Liver and Friends Flashcards
1
Q
What are the 7 functions of the liver?
A
- Oestrogen regulation
- Detoxification
- Metabolism of carbohydrates
- Albumin production
- Clotting factor production
- Bilirubin regulation
- Immunity
2
Q
What is alcoholic liver disease?
A
Liver damage caused by excess alcohol intake
3
Q
Describe the epidemiology of alcoholic liver disease
A
- Most common cause of chronic liver disease in the western world
- Usually presents in men in 40s-50s
4
Q
What are 2 causes of alcoholic liver disease?
A
- Excessive consumption of alcohol (main)
- Some genetic predisposition
5
Q
Describe the order of alcoholic liver disease
A
Fatty liver (steatosis) –> alcoholic hepatitis –> cirrhosis
6
Q
Describe the pathophysiology of fatty liver (steatosis)
A
- Metabolism of alcohol produces fat in the liver
- Cells become swollen with fat with large amounts of alcohol
- Fat disappears on alcohol abstinence
- Alcohol directly affects stellate cells (fat-storing cells) which are transformed into collagen-producing myofibroblast cells
7
Q
Describe the pathophysiology of alcoholic hepatitis
A
- Fatty change and infiltration by polymorphonuclear leukocytes and hepatocyte necrosis
- Presence of mallory bodies and giant mitochondria
8
Q
What are 4 signs of alcoholic liver disease?
A
- Hepatomegaly
- Ascites
- Rapid onset jaundice
- Encephalopathy
9
Q
What are the symptoms of fatty liver (alcoholic liver disease)?
A
Usually asymptomatic
10
Q
What are 4 symptoms of alcoholic hepatitis (alcoholic liver disease)?
A
- Nausea and vomiting
- Diarrhoea
- Anorexia
- RUQ pain
11
Q
What is a symptom of alcoholic cirrhosis (alcoholic liver disease)?
A
May be asymptomatic
- Spider naevi (clusters of RBCs visible under skin)
12
Q
What are 8 investigations for patients with alcoholic liver disease?
A
- LFTs
- FBC
- PTT (increased)
- Abdominal ultrasound
- Fibroscan
- Endoscopy
- CT/MRI
- Liver biopsy
13
Q
Describe LFT results for patients with alcoholic liver disease
A
- GGT = very raised
- AST:ALT = raised ratio (2:1)
14
Q
Describe FBC results for patients with alcoholic liver disease
A
- Macrocytic anaemia (elevated MCV)
- Thrombocytopenia
- Hypoglycaemia
15
Q
What is the recommended alcohol consumption?
A
- No more than 14 units per week for both men and women
- No more than 5 units in a day
16
Q
How do you calculate the number of units of alcohol?
A
Strength (ABV) x volume (ml) / 1,000
17
Q
What is the treatment for alcoholic liver disease?
A
- Lifelong abstinence from alcohol
- Corticosteroids (to control inflammation)
- IV thiamine
- Diet high in vitamins and proteins
- Liver transplant
18
Q
What are 5 complications of alcoholic liver disease?
A
- Liver failure
- Wernicke-Korsakoff encephalopathy
- Acute/chronic pancreatitis
- Mallory-Weiss tear
- Increased risk of cancers (particularly breast/mouth/throat)
19
Q
What is non-alcoholic fatty liver disease?
A
Range of conditions caused by a build-up of fat in the liver
20
Q
Describe the epidemiology of non-alcoholic fatty liver disease
A
25% of population
21
Q
What are 3 risk factors for non-alcoholic fatty liver disease?
A
- Obesity
- Diabetes
- Hyperlipidaemia
22
Q
Describe the order of non-alcoholic fatty liver disease
A
Healthy –> steatosis –> steatohepatitis –> fibrosis –> cirrhosis
23
Q
What are 2 signs of non-alcoholic fatty liver disease?
A
- Hepatomegaly
- Jaundice
24
Q
What are 5 symptoms of non-alcoholic fatty liver disease?
A
Asymptomatic
1. Nausea and vomiting
2. Diarrhoea
3. Fatigue
4. Malaise
5. RUQ pain
25
What are 4 investigations for patients with non-alcoholic fatty liver disease?
1. LFTs (raised ALT and sometimes AST)
2. CT/MRI
3. Ultrasound
4. Liver biopsy
26
What is the treatment for non-alcoholic fatty liver disease?
- Reduce weight
- No effective drug treatments
27
What is autoimmune hepatitis?
Liver inflammation due to the immune system attacking hepatocytes
28
Describe the epidemiology of autoimmune hepatitis
- Type 1 occurs in adults (typically women in 40s-50s around/after menopause)
- Type 2 occurs in children (typically teenagers/early 20s)
29
What are 4 signs and symptoms of autoimmune hepatitis?
1. Hepatosplenomegaly
2. Fatigue
3. Fever
4. Jaundice
30
What are 5 investigations for patients with type 1 autoimmune hepatitis?
1. Biopsy
2. Raised ALT and AST
3. Anti-nuclear antibodies (ANA)
4. Anti-smooth muscle antibodies (acin-acin)
5. Anti-soluble liver antigen (anti-SLA/LP)
31
What are 4 investigations for patients with type 2 autoimmune hepatitis?
1. Biopsy
2. Raised ALT and AST
3. Anti-liver kidney microsomes-1 (anti-LKM1)
4. Anti-liver cytosol antigen type 1 (anti-LC1)
32
What is the treatment for autoimmune hepatitis?
- High dose steroids e.g. prednisolone, azathioprine
- Usually successful in inducing remission but is required life long
- Liver transplant (can recur)
33
What is viral hepatitis?
Inflammation of the liver as a result of direct viral infection
34
Describe the epidemiology of Hepatitis A
- Most common viral hepatitis worldwide
- Common in Africa and South America
35
How is Hepatitis A spread?
- Faecal oral route (facilitated by overcrowding and poor sanitation)
- Arises from ingestion of contaminated food/water e.g. shellfish
36
What are 3 risk factors for Hepatitis A?
1. Shellfish
2. Travellers
3. Food handlers
37
Describe the pathophysiology of Hepatitis A
- Small, unenveloped RNA virus (picornavirus)
- Incubation period of 28 days
- Viral replication occurs in infected hepatocytes
- Hepatocytes destroyed due to immune response and viral life cycle
- = acute hepatitis
- Viral particles excreted in bile then faeces
38
What are 3 signs of Hepatitis A?
1. Cholestasis
2. Jaundice (after 2 weeks)
3. Hepatomegaly (after 2 weeks)
39
What are 4 symptoms of Hepatitis A?
1. Nausea and vomiting
2. Anorexia
3. Abdominal pain
4. Dark urine and pale stool (after 2 weeks)
40
What are 2 investigations for patients with Hepatitis A?
1. Raised ALT
2. Antibody to HAV (IgM)
41
What is the treatment for Hepatitis A?
- Resolves without treatment in 1-3 months
- Treat symptoms = analgesia
- Avoid alcohol
- Vaccination
42
What are 2 complications of Hepatitis A?
1. Cholestatic hepatitis
2. Acute liver failure (rare)
43
How is Hepatitis B spread?
Direct contact with blood/bodily fluids (needles, sexual, IV drugs users, vertical transmission etc.)
44
What are 6 risk factors for Hepatitis B?
1. Healthcare personnel
2. Emergency/rescue teams
3. CKD/dialysis patients
4. Travellers
5. Homosexual men
6. IV drug users
45
Describe the pathophysiology of Hepatitis B
- Enveloped DNA virus
- HBsAg produced in excess by infected hepatocytes
- Virus penetrates into hepatocytes and loses its coat
- Virus core is transported to nucleus without processing
= acute and chronic hepatitis
46
What are 2 signs of Hepatitis B?
1. Jaundice
2. Hepatomegaly
47
What are 3 symptoms of Hepatitis B?
1. Flu-like symptoms
2. Fever
3. Pruritus (itchy skin)
48
What are 3 investigations for patients with Hepatitis B?
1. Blood = raised ALT
2. Antibody tests:
- HBsAG (Hep B surface antigen)
- HBsAb (Hep B surface antibody)
49
What is the treatment for Hepatitis B?
- Most people fully recover from infection within 2 months
- Vaccination
- Stop smoking and drinking alcohol
- 10% go on to become chronic hepatitis B carriers
- Antiviral medication e.g. pegylated interferon-alpha 2a (48 week long treatment course)
50
What are chronic hepatitis B carriers?
Hepatitis B virus DNA has integrated into the host's DNA so viral proteins continue to be produced
51
What are 2 complications of Hepatitis B?
- Cirrhosis and chronic infection --> hepatocellular carcinoma
- Fulminant hepatic failure (rare)
52
Describe the epidemiology of Hepatitis C
Six numbered genotypes = no.1 affects 50% of cases, no.2 and 3 make up 40%
53
How is Hepatitis C spread?
Via blood/bodily fluids (e.g. poorly sterilised instruments, shared needles etc.)
54
Describe the pathophysiology of Hepatitis C
- Enveloped RNA virus
- Incubation period of 6-9 weeks
- Chronic infection causes a slowly progressive fibrosis over years
55
What are 3 symptoms of Hepatitis C?
Often asymptomatic in acute phase but in chronic:
1. Malaise
2. Weakness
3. Anorexia
56
What are 2 investigations for patients with Hepatitis C?
1. Hepatitis C antibody screening test
2. Hepatitis C RNA testing
57
What is the treatment for Hepatitis C?
- 1/4 fight off and make full recovery
- 3/4 become chronic
- No vaccine available
- Stop smoking and drinking alcohol
- Curable with direct acting antiviral medications e.g. pegylated interferon-alpha 2a (8-12 week course)
58
What are 2 complications of Hepatitis C?
1. Liver cirrhosis
2. Hepatocellular carcinoma
59
How is Hepatitis D spread?
Blood-borne transmission (sexually, IVDU)
60
Describe the pathophysiology of Hepatitis D
- RNA virus
- Only occurs in people who are also infected with Hepatitis B
- Attaches to HBsAg to survive
61
What are 2 signs of Hepatitis D?
Signs of acute HBV infection:
1. Jaundice
2. Hepatomegaly
62
What are 3 symptoms of Hepatitis D?
Symptoms of acute HBV infection:
1. Flu-like symptoms
2. Fever
3. Pruritus
63
What is the treatment of Hepatitis D?
Same treatment as HBV:
- Stop smoking and drinking alcohol
- Antiviral medication e.g. pegylated interferon-alpha 2a (48 week long treatment course)
64
What is the complication of Hepatitis D?
Increases complications/disease severity of hepatitis B
65
Describe the epidemiology of Hepatitis E
Most common cause of acute hepatitis in the UK
66
How is Hepatitis E spread?
Faecal-oral transmission (raw/undercooked pork meat)
67
Describe the pathophysiology of Hepatitis E
- RNA virus
- Usually results in self-limiting acute hepatitis
68
What are the symptoms for Hepatitis E?
95% of cases are asymptomatic (usually self-limiting)
69
What is the investigation for patients with Hepatitis E?
Serology
70
What is the treatment for Hepatitis E?
- Often not required
- Treat symptoms
- No vaccine available
71
What is a complication of Hepatitis E?
Can progress to cirrhosis in immunocompromised (rare)
72
What is liver cirrhosis?
Scarring of the liver (fibrosis) caused by continuous, long-term liver damage - chronic inflammation and damage to hepatocytes
73
What are the 4 main causes of liver cirrhosis?
1. Alcoholic liver disease
2. Non-alcoholic fatty liver disease
3. Hepatitis B
4. Hepatitis C
74
What are 2 rarer causes of liver cirrhosis?
1. Hereditary haemochromatosis (HH)
2. Wilson's disease (WD)
75
Describe the pathophysiology of liver cirrhosis
- Damaged hepatocytes are replaced with scar tissue (fibrosis)
- Fibrosis affects the structure and blood flow through the liver
- This increases resistance in the vessels leading into the liver (portal hypertension)
76
What are 4 signs of liver cirrhosis?
1. Jaundice
2. Hepatosplenomegaly
3. Ascites
4. Asterixis (inability to maintain sustained posture with subsequent brief, shock-like, involuntary movements)
77
What are 5 symptoms of liver cirrhosis?
1. Spider naevi/caput medusae
2. Palmar erythema
3. Gynaecomastia
4. Testicular atrophy
5. Bruising
78
What are the 7 investigations for patients with liver cirrhosis?
1. Bloods
2. Imaging (CT/MRI/ultrasound/fibroscan)
3. Endoscopy
4. Liver biopsy
5. Enhanced liver fibrosis blood test
6. Child-Pugh score
7. MELD score
79
What are the results of bloods in patients with liver cirrhosis?
- High AST:ALT ratio
- High bilirubin
- Decreased albumin
- Increased PTT/NR
- Thrombocytopenia
- Hyponatraemia
80
What is the enhanced liver fibrosis blood test?
< 7.7 = none to mild fibrosis
7.7-9.8 = moderate fibrosis
>9.8 = severe fibrosis
81
What does the child-pugh score calculate?
Indicates severity of cirrhosis
82
What does the MELD score calculate?
Gives a percentage estimated 3 month mortality
83
What is the treatment for liver cirrhosis?
- High protein, low sodium diet
- Fluids
- Analgesia
- Alcohol abstinence
- Liver transplant
84
What are 6 complications of liver cirrhosis?
1. Malnutrition
2. Portal hypertension, varices, variceal bleeding
3. Ascites and spontaneous bacterial peritonitis
4. Hepato-renal syndrome
5. Hepatic encephalopathy
6. Hepatocellular carcinoma
85
What is jaundice?
Yellow discolouration of sclera and skin due to hyperbilirubinaemia
86
What is the jaundice threshold?
Occurs at bilirubin levels roughly greater than 50 umol/L
87
What are 3 causes of pre-hepatic jaundice?
1. Haemolytic anaemia (e.g. sickle cell, malaria)
2. Gilbert's syndrome
3. Criggler-Najjar syndrome
88
What are 6 causes of intrahepatic jaundice?
1. Alcoholic liver disease
2. Viral/autoimmune hepatitis
3. Iatrogenic
4. Hereditary haemochromatosis
5. Primary biliary cirrhosis/primary biliary cholangitis
6. Hepatocellular carcinoma
89
What are 3 causes of posthepatic jaundice?
1. Intra-luminal causes e.g. gallstones
2. Mural causes e.g. cholangiocarcinoma, stricture, drug-induced cholestasis
3. Extra-mural cases e.g. pancreatic cancer, abdominal masses (e.g. lymphoma)
90
What is bilirubin?
Breakdown product from catabolism of haem (formed from destruction of RBCs)
91
What usually happens to bilirubin?
- Conjugates with liver (becomes water soluble)
- Excreted via bile into GI tract
- Excreted in faeces as urobilinogen/stercobilin
- 10% of urobilinogen is reabsorbed into bloodstream and excreted via kidneys
92
Describe the pathophysiology of pre-hepatic jaundice
- Excessive breakdown of RBCs
- Liver overwhelmed to conjugate bilirubin
- = unconjugated hyperbilirubinaemia
- = unconjugated bilirubin in bloodstream
93
Describe the pathophysiology of intrahepatic jaundice
- Dysfunction of hepatic cells
- Liver loses ability to conjugate bilirubin
- Cirrhotic liver can cause obstruction
- = unconjugated and conjugated bilirubin in bloodstream
94
Describe the pathophysiology of post-hepatic jaundice
- Obstruction of biliary drainage
- Bilirubin is conjugated but cannot be excreted
- = conjugated bilirubin in bloodstream
95
Describe the clinical presentation/investigations of pre-hepatic jaundice
- Yellow sclera and skin
- Normal urine and stool
- No itching
- Normal LFTs
96
Describe the clinical presentation of intrahepatic jaundice
- Yellow sclera and skin
- Dark urine and pale stool
- Itching
- Abnormal LFTs
97
Describe the clinical presentation of post-hepatic jaundice
- Yellow sclera and skin
- Dark urine and pale stool
- Itching
- Abnormal LFTs
98
What are the 2 causes of Wernicke's encephalopathy and Korsakoff syndrome?
1. Alcohol excess
2. Thiamine (vitamin B1) deficiency
99
Describe the pathophysiology of Wernicke's encephalopathy and Korsakoff syndrome
- Thiamine is poorly absorbed in the presence of alcohol
- Thiamine deficiency causes damage to the hypothalamus and thalamus
- Further deficiency leads to damaged nerve cells and supporting cells in the CNS
- Wernicke's = acute phase
- Korsakoff = chronic phase
100
What are 3 symptoms of Wernicke's encephalopathy?
1. Confusion
2. Ataxia (difficulty with coordinated movements)
3. Oculomotor disturbances
101
What are 3 symptoms of Wernicke's encephalopathy?
1. Confusion
2. Ataxia (difficulty with coordinated movements)
3. Oculomotor disturbances
102
What are 2 symptoms of Korsakoff's syndrome?
1. Memory impairment
2. Behavioural changes
103
What is the treatment for Wernicke's encephalopathy?
- Thiamine supplementation
- Abstaining from alcohol
104
What are complications of Wernicke's encephalopathy and Korsakoff's syndrome?
- Death if left untreated
- Korsakoff's syndrome is irreversible and usually results in patients requiring full time institutional care
105
What is hepatic encephalopathy?
Changes in the brain that occur due to build up of toxins in the blood, particularly ammonia
106
What are 6 causes of hepatic encephalopathy?
1. Constipation
2. Electrolyte disturbance
3. Infection
4. GI bleed
5. High protein diet
6. Medications (particularly sedatives)
107
Describe the pathophysiology of hepatic encephalopathy
- Liver cirrhosis causes functional impairment of hepatocytes
- Hepatocytes cannot metabolise ammonia into harmless waste products
- Collateral circulation occurs in chronic liver disease (consequence of portal hypertension)
- Collateral vessels are made between portal and systemic circulation so ammonia can bypass liver
108
What are 2 symptoms of acute hepatic encephalopathy?
1. Confusion
2. Reduced consciousness
109
What are 3 symptoms of chronic hepatic encephalopathy?
1. Personality changes
2. Memory changes
3. Mood changes
110
What is the treatment for hepatic encephalopathy?
- Laxatives (to clear ammonia from gut)
- Antibiotics (to reduce no. of bacteria in gut that produce ammonia)
- Nutritional support
111
Describe the epidemiology of pancreatic cancer
- 99% in exocrine component of pancreas
- More common in males >60
- Majority are adenocarcinomas
112
What are 7 risk factors for pancreatic cancer?
1. Smoking
2. Excessive alcohol/coffee intake
3. Excessive use of aspirin
4. Diabetes
5. Chronic pancreatitis
6. Family history
7. Genetic mutations (presence of PRSS-1 mutation)
113
Describe the pathophysiology of pancreatic cancer
- Originates in ductal epithelium and evolves from pre-malignant lesions to full-invasive cancer
- 60% arise in pancreatic head
- 25% arise in body
- 15% arise in tail
- Tumour in head can grow large enough to compress the bile ducts and cause obstructive jaundice
- Tend to spread and metastasise early, particularly to liver, peritoneum, lungs and bones
114
What are 2 signs of pancreatic cancer?
1. Acute pancreatitis
2. Courvoisier's law = palpable gallbladder and jaundice is usually caused by cholangiocarcinoma or pancreatic cancer
115
What are 3 symptoms of pancreatic cancer?
1. Anorexia
2. Weight loss
3. Jaundice
116
What is a symptom of pancreatic cancer (body and tail)?
Epigastric pain that radiates to the back that is relieved by sitting forward
117
What are 3 symptoms of pancreatic cancer (head)?
Painless obstructive jaundice:
1. Yellow skin and sclera
2. Dark urine and pale stools
3. Generalised itching
118
What are 3 investigations for pancreatic cancer?
1. Abdominal ultrasound/CT
2. Biopsy
3. Carbohydrate antigen 19-9 tumour marker = raised
119
What is the treatment for pancreatic cancer?
- Surgery
- Palliative therapy (often diagnosed late and has a very poor prognosis - 5% 3 year survival)
120
What are 4 surgeries for pancreatic cancer?
1. Total pancreatectomy
2. Distal pancreatectomy
3. Radical pancreaticoduodenectomy (Whipple procedure)
4. Pylorus-preserving pancreaticoduodenectomy (PPPD - modified Whipple procedure)
121
What are 5 palliative therapies for pancreatic cancer?
1. Stents (to relieve biliary obstruction)
2. Surgery to improve symptoms (e.g. bypassing biliary obstruction)
3. Palliative chemotherapy
4. Palliative radiotherapy
5. End of life care
122
Describe the epidemiology of hepatocellular carcinoma
80% of primary liver cancers
123
What are 4 risk factors for hepatocellular carcinoma?
Liver cirrhosis due to:
1. Viral hepatitis B and C
2. Alcohol
3. Non alcoholic fatty liver disease
4. Other chronic liver disease
124
What are 2 signs of hepatocellular carcinoma?
1. Hepatomegaly
2. Ascites
125
What are 9 symptoms of hepatocellular carcinoma?
Often remains asymptomatic for a long time
1. Fever
2. Malaise
3. Weight loss
4. Abdominal pain
5. Anorexia
6. Nausea/vomiting
7. Jaundice
8. Pruritus
9. RUQ pain
126
What are 4 investigations for patients with hepatocellular carcinoma?
1. CT/MRI liver
2. Biopsy
3. Alpha-fetoprotein (ATP) = raised
4. Bloods = clotting abnormalities/deranged LFTs
127
What is the treatment for hepatocellular carcinoma?
- Poor prognosis
- Surgical resection
- Radiofrequency ablation
- Chemo/radiotherapy (palliative)
- TACE (transarterial chemoembolism - blocks blood supply to tumour)
- Kinase inhibitors (inhibits proliferation of cancer cells)
- Liver transplant
128
Describe the epidemiology of cholangiocarcinoma
- 20% of primary liver cancers
- Usually >50
- 90% = ductal adenocarcinomas
- 10% = squamous cell carcinomas
129
What are 2 risk factors for cholangiocarcinoma?
1. Associated with primary sclerosing cholangitis (10%)
2. Viral hepatitis B and C
130
What are 2 signs of cholangiocarcinoma?
1. Hepatomegaly
2. Ascites
131
What are 4 symptoms of cholangiocarcinoma?
1. Painless jaundice
2. Fever
3. Malaise
4. RUQ pain
132
What are 4 investigations for patients with cholangiocarcinoma?
1. CT/MRI liver
2. Biopsy (ERCP - endoscopic retrograde cholangiopancreatography)
3. CA19-9 tumour marker = raised
4. Bloods = clotting abnormalities/deranged LFTs
133
What is the treatment for cholangiocarcinoma?
- Poor prognosis
- Surgical resection
- ERCP - stent placed in bile duct
- Chemo/radiotherapy (palliative)
134
What is Gilbert's syndrome?
Higher than normal levels of bilirubin in the blood
135
Describe the epidemiology of Gilbert's syndrome
- Most common cause of hereditary jaundice
- More common in males
136
What is the cause of Gilbert's syndrome?
Genetic - autosomal recessive mutation in UDP1A1 gene (UGT enzyme deficiency)
137
What are 2 risk factors for Gilbert's syndrome?
1. Type 1 diabetes
2. Being male
138
Describe the pathophysiology of Gilbert's syndrome
- Deficient/abnormal UGT
- Causes unconjugated hyperbilirubinaemia
139
What are 4 symptoms of Gilbert's syndrome?
30% asymptomatic
1. Painless jaundice = yellow skin and sclera
2. Criggler Najjar = jaundice
3. Criggler Najjar = nausea/vomiting
4. Criggler Najjar = lethargy
140
What is the treatment for Gilbert's syndrome/Criggler Najjar?
- Gilbert's = fine without treatment
- Criggler Najjar = phototherapy (breaks down unconjugated bilirubin)
141
What is an inguinal hernia?
Protrusion of abdominal cavity contents through the inguinal canal
142
Describe the epidemiology of inguinal hernias
- Commonest type of hernia
- More common in males >40
- Direct (20%) and indirect (80%)
- Accounts for ~70% of all abdominal hernias
143
What are 5 risk factors for inguinal hernias?
1. Male
2. Chronic cough
3. Constipation
4. Heavy weight lifting
5. Ascites
144
Describe the pathophysiology of an indirect inguinal hernia
- High abdominal pressure causes the internal organs to push through a weakened section of the abdominal wall
- Protrudes through the deep inguinal ring
- Lateral to inferior epigastric vessels
- Can strangulate
145
Describe the pathophysiology of a direct inguinal hernia
- High abdominal pressure causes the internal organs to push through a weakened section of the abdominal wall
- Protrudes directly into inguinal canal
- Medial to inferior epigastric vessels
- Rarely strangulates
146
Describe the clinical presentation of inguinal hernias
- Swelling of groin associated with coughing/straining (due to bowel movements/heavy lifting)
- Points to groin
- Appearance of lump
- Usually asymptomatic
- Pain indicates strangulation (indirect)
147
What are 2 investigations for patients with inguinal hernias?
1. Look for lump
2. Ultrasound
148
What is the treatment for inguinal hernias?
- Truss (belt to keep hernia contained and prevent further progression)
- Surgical repair
149
What are 2 complications of all hernias?
1. Incarceration (part of intestine/abdominal tissue becomes trapped in hernia sac)
2. Strangulation (cuts off blood flow)
150
What is a femoral hernia?
Protrusion of abdominal cavity contents through femoral canal
151
Describe the epidemiology of femoral hernias
- More common in females - middle age and elderly
- Idiopathic
152
Describe the pathophysiology of femoral hernias
- High abdominal pressure causes the internal organs to push through a weakened section of abdominal wall
- Bowel enters femoral canal
153
Describe the clinical presentation of femoral hernias
- Mass in upper medial thigh or above inguinal ligament (lateral and inferior to pubic tubercle)
- Points down leg
- Increase in swelling on cough
154
What are 2 investigations for patients with femoral hernias?
1. Look for lump
2. Ultrasound
155
What is the treatment for femoral hernias?
- Surgical repair
- Herniotomy (ligation and excision of sac)
156
What is an umbilical hernia?
Protrusion of abdominal cavity contents at umbilicus
157
Describe the epidemiology of umbilical hernias
10-30% of all hernias
158
What are 3 causes of umbilical hernias?
1. Occur congenitally as a result of developmental error
2. 90% in adults acquired through multiple and difficult pregnancies
3. Also occur in adults with ascites and obesity
159
Describe the pathophysiology of umbilical hernias
Organs develop outside of abdominal cavity and enter through an opening in the umbilicus
160
Describe the clinical presentation of umbilical hernias
- Mass at site of umbilicus
- Can be asymptomatic
- Pain if abdominal wall contracts (worsened on straining)
161
What are 2 investigations for patients with umbilical hernias?
1. Look for lump
2. Ultrasound
162
What is the treatment for umbilical hernias?
<1cm = usually close spontaneously by 5 years
1-5cm = usually requires repair with preservation of umbilicus
163
What is an incisional hernia?
Protrusion of abdominal cavity contents at site of an incision from previous surgery
164
Describe the epidemiology of incisional hernias
15% of abdominal operations result in incisional hernias
165
What is the cause of incisional hernias?
Failure of wound to heal following an abdominal operation
166
Describe the pathophysiology of incisional hernias
Internal organs push through improperly healed sections of abdominal wall
167
Describe the clinical presentation of incisional hernias
- Mass
- Location depends on location of incision
168
What is the investigation for patients with incisional hernias?
Look for lump (observation)
169
What is the treatment for incisional hernias?
Urgent repair with reinforcing mesh
170
What is an additional complication of incisional hernias?
Recurrence in 50% of large hernias
171
What is an epigastric hernia?
Protrusion of abdominal cavity contents in the epigastric area
172
Describe the epidemiology of epigastric hernias
Most common in men between 20-50
173
What is the main risk factor for epigastric hernias?
Obesity
174
Describe the pathophysiology of epigastric hernias
Internal organs push through a weakened section of abdominal muscle
175
Describe the clinical presentation of epigastric hernias
- Mass above umbilicus in the linea alba
- Usually asymptomatic
- Can be made to bulge by asking the patient to strain
- Possible bloating, nausea and vomiting (often after meals)
176
What are 3 investigations for patients with epigastric hernias?
1. Look for lump
2. Ultrasound
3. CT
177
What is the treatment for epigastric hernias?
Surgical correction
178
What is a hiatus hernia?
Protrusion of part of the stomach through the oesophageal hiatus of the diaphragm
179
Describe the epidemiology of hiatus hernias
- 30% of patients >50
- Sliding and rolling/para-oesophageal (uncommon)
180
What is the main risk factor for hiatus hernias?
Obesity
181
Describe the pathophysiology of sliding hiatus hernias
Oesophageal-gastric junction slides through the hiatus and lies above the diaphragm
182
Describe the pathophysiology of rolling/para-oesophageal hiatus hernias
- Gastric fundus rolls up through hiatus alongside oesophagus
- Gastro-oesophageal junction remains below level of the diaphragm
183
Describe the clinical presentation of hiatus hernias
Asymptomatic except for symptomatic GORD
184
What are 2 investigations for patients with hiatus hernias?
1. Barium swallow
2. Upper GI endoscopy
185
What is the treatment for hiatus hernias?
- Lose weight
- Treat reflux symptoms
- Surgery
186
What is biliary colic?
Obstruction of the cystic or common bile duct by a stone migrating from the gallbladder
187
Describe the epidemiology of gallstones
- 10-20% of the population
- More common in females
- Prevalence increases with age
- Cholesterol gallstones = 80% of the western world's gallstones
188
What are 3 causes of cholesterol gallstones?
Multifactorial
1. Cholesterol super saturation
2. Nucleation factors
3. Reduced gallbladder motility
189
What are 3 causes of pigment gallstones?
1. Chronic haemolysis (increased bilirubin production)
2. Cirrhosis
3. Complication of a cholecystectomy/with duct strictures
190
What are the 5 risk factors for gallstones?
5Fs
1. Fat
2. Fertile (pregnant)
3. Forty (or over)
4. Female
5. Family history
191
Describe the pathophysiology of cholesterol gallstones
- Cholesterol held in solution by detergent action of bile salts and phospholipids
- Forms micelles and vesicles
- Excess of cholesterol (lithogenic bile) = lack of bile salts and phospholipids
- Cholesterol crystals and gallstones form
192
Describe the pathophysiology of pigment gallstones
- Bilirubin polymers and calcium bilirubinate
- Caused by excess of bilirubin
193
What are the 2 symptoms of gallstones?
70% are asymptomatic
1. Biliary colic
2. Acute cholecystitis
194
What are 2 symptoms of biliary colic?
1. 'Colicky' RUQ pain that is worse after eating large/fatty meals and may radiate to epigastrium/back
2. Nausea and vomiting
195
What is a symptom of acute cholecystitis?
Distension of gallbladder leading to necrosis and ischaemia
196
What is the diagnostic test for gallstones/biliary colic?
Ultrasound:
- Stones
- Gallbladder wall inflammation
- Duct dilation suggests distal blockage
197
What are 3 other investigations for gallstones/biliary colic?
1. FBC and CRP = signs of inflammatory response
2. LFTs = raised ALP but normal bilirubin/ALT
3. Amylase = check for pancreatitis
198
What is the treatment for gallstones?
- NSAIDs/analgesia
- IV antibiotics
- Optional laparoscopic cholecystectomy
199
What are 6 complications of gallstones?
1. Obstructive jaundice
2. Acute cholecystitis
3. Acute cholangitis
4. Pancreatitis
5. Gallstone ileus (occludes intestinal lumen)
6. Empyema (obstructed gallbladder fills with pus)
200
What is cholecystitis?
Cystic duct impaction - inflammation of the gallbladder due to obstruction of bile ducts and build-up of bile
201
Describe the pathophysiology of cholecystitis
- Stone blocks bile ducts
- Bile builds up and distends gallbladder
- Vascular supply may be reduced as a result of distension
- Retained bile leads to inflammation of gallbladder
202
What are 3 symptoms of cholecystitis?
1. Generalised epigastric pain migrating to severe RUQ pain
2. Signs of inflammation e.g. fever/fatigue
3. Pain associated with tenderness and guarding from inflamed gallbladder and local peritonitis
203
What are 3 investigations for patients with cholecystitis?
1. Positive Murphy's sign (severe pain on deep inhalation with hand pressed into RUQ)
2. FBC and CRP = inflammatory markers
3. Ultrasound = thick gallstone walls (inflammation)
204
What is the treatment for cholecystitis?
- IV antibiotics
- Heavy analgesia
- IV fluids
- Cholecystectomy
205
What is acute cholangitis?
Bacterial infection of biliary system due to prolonged bile duct blockage
206
Describe the epidemiology of acute cholangitis
5-10% mortality
207
Describe the pathophysiology of acute cholangitis
- The bile duct is blocked and so prevents bile from being able to 'flush' out into GI tract
- Bacteria can climb up from the GI tract and cause biliary tree infection and consolidation
- Infection can affect pancreas as it shares ducts with the gallbladder
208
What are 3 signs of acute cholangitis?
1. Sepsis
2. Pancreatitis
3. Reynolds pentad
209
What is Reynolds pentad (acute cholangitis)?
1. Charcot's triad
2. Confusion
3. Septic shock
210
What are 3 symptoms of acute cholangitis?
Charcot's triad:
1. Jaundice
2. Severe RUQ pain
3. Fever (rigors)
211
What are 3 investigations for patients with acute cholangitis?
1. FBCs, LFTs and CRP = leukocytosis, raised ALP/bilirubin/CRP
2. Blood cultures/MC&S = work out what pathogen it is
3. Ultrasound +/- ERCP
212
What is the treatment for acute cholangitis?
- Treat sepsis (IV antibiotics)
- ERCP and stenting (to mechanically clear blockage)
- Surgery - cholecystectomy
213
What is primary biliary cholangitis also known as?
Primary biliary cirrhosis
214
Describe the epidemiology of primary biliary cholangitis
- 90% of patients = women aged 40-50
- More common in females (9:1)
- Typical presentation at 50
215
What are 5 risk factors of primary biliary cholangitis?
1. Family history
2. Many UTIs
3. Smoking
4. Past pregnancy
5. Autoimmune disease
216
Describe the pathophysiology of primary biliary cholangitis
- Interlobar bile ducts are damaged by chronic autoimmune granulomas
- This results in inflammation and leakage of bile into the blood and hepatocytes
- = inflammation and bile stasis
217
What are 5 clinical presentations of primary biliary cholangitis?
1. Hepatomegaly
2. Variceal bleeding
3. Asymptomatic
4. Lethargy and fatigue
5. Joint pain and arthropathy
218
What are 2 clinical presentations of primary biliary cholangitis due to leakage of bile?
1. Pruritus (itchy skin)
2. Jaundice
219
What are 3 clinical presentations of primary biliary cholangitis due to leakage of cholesterol?
1. Pigmented xanthelasma (yellow fat deposited under skin around eyelids)
2. Xeropthalmia (dryness of conjunctiva and cornea)
3. Corneal arcus (white/grey ring around cornea)
220
What are 4 investigations for patients with primary biliary cholangitis?
1. Antibody tests = presence of anti-mitochondria antibodies (AMA) and raised serum IgM
2. LFTs = raised ALP/GTT/cholesterol
3. Ultrasound
4. Liver biopsy
221
What is the treatment for primary biliary cholangitis?
- Ursodeoxycholic acid (reduces cholesterol absorption and improves bilirubin and aminotransferase levels)
- Cholestyramine (reduces cholesterol absorption)
- Bisphosphonates (for osteoporosis)
- Vitamin ADEK supplementation
- Liver transplant
222
What is primary sclerosing cholangitis?
Progressive sclerosis of the biliary tree leading to chronic cholestasis and end-stage liver disease
223
Describe the epidemiology of primary sclerosing cholangitis
- Associated with IBD, mainly UC
- More common in males
- Median age = 35
224
Describe the pathophysiology of primary sclerosing cholangitis
- Chronic inflammation and fibrosis of bile ducts
- Progressive obliterative fibrosis in the intra- and extra-hepatic ducts can cause strictures of the ducts
225
What are 4 symptoms of primary sclerosing cholangitis?
50% asymptomatic until advanced disease
1. Non-specific pruritus
Charcot's triad:
2. Fever (with chills)
3. RUQ pain
4. Jaundice
226
What are 3 investigations for patients with primary sclerosing cholangitis?
1. Ultrasound (can show bile duct dilatation)
2. ERCP (endoscopic retrograde cholangio pancreatography)
3. LFT (elevated ALP/GGP and serum albumin levels drop with progression)
227
What is the treatment for primary sclerosing cholangitis?
- Manage symptoms of liver failure
- ERCP (can dilate extra-hepatic strictures to slow progression)
- High dose ursodeoxycholic acid (can slow progression)
- Liver transplant
228
What are 4 complications of primary sclerosing cholangitis?
1. Liver failure
2. Hepatocellular carcinoma
3. Cholangiocarcinoma
4. Colorectal cancer
229
What is acute pancreatitis?
Sudden inflammation of the pancreas leading to autodigestion of the gland
230
What are the 11 causes of acute pancreatitis?
I GET SMASHED
1. Idiopathic (20%)
2. Gallstones (40%)
3. Ethanol (alcohol - 30%)
4. Trauma
5. Steroids
6. Mumps/malignancy
7. Autoimmune
8. Scorpion stings
9. Hypertriglyceridemia/hypercalcaemia
10. (post) ERCP
11. Drugs (tobacco/thiazides)
231
Describe the pathophysiology of alcohol induced acute pancreatitis
- Pancreas releases exocrine enzymes (zymogen and trypsinogen) that cause autodigestion of the organ
- Increased intracellular calcium leads to activation of intra-cellular proteases and release of pancreatic enzymes
- Can result in acinar cell injury and necrosis
232
Describe the pathophysiology of gallbladder induced acute pancreatitis
- Gallstones block the bile duct (sphincter of Oddi)
- This causes back pressure in the pancreatic duct
233
What are 5 signs of acute pancreatitis?
1. Cullen's sign (bruising around periumbilical region)
2. Grey Turner's sign (bruising on flanks)
3. Tachycardia
4. Distension
5. Abdominal guarding and tenderness
234
What are 3 symptoms of acute pancreatitis?
1. Epigastric/upper abdominal pain radiating through to the back
2. Nausea and vomiting
3. Fever with chills
235
What are 3 investigations for patients with acute pancreatitis?
1. CT abdomen (evidence of inflammation, necrosis and pseudocyst)
2. Abdominal Xray (shows no psoas shadow due to raised retroperitoneal fluid)
3. LFTs (serum amylase 3x greater, raised lipase/ALT/AST)
236
What is the treatment for mild acute pancreatitis?
- Pain relief
- IV fluids
237
What is the treatment for severe acute pancreatitis?
- IV antibiotics if necrotising
- Nasogastric tube
238
What are 7 complications of acute pancreatitis?
1. Pancreatic necrosis
2. Pancreatic ascites
3. Pancreatic pseudocysts (25%)
4. Insulin dependent DM
5. ARDS
6. DIC
7. Sepsis
239
What is chronic pancreatitis?
Long standing inflammation of the pancreas from irreversible damage
240
What are 4 causes of chronic pancreatitis?
1. Excess alcohol consumption (main)
2. Hereditary
3. Autoimmune
4. Complication of cystic fibrosis
241
Describe the pathophysiology of chronic pancreatitis
- Pancreas releases exocrine enzymes (zymogen and trypsinogen) that cause autodigestion of the organ
- Leads to precipitation of protein plugs within duct lumen, forming a nidus for calcification
- This leads to ductal hypertension and pancreatic damage
- Small and large duct pancreatitis (large associated with calcification)
242
What are 2 signs of chronic pancreatitis?
1. Diabetes
2. Steatorrhoea (increase in fat excretion in blood)
243
What are 4 symptoms of chronic pancreatitis?
1. Severe epigastric abdominal pain radiating through to the back
2. Severe weight loss (due to malabsorption)
3. Jaundice
4. Nausea and vomiting
244
What are 2 investigations for patients with chronic pancreatitis?
1. Xray/CT (display calcification)
2. Secretin stimulation test (detects if pancreas exocrine function is damaged)
245
What is the treatment for chronic pancreatitis?
- Pain relief
- Cessate alcohol
- Replace pancreatic enzymes
- Surgery - local resection to alleviate duct dilatation
246
What are 2 complications of chronic pancreatitis?
1. Insulin dependent DM
2. Pancreatic carcinoma
247
What is ascites?
Fluid in the peritoneal cavity
248
Describe the pathophysiology of ascites
Increased pressure in portal system causes fluid to leak out of the capillaries in the liver and bowel and into the peritoneal cavity
249
How is the cause of ascites determined?
SAAG - serum-ascites albumin gradient
250
Describe what it means if there is a high SAAG value (>1.1)
- Not much albumin
- Fluid = hepatic lymph
- Fluid produced by a sinusoid with increased hydrostatic pressure and decreased oncotic pressure
CAUSE = PORTAL HYPERTENSION
251
Describe what it means if there is a low SAAG value (<1.1)
- Lots of albumin
- Fluid = plasma
CAUSE = EXTRAHEPATIC SOURCE:
- Malignancy
- Infection (TB)
- Trauma
- Pancreatitis
252
What are 2 investigations for patients with ascites?
1. Fluid sample
2. Ultrasound/MRI/CT
253
What is portal hypertension?
Elevated pressure in the portal venous system
254
What is a pre-hepatic cause of portal hypertension
Portal vein thrombosis
255
What are 4 intra-hepatic causes of portal hypertension?
1. Cirrhosis
2. Schistosomiasis
3. Budd Chiari syndrome
4. Sarcoidosis
256
What are 3 post-hepatic causes of portal hypertension?
1. RH failure
2. IVC obstruction
3. Constrictive pericarditis
257
Describe the pathophysiology of portal hypertension
- Following liver injury and fibrogenesis, activated myofibroblasts contract (mediated by endothelin, NO and prostaglandins)
- This increases resistance to blood flow
- = portal hypertension
- Splanchnic vasodilation causes a drop in BP
- CO increases to compensate for low BP
- Salt and water are retained to increase blood volume
- = hyperdynamic circulation
- Causes formation of collaterals between portal and systemic systems
258
What is the difference between compensated and decompensated portal hypertension?
Compensated - liver can still function effectively
Decompensated - when the liver is damaged to the point that it cannot function adequately
259
What are 9 presentations of portal hypertension?
1. Ascites/oedema
2. Hepatic encephalopathy
3. Splenomegaly
4. Oesophago-gastric varices (and GI bleeding)
5. Xanthelasma (yellow fat deposits under skin of eyelids)
6. Spider naevi
7. Palmar erythema
8. Finger clubbing
9. Leukonychia (white discolouration on nails)
260
What is a symptom of portal hypertension?
Bruising
261
What are 3 symptoms of compensated portal hypertension?
Usually asymptomatic
1. Weight loss
2. Weakness
3. Fatigue
262
What are 3 symptoms of decompensated portal hypertension?
1. Jaundice
2. Pruritus
3. Abdominal pain (due to ascites)
263
What are 3 investigations for patients with portal hypertension?
1. Liver biopsy
2. LFTs (raised bilirubin/AST/ALT)
3. Ultrasound/MRI/CT
264
What is the treatment for portal hypertension?
- Treat underlying cause
- Liver transplant
- Good nutrition
265
What are 5 complications of portal hypertension?
1. Coagulopathy (fall in clotting factors II, VII, IX and X)
2. Encephalopathy
3. Thrombocytopenia
4. Hepatocellular carcinoma
5. Hypoalbuminemia
266
What are oesophageal varices?
Dilated veins at the junction between the portal and systemic venous systems leading to variceal haemorrhage
267
Describe the epidemiology of oesophageal varices
- 90% of patients with cirrhosis develop this over 10 years (but only a third bleed)
- 10-20% of all upper GI bleeding
268
What are 2 causes of oesophageal varices?
1. Chronic liver disease
2. Portal hypertension
269
Describe the pathophysiology of oesophageal varices
- High pressure in portal vein
- Vessels are thin and not meant to transport higher pressure blood
- This causes damage and can lead to bleeding from the varices into the oesophagus
- Rupture --> haematemesis --> blood digested --> melaena
270
Describe the clinical presentation of oesophageal varices
- Liver disease
- Shock (low BP, high HR)
- Haematemesis (vomiting blood)
- Pallor
271
What is the investigation for oesophageal varices?
Upper GI endoscopy
272
What is the medicinal treatment for oesophageal varices?
- Resuscitation/maintain airway
- Beta blocker (to reduce CO and portal pressure)
- Nitrate (to reduce portal pressure)
273
What is the surgical treatment for oesophageal varices?
- Band ligation
- Trans jugular intrahepatic portosystemic shunt (TIPSS)
274
What are 2 complications of oesophageal varices?
1. 70% chance of rebleeding
2. Significant risk of death
275
What is spontaneous bacterial peritonitis?
Infection of ascitic fluid and peritoneal lining without any clear cause
276
Describe the epidemiology of spontaneous bacterial peritonitis
- Occurs in 10% of patients with ascites secondary to cirrhosis
- Mortality of 10-20%
277
What are the 3 most common causes of spontaneous bacterial peritonitis?
1. E. coli
2. Klebsiella pneumoniae
3. Gram positive cocci e.g. staphylococcus and enterococcus
278
What are 2 signs of spontaneous bacterial peritonitis?
1. Ileus (temporary lack of normal muscle contractions of intestines)
2. Hypotension
279
What are 2 symptoms of spontaneous bacterial peritonitis?
Can be asymptomatic
1. Fever
2. Abdominal pain
280
What are the investigations for patients with spontaneous bacterial peritonitis?
Bloods:
- Raised WBC/CRP/creatinine
- Metabolic acidosis
281
What is the treatment for spontaneous bacterial peritonitis
IV cephalosporin e.g. cefotaxime
282
What is haemochromatosis?
Multi system disorder of dysregulated dietary iron absorption and increased iron release from macrophages
283
Describe the epidemiology of haemochromatosis
Usually presents between 40-60
284
What are 2 causes of haemochromatosis?
1. HFE gene mutations (C282Y and H63D - autosomal recessive)
2. Secondary iron overload due to multiple transfusions
285
Describe the pathophysiology of haemochromatosis
- Deficiency of hepcidin (iron regulatory hormone)
- Increased intestinal iron absorption
- Iron accumulates in liver, joints, pancreas, heart, skin and gonads
286
What are 4 signs of haemochromatosis?
1. Arrhythmia
2. Hepatomegaly
3. Heart failure
4. Chronic liver disease
287
What are 5 symptoms of haemochromatosis?
Often asymptomatic until later stages
1. Slate grey (brown/bronze) skin
2. Fatigue
3. Weakness
4. Hypogonadism e.g. erectile dysfunction
5. Arthralgia
288
What are 4 investigations for haemochromatosis?
1. Blood (iron study)
2. Genetic testing
3. Liver biopsy (gold standard)
4. MRI
289
What is the treatment for haemochromatosis?
- 1st line = venesection
- 2nd line = iron chelation
- Liver transplant
290
What are 4 complications of haemochromatosis?
1. Cirrhosis
2. Hepatocellular carcinoma
3. Diabetes
4. Heart disease
291
What is Wilson's disease?
Disorder of copper metabolism resulting in a build-up of copper in the liver and CNS
292
Describe the epidemiology of Wilson's disease
Onset usually in 20s-30s
293
What is the cause of Wilson's disease?
Mutation in the gene defecting the enzyme involving in biliary excretion of excess copper (autosomal recessive)
294
Describe the pathophysiology of Wilson's disease
Copper accumulates in liver, basal ganglia and cornea
295
What are 3 signs of Wilson's disease?
1. Cirrhosis
2. Hepatitis
3. Psychiatric/neurological presentation = Parkinsonian
296
What are 7 symptoms of Wilson's disease?
1. Psychiatric/neurological presentation = depression
2. Kayser-Fleischer rings (green/brown ring around cornea)
3. Tremor
4. Reduced memory
5. Involuntary movements
6. Dysphagia (swallowing difficulties)
7. Dysarthria (difficult/unclear articulation of speech)
297
What are 3 investigations for patients with Wilson's disease?
1. 1st line = 24 hour urine copper and blood ceruloplasmin = high copper, low ceruloplasmin
2. Liver biopsy
3. Slit lamp analysis
298
What is ceruloplasmin?
Protein that stores and carries copper from the liver to other parts in the blood
299
What is the treatment for Wilson's disease?
- Avoid high copper foods (e.g. liver, chocolate, nuts, mushroom, shellfish)
- Avoid alcohol
- Penicillamine (copper chelation)
- Liver transplant
300
What are 3 complications of Wilson's disease?
1. Liver failure
2. Neurological problems
3. Death if left untreated
301
What is alpha-1 antitrypsin deficiency?
Deficiency of serine protease inhibitor alpha-1 antitrypsin
302
Describe the epidemiology of alpha-1 antitrypsin deficiency
- Presents between 30s-50s (earlier in smokers)
- More common in white people
303
What is the cause of alpha-1 antitrypsin deficiency?
Mutation in SERPINA1 gene (autosomal recessive)
304
Describe the pathophysiology of alpha-1 antitrypsin deficiency
- A1AT inhibits the action of neutrophil elastase
- This is a proteolytic enzyme produced by neutrophils in the presence of inflammation/infection/smoking
- When this is deficient, elastase can break down elastin without inhibition
- Alveolar walls and liver are destroyed
305
What are 4 signs of alpha-1 antitrypsin deficiency?
1. Early onset emphysema
2. Neonates may present with hepatitis
3. Cirrhosis
4. Liver failure
306
What are 2 symptoms of alpha-1 antitrypsin deficiency?
1. COPD like symptoms e.g. SOB
2. Neonates may present with jaundice
307
What are 2 investigations for patients with alpha-1 antitrypsin deficiency?
1. Serum levels of A1AT
2. Liver biopsy
308
What is the treatment for alpha-1 antitrypsin deficiency?
- Smoking cessation
- Corticosteroids
- Bronchodilators
- Treat infection
- Drinking cessation
- Possible liver transplant
309
Describe the normal physiology of paracetamol metabolism
- 95% is converted into non-toxic metabolites via phase II metabolites which conjugates with sulfate and glucuronide
- The other 5% is converted to a highly reactive intermediate NAPQI by cytochromes P450 2E1 and 3A4
- NAPQI is detoxified by conjugation with glutathione to form cysteine and mercapturic acid conjugates
310
Describe the pathophysiology of paracetamol overdose
- Phase II metabolic pathways become saturated
- More paracetamol is shunted to the cytochrome P450 system to produce NAPQI
- Hepatocellular supplies of glutathione become depleted due to the higher demand
- Lack of glutathione results in NAPQI remaining in the liver (toxic form)
- NAPQI reacts with cellular membrane molecules to result in widespread hepatocyte damage and death
311
What are 2 signs of paracetamol overdose?
1. Fulminant liver failure
2. Hepatic encephalopathy
312
What are 3 symptoms of paracetamol overdose?
Asymptomatic
1. Vomiting
2. RUQ pain
3. Jaundice
313
What is the treatment for paracetamol overdose?
- Activated charcoal
- IV N-acetylcysteine
- Liver transplant
314
What is the difference between acute and chronic liver failure?
Acute - rapid onset (no evidence of prior liver disease)
Chronic - progressive (evidence of prior liver disease)
315
What are 4 causes of acute liver failure?
1. Paracetamol DILI
2. Alcohol
3. Viral hepatitis
4. Drugs
316
What is the difference between hyperacute, acute and subacute liver failure?
Hyperacute = onset of encephalopathy less than 7 days after the development of jaundice
Acute = onset of encephalopathy 8-28 days after the development of jaundice
Subacute = onset of encephalopathy 28-72 days after the development of jaundice
317
What are 2 signs of acute liver failure?
1. Hypoglycaemia (rarer)
2. Asterixis (inability to maintain sustained posture with subsequent brief, shock-like involuntary movements - rarer)
318
What are 4 common symptoms of acute and chronic liver failure?
1. Malaise
2. Anorexia
3. Jaundice
4. Pruritus
319
What are 4 other symptoms of acute liver failure?
1. Nausea
2. Confusion (rarer)
3. Bleeding (rarer)
4. RUQ pain (rarer)
320
What are 2 investigations for liver failure?
1. LFTs (high bilirubin, low albumin, high PTT)
2. Liver hepatic enzymes (raised AST/ALT/ALP)
321
What is the treatment for liver failure?
- Fluids
- Analgesia
- Liver transplant
322
What is the treatment for complications of liver failure?
- Ascites = diuretics
- Cerebral oedema = mannitol
- Bleeding = vitamin K
- Encephalopathy = lactulose
- Sepsis = antibiotics
- Hypoglycaemia = dextrose
323
What are 7 causes of chronic liver failure?
1. Alcohol
2. Viral hepatitis
3. Autoimmune (PBC/PSC)
4. Metabolic (HH, WD, A1AT def)
5. Neoplastic
6. NAFLD
7. AFLD
324
Describe the progression of chronic liver failure
Chronic liver condition --> liver damage --> liver symptoms --> liver cirrhosis (if prolonged) --> liver failure
325
What are 2 signs of chronic liver failure?
1. Ascites/oedema
2. Hepatosplenomegaly
326
What are 8 symptoms of chronic liver failure?
1. Gynaecomastia
2. Dupuytren's contracture (one/more fingers bent towards palm)
3. Clubbing/leukonychia (white nails)
4. Palmar erythema
5. Xanthelasma (yellow growths on/near eyelids)
6. Spider naevi/caput medusae
7. Haematemesis
8. Easy bruising
327
What is a complication of chronic liver failure?
Higher risk of hepatocellular carcinoma
