Liver Flashcards
1
Q
Functions of the Liver
A
storage
Protein synthesis (albumin)
Carb & lipid metabolism
Immunity
Detoxification and excretion
2
Q
Causes of acute liver injury
A
Viral: hepatitis (A/B)
Drugs - paracetamol overdose , Ab
Vascular - budd chiari
Obstruction
congestion
3
Q
Causes of chronic liver injury
A
Alcohol
Viral (B,C)
Metabolic - wilson’s
Autoimmune - sclerosing cholangitis
4
Q
Presentation of acute liver injury
A
Malaise
Nausea
Anorexia
Jaundice
5
Q
Presentation of chronic liver injury
A
Ascites
Oedema
Haematemesis
Anorexia
Easy bruising
6
Q
Define liver failure
A
Where the liver loses its ability to repair and regenerate leading to decompensation
7
Q
What are the 3 markers of liver function
A
Serum
* Bilirubin (high = damage)
* Albumin (low=damage)
* Prothrombin time (high=damage)
8
Q
How are serum liver enzymes used in the investigation of liver function
A
- They’re non specific to liver function but can show the likelihood of liver damage
9
Q
Give examples of serum enzymes used in LFTs
A
- Cholestatic: alkaline phosphatase, gamma-GT
- Hepatocellular: transaminases - Aspartate transaminase (AST) and alanine transaminase (ALT)
10
Q
What are the 3 types of jaundice and state whether they’re unconjugated or conjugated
A
- Prehaptic (unconjugated)
- Hepatic (conjugated)
- Post-hepatic (conjugated)
11
Q
Give 2 causes of prehepatic jaundice
A
- Haemolytic anaemias
- Gilbert’s syndrome
12
Q
List 3 causes of hepatic jaundice
A
- Hepatitis
- Neoplasm
- Ischaemia
13
Q
Give 2 causes of post-hepatic jaundice
A
- Gallstone
- Stricture
14
Q
Describe the urine and stool sample for conjugated jaundice
A
- Dark urine
- Pale stool
15
Q
Which type of jaundice may experience itching
A
Conjugated
16
Q
Signs and symptoms of acute liver failure
A
- Jaundice
- Coagulopathy
- Hepatic encephalopathy - altered mood, dyspraxia etc
- Renal failure
- Hypoalbuminaemia
17
Q
Describe the investigation of acute liver failure
A
- LFTs - raised ALT/AST and PT/INR
- USS
- FBC - Leukocytosis. anaemia, thrombocytopenia
- Microbiology to rule out infection - blood culture, peritoneal tap
- ABG - metabolic acidosis
18
Q
Describe the management of acute LF
A
- Treat underlying cause and complications
- ABCDE
- Anaglesia
19
Q
State complications of LD and how each would be treated
A
- Encephalopathy - lactulose
- Cerebral oedema - mannitol
- Haemorrhage - Vit K
- Ascites - diuretics
- Sepsis - sepsis 6
20
Q
Presentation of chronic liver disease
A
- spider naevi - dilated blood vessel due to excess oestrogen
- Splenomegaly
- Palmar erythema - red palm
- Jaundice
- Abdo distension
- Leukonychia (white nails)
21
Q
Describe the investigation of CLD
A
- GS: Liver Biopsy
- Raised AST and ALT
- FBC - low albumin, thrombocytopenia and prolonged INR
- Abdo USS - nodularity, atrophy, signs of advanced cirrhosis
22
Q
Management of CLD
A
- Treat complications
- Liver transplant
- Lifestyle modifications
23
Q
What is Gilbert’s syndrome
A
- Auto Rec inheritance of UGT1A1
- affects the liver’s ability to metabolise bilirubin
- Raised unconjugated bilirubin
24
Q
How does Gilbert’s syndrome typically present
A
Painless jaundice at a young age
25
What are ascites
Accumulated free fluid in the abdo cavity (peritoneal)
26
Causes of ascites
* infection - TB
* Neoplasia - ovary, pancreas
* congestive HF, pericarditis - fluid leaks out
* low albumin (nephrotic syndrome)
* liver cirrhosis
* Budd-Chiari syndrome
27
Signs and symptoms of ascites
* Abdo distension
* Shifting dullness (flanks)
28
Describe the investigation of ascites
* Shifting dullness
• CT/USS/MRI - diagnostic
* Ascitic fluid tap - cytology (high WBC) and protein levels
* Serum ascites albumin gradient:
- Transudate: >11g/L, clear fluid (increased HP, portal HTN)
- Exudate: <11g/L, cloudy fluid (inflammation mediated)
29
How are ascites treated
* Treat underlying cause - e.g. Ab for bacterial infection
* Diuretics to increase renal Na excretion (e.g. spironolactone)
* Paracentesis - drain fluid
* reduce dietary sodium
30
Describe the ascites percussion test
* Percuss centrally to laterally until dull sound
* Keep finger at dull spot and ask patient to lean to opposite side
* If the dullness is fluid , it will have moved and the previously dull area will be resonant
31
Describe the progression of alcoholic liver disease
1) Alcohol related fatty liver - reversible
2) Alcoholic hepatitis - usually reversible
3) Cirrhosis - scar tissue
32
What is the recommended alcohol consumption per week
14 units
No more than 5 units per day
33
What questionnaires can be used to screen for alcohol dependency
1) CAGE (2<) - Cut down, Annoyed, Guilty, Eye opener
2) AUDIT - MCQ, 8/10 = harmful use
34
Describe the CAGE questionnaire
* should Cut down
* are people Annoyed by your drinking
* feel Guilty about drinking
* you drink in the morning (eye opener)
35
Signs and symptoms of ALD
* Alcohol dependency
* Jaundice
* Spider naevi
* Hepatomegaly
* Palmar erythema - red palms
36
Describe the investigation of ALD
* FBC - macrocytic non-megaloblastic anaemia
* LFTs - ^ gamma-GT, ^ AST/ALT, low albumin
* Elevated prothrombin time
* Biopsy - inflammation, necrosis, mallory bodies
37
Describe the management of ALD
* alcohol abstinence
* Diet - vitamins and high protein
* Treat complications of cirrhosis - ascites, portal HTN
* Short term steroids
* Liver transplant
38
What is delirium tremens
Medical emergency associated with alcohol withdrawal
39
Describe the presentation of delirium tremens
* Ataxia - uncoordinated movements
* Tremors
* Severe agitation
* Seizures
* Delusions and hallucinations
40
Treatment for delirium tremens
Chlordiazepoxide
Diazepam (less common)
41
How can ALD cause Wernicke-Korsakoff Syndrome (WKS)
* Excess alcohol leads to thiamine (Vit B1) deficiency
* B1 is poorly absorbed in the presence of alcohol
* 1st Wernicke's encephalopathy then Korsakoff's syndrome
42
Give 3 features of Wernicke encephalopathy
* Confusions
* Oculomotor disturbances
* Ataxia
43
Give 2 features of Korsakoff's syndrome
* Memory impairment - unable to form new memories and unable to recall past memories
* Behaviour changes
44
Give 3 RFs of ALD
Alcohol
Obesity
Smoking
45
Give 4 RFs of Non alcoholic fatty LD
* Obesity
* T2DM
* HTN
* Drugs - NSAIDS
46
Describe the stages of NAFLD
1. NAFLD
2. Non-alcoholic steatohepatitis
3. Fibrosis
4. Cirrhosis
47
How does NAFLD typically present
* Usually asymptomatic and found incidentally
* Severe = signs of LF
48
Describe the investigation of NAFLD
* Liver biopsy
* Abnormal LFTs - particularly raised gamma GT
* Abdo US - confirm fatty liver
* Non-invasive liver screen
* Enhanced liver fibrosis blood test
49
Describe the management of NAFLD
* Weight loss
* Control diabetes, HTN and cholesterol
* exercise
* Vit E
50
What is viral hepatitis
Inflammation of the liver as a result of viral replication within hepatocytes
51
What is the most common viral hepatitis
Hep A
52
What type of virus is Hep A
Acute RNA picornavirus
53
How is Hep A transmitted
Faeco-oral - usually contaminated water and food (uncooked shellfish)
54
Give 3 RFs of Hep A
* Overcrowding
* Shellfish
* Travel
55
Describe the presentation of Hep A
Prodromal (1-2w):
* N+V, Fever, Malaise
Icteric (up to 3m):
* Jaundice, dark urine, pale stools
56
Describe the investigation of Hep A (HAV)
* LFTs - ^ bilirubin
* Serology - HAV IgM = acute infection, IgG anti-HAV
* Bloods - raised ESR
57
How is Hep A treated
* Self-limiting: usually clears up within 3 months
* Travellers vaccine available
* Rest, analgesia
58
What type of virus is Hep C (HCV)
* RNA flavivirus
* can be acute or chronic
59
How is HCV transmitted
* Blood
* Bodily fluids - sex
* IV drug use
60
Describe the presentation of acute HCV
* Usually Asymptomatic
* May have flu like symptoms - N+V, fever, diarrhoea
61
How does chronic HCV present
Chronic liver signs and hepatosplenomegaly
62
Describe the diagnosis of HCV
Serology:
* HCV RNA - active infection
* HCV Ab +ve = active/ resolved
63
Describe the management of HCV
* Direct acting antivirals (DAA) + Ribavirin
64
Give 2 complications of HCV
* Liver cirrhosis
* Hepatocellular carcinoma
65
What type of virus is Hep E (HEV)
Acute single stranded RNA
66
How is HEV transmitted
Faeco-oral spread
* Undercooked pork
* Contaminated water
67
Presentation of HEV
Mild illness usually asymptomatic
68
Describe the investigation of HEV
HEV IgM = acute infection
HEV RNA
69
How is HEV treated
* Self limiting infection so usually clears up within a month
* Supportive
70
Who is particularly susceptible to complications following HEV
* Immunocompromised - chronic LF
* Pregnancy - increased mortality
71
What type of virus is Hep B (HBV)
* Double stranded DNA virus
* Acute and chronic
72
How is HBV transmitted
* Blood
* Bodily fluids - sex
* Needles - IVDU, tattoos
* Vertical - mother to child
73
Give 3 RFs of HBV
* Healthcare workers
* Dialysis patients
* IVDU
74
Describe the presentation of HBV
* Prodromal (1-2w) - n+v, RUQ pain, fever
* Icteric (up to 6m) - jaundice, dark urine, arthralgia, pale stool
75
Describe the HBV serology
* Surface antigen (HBsAg) - active infection
* Surface antibody (HBsAb) - past/current infection/ vaccine
* Core Ab (HBcAb) - past/current infection
- IgM = active/ recent + chronic
- IgG = resolved/ chronic
* E antigen (HBeAg) - marker of viral replication and implies high infectivity
* HBeAb - chronic or been through active phase
* HBV DNA - direct count of viral load
76
How does HBcIgM differ in acute and chronic infection
Acute = high titre
Chronic = low titre
77
Describe the management of HBV
* Vaccination HBsAg - 3 doses
* Anti-viral meds - Pegylated interferon alpha 2a
* Entecavir (anti-viral)
* Liver transplant
78
What type of virus is Hep D (HDV)
SSRNA virus
79
What is the only way HDV can survive
Only survive in patients who also have HBV
80
Describe the pathophysiology of HDV
* It attaches itself to HBsAg (can't survive without this)
* HBV + HBD = greater chance of cirrhosis/ HCC
81
Describe the serology of HDV
Manifests as co-infection
* IgM HDV
* IgM HBV
82
What is autoimmune hepatitis
Possible genetic predisposition triggered by a viral infection that causes an autoimmune response against hepatocytes
83
What are the 2 types of autoimmune hepatitis
Type 1 (80%) - adult females (>45)
Type 2 - younger females
84
What autoantibodies are involved in type 1 autoimmune hepatitis
* Anti-nuclear Ab (ANA)
* Anti-smooth muscle Ab (ASMA)
* Anti-soluble liver Ag (ASLA)
85
What autoantibodies are involved in type 2 autoimmune hepatitis
* Anti-Liver Kidney Microsomes-1 (ALKM1)
* Anti-Liver Cytosol Antigen Type 1 (ALC1)
86
Describe the presentation of autoimmune hepatitis
* May be asymptomatic or have features of liver disease
* Fatigue in type 1 following menopause
87
How is AIH investigated
* Serology - autoantibodies
* LFTs - elevated ALT and AST with normal/ mildly elevated ALP
88
Treatment for AIH
* Steroids (prednisolone) + immunosuppressants (azathioprine)
* Liver transplant
89
Describe the CAGE questionnaire
* should Cut down
* are people Annoyed by your drinking
* feel Guilty about drinking
* you drink in the morning (eye
90
What is biliary colic
Intermittent right upper quadrant pain caused by temporary obstruction of gallstones irritating the bile ducts
91
Describe the composition of gallstones
* Form from concentrated bile in the bile duct
* Most are made of cholesterol but can also be made out of pigment or mixed
92
Give 5 RFs for gallstones
5 Fs:
* Female
* Fat (overweight)
* Forty (or older)
* Fair (Caucasian)
* Fertile (pre-menopausal)
93
What is acute cholecystitis
Acute inflammation of the gallbladder (95% involve gallstones)
94
Describe the presentation of gallstones
* Colicky RUQ pain
* Worse after eating fatty meals
* N+V
* Episodes of pain for 30+ mins
95
Describe the investigation of gallstones
* 1st line: Ultrasound - stones, duct dilation
* Raised alkaline phosphatase - non specific marker
96
How are gallstones treated
* NSAIDS for mild pain (diclofenac)
* Cholecystectomy
* ERCP - remove stones
* Reduced fatty diet
97
Describe the presentation of acute cholecystitis
* RUQ pain which may radiate to right shoulder
* Fever
* +ve Murphy's sign
98
Describe a +ve murphy's sign
* Ask patient to breathe in and hold a deep breath while palpating the RUQ
* Severe pain = +ve
99
Describe the investigation of acute cholecystitis
* Abdo ultrasound - Fluid around GB, stones/sludge in GB,
thickened GB wall (>3mm)
* LFT - normal
* FBC - Leukocytosis and neutrophilia
* Magnetic resonance cholangiopancreatography (MRCP) - if common bile duct stone is suspected but not seen in US
100
Treatment for cholecystitis
* Before surgery: IV fluids, analgesia, IV Ab if needed
* Laproscopic cholecystectomy within 1 week (usually 72h)
101
What is acute cholangitis
Inflammation of the bile ducts (85% due to gallstones)
102
Explain how acute cholangitis increase the risk of sepsis
* Prolonged bile duct blockage leads to bile stasis
* Bacteria (typically E.coli) from the duodenum climbs up through the ampulla of Vater and causes biliary tree infection
103
Describe the presentation of acute cholangitis
* Charcot's triad: fever, jaundice, RUQ pain
* Obstructive: Reynolds pentad - Charcot's triad + altered mental state and hypotension (sepsis)
104
Investigations of acute cholangitis
* FBC - leukocytosis
LFTs - raised ALP and bilirubin
* CRP raised
* Endoscopic abdo ultrasound - common bile duct dilation and gallstones
* endoscopic retrograde cholangiopancreatography
105
Treatment of acute cholangitis
* Treat sepsis - IV fluids + Abx
* ERCP and stenting to clear blockage
* Cholecystectomy
106
What is primary biliary cholangitis (cirrhosis)
Condition where the immune system attacks the small bile ducts within the liver
107
Explain the pathophysiology of PBC
* Immune system attacks intralobar bile ducts (canals of Hering)
* This causes obstruction of the outflow of bile (cholestasis)
* Back-pressure of bile obstruction and overall disease process leads to fibrosis, cirrhosis and LF
108
Give 3 RFs for PBC
* 40-50 y/o women
* Other autoimmune conditions (e.g. coeliac)
* Rheumatoid conditions (e.g. sjogrens and arthritis)
109
Describe the presentation of PBC
* Pruritus - itchy skin (bile acids)
* Fatigue
* Jaundice (bilirubin)
* Xanthelasma - cholesterol deposits in skin
* Pale stools
* GI disturbances and abdo pain (lack of bile acids)
* Signs of cirrhosis and LF
110
Dx of PBC
* LFT - raised ALP, bilirubin
* Serology: raised AMA (anti-mitochondrial Ab)
* Liver biopsy - diagnose and stage disease
* Raised IgM
111
Describe the management of PBC
* 1st: ursodeoxycholic acid - reduces intestinal absorption of cholesterol
* Colestyramine - decreases bile acid reabsorption in SI helps with pruritus
* Liver transplant in end stage
* Immunosuppression - with steroids
112
What is primary sclerosing cholangitis
Autoimmune chronic LD where the intrahepatic or extra-hepatic bile ducts become inflamed, strictured and fibrotic
113
Give 4 RFs of PSC
* Ulcerative colitis - strong link (70%)
* Male
* age 40-50
* FHx
114
Describe the presentation of PSC
* Initially asymptomatic
* Pruritus
* Fatigue
* Jaundice
* Chronic RUQ pain
115
How is PSC investigated
* LFT - raised ALP, GGT
* Autoantibodies: p-ANCA, antinuclear Ab
* GS: MRCP
116
Describe the management of PSC
* Colestyramine for pruritus
* ERCP can be used to dilate and stent strictures
* Liver transplant
* Fat soluble vitamin supplements
117
Give 3 complications of PSC
* Fat soluble deficiencies
* Acute bacterial cholangitis
* Cholangiocarcinoma
118
What is liver cirrhosis
* The result of chronic inflammation and damage to liver cells
* When liver cells are damaged they're replaced with fibrosis and nodules of scar tissue from within the liver
* Irreversible
119
Give 4 common causes of liver cirrhosis
* ALD
* NAFLD
* Hep B
* Hep C
120
Give 3 metabolic conditions that cause liver cirrhosis
* Haemochromatosis
* Wilson's disease
* Alpha 1 antitrypsin deficiency
121
Signs and symptoms of liver cirrhosis
* Jaundice
* Hepatomegaly
* Splenomegaly
* Spider naevi
* Palmar erythema
* Ascites
122
Describe the investigation of cirrhosis
* US and CT - splenomegaly, nodularity, enlarged portal vein
* Biopsy - diagnostic
* Bloods - low albumin, high INR
123
Name and describe the score used to indicate the severity of cirrhosis
Child-Pugh score
* 5 factors: bilirubin, albumin, INR, ascites & encephalopathy
* Give score of 1/2/3
124
Describe the management of cirrhosis
* Liver transplant
* Alcohol abstinence
* Treat complications
* Screen for hepatocellular carcinoma every 6m (marker=AFP)
125
Give 5 complications of cirrhosis
* Ascites and spontaneous bacterial peritonitis
* Portal HTN
* Varices/ variceal bleeding
* Hepatocellular carcinoma
* Malnutrition
126
What is portal HTN
Elevated pressure in the portal venous system
127
Give a prehepatic cause of portal HTN
Portal vein thrombosis
128
Give 3 intrahepatic causes of portal HTN
* Cirrhosis (mc)
* Schistosomiasis (parasitic disease)
* Budd Chiari syndrome
129
Explain how cirrhosis causes portal HTN
* Cirrhosis increases resistance of blood flow in liver
* Results in increased back-pressure into portal system = portal HTN
130
How does portal HTN typically present
* Mostly asymptomatic
* Can present with oesophageal varices rupture
131
What are oesophageal varices
* Dilated and tortuous collateral blood vessels
* Direct consequence of portal HTN
132
What is a DDx of oesophageal varices and how would they be distinguished
Mallory Weiss tear - acute Hx
Varices - long Hx of alcoholism/ cirrhosis
133
What is the GS investigation for oesophageal varices
Upper GI endoscopy
134
How are stable (non-bleeding) varices treated
* Non-selective BB (e.g. propranolol + nitrates) - reduce portal HTN
* Elastic band ligation
* Injection of sclerosant
135
How is a ruptured oesophageal varices treated
* Resuscitation: ADH analogue (e.g IV terlipressin) - vasoconstriction = slow bleeding
* Variceal banding
* Sclerotherapy
* Transjugular intra-hepatic portosystemic shunt (TIPS) - decrease portal pressure by diverting blood
136
What are the signs of a ruptured oesophageal varices
* Haematemesis (coffee ground)
* Melena
137
What is acute pancreatitis
* Acute inflammation of the pancreas
138
What are the 3 main RFs of acute pancreatitis
* Gallstones
* Alcohol
* Post-ERCP
139
Describe the pathophysiology of gallstones pancreatitis
* Gallstones get trapped at the ampulla of vater which blocks the flow of bile and pancreatic juices into duodenum
* Reflux of bile into pancreatic duct and prevention of pancreatic enzymes being secreted results in inflammation
* Autodigestion of pancreas if host defences against pancreatic enzymes (trypsin) are overwhelmed
140
How does alcohol cause pancreatitis
Alcohol is directly toxic to pancreatic cells
* mc in men and young ppl
141
Causes of pancreatitis
I get smashed
* Idiopathic
* Gallstones
* Ethanol (alcohol)
* Trauma
* Steroid use
* Mumps
* Autoimmune
* Scorpion stings
* Hypercalcemia/ hyperlipidaemia
* ERCP
* Drugs
142
Name 3 drugs that can cause pancreatitis
Furosemide
mesalazine
Thiazide diuretic
Azathioprine
143
Describe the presentation of acute pancreatitis
* Severe epigastric pain that may radiate to the back
* Tachycardia
* Fever
* N+V
* Jaundice
* Grey turner sign (flank bruising)
* Cullen sign (Periumbilical bruising)
144
Describe how acute pancreatitis is investigated
- raised serum amylase
- raised serum lipase (GS, more specific)
* Raised CRP - level of inflammation
* LFTs - raised ALP suggests gallstones as cause
* US - assess for gallstones
145
What are 2 scoring systems for acute pancreatitis
* APACHE 2
* Glasgow score
146
Describe the glasgow score
* Assess severity of pancreatitis
* Gives numerical score for each factor
* 3< = severe pancreatitis
P – Pa02
A – Age > 55
N – Neutrophils
C – Calcium
R – uRea
E – Enzymes
A – Albumin
S – Sugar
147
Describe the treatment of acute pancreatitis
* Nil by mouth
* IV fluid
* Analgesia - morphine
* Treat GS - ERCP
* Treat complications (eg drain large collections)
148
Give 4 complications of acute pancreatitis
* Necrosis
* Chronic pancreatitis
* Pseudocysts - same presentation
* Acute peripancreatic fluid collections
149
What is chronic pancreatitis
* Chronic inflammation of the pancreas
* 3m Hx of pancreatic deterioration
150
Describe the pathophysiology of chronic pancreatitis
* Persistent inflammation of the pancreas due to changes in pancreatic structure
* Fibrosis and reduced function of pancreas
151
Give 4 causes of chronic pancreatitis
* Alcohol (mc)
* CKD
* genetics - CF and haemochromatosis
* Recurrent acute pancreatitis
152
Describe presentation of chronic pancreatitis
* Epigastric pain radiation to back
* Similar Sx to acute p but less intense and longer lasting
* Sx worse after alcohol and better on leaning forward
* Steatorrhea (fat in stool)
* weight loss and malnutrition
153
Describe the investigation of chronic pancreatitis
* Serum amylase and lipase unlikely to be high in severe cases
* MRI/CT abdo - pancreatic calcifications, ductal dilation
* transabdo US - ductal/ parenchymal abnormalities, fibrosis
* MRCP - used if CT is inconclusive, alternating dilation and stenosis of pancreatic ducts
* ERCP - chain of lakes ( tortuous and dilated pancreatic duct)
154
How is chronic pancreatitis treated
* Alcohol cessation
* pancreatic enzyme supplements - e.g. pancreatin with PPi (omeprazole) to increase absorption
* Surgery
* Simple analgesia
155
How can chronic pancreatitis cause steatorrhea
* Lack of pancreatic enzymes (lipase)
* Malabsorption of fat
* Steatorrhea
* Deficiency in fat soluble vitamins
156
What condition can develop as a result of loss of endocrine function in chronic pancreatitis
DM due to lack of insulin
157
What is peritonitis
Inflamed peritoneal cavity
158
What is spontaneous bacterial peritonitis
Infection in the ascitic fluid and peritoneal lining
159
Give 3 bacteria that cause SBP
* Gram -ve: E.coli, klebsiella pneumoniae
* Gram +ve: Staph aureus (cocci)
160
Describe the signs and symptoms of peritonitis
* Sudden severe abdo pain
* Guarding
* May have ascites
* Fever
161
Describe the investigation of peritonitis
* Ascitic tap - raised ascitic neutrophilia
* Blood cultures - causative organism
* FBC - anaemia, leukocytosis
162
How is SBP treated
* ABCDE
* IV Abx (eg cefotaxime or vancomycin if sepsis)
* Iv fluids/ albumin
* Paracentesis - remove peritoneal fluid through needle
163
What is a complication of SBP
septicaemia
164
What is hepatic encephalopathy
Build up of toxins (ammonia) that affect the brain
165
Why does hepatic encephalopathy occur in liver cirrhosis
* Functional impairment of liver cells prevents ammonia being metabolised into harmless waste products
* Collateral vessels between portal and systemic circulation mean that ammonia bypasses liver and enters systemic system directly
166
Give 4 ways which hepatic encephalopathy presents
* Reduced consciousness
* Confusion
* Memory problems
* Mood/ personality changes
167
Give 4 things that can trigger the onset of hepatic encephalopathy
* Constipation
* GI bleed
* Electrolyte imbalance
* Sedative meds
168
How is hepatic encephalopathy treated
* Laxatives (i.e. lactulose) - promote excretion of ammonia
* Ab (i.e. rifaximin) - reduce n.o intestinal bacteria
169
What is haemochromatosis
Genetic disorder that causes the body to absorb and store excess iron
170
What causes haemochromatosis
Autosomal recessive inheritance of mutated human haemochromatosis gene (HFE) on chrom 6
171
Give 4 RFs of haemochromatosis
* Middle age (40<)
* Male
* FHx
* White ancestry
172
Explain the pathophysiology of haemochromatosis
Excess iron is absorbed by the body and deposited in multiple organs
173
Describe the presentation of haemochromatosis
* Joint pain
* Fatigue
* Sexual problems (e.g. amenorrhoea) - hypogonadism
* Bronze/ slate-grey discolouration
174
Describe the investigation of haemochromatosis
* Raised serum ferritin and transferrin
* Liver biopsy (prior GS but no longer necessary due to genetic testing)
* Genetic testing
175
How is haemochromatosis treated
* Venesection - weekly removing blood
* Iron chelation (deferasirox)
* Lifestyle: limit Vit C
176
Complications haemochromatosis
* T1DM - Fe affects function of pancreas
* Liver cirrhosis
* Hypogonadism
* Hypothyroidism
* HCC
177
Give a contraindication to venesection
Anaemia
178
What is Wilson's disease
Excessive accumulation of copper in the body and tissues
179
What causes wilson's disease
* Autosomal recessive mutation of ATP7B copper binding protein on chromosome 13
* Results in reduced removal of Cu in liver
180
Give 2 RFs of Wilson's disease
* Male
* FHx
181
Describe the hepatic presentation of Wilson's disease
Hx of chronic hepatitis
cirrhosis - ascites (uncommon)
Jaundice (uncommon)
182
Describe the neurological signs and Sx of wilson's disease
* Most Cu is deposited in basal ganglia
* Tremor and chorea
* Dysarthria - speech issues
* behavioural problems
* Incoordination - sloppy handwriting, walking, eating
183
Give 2 psychiatric symptoms of Wilson's disease
* Mild depression
* Psychosis
184
What is parkinsonism
Umbrella term used to describe Sx of:
* Tremor
* Rigidity
* Bradykinesia (slowness of movement)
185
What is an ophthalmological signs of Wilson's disease
Kayser Fleischer rings
Cu deposits in cornea giving greenish brown appearance
186
Describe the investigation of Wilson's disease
* Serum ceruloplasmin (protein that carriers copper) and CU - decreased levels
* Liver biopsy
* 24h urine Cu assay - elevated
* Genetic analysis - ATP7B gene
* Slit lamp exam - KF rings
187
How is Wilson's disease managed
* Cu chelation - penicillamine or trientine hydrochloride
* Avoid high Cu foods e.g. shellfish & mushrooms
* Liver transplant as last resort
188
Define alpha 1 antitrypsin (A1AT) deficiency and state the cause
* Deficiency of alpha 1 antitrypsin enzyme
* Autosomal codominant inheritance of protease inhibitor gene (serpine-1 gene)
189
What chromosome is the gene for alpha 1 antitrypsin coded on
14
190
Explain the pathophysiology of A1AT deficiency
* Elastase is an enzyme secreted by neutrophils that digests CT
* A1AT is produced in the liver and inhibits neutrophil elastase
* Reduced A1AT affect 2 main organs:
- Lungs
- Liver
191
How does A1AT deficiency affect the lungs
* Excess breakdown of elastic fibres in lungs
* Leads to emphysema and bronchiectasis
192
How does A1AT deficiency affect the liver
* Accumulation of abnormal A1AT causes liver damage
* Progresses to cirrhosis
* Can lead to HCC
193
How does A1AT deficiency present
* COPD like Sx: dyspnoea, chronic cough, sputum
* Jaundice
194
Describe the investigation of A1AT deficiency
* Low serum A1AT
* Genetic testing - A1AT gene mutation
* CT thorax - emphysema
* Pulm function - reduced FEV1, FVC etc
195
How is A1AT deficiency treated
* Smoking cessation
* Liver transplant for end stage
Symptomatic management e.g. inhalers for emphysema
196
What is a hernia
Protrusion of an organ (e.g. bowel) through a weak point in a cavity wall
197
What is a reducible hernia
Lump may be pushed back into normal place
198
What is an irreducible hernia
One that can't be reduced back into it's proper position
* Bowel trapped in herniated position (incarcerated)
199
What can incarceration lead to
* Obstruction - hernia causes a blockage in passage of faeces
* Strangulated - hernia is non-reducible and the base of the hernia becomes so tight it cuts off the blood supply causing ischaemia
200
What is an indirect inguinal hernia
* Bowel herniates through inguinal ring
* passes lateral to inferior epigastric artery
201
What is a direct inguinal hernia
* Hernia protrudes directly through abdominal wall (hesselbach's triangle)
* Passes medial to inferior epigastric artery
* pressure over deep inguinal ring will not stop herniation
202
What are the boundaries of Hesselbach's triangle
RIP:
* Rectus abdominis - medial
* Inferior epigastric vessels - superior/lateral
* Poupart's (inguinal) ligament - inferior
203
What is a femoral hernia
* Herniation of bowel through femoral canal
* protrudes below inguinal ligament
* High risk of strangulation due to rigid femoral canal borders
204
What are hiatus hernias
* Herniation of stomach up through the diaphragm
205
What are the 2 main types of hiatal hernia
* Sliding - stomach slides up through diaphragm
* Rolling - separate portion (fundus) fold around and enters diaphragm
206
How are hiatus hernias investigated
* Barium swallow
* CXR
207
RFs of hiatal hernia
* Pregnancy
* Obesity
* Over 50
208
How are hernias treated
Surgery - tension free
209
What is the most typical pancreatic cancer
Typically adenocarcinoma occurring in head of pancreas
210
Give 4 RFs of pancreatic cancer
* Smoking
* Over 60
* Chronic pancreatitis
* T2DM
211
Describe the presentation of pancreatic cancer
* Painless obstructive jaundice: tumour blocks flow of bile - jaundice, pale stool, dark urine, itching
* Courvoisier's signs - palpable gallbladder and jaundice
* Steatorrhoea
* many present in non-specific way: anorexia, weight loss, epigastric pain
212
Investigation of pancreatic cancer
* GS: Pancreatic CT - mass
* CA 19-9
* abdo US - dilated bile and pancreatic ducts
* Tumour marking - monitor progression
213
How is pancreatic cancer managed
Poor prognosis as usually identified late tends to metastasise early to liver and lungs
* Surgery to remove tumour - pancreaticoduodenectomy (whipple procedure)
* Palliative - chemo/radiotherapy
214
What type of liver cancer is most common
Secondary
MC a result of metastases
215
Define hepatocellular carcinoma (HCC)
Primary hepatocyte neoplasia arising from liver parenchyma
* Accounts for 90% of all primary liver cancers
216
4 causes of HCC
* HBV - mc worldwide
* HCV - mc Europe
* Liver cirrhosis (RF)
* Aflatoxin - poisonous carcinogens produced by moulds often in grains and seeds
217
Where do HCC typically metastasise to
Via hepatic/portal veins
* Lymph nodes
* Bones
* Lungs
218
Signs and symptoms of HCC
* Jaundice
* Ascites
* Weight loss
219
Investigations of HCC
* 1ST: US - identify lesions
* GS: CT - confirmation if lesion is large
* Raised serum alpha-fetoprotein
220
How is HCC treated
* Liver transplant
* Resection
221
Which vaccine should be taken to prevent HCC
HBV vaccine
222
Where are secondary liver tumours typical from
* GIT
* Breast
* Lungs (bronchus)
223
What is cholangiocarcinoma
Biliary tree cancer
224
Causes/ RFs of cholangiocarcinoma
* Infestation with parasitic worms (flukes)
* PSC
* HBV & HCV
* IBD (RF)
225
Signs and symptoms of cholangiocarcinoma
* colicky Abdo pain
* Weight loss
* Palpable mass in RUQ
* Fever
* Jaundice and ascites
Late onset of Sx as tumour is slow growing
226
Investigation of cholangiocarcinoma
* CT and US
* GS: ERCP
* Raised bilirubin and ALP
227
Treated of cholangiocarcinoma
* Surgery not possible in 70% of patients due to late presentation
* ERCP - stenting
228
3 Features of budd-chiari syndrome
Triad of:
*ascites
* abdo pain
* tender hepatomegaly
