Liver Flashcards
1
Q
What are five main functions of the liver?
A
Albumin and clotting factor production, bilirubin metabolism, drug metabolism, detoxification, storage of vitamins/metals.
2
Q
What two broad categories are measures of liver function?
A
LFTs and hepatic enzymes.
3
Q
Which three LFTs are markers of liver function?
A
Bilirubin, albumin and prothrombin time (clotting factors).
4
Q
What are LFTs if there is liver damage and why?
A
Bilirubin is increased, albumin is decreased and prothrombin time is increased.
-Bilirubin isn’t metabolised so remains in the blood.
-Albumin isn’t produced so there are low levels.
-Clotting factors aren’t produced so prothrombin time is increased.
5
Q
Which enzymes are related to liver damage?
A
-Aminotransferases - ALT and AST.
-ALP.
-GGT.
6
Q
Why are aminotransferases high in the blood in liver damage?
A
When hepatocytes are damaged, they leak into the blood.
7
Q
When is ALP (alkaline phosphate) raised?
A
In biliary tree damage.
8
Q
What is liver failure?
A
Liver loses it’s ability to repair and regenerate leading to compensation.
9
Q
What are the types of liver failure?
A
Acute and chronic.
10
Q
What is acute liver failure?
A
The loss of liver function that occurs quickly (days/weeks) in someone with no previous liver disease.
11
Q
What is chronic liver failure?
A
Progressive decline in liver function over 6 months in someone with existing liver disease.
12
Q
What are the three main causes of acute liver failure?
A
Viral (hepatitis, CMV and EBV).
Autoimmune hepatitis.
Paracetamol overdose.
13
Q
What are the four main causes of chronic liver failure?
A
ALD (mc), NAFLD, viral hepatitis, alcohol.
14
Q
What are three other causes of chronic liver failure?
A
Autoimmune (PBC, PSC)
Metabolic (haemochromatosis, Wilson’s, A1ATD)
Malignancy.
15
Q
How does acute liver failure present?
A
Jaundice, nausea, anorexia and malaise.
16
Q
What are the 12 signs/symptoms of chronic liver failure?
A
Jaundice, pruritus, malaise, anorexia.
Oedema, gynecomastia, clubbing, palmar erythema.
Xanthelasma, spider naevi/caput medusae.
Hepatosplenomegaly and easily bruising/bleeding.
17
Q
Explain the prognosis of acute liver failure.
A
90% of acute liver failure leads to recovery.
10% leads to fulminant liver failure.
18
Q
Explain the progression of chronic liver failure.
A
A liver disease either leads to fibrosis and then cirrhosis and eventually liver failure.
-Some will resolve.
-Some will progress to liver failure.
19
Q
What is ESLD and what is it a risk factor for?
A
End stage liver disease which is decompensated cirrhosis.
Big risk factor for HCC.
20
Q
What investigations are done for liver failure?
A
LFTs - inc. bilirubin, dec. albumin, inc. INR.
Imaging and microbiology (investigate infections).
21
Q
How is acute liver failure treated?
A
Acutely with ABCDE, fluid and analgesia.
-Treat the underlying cause and any complications.
22
Q
What are the five main complications of acute liver failure?
A
Increased ICP, HE, ascites, haemorrhage and sepsis.
23
Q
What is the gold standard diagnosis for chronic liver disease?
A
Liver biopsy (determines disease extent).
24
Q
What are two other investigations for chronic liver failure?
A
LFTs and imaging (USS).
25
What is the main treatment for chronic liver failure?
Prevent the progression of the disease by lifestyle modifying (no alcohol, decrease BMI, avoid drugs).
-Manage complications.
26
When would a liver transplant be considered for chronic liver failure?
If decompensated - ESLF.
27
What is liver cirrhosis?
Replacement of hepatocytes with scar tissue as a result of chronic inflammation of the liver.
28
What are the common causes of liver cirrhosis?
ALD, NAFLD, hepatitis B/C.
29
What are the seven other causes of liver cirrhosis?
Autoimmune hepatitis, PBC, PSC, drugs.
Metabolic - Haemochromatosis, Wilson's, A1ATD.
30
What are the 9 symptoms of liver cirrhosis?
-The same as chronic liver failure:
Jaundice, hepatosplenomegaly, spider naevi, palmar erythema, gynecomastia, bruising, ascites, caput medusae.
31
What are the investigations for liver cirrhosis?
The same as chronic liver failure:
-Liver biopsy, bloods (LFTs), USS/CT/MRI - hepatosplenomegaly.
32
What is the definitive treatment for liver cirrhosis?
Liver transplant.
33
In someone with liver cirrhosis, what is screened for and how often?
HCC every 6 months.
34
How is liver cirrhosis managed?
Conservatively with fluids, analgesia, no alcohol and better diet.
-Manage complications.
35
What are the five main complications of liver cirrhosis?
Ascites, portal hypertension and oesophageal varices, HE, HCC, SBP.
36
What is alcoholic liver disease?
The result from the effects of long term excessive consumption of alcohol on the liver.
37
What is the most common cause of liver failure?
Alcoholic liver disease.
38
How are alcohol units calculated?
Strength (ABV) x volume (ml) divided by 1000.
39
What are the three main risk factors for alcohol liver disease?
Chronic alcohol, obesity and smoking.
40
Explain the pathophysiology of alcoholic liver disease.
Steatosis (undamaged fatty liver) - Alcohol hepatitis (Mallory bodies) - Alcohol cirrhosis (micronodular).
41
What are the early symptoms of alcoholic liver disease?
Asymptomatic.
42
What are the symptoms of late stage alcoholic liver disease?
Chronic liver symptoms and alcohol dependence.
43
What is a quick screen for harmful alcohol consumption?
CAGE questions:
-Cut down? (thought)
-Annoyed? (when others comment)
-Guilty?
-Eye opener? (drink in morning to help nerves).
44
How is alcoholic liver disease diagnosed?
History of heavy drinking:
-LFTs with inc. GGT and AST:ALT ratio >2.
-FBC (raised MCV).
-Biopsy to confirm if ALD or hepatitis.
-CT/MRI.
45
How is ALD managed conservatively?
Stop drinking alcohol permanently, healthy diet, lower BMI, vitamins.
46
When would liver transplant be considered in someone with ALD?
With ESLD and abstinence for 3+ months.
47
What are 7 complications of drinking too much alcohol?
Pancreatitis, HE, Wernicke-Korsakoff syndrome, cirrhosis/HCC, Mallory Weiss tear, ALD and alcohol dependence, alcoholic cardiomyopathy.
48
If someone has alcohol dependence, what happens if they stop drinking?
They will develop withdrawal symptoms.
49
Describe the stages of alcohol withdrawal and their symptoms.
6-12h - Tremor, sweating, headache, craving and anxiety.
12-24h - Hallucinations.
24-48h - Seizures.
24-72h - Delirium tremens.
50
What is delirium tremens?
Medical emergency associated with alcohol withdrawal.
51
What is the mortality rate of delirium tremens?
35%.
52
Describe the pathophysiology of delirium tremens.
Alcohol stimulates GABA receptors, relaxing the brain.
Alcohol inhibits glutamate receptors, further relaxing.
Chronic alcohol use results in GABA system being down-regulated and glutamate system being up-regulated to balance the effects of alcohol.
-When alcohol is removed, GABA under-functions and glutamate over-functions causing extreme excitability in the brain (excess adrenergic activity).
53
How does delirium tremens present?
Neuro - confusion, agitation, delusion/hallucination, ataxia, tremor.
-Tachycardia, hyperthermia, arrhythmias, hypertension.
54
How is alcohol withdrawal managed?
With benzodiazepines and high dose IV B, followed by oral thiamine.
55
What is not-alcoholic fatty liver disease?
Chronic liver disease due to fat deposits in the hepatocytes (not due to alcohol).
56
What are seven risk factors for NAFLD?
Obesity, hypertension, T2DM, drugs, FHx, endocrine disorders, hyperlipidaemia.
57
What are the stages of NAFLD?
1. NAFLD
2. Non-alcoholic steatohepatitis
3. Fibrosis
4. Cirrhosis
58
How does NAFLD present?
Typically Asx.
-If very severe, signs of chronic liver failure.
59
How is NAFLD diagnosed?
1st line - Imaging (USS).
Bloods and LFTs.
60
How is NAFLD treated?
Weight loss, exercise, stop smoking, control diabetes, BP and cholesterol, avoid alcohol.
61
What is hepatitis? What are the different types?
Inflammation of the liver.
Can be chronic or acute and viral or autoimmune.
62
What causes acute hepatitis?
-Viral (Hep A-E, herpes viruses etc.).
-Other infection (Bacteria, parasites).
-Autoimmune hep, metabolic.
-Drugs, pregnancy.
-NAFLD.
63
What causes chronic hepatitis?
-Viral hepatitis.
-Drugs, autoimmune hep, metabolic.
-NAFLD.
64
Describe the hepatitis A infection.
Acute and mild, self limiting with no chronic disease.
-100% immunity after infection.
65
Does hepatitis A cause fulminant liver failure?
Very rarely.
66
How is hepatitis A spread?
Faeco-oral spread, associated with contaminated food and water
67
What are the three main risk factors for hepatitis A?
Overcrowding, poor saturation, exotic travel (Africa).
68
How does hepatitis A usually present?
Usually symptomatic:
-Malaise, fever, N+V, jaundice.
69
How is hepatitis A diagnosed?
Bloods - leukopenia and increased ESR.
LFTs - Increased bilirubin.
HAV serology (anti-HAV antibodies IgM and IgG).
70
What is the management for hepatitis A?
Supportive treatment:
-Monitor LFTs.
-Travel vaccine (primary prevention).
71
Describe the genetics of the hepatitis A virus.
RNA virus.
72
Describe the genetics of the hepatitis B virus.
DNA virus.
73
Describe the hepatitis B infection.
Acute and chronic virus that is blood borne.
74
How is hepatitis B spread?
Via blood and body fluids:
-Vertical transmission.
-Needles.
-Sexually.
-Contaminated household.
75
What are the risk factors for hepatitis B infection?
IVDU, gay sex, healthcare workers.
76
Describe the progression of hepatitis B infection.
Most people fully recover within two months.
10% becomes chronic hepatitis B carriers which can lead to cirrhosis, liver failure and risk of HCC.
77
How is hepatitis B prevented?
Vaccination.
78
Which antibodies is tested for in HepB screening?
HBcAb (core antibody) for previous infection.
HBsAb (surface antibody) for current infection.
79
If hepatitis B screening is positive, what is further tested?
-HBeAg (antigen) that implies infectiousness/viral replication.
-HBV DNA (DNA) which counts direct viral load.
80
What is given in a hepatitis B vaccine?
HBsAg is given in the vaccine.
81
What does presence of HbsAb in the blood imply?
An immune response against HbsAg so vaccination or previous/current infection.
82
What marker of Hepatitis B can be helpful in distinguishing the level of infection?
HbcAb (core antibodies):
-IgM of it implies current infection.
-IgG of it implies past infection (when HbsAg is negative).
83
What does positive HBeAg mean in a hepatitis B infection?
Implies the patient is in the acute phase of the infection and the virus is replicating.
84
In hepatitis B, what does negative HBeAg and positive HBeAb signify?
The active phase has stopped, the virus has stopped replicating.
-Less infectious.
85
In Hepatitis B serology, what is the marker for an active infection?
HBsAg (surface antigen).
86
In hepatitis B serology, what implies vaccination or past infection?
HBsAb (surface antibody).
87
In hepatitis B serology, what is a marker of viral replication and a marker for infectivity?
HBeAg (E antigen).
88
In hepatitis B serology, what signifies the active phase is finished?
HBeAb (E antibody).
89
In hepatitis B serology, what signifies past and current infection?
HBcAb (core antibodies).
-IgM is active.
-IgG is past.
90
In hepatitis B serology, what is a markers of direct viral load?
HBV DNA.
91
What are the investigations for hepatitis B?
LFTs and serology.
92
How is hepatitis B managed?
Vaccine is available.
Antivirals if needed.
-Improve diet and lifestyle.
-Treat complications.
93
Describe the hepatitis D infection.
Acute and chronic, blood borne RNA virus which can only survive if someone is infected with Hepatitis B.
94
Why does hepatitis D only survive if a patient is already infected with hepatitis B?
It attaches to HBsAg and uses HBV to replicate.
95
How common is hepatitis D in the UK?
Very low rates.
96
What are the complications of hepatitis D?
Will make hepatitis B infection worse and increase the chances of cirrhosis.
97
How is hepatitis D treated?
No specific treatment.
98
Describe the hepatitis C virus/infection.
Acute and chronic blood borne RNA virus.
99
Is there a vaccine available for hepatitis C?
No.
100
What is the most common hepatitis virus in the UK?
Hepatitis C.
101
How is hepatitis C spread?
Blood borne - IVDU, vertical transmission, sex.
102
Describe the prognosis of hepatitis C.
25% make a recovery.
75% progress to liver cirrhosis and can turn into HCC.
103
How is hepatitis C diagnosed?
LFTs and serology:
-HCV antibody.
-HCV RNA.
104
What is the management for hepatitis C?
Treated with directly acting antivirals.
-Improve lifestyle.
-Treat complications.
105
Is hepatitis E common in the UK?
No, very rare.
106
Describe hepatitis E.
RNA virus spread via the faeco-oral route.
107
How severe is hepatitis E? Does it need treatment?
Not severe, only producing a mild illness which can be asymptomatic.
-No treatment required.
-No vaccine.
108
How often does hepatitis E progress to chronic liver disease?
Tends to only be in those who are immunocompromised.
109
Which hepatitis infections are notifiable diseases?
All of them.
110
Compare all viral hepatitis infections.
A - RNA, faeco-oral. 100% immunity,
B - DNA, blood-borne. 10% fulminant liver failure.
C - RNA, blood-borne. 75% fulminant liver failure.
D - RNA, blood-borne. Binds to HBVsAg to survive.
E - RNA, faeco-oral. 100% immunity.
111
What is autoimmune hepatitis?
A T-cell mediated response against hepatocytes which results in chronic hepatitis.
112
What causes autoimmune hepatitis?
No clear cause, thought to be genetic predisposition and triggered by viral infection.
113
How rare is autoimmune hepatitis?
Very rare cause of chronic hepatitis.
114
What are the four risk factors for autoimmune hepatitis?
Females, autoimmune diseases, viral hepatitis, HLADR3/4.
115
What are the types of autoimmune hepatitis?
Type 1 - Adult females (40s/50s). Less acute.
Type 2 - Young females (teens/20s). Acute.
116
Which autoantibodies are present in type 1 autoimmune hepatitis?
1. Anti-nuclear autoantibodies (ANA).
2. Anti-smooth muscle autoantibodies (ASMA).
3. Anti-soluble liver antigen (ASLA/LP).
117
Which autoantibodies are present in type 2 autoimmune hepatitis?
1. Anti-liver kidney microsomes-1 (ALKM1).
2. Anti-liver cytosol antigen type 1 (ALC1).
118
How is autoimmune hepatitis diagnosed?
Liver biopsy and serology (for autoantibodies).
119
What are the symptoms/signs of autoimmune hepatitis?
25% asymptomatic.
-Jaundice, fever, hepatosplenomegaly.
120
How is autoimmune hepatitis treated?
High dose steroids (prednisolone).
-Hepatitis vaccinations.
-If ESLD, liver transplant.
121
What is biliary tract disease?
Three main conditions that affect the biliary tree:
-Gallstones.
-Cholecystitis.
-Ascending cholangitis.
122
In biliary tract disease, when do you get RUQ pain?
All 3 conditions:
-Gallstones
-Cholecystitis
-Ascending cholangitis
123
In biliary tract disease, when do you get fever?
With ascending cholangitis and cholecystitis.
124
In biliary tract disease, when do you get jaundice?
With ascending cholangitis.
125
What is biliary colic?
The temporary blockage of the cystic or common bile duct by gallstones which causes pain.
126
What are the contents of gallstones?
Cholesterol (80%), pigment, bilirubin, bile salts.
127
What are the risk factors for biliary colic?
5Fs - Fat, forty, female, family history, fertile.
-T2DM, NAFLD.
128
What are the symptoms of gallstones?
RUQ pain often after fatty meals.
-Referred shoulder pain and N+V.
129
How are gallstones diagnosed?
Abdo USS - thick wall, stones and duct dilation.
130
How are gallstones managed?
Elective laparoscopic cholecystectomy if symptomatic.
-Analgesia for pain and decrease fat in diet.
131
What is cholecystitis?
Blockage of the cystic duct which causes a buildup of bile and causes inflammation of the gallbladder.
132
How does cholecystitis present?
RUQ pain with fever and referred pain to tip of right shoulder.
133
How is cholecystitis diagnosed?
FBC, CRP and LFTs show leukocytosis and neutropenia.
-Positive Murphy sign - press on gallbladder and ask patient to inhale - PAIN.
-Abdo USS - thick gallbladder wall.
134
How is cholecystitis managed?
Laparoscopic cholecystectomy within a week.
-IV fluid, analgesia, IV antibiotics.
135
What is ascending cholangitis?
Prolonged bile duct blockage so instead of being flushed into GI tract by bile, bacteria ascend through the biliary tree which can lead to sepsis.
136
What is the presentation of ascending cholangitis?
Charcot's triad - RUQ pain, fever and jaundice (post - dark piss, pale shite).
If sepsis - Reynold' pentad (Charcot's triad, confusion, sepsis).
137
What is the gold standard investigation for ascending cholangitis?
MRCP - magnetic resonance cholangio pancreatography.
138
What is the first line investigation for ascending cholangitis?
USS abdo.
139
What other investigations are done for ascending cholangitis?
Bloods: LFTs, FBC, CRP.
-Blood cultures for sepsis.
140
What is the treatment for ascending cholangitis?
ERCP (Endoscopic retrograde cholangiopancreatography) and laparoscopic cholecystectomy.
141
What is jaundice?
The yellowing of the skin and eyes due to the accumulation of bilirubin.
142
What are the types of jaundice?
Pre-hepatic.
Hepatic.
Post-hepatic.
143
How does pre-hepatic jaundice present?
Normal urine and stool colour, no itching and generally normal LFTs.
144
How does hepatic and post-hepatic jaundice present?
Dark urine, pale stools, can itch, abnormal LFTs.
145
What causes pre-hepatic jaundice?
Increased RBC breakdown - Gilbert's syndrome and haemolytic anaemias:
-Sickle cell, G6DP deficiency, thalassaemia, malaria.
146
What causes hepatic jaundice?
Disease of the liver itself:
-HCC, ALD, NAFLD, hepatitis, drugs.
147
What causes post-hepatic jaundice?
Biliary tree obstruction:
-Pancreatic cancer, autoimmune (PBC, PSC), gallstones.
148
Explain the pathophysiology of pre-hepatic jaundice.
Increased RBC breakdown overwhelms the liver's ability to conjugate bilirubin, causing unconjugated bilirubinemia.
149
Explain the pathophysiology of intra-hepatic jaundice.
Dysfunction of the hepatocytes and they lose their ability to conjugate bilirubin. Causing both unconjugated and conjugated bilirubinaemia.
150
Explain the pathophysiology of post-hepatic jaundice.
Obstruction of biliary tree, so the bilirubin that is excreted has already been to the liver so this causes conjugated bilirubinaemia.
151
What is primary biliary cholangitis?
The progressive autoimmune destruction of the small bile ducts within the liver. This obstructs the outflow of bile leading to cholestasis and eventually fibrosis, cirrhosis and liver failure.
152
What are the four risk factors for primary biliary cholangitis?
Female, 40-50y, other autoimmune disease, smoking.
153
How does primary biliary cholangitis present?
Initially asymptomatic.
-Pruritus, itching, jaundice, hepatosplenomegaly.
-Xanthelasma and xanthoma (lipid deposits on skin).
154
Explain the pathophysiology of the symptoms of primary biliary cholangitis.
Bile usually contains bile salts, cholesterol and bilirubin. When the outflow of bile is obstructed, these build up.
-Bile salts cause itching.
-Cholesterol deposits in the skin and causes xanthoma/xanthelasma.
- Bilirubin causes jaundice.
155
How is primary biliary cholangitis diagnosed?
1. LFTs - alkaline phosphatase, bilirubin.
2. Liver biopsy.
3. Autoantibodies (AMA, ANA).
156
What is the management for primary biliary cholangitis?
1. Ursodeoxycholic acid (reduces cholestasis).
2. Cholestyramine (binds to bile to prevent absorption in gut, helps pruritus).
3. Steroids considered in some.
4. ESLD - liver transplant.
157
What is primary sclerosing cholangitis?
When the intrahepatic and extrahepatic ducts become stiff and fibrotic.
This leads to the obstruction of bile outflow and eventually hepatitis, fibrosis and cirrhosis.
158
Which condition is associated with primary sclerosing cholangitis?
Ulcerative colitis (70% of people).
159
What causes primary sclerosing cholangitis?
Unclear.
-Thought to be a combination of genetic, autoimmune and environmental.
160
What are the four risk factors for primary sclerosing cholangitis?
Male, 30-50, ulcerative colitis and FHx.
161
How does primary sclerosing cholangitis present?
Initially asymptomatic:
-Jaundice, fatigue, pruritus, RUQ pain, hepatosplenomegaly.
162
How is PSC diagnosed?
GS - MRCP.
LFTs show 'cholestatic' - ALP raised first.
Serology - pANCA positive in 95%, ANA in 75%.
163
How is PSC managed?
1. Cholestyramine (binds to bile to prevent it being absorbed).
2. ERCP - dilation and stenting of ducts.
3. Manage complications.
4. If ESLD - liver transplant.
164
What is pancreatitis?
Inflammation of the pancreas.
165
What are the types of pancreatitis?
Acute and chronic pancreatitis.
166
What is acute pancreatitis?
Rapid onset of pancreas inflammation and symptoms. Normal function usually returns after.
167
What is chronic pancreatitis?
Long-term inflammation and symptoms with a progressive and permanent decrease in pancreas function.
168
What are the three main causes of acute pancreatitis?
Gallstones, alcohol, post-ERCP.
169
How do gallstones cause acute pancreatitis?
Gallstones obstruct flow of pancreatic secretions and digestive enzyme accumulate in pancreas. Ca2+ is released, activates trypsinogen and autodigestion.
170
How does alcohol cause pancreatitis?
Directly toxic to pancreatic cells, resulting in inflammation.
-Also contracts ampulla of vater.
171
What are the causes of acute pancreatitis? (acronym).
IGETSMASHED:
Idiopathic, gallstones, ethanol (alcohol), trauma, steroids, mumps/malignancy, autoimmune, scorpion venom, hyperlipidaemia, ERCP, drugs.
172
How does someone with acute pancreatitis present?
Sudden severe epigastric pain which radiates to the back.
-Jaundice, pruritus, N+V, tachycardia.
-Grey turner and Cullen sign - bleeding.
173
How is pancreatitis diagnosed?
GS - Bloods (increased serum amylase/lipase, CRP).
-CT/USS to assess cause/complications.
174
How is acute pancreatitis managed?
ABCDE - NBM, IV fluids, analgesia, prophylactic Abx.
-Treat cause (gallstones - ERCP).
175
What are three complications to acute pancreatitis?
Infection, abscess, chronic pancreatitis.
176
What is the most common cause of chronic pancreatitis?
Alcohol.
177
How does chronic pancreatitis present?
The same as acute pancreatitis but usually less intense and more longer lasting.
178
What are features of chronic pancreatitis that don't manifest in acute pancreatitis?
Exocrine and endocrine dysfunction, abscess formation and chronic epigastric pain.
179
How is chronic pancreatitis managed?
1. Abstaining from alcohol and smoking.
2. Analgesia for pain (NSAIDs).
3. Replace enzymes and hormones that.
4. Treat complications.
180
What is ascites?
Accumulation of fluid in the peritoneal cavity.
181
What are the four main causes of ascites?
Liver cirrhosis, malignancy (GI, ovary, lymphoma), heart failure, nephrotic syndrome.
182
How does ascites present?
Abdominal distension, weight gain, respiratory distress, N+V, discomfort.
-Signs of underlying cause.
183
How is ascites diagnosed?
Examination:
-Shifting dullness to flanks.
-Ascitic tap and cytology.
184
How is ascites managed?
Treat underlying cause.
-Spironolactone, loop diuretics.
-Paracentesis.
-Surgery and liver transplant.
185
What is the main complication of ascites? In how many people does it occur and what is the mortality rate?
Spontaneous bacterial peritonitis (SBP):
-Occurs in 20% of patients and mortality rate is 20%.
186
What is Wernicke's encephalopathy?
A degenerative brain disorder caused by the lack of thiamine (vitamin B1) which usually results from alcohol abuse.
187
What usually causes Wernicke's encephalopathy?
Alcohol abuse as vitamin B1 is poorly absorbed in the presence of alcohol and they tend to have poor diets.
188
How does someone with Wernicke's encephalopathy present and why?
With confusion, ataxia, and oculomotor disturbances as B1 deficiency causes damage to the brain.
189
Is Wernicke's encephalopathy permanent?
No, it can be reversed if treated fast.
190
What is Korsakoff syndrome? What usually precedes it?
An irreversible disabling memory disorder and form of dementia that results from vitamin B1 deficiency and alcoholism.
-Occurs after Wernicke's encephalopathy.
191
How does Korsakoff syndrome present?
With memory impairment and behavioural changes.
192
Explain the pathophysiology of Korsakoff syndrome.
Lack of vitamin B1 damages both the nerve cells and supporting cells in the brain and spinal cord.
193
What is hepatic encephalopathy?
Changes in the brain that occur in patients with liver disease due to the build up of toxins (mostly ammonia).
194
How does hepatic encephalopathy usually present?
With personality changes, intellectual impairment, impaired memory and coma.
195
What is the most common cause of hepatic encephalopathy?
Chronic liver disease.
196
How is hepatic encephalopathy treated?
-Laxatives - helps to clear ammonia from the gut before being absorbed.
-Antibiotics - reduces bacteria in gut producing ammonia.
197
What are three common metabolic liver diseases?
1. Haemochromatosis.
2. Wilson's disease.
3. A1ATD.
198
What is haemochromatosis?
Iron storage disorder that results in excessive total iron in the body and iron deposits in the tissues.
199
Explain the pathophysiology of haemochromatosis.
Autosomal recessive mutation of HFE gene on chromosome 6. HFE gene usually regulates iron storage. When the gene is faulty the storage of iron is impaired.
200
Who is haemochromatosis more common in?
Men as women lose iron in menstruation.
201
What are the nine signs/symptoms of haemochromatosis?
Fatigue, joint pain, hypogonadism, slate grey/bronze skin, osteoporosis, heart failure, hair loss and memory/mood disturbance.
202
How is haemochromatosis diagnosed?
Iron studies:
-High serum ferritin.
-High transferrin saturation.
Genetic testing for HFE gene, liver biopsy with Perl's stain.
CT/MRI.
203
How is haemochromatosis treated?
1.Venesection (to remove iron) and iron chelation.
2. Avoid alcohol.
3. Monitor and treat complications.
204
What are six complications of haemochromatosis?
T1DM, liver cirrhosis, cardiomyopathy, HCC, hypothyroidism, pseudogout.
205
What is Wilson's disease?
Excessive accumulation of copper in the body and tissues.
206
Explain the pathophysiology of Wilson's disease.
Autosomal recessive mutation of ATP7B gene on chromosome 13. Defective enzyme and impaired copper secretion.
207
What are the typical patients with Wilson's disease?
Young (20y) with family history.
208
How does Wilson's disease present?
-Neuro problems - parkinsonism, memory issues, dystonia, dysarthria.
-Psychiatric - From depression to psychosis.
-Hepatic - Hepatitis and cirrhosis.
-Ophthalmic - Kayser Fleischer rings.
-Other - Haemolytic anaemia, osteopenia, RTA.
209
How is Wilson's disease diagnosed?
Serum caeruloplasmin is low, low serum copper.
GS - Liver biopsy.
-24h urine copper assay.
210
How is Wilson's disease treated?
Copper chelation with penicillinamine and trientine.
211
What is the last resort treatment for Wilson's disease?
Liver transplant.
212
What is alpha-1 antitrypsin deficiency?
A deficiency of alpha-1 antitrypsin enzymes caused by an autosomal recessive mutation of protease inhibitor gene on chromosome 14.
213
Explain the pathophysiology of A1ATD.
A1AT usually inhibits neutrophil elastase enzymes which digest connective tissues.
If there is decreased A1AT, there will be increased NE which affects the lungs and the liver.
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How does A1ATD affect the lungs?
Lack of A1AT leads to excess protease enzymes which attack connective tissue in the lung and causes bronchiectasis and emphysema.
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How does A1ATD affect the liver?
A mutant version of A1AT is produced and builds up in the liver and causes damage, eventually cirrhosis and then risk of HCC.
216
How does A1ATD present?
A young/middle aged male with little/no smoking history with COPD like symptoms:
-SOB, chronic cough and sputum.
-May have liver Sx (jaundice).
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How is A1ATD diagnosed?
-Serum A1AT is decreased.
-Liver biopsy - cirrhosis and acid-Schiff-positive staining globules.
-CT thorax - emphysema and bronchiectasis.
-Genetic test for gene.
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How is A1AT disease managed?
Stopping smoking, managing symptoms.
-If ESLD consider liver transplant.
-Monitor for complications (HCC).
219
What is portal hypertension?
Increased pressure in the pressure in the portal vein.
220
What are the three main groups of causes for portal hypertension?
Prehepatic, hepatic and post-hepatic.
221
What is the main cause of pre-hepatic portal hypertension?
Portal vein thrombosis.
222
What are the two causes of hepatic portal hypertension? (UK vs worldwide).
-Liver cirrhosis (mc in UK).
-Schistomiasis (mc world).
223
What are the two main post-hepatic causes of portal hypertension?
Budd Chiari, right sided heart failure.
224
Explain how liver cirrhosis leads to portal hypertension and oesophageal varices.
Cirrhosis causes increased resistance to flow so dilation and increased CO to compensate, there is a fluid overload in the portal vein which means blood shunts to the gastroesophageal vein, causing oesophageal varices.
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How does portal hypertension present?
Mostly asymptomatic until oesophageal varices rupture.
226
How often does portal hypertension cause oesophageal varices and how many rupture?
90% cause varices,
1/3rd rupture.
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What are oesophageal varices?
Thin dilated veins that develop in the lining of the lower oesophagus.
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When are oesophageal varices symptomatic?
When they rupture.
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What is the most common complication of oesophageal varices?
Rupture and bleeding.
230
How are oesophageal varices diagnosed?
Oesophagogastroduodenoscopy (OGD - upper GI).
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How do patients with ruptured oesophageal varices present?
With hematemesis (vomiting blood) and melaena (black turds due to upper GI bleeding).
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How are bleeding oesophageal varices treated?
IV terlipressin, variceal banding, vitamin K.
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How are stable oesophageal varices treated?
Beta-blockers, nitrates and banding.
-TIPS (transjugular intrahepatic portosystemic shunt) - shunt from portal vein to hepatic vein to relieve pressure on portal system.
234
What is perotinitis?
Inflammation of the peritoneum.
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What are the causes of perotinitis?
Primary - SBP (mc) and ascites.
Secondary - due to underlying cause.
236
What are the bacterial causes of SBP?
Staph aureus, E. coli and klebisella.
237
What are three chemical causes of peritonitis?
Ruptured ectopic pregnancy, intestine perforation, bile.
238
How does peritonitis usually present?
Sudden onset severe abdo pain.
-Then collapse, septic shock, fever.
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How is peritonitis diagnosed?
Ascitic tap shows neutrophilia, cultures show causative organisms.
-Bloods: Increased ESR and CRP.
-Exclusion of pregnancy and bowel obstruction.
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How is peritonitis treated?
Emergency - ABCDE, IV fluid and IV antibiotics.
-Treat underlying cause.
241
What are two complications of peritonitis?
Sepsis and abscesses.
242
Explain the pathophysiology of pancreatic cancer.
Adenocarcinoma of exocrine pancreas of ductal origin.
243
In pancreatic cancer, which areas are most commonly affected?
60% head, 25% body, 15% tail.
244
Who is pancreatic cancer most common in?
Males above 60.
245
What are five risk factors for pancreatic cancer?
Smoking, alcohol, diabetes, family history, chronic pancreatitis.
246
What is the average 5y survival for pancreatic cancer?
3%.
247
Why is pancreatic cancer dangerous?
It metastasises early and quickly.
248
What is the key presenting symptom of pancreatic cancer?
Painless obstructive jaundice.
-With jaundice, pale stools, dark urine and itching.
249
What are six other signs/symptoms of pancreatic cancer?
N+V, abdo pain, Courvoisier sign (palpable GB and jaundice), change in bowel habit, weight loss, new onset diabetes or worsening of T2DM.
250
What are the investigations for pancreatic cancer? 1st line and GS?
Abdo USS (1st),CT scan (GS).
-Pancreatic biopsy.
-CA19-9 tumour marker may be raised.
251
How is pancreatic cancer treated?
Surgery considered in small tumours.
-Chemo and palliative care.
252
What is Gilbert's syndrome?
Hereditary jaundice caused by autosomal recessive mutation in UDP1A1 gene which leads to UGT enzyme deficiency.
253
What are the two risk factors for Gilbert's syndrome?
Male, T1DM.
254
Describe the pathophysiology of Gilbert's syndrome.
UGTs are enzymes that catalyse phase II glucuronidation reactions in the liver so they bilirubin isn't metabolised, leading to unconjugated hyperbilirubinaemia and jaundice.
255
What is the most common cause of hereditary jaundice?
Gilbert's syndrome.
256
What are the symptoms of Gilbert's syndrome?
Presents with painless jaundice at a young age.
30% asymptomatic.
257
What is Criggler Najjar?
A more severe and rare hereditary jaundice.
258
How is Gilbert's syndrome and Criggler Najjar treated?
Gilbert's - no treatment.
CN - phototherapy (breaks down bilirubin).
259
What are the primary liver cancers?
Hepatocellular carcinoma (80%) and cholangiocarcinoma (20%).
260
What are the most common primary locations for cancers to metastasise to the liver?
GI tract, breast, lung.
261
Is primary or secondary lung cancer more common?
Secondary.
262
What are six risk factors for primary liver cancer?
Liver cirrhosis, hepatitis (viral/auto), alcohol, ALD, NAFLD, haemochromatosis.
263
What are three main signs of liver cancer?
-Decompensated liver failure (jaundice, ascites, HE).
-Signs of cancer (TATT, weight loss, night sweats).
-Hepatosplenomegaly.
264
What are three symptoms of liver cancer?
Non-specific symptoms - abdo pain, anorexia, N+V.
265
What are two risk factors for cholangiocarcinoma?
Parasites, PSC.
266
What investigations are used for liver cancer? 1st line and GS?
GS - ERCP (chol), CT (HCC).
1st - USS.
Tumour markers:
-Alpha-feroprotein (HCC), CA19-9 (chol).
267
How is liver cancer treated?
Surgical resection of tumour.
Chemo, radio, stents.
268
What are two benign liver cancers?
Haemangioma and hepatic adenoma.
269
What is a hernia?
Protrusion of an organ through defect in its containing cavity (usually the bowel).
270
What are the six types of hernia?
Inguinal, femoral, hiatal, epigastric, incisional, umbilical.
271
What can hernias be?
Reducible - pushed back into place.
Irreducible - obstructed, strangulated, incarceration.
272
What is the curative option of hernias?
Surgery.
273
What is a hiatal hernia?
When the stomach herniates through the diaphragm aperture.
274
What is the most common type of hernia?
Inguinal (direct and indirect).
275
How would you diagnose ALD with LFTs?
AST:ALT ratio is 2:1.
276
What is Wernicke's encephalopathy triad?
Ataxia (wide base), encephalopathy (confusion) and ophthalmoplegia (not following finger with eyes).
