• CA19-9 Biomarker: Elevated
• Biopsy: Confirm pancreatic ductal adenocarcinoma
v) Pancreatic Adenocarcinoma
```
vi)
• Surgical resection
+
• Pancreatic enzyme replacement
+
• Biliary stenting
+
• Radio/Chemotherapy
```
189
Which of the following diseases is described: inherited defect in UDPGT enzyme causing impaired conjugation of bilirubin?
A. Crigler-Najjar Syndrome
B. Gilbert's Syndrome
C. Dubin-Johnson Syndrome
D. Tay-Sachs Disease
B. Gilbert's Syndrome
190
Which of the following diseases is described: inherited defect in UDPGT enzyme causing no/litte conjugation of bilirubin?
A. Crigler-Najjar Syndrome
B. Gilbert's Syndrome
C. Dubin-Johnson Syndrome
D. Tay-Sachs Disease
A. Crigler-Najjar Syndrome
191
Which of the following diseases is described: inherited defect in MRP2 thus no export of conjugated bilirubin?
A. Crigler-Najjar Syndrome
B. Gilbert's Syndrome
C. Dubin-Johnson Syndrome
D. Tay-Sachs Disease
C. Dubin-Johnson Syndrome
192
A 22 year old male presents with jaundice. He has no other remarkable FHx or PMHx other than his father and uncle also have episodic Jaundice when stressed.
O/E you identify conjunctiva icterus, mucosal icterus and icterus of the skin. There is an absence of hepatosplenomegaly and no stigmata of chronic liver disease.
i) What are his risk factors?
ii) What are his clinical features?
iii) What disease might he have?
iv) What investigations would you run and what would you expect?
The results show deranged LFTs, a normal LDH, Negative Coomb's test and a mutational change.
v) What is your Ddx? What mutation might this be?
vi) Outline your management plan for this patient.
i)
- Male
- FHx of GS
- Post-pubertal age
ii)
- Jaundice
- Absence of HSM
- No signs of liver disease
iii)
CN Syndrome
Gilbert's Syndrome
Inherited LSD?
```
iv)
• LFTs: Unconjugated bilirubin elevated (<51umol/L ≈ <3mg/dL)*
GGT normal/ASP normal/ALT normal/ALP normal
• LDH: normal
• FBC: Normal
• Peripheral blood smear: Normal
• Direct Coomb’s test: Negative*
• UGT1A1 genotyping: Mutation present*
```
v) Gilbert Syndrome
UGT1A1
vi)
Patient education: Avoid triggers: Fasting/ Stress/ Dehydration/ Sleep deprivation/ Heavy physical exertion/ Surgery/ Concurrent illness/ Menstruation/ Psychological stress
193
A neonate is born and has neonatal jaundice. In the PMHx there is nothing remarkable but the FHx you identify that several family members have had neonatal jaundice.
O/E you identify abdominal pain, pruritus, weight loss. The nurse mentions the neonate has had pale stools and dark urine.
i) What risk factors does this patient have?
ii) What are the clinical features of this patient?
iii) What investigations would you wish to conduct and what might you expect?
The results come back with unconjugated hyperbilirubinemia, prolonged PT time, UGT1A1 mutation present and UDPGT enzyme activity reduced.
iv) What is your DDx?
v) Outline your management plan for this patient.
i)
FHx Jaundice
Neonate
```
ii)
Neonatal jaundice
Pruritus
Weight loss
Pain
Pale stools
Dark urine
```
```
iii)
• LFTs: Unconjugated hyperbilirubinemia*
• PT time: May be increased*
• Direct Coomb’s test: Negative*
• UGT1A1 genotyping: Mutation present*
• UDPGT enzyme activity: Reduced activity*
• LDH: normal
• FBC: Normal
• Peripheral blood smear: Normal
```
iv) Crigler-Najjar Syndrome
v)
• Phototherapy: Reduce bilirubin molecules in the skin
• Plasmapheresis
• Liver transplantation
194
Mr. Akrami, an Iranian Jew, presents with intermittent jaundice. On asking if an itch is present, he says there has been no itch. There is no remarkable PMHx however he says he has recently had a throat infection. He mentions his family have a history of this disease but assures you it is not anything to worry about.
i) What are his risk factors?
ii) What are his clinical features?
iii) What investigations might you run and expect?
The results come back as normal LFTs but raised conjugated bilirubin. Additionally there is a mutational change.
iv) What is your DDx and why? Identify the likely mutation based on your DDx.
v) Outline your management
i)
- Iranian
- Jewish
- FHx
ii)
- Jaundice
- X Pruritus
- Recent infection
```
iii)
• LFTs: Hyperbilirubinemia
--> Normal ALT/ALP/AST/GGT
• Serum bile acids: Normal
• Clotting profile: Normal
• Genotype of MRP2 gene (ABCC2 gene): Mutation in ABCC2 gene
```
iv) Dubin-Johnson Syndrome
- Jaundice
- Elevated conjugated bilirubin
- No deranged LFTs
- Mutation in ABCC2 gene (MRP2) ≈ DJ syndrome
v)
Confirmation of Diagnosis + Reassurance
195
What is jaundice defined as?
A. < 1.2 mg per dL
B. ≥ 1.2mg per dL
C. ≥ 1.2mg per L
D. < 1.2 mg per L
B. ≥ 1.2mg per dL
196
What is the normal bilirubin range?
A. 2.4-18umol/L
B. 2.2-18umol/L
C. 3.4-20umol/L
D. 3.4-22umol/L
C. 3.4-20umol/L
197
List the clinical signs of jaundice.
* Icterus (Jaundice)
* Darkening of urine (haemoglobinuria)
* Pruritus
* Steatorrhea
* Weight loss
* Acholic stools
198
List the components of bile.
* Bilirubin
* Bile salts --> family of steroid molecules ending in carboxylic acids and several hydroxyl groups
* Phospholipids
* Cholesterol
* Proteins
* Fatty acids
199
How many days do RBCs last for?
A. 200 Days
B. 110 Days
C. 120 Days
D. 80 Days
C. 120 Days
200
What is the role of ferrochelatase?
A. Insert Hb into PPIX
B. Insert Fe into PPIX
C. Insert Cu into PPIX
B. Insert PPIX into RBC
B. Insert Fe into PPIX
201
List the three Bilirubin fractions.
* Total Bilirubin = Conjugated + Unconjugated
* Direct Bilirubin = Conjugated
* Indirect Bilirubin = Unconjugated
202
Outline enterohepatic circulation.
Circulation of biliary acids, bilirubin and other substances from liver to gall bladder to SI to enterocyte back to liver
203
Which of the liver enzymes are found in the cytoplasms of hepatocytes?
A. GGT
B. ALP
C. Bilirubin
D. AST
D. AST
204
Which of the liver enzymes are found in the cytoplasms of hepatocytes?
A. GGT
B. ALP
C. Bilirubin
D. ALT
D. ALT
205
Which of the liver enzymes are found in the biliary canalicular membrane?
A. GGT
B. ALP
C. AST
D. ALT
B. ALP
206
Which of the liver enzymes are found in the biliary canalicular membrane co-localised with another?
A. GGT
B. ALP
C. AST
D. ALT
A. GGT
207
Which of the liver enzymes are present only in the cytoplasm of hepatocytes?
A. GGT
B. ALP
C. AST
D. ALT
D. ALT
208
Which of the liver enzymes are present in bone and placenta?
A. GGT
B. ALP
C. AST
D. ALT
B. ALP
209
Which of the liver enzymes are present in myocytes?
A. GGT
B. ALP
C. AST
D. ALT
C. AST
210
Which of the liver enzymes are induced by drugs and non-specific?
A. GGT
B. ALP
C. AST
D. ALT
A. GGT
211
Name 2 lifestyle choices that make Homer (and many others in developed countries) at higher risk of liver disease
obesity, alcohol
212
Name 3 stigmata of chronic liver disease (3 pts)
gynaecomastia,spider naevi, palmar erythema, Duypteren’s contracture, ascites, hepatic flap, hypothenar atrophy, cachexia
213
What blood test is most typically diagnostic of Primary Biliary Cirrhosis/Cholangitis?
AMA (anti-mitochondrial antibodies)
IgM
214
What is the main cause of abdominal swelling in chronic liver disease?
ascites
215
Name one treatment for abdominal swelling in chronic liver disease.
spironolactone or eplerenone
drainage
216
Is serum albumin high or low in chronic liver disease?
low
217
Does haemolytic anaemia cause conjugated or unconjugated hyperbilirubinaemia?
unconjugated jaundice
218
The typical urinalysis would show in haemolytic anaemia
A. high bilirubin and high urobilinogen
B. No bilirubin and high urobilinogen
C. High bilirubin and no urobilinogen
B – no bilirubin high urobilinogen
219
Name 2 causes of haemolytic anaemia
from thalassemia , sickle cell disease, drug – induced, auto-immune
220
In pure obstructive jaundice which is the most characteristic raised liver blood test ?
raised Alk Phos (ALP)
221
What is the commonest benign cause of obstructive jaundice?
Cholelithiasis (gallstones) affecting CBD
222
What is the commonest malignant cause of obstructive jaundice?
Carcinoma head of pancreas
223
What is Charcot’s triad?
Jaundice, fever, RUQ tenderness
224
Name the first line imaging technique in suspected obstructive jaundice?
Abdominal USS
225
What procedure is commonly used to resolve obstructive jaundice?
ERCP
226
Which hepatitis is commonly spread by the faeco-oral route?
Hep A
227
Name 2 types of hepatitis transmitted by needles or sexual contact?
Hep B and C
228
Can an ALT of over 10,000 u/l occur in viral hepatitis?
YES
229
Name 2 causes of hepatitis which are non-viral.
Includes alcoholic, auto-immune, drug-induced, steatic
230
Name a virus that can cause sore throat and hepatitis.
EBV
