Hepatobiliary Flashcards

(243 cards)

1
Q

Define cholecystitis

A

Inflammation of the gallbladder

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2
Q

Main cause of cholecystitis

A

Gallstone impaction into the cystic duct or neck of gallbladder

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3
Q

First line investigations for cholecystitis

A
  • Abdominal ultrasound scan
  • FBC (high WBC count)
  • LFTs (exclude liver/bile duct pathology)
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4
Q

Results of imaging in a patient with cholecystitis

A
  • Thickened gallbladder
  • Stones or sludge in gallbladder
  • Fluid around the gallbladder
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5
Q

Patient presents with RUQ that radiates to the right shoulder, positive Murphy’s sign, tachycardic and fever. Diagnosis?

A

Cholecystitis

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6
Q

Gold standard imaging for cholecystitis diagnosis

A

Mangnetic resonance cholangiopancreatography (MRCP)

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7
Q

Differential diagnoses for cholecystitis

A
  • Acute cholangitis
  • Chronic cholangitis
  • Appendicitis
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8
Q

First line management for cholecystitis

A
  • IV fluids
  • Analgesia
  • Antibiotics
  • Endoscopic retrograde cholangio-pancreatography (ERCP)
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9
Q

Complications of cholecystitis

A
  • Sepsis
  • Gangrenous bladder
  • Perforation -> peritonits
  • Gallbladder empyema
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10
Q

What positive clinical examination sign is suggestive of cholecystitis?

A

Murphy’s sign
(acute pain + sudden stopping of inspiration)

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11
Q

Define biliary colic

A
  • Term to describe pain associated with temporary obstruction of the cystic or common bile duct by a stone migrating from the gallbladder
  • Sudden onset, serve but constant, has a crescendo characteristic
  • Pain is temporary -> stops when gallstone dislodges
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12
Q

What is ascending/acute cholangitis?

A

Life-threatening condition - caused by an ascending bacterial infection of the biliary tree
(High mortality - sepsis + septicaemia)

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13
Q

Causes of acute/ascending cholangitis

A
  • Gallstones in common bile duct (stops bile flow -> jaundice)
  • Infection introduced during ERCP procedure
  • Benign + malignant strictures
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14
Q

Most common organisms to cause ascending/acute cholangitis

A
  • Escherichia coli
  • Klebsiella species
  • Enterococcus species
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15
Q

Risk factors for ascending/acute cholangitis

A

4Fs:
* Female
* Fat
* Fertile = pregnancy, under 40
* Fair = Northern European and Hispanic

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16
Q

Pathophysiology of ascending/acute cholangitis

A
  • Obstruction of common bile duct → stasis of bile → invasion of bacteria from duodenum
  • High pressure on the CBD (due to the obstruction) can cause spaces between the cells to widen which allows the bacteria and the bile access to the blood stream → bacteraemia + jaundice
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17
Q

Key signs of ascending/acute cholangitis
(name of triad)

A

Charcot’s triad
* RUQ pain
* Fever
* Jaundice (dark urine + pale stools) (raised bilirubin)

(Theres also shock - hypotension + tachycaridia)

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18
Q

Symptoms of ascending cholangitis

A
  • Reynold’s pentad: bacteria → septic shock → leaky vessels → hypotension → less blood flow to organs e.g. brain → confusion
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19
Q

First line investigations for ascending cholangitis

A
  • Transabdominal ultrasound (dilated bile duct, common bile duct stones)
  • FBC (raised WBCs)
  • ESR + CRP (raised)
  • Serum bilirubin (raised)
  • Blood cultures (usually gram-negative)
  • Serum LFTs (raised transaminases + alkaline phosphatase)
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20
Q

Management for ascending cholangitis

A
  • Endoscopic reterograde cholangiopancreatography (ERCP) - to remove stones from bile duct
  • IV fluids
  • IV antibiotics (co-amoxiclav)
  • If this fails → laparoscopic/open cholecystectomy
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21
Q

Complications of ascending cholangitis

A
  • Aute pancreatitis
  • Sepsis
  • Septicaemia
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22
Q

What is the condition that involves intrahepatic + extrahepatic ducts become strictured + fibrotic?

A

Primary sclerosing cholangitis
* (Sclerosing → stiffening, hardening)
* (Cholangitis → inflammation of bile ducts)

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23
Q

Who is the typical patient that presents with primary sclerosing cholangitis and primary biliary cholangitis/cirrhosis?

A
  • Primary sclerosing cholangitis → young and middle aged men (often with IBD)
  • Primary biliary cirrhosis → middle-aged women (other autoimmune, rheumatoid conditions)
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24
Q

What condition are 70% of primary sclerosing cholangitis cases associated with?

A

Ulcerative colitis
(So if a patient has UC and has liver symptoms → think about primary sclerosing cholangitis)

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25
Risk factors for primary sclerosing cholangitis
* Male * Aged 30-40 * Ulcerative colitis (IBD) * Family history - genetic predisposition
26
A 32 y/o male patient presents with jaundice, chronic RUQ pain, and is feeling itchy (pruritus). You examine him and find that he has hepatomegaly. DIagnosis?
Primary sclerosing cholangitis
27
What does a 'cholestatic picture' on LFTs indicate?
Decreased or slowed release of bile (Liver cells = are not being directly injured - but are instead responding to bile stasis) * ↑ALP, ↑GGT, ↑Bilirubin * Mild ↑/ normal ALT, AST
28
What does a 'obstructive picture' on LFTs indicate?
Obstruction of the bile ducts (Bile builds up in the liver, leading to inflammation + injury) * ↑ALP, ↑GGT * Mild ↑/ normal ALT, AST
29
First line investigations for primary sclerosing cholangitis
LFTs * ↑ALP (most deranged) * ↑GCT * ↑Bilirubin * ↑/N ALT + AST Abdominal ultrasound
30
Gold standard investigation for primary sclerosing cholangitis
Magnetic resonance cholangiopancreatography (MRCP) (May show bile duct lesions or strictures
31
Antibodies for primary sclerosing cholangitis
No specific antibodies for PSC! * Antineutrophil cytoplasmic antibody (**p-ANCA**) in up to 94% * Antinuclear antibodies (**ANA**) in up to 77%
32
First line management for primary sclerosising cholangitis
* ERCP = dilate + stent strictures * Ursodeoxycholic acid = slow progression * Cholestyramine = a bile sequestrate (reduce pruritus) Curative: Liver transplant
33
Complications of primary sclerosing cholangitis
* Cirrhosis -> end-stage liver disease * Ascites * Oesophageal varcies * Acute bacterial cholangitis * Cholangiocarcinoma
34
What is primary biliary cholangitis/cirrhosis?
Autoimmune destruction of the bile ducts → cirrhosis
35
What is the typical patient to present with primary biliary cholangitis/cirrhosis?
Middle aged woman with other immune or rheumatoid conditions
36
A middle aged woman with rheumatoid arthritis presents with pruritus, abdominal pain (RUQ), jaundice, pale stools, fatty yellow deposits on her eyelids (Xanthelasma) and signs of cirrhosis (ascites and splenomegaly). Diagnosis?
Primary biliary cirrhosis/cholangitis
37
What is the most specifc antibody test for primary biliary cholangitis/cirrhosis?
Anti-mitochondria antibodies (AMA)
38
Investigations for primary biliary cholangitis/cirrhosis
Antibody tests * Anti-mitochondrial antibodies * Anti-nuclear antibodies LFTs * ↑ALP (first to be raised - obstructive pathology) * ↑GCT * ↑Bilirubin (later) Abdominal ultrasound Liver biopsy - diagnosing + staging ESR (raised) IgM (raised)
39
First line management for primary biliary cholangitis/cirrhosis
* Ursodeoxycholic acid * Cholestyramine * ADEK supplementation * Biphosphonates (for osteoporosis)
40
Complications for primary biliary cholangitis/cirrhosis
* Advanced liver cirrhosis * Portal hypertension * Osteoporosis * Hyperlipidaemia * Fat soluble vitamin deficiencies * Steatorrhoea (greasy stools due to lack of bile salts to digest fats)
41
What is the mnemonic for the causes of pancreatitis?
I GET SMASHED - **I** – **I**diopathic - **G** – **G**allstones - **E** – **E**thanol (alcohol consumption) - **T** – **T**rauma - **S** – **S**teroids - **M** – **M**umps - **A** – **A**utoimmune - **S** – **S**corpion sting - **H** – **H**yperlipidaemia - **E** – **E**RCP - **D** – **D**rugs (furosemide, thiazide diuretics and azathioprine)
42
What people are likely to present with acute pancreatitis? (Men and women)
- Gallstones → women + older - Alcohol → men + younger
43
Define acute pancreatitis
Sudden inflammation + haemorrhaging of the pancreas - due to its autodigestion
44
Where do gallstones get lodged to cause acute pancreatitis?
The spincter of Odi (Blocks the release of pancreatitis juices)
45
A patient presents with acute onset of severe epigastric pain that radiates to the back. They have a low grade fever, N+V and slightly tachcardic. On examination the abdomen is distended and they have bruising around the periumbilical region and on flanks. Diagnosis?
Acute pancreatitis
46
What are the two clinical signs associated with acute pancreatitis?
* Cullen's sign (bruising around periumbilical region) * Grey Turner's sign (bruising on flanks)
47
What is the key lab test for pancreatitis?
Lipase (more specific + sensitive than amylase) X3 upper limit
48
What are the factors that cause alcoholic liver disease?
* Alcohol comsumption (prolonged and heavy) * Genetic predisposition * Immunological immune response * (Rx - Hepatitis C)
49
In 3 threes what is the pathophysiology of alcoholic liver disease?
Fatty liver → hepatitis → cirhhosis Fatty liver → Alcoholic hepatitis → alcoholic steatosis (cells become swollen with fat) → cirrhosis
50
Metabolism of alcohol produces what in the liver?
Fat
51
What is the cellular pathway in which alcohol causes cirrhosis?
* Alcohol = transforms stellate cells into collagen-producing myofibroblast cells * Some cases: Collagen = laid down around the central hepatic veins → cirrhosis without preceding hepatitis
52
What are the signs and symptoms of alcoholic liver disease?
Signs: * **Hepatomegaly** * **Ascites** * **Jaundice** * Weight loss/ gain Symptoms: * **RUQ pain** * N + V * Diarrhoea * Fatigue
53
Name some complications (/conditions) from alcohol
* Alcoholic liver disease * Cirrhosis and the complications of cirrhosis including hepatocellular carcinoma * Alcohol Dependence and Withdrawal * Wernicke-Korsakoff Syndrome (WKS) * Pancreatitis
54
Name the signs of liver disease
* Jaundice * Hepatomegaly * Spider Naevi * Bruising (due to abnormal clotting) * Ascites * Gynaecomastia * Palmar Erythema * Caput Medusae – engorged superficial epigastric veins * Asterixis – “flapping tremor” in decompensated liver disease
55
What blood tests what indicate alcoholic liver disease?
* FBC (raised MCV) * LFTs - Transaminases (ALT + AST) (elevated): **AST>ALT ratio (usually 2:1)** - **Gamma-GT (very elevated)** - ALP (raised in later disease) * Bilirubin (elevated - in cirrhosis) * Serum albumin (low - impaired function of liver) | U+Es = deranged in hepatorenal syndrome
56
What imaging Ix can be used in alcoholic liver disease?
* Ultrasound (see fatty changes) - '**FibroScan** (check stiffness of liver → gives indication of fibrosis) * CT and MRI scans (look for fatty infiltration of liver, hepatocellular carcinoma, hepatosplenomegaly, abnormal blood vessel changes, ascites)
57
What is the gold standard Ix for alcoholic liver disease?
Liver biopsy
58
What Ix is used to confirm a diagnosis of alcohol-related hepatitis or cirrhosis?
Liver biopsy
59
DDx for alcoholic liver disease
* Cholecystitis * Hepatits B virus * Hepatitis C * Hepatitis A * Acute liver failure
60
What are the stages of alcohol withdrawal?
6-12 hours: **tremor, sweating, headache, craving** and **anxiety** 12-24 hours: **hallucinations** 24-48 hours: **seizures** 24-72 hours: **“delirium tremens”**
61
How does delirium tremens present?
* Acute confusion * Severe agitation * Delusions + hallucination * Tremor * Tachycardia * Hypertension * Hyperthermia * Ataxia (difficulties with coordinated movements) * Arrhythmias
62
Managment of alcoholic liver disease
* Acohol abstinence (delirium tremens risk → treat with diazepam) * IV thiamine → prevent Wernicke-Korsakoff encephalopathy * Diet high in vitamins + protein * Liver transplant
63
What is the triad of Wernecke-Korsakoff encephalopathy?
* Confusion * Ataxia (poor muscle control) * Nystagmus (rhythmica, repetitive and involuntary movement of the eyes)
64
Complications of alcoholic liver disease
* Cirrhosis * Liver failure * Hepatic encephalopathy * GI bleeding * Portal hypertension
65
What is NAFLD?
Non-alcoholic fatty liver disease = NAFLD * Disease due to accumulation in liver → associated with inflammation → interfere with functioning of liver cells
66
What can NAFLD progress to?
* Hepatitis * Cirrhosis
67
What are the stages of NAFLD?
Non-alcoholic steatohepatitis (NASH) → fibrosis → cirrhosis * NAFLD = steatosis without inflammation * NASH = steatosis + hepatic inflammation - indistinguishable from alcoholic steatohepatitis
68
What are the Rx of NAFLD?
Same as cardiovascular disease: * Obesity * Type 2 diabetes * Hyperlipidaemia * High cholesterol
69
What are the signs and symptoms of NAFLD?
Signs: * Hepatomegaly * RUQ pain * Jaundice Symptoms: * Fatigue + malaise
70
What do the LFTs look like in NAFLD?
All a little elevated Serum ALT>AST level = NAFLD
71
What are the proportions/ratio for the LFTs (transaminases) in alcoholic liver disease and NAFLD?
* Alcoholic liver disease (AST>ALT → 2:1) * NAFLD (ALT>AST)
72
How do you distinuish between NAFLD and NASH on a liver biopsy?
> 5% fat content → NAFLD
73
What imaging Ix can you perform for NAFLD?
Ultrasound liver (Confirm hepatic steatosis (fatty liver))
74
What is the fiest line Ix to measure fibrosis in the liver?
Enhanced Liver Fibrosis (ELF) blood test (ELF - santa is not an alcoholic) | NAFLD fibrosis score is the 2nd line assessment for liver fibrosis
75
How do you differentiate between alcoholic liver disease and NAFLD?
Quantification of alcohol intake
76
What are the pharmacological treatment for NAFLD?
No licensed drug treatment (Just lifestyle changes and maybe statins for hyperlipidaemia)
77
What is the treatment of liver fibrosis?
* Vitamin E * Pioglotazone
78
What is liver cirrhosis characterised by?
* Loss of normal hepatic architecture * Nodular regneration * Bridging fibrosis
79
Brief pathology of liver cirrhosis and portal hypertension
- Liver cirrhosis = result of chronic inflammation + damage to liver cells - Damaged liver cells = replaced with scar tissue (fibrosis) - Nodules of scar tissue = form within the liver - Fibrosis = affects the structure + blood flow through the liver → causing increased resistance in the vessels leading to the liver - = Portal hypertension
80
What are tissue macrophages in the liver called?
Kupffer cells
81
What is the pathology of cirhosis caused by chronic liver injury and liver injury?
- Chronic liver injury results in inflammation + matrix deposition + necrosis + angiogenesis → fibrosis - Liver injury = causes necrosis + apoptosis → releasing cell contents + reactive oxygen species (ROS)
82
What causes micronodular and macronodular cirrhosis?
* Micronodular = alcohol + biliary tract disease * Macronodular = chronic viral hepatitis
83
What does 'compensated' mean in terms of liver function?
When the liver can still function effectively + there are no (or few) noticeable clinical symptoms
84
What does 'decompensated' mean regarding liver function?
- When the **liver is damaged to the point that it cannot function adequately and overt clinical complications** (such as jaundice, ascites, variceal haemorrhage and hepatic encephalopathy) are present. - Events causing decompensation include **infection, portal vein thrombosis** and **surgery**
85
What are the signs of liver cirrhosis? Mneumonic?
BASH OJ - **Bruising** – due to abnormal clotting - **Ascites** - **Splenomegaly** – due to portal hypertension - **Hepatomegaly** – however the liver can shrink as it becomes more cirrhotic - **************Oedema************** - decreased albumin in blood - **Jaundice** – caused by raised bilirubin Symptoms: * Compensated → asymptomatic; non-specific (weight loss, fatigue) * Decompensated → Pruritis, abdominal pain (due to ascites)
86
What vassopressin analogue is used to causes vasoconstriction in oesophageal varices?
Terlipressin
87
What is the gold standard Ix to confirm liver cirrhosis?
Liver biopsy....Obvs!
88
Patient has liver cirrhosis and the blood tests come back. What what they look like?
- Serum albumin + prothrombin time = best indicators of liver function - Albumin (low) - Prothrombin (elevated) - Bilirubin (raised) - AST (raised) - ALT (raised)
89
Why might you have hyponatraemia in severe liver disease?
Hyponatraemia = indicates fluid retention in severe liver disease. (Also, Urea and creatinine become deranged in hepatorenal syndrome)
90
What scan do NICE recommned for patients that are risk of liver cirrhosis every two years?
FibroScan ('Transient elastography')
91
Why might you perform an upper GI endoscopy on someone with liver cirrhosis?
When portal hypertension is suspected → for oesophageal varices
92
What scoring system is used to classify the severity of liver cirrhosis?
Child-Pugh Score
93
What scoring system gives you the estimated 3 month mortality for someone with liver cirrhosis?
MELD Score (Helps guide referral for liver transplant)
94
What is the management for liver cirrhosis?
- Treat underlying cause - Alcohol abstinence - Antivirals for Hep C - **Spironolactone for ascites** - Good nutrition - **high protein + low sodium diet** - Liver transplant Every 6 months: - Ultrasound screening for hepatocellular carcinoma - MELD score - Endoscopy every 3 years in patients without known varices
95
What are the complications of liver cirrhosis?
* Coagulopathy - fall in clotting factors clotting factors II, VII, IX, and X * Thrombocytopenia * Hypoalbuminaemia * Portal hypertension - Ascites - Oesophageal varices - Variceal bleeding * Hepatocellular carcinoma
96
What is jaundice a result of?
Raised/excess bilirubin
97
What is bilirubin a component of?
Bile
98
What is pre-hepatic jaundice? Causes?
Excess of Hb → increases total unconjugated bilirubin * Malaria * Sickle cell anaemia
99
What is hepatic jaundice? Causes?
Failure of hepatocytes to take up, metabolise or excrete bilirubin * Viral hepatitis * Drugs, alcohol * Cirrhosis Increased unconjugated + conjugated bilirubin
100
How does hepatic jaundice present?
* Dark urine * Normal/pale stools
101
What is post-hepatic jaundice? Causes?
Obstruction in the biliary tree * Gallstones * Pancreatitis (head pf pancreas blocks common bile duct) Increased conjugated bilirubin
102
What does dark urine, pale stool and itching indicate?
Hepatic or post-hepatic jaundice
103
A patients test results come back: * Cholestatic pattern of serum LFTs (elevated ALP, GGT) * Anti-mitochondrial antibody Diagnosis?
Primary biliary cholangitis
104
What is Cullen's sign and which condition does it present in?
Bruising around the preumbilical region (Acute pancreas)
105
What is Grey Turner's sign and which condition does it present in?
Bruising on flanks (Acute pancreatitis)
106
First line tests for acute pancreatitis?
LFTs: * Raised serum lipase (lipase to amylase > 2 suggests alcoholic) * Raised ALT and AST (elevated ALT>150 suggests gallstones) CRP Transabdominal ultrasound (initial assessment of gallstones)
107
What scoring system is used to assess the severity of pancreatitis?
Glasgow score * (0-1: mild pancreatitis) * (2: Moderate pancreatitis) * (3 or more: severe pancreatitis)
108
Management for acute pancreatitis?
* Initial resuscitation (ABCDE approach) * IV fluids * Analgesia * Treatment of gallstones in gallstone pancreatitis (ERCP/cholecystectomy) * Antibiotics (evidence of infection) * Treatment of complications (endoscopic or percutaneous drainage of large collections)
109
What is chronic pancreatitis?
* Chronic inflammation due to structural changes (fibrosis, atrophy, calcification) * Results in fibrosis + reduced function in pancreatic tissue * Generally irreversible
110
Difference between acute and chronic pancreatitis?
* Acute pancreatitis = a self-limiting and reversible pancreatic injury associated with mid-epigastric abdominal pain and elevated serum pancreatic enzymes * Chronic pancreatitis = characterised by recurrent or persistent abdominal pain and progressive injury to the pancreas and surrounding structures, resulting in scarring and loss of function.
111
Causes of chronic pancreatitis?
- Alcohol use (most common) - Repeated bouts of acute pancreatitis - Cystic fibrosis - main cause in children - Tumours - Pancreatic trauma
112
Rx for chronic pancreatitis?
* Alcohol * Smoking * Family history * Coeliac disease
113
What are the signs and symptoms of chronic pancreatitis?
Similar to acute pancreatitis - but less intense + longer lasting * **Epigastric pain that radiates to the back** (may be linked to eating meals) * N + V * Endocrine dysfunction: Malabsorption (**weight loss, steatorrhoea, vitamin deficiency**) * Exocrine dysfunction: **Diabetes Mellitus**
114
Ix for chronic pancreatitis
* Lab tests - May have low lipase + amylase (as there may not be enough healthy tissue to make enzymes) - Faecal elastase * Transabdominal ultrasound * Abdominal X-ray (calcifications) * CT abdomen * ERCP/MRCP
115
What is the management of chronic pancreatitis?
* **Lifestyle modification → stopping smoking + alcohol** - **Analgesia** - pain management - **Replace digestive enzymes + nutritional supplements (i.e. lipase)** - Otherwise lack of enzymes = leads to malabsorption of fat, greasy stools (steatorrhea) + deficiency in fat-soluble vitamins * **ERCP with stenting** (treat strictures + obstruction to the biliary system + pancreatic) * **Surgery** (pseudocysts, abscesses, severe chronic pain)
116
What are the complications of chronic pancreatitis?
- ***Chronic epigastric pain*** - ***Loss of exocrine function***, resulting in a lack of pancreatic enzymes (particularly lipase) secreted into the GI tract - ***Loss of endocrine function***, resulting in a lack of insulin, leading to diabetes - ***Damage*** and ***strictures*** to the duct system, resulting in obstruction in the excretion of pancreatic juice and bile - Formation of ***pseudocysts*** or ***abscesses***
117
What is ascites?
Abnormal accumulation of fluid within the peritoneal cavity
118
What conditions can impair blood flow and result in ascites?
* Cirrhosis i.e. portal hypertension * Cardiac failure * Constrictive pericarditis
119
What conditions can decrease the oncotic pressure (low protein), leading to ascites?
* Hypoalbuminaemia * Malnutrition * Nephrotic syndrome
120
What serious condition that causes local inflammation causes ascites?
Peritonitis
121
What is exudate ascites?
* High protein * Extremely bad * Causes: Cancer, sepsis, TB, nephrotic syndrome
122
What is transudate ascites?
* Low protein fluid * Causes: Cirrhosis
123
How do you manage ascites? Briefly
* Treat underlying cause * Diet * Diuretic * Drainage
124
What are the signs of ascites?
* **Abdominal swelling ** * **Shifting dullness** - Flank dullness - Bulging flanks * Jaundice * Oedema (Abdominal swelling with shifting dullness)
125
What should you suspect if a patient with ascites complains of severe pain?
Bacterial peritonitis
126
What are Ix for ascites?
* Presence of fluid = confirmed by demonstrating shifting dullness * Ultrasound * Ascitic aspiration → culture, gram stain, cytology, protein, amylase * Serum Ascites Albumin Gradient (SAAG)
127
What SAAG tell you?
Serum Ascitic Albumin Gradient (SAAG) * Defines the presence of portal hypertension in patients with ascites
128
A patient presents with ascites, they have a history of liver disease, what Ix do you perform to establish whether portal hypertension is also present?
SAAG (Serum Ascites Albumin Gradient)
129
What are the management options to treat ascites?
**Treat underlying cause, diet, diuretic, drainage** * Treat underlying cause * /Low sodium diet * **Aldosterone antagonis**t diuretics (**spironolactone**) * **Paracentesis** (**ascitic tap** or **ascitic drain**) * **Prophylactic antibiotics** against **spontaneous bacterial peritonitis** (ciprofloxacin)
130
What is spontaneous bacterial peritonitis (SBP)?
Infection develops in the ascitic fluid + peritoneal lining without a clear cause (not secondary to ascitic drain or bowel perforation)
131
An ascitic patient starts to deteroriate, what should you suspect? (They develop hypotension)
Spontaneous bacterial peritonitis (SBP)
132
What are the common organisms that can cause spontaneous bacterial peritonitis?
Escherichia coli Klebsiella pneumoniae Gram positive cocci (staphylococcus + enterococcus)
133
What is the prophylaxis and management of SBP?
Prophylaxis: Ciprofloxacin Management: IV cephalosporin (cefataxime)
134
What infection is commonly seen in patients with end-stage liver disease?
Spontanous Bacterial Peritonitis?
135
Rx for SBP?
* Decompensated hepatic state (usually cirrhosis) * GI bleeding * Endoscopic sclerotherapy for oesophageal varices * Low ascitic protein/complement
136
What are the signs and symptoms for SBP?
Signs: * Ascites * Deranged bloods - raised: WBC, CRP, Creatinine, metabolic acidosis * Altered mental status * GI bleeding * Hypotension * Ileus Symptoms: * Fever * Abdominal pain * Vomiting
137
Read: Ix results for SBP
- FBC - Leukocytosis (maybe) - Anaemia (potential GI bleeding) - Blood cultures - Growth of causative organism - LFTs - Liver enzymes (elevated) - Bilirubin (elevated) - Albumin (decreased)
138
What are two major complications of SBP?
* Sepsis * Renal failure
139
What is portal hypertension?
Increased back pressure into the portal system - Liver cirrhosis = ↑resistance of blood flow in the liver - As a result, ↑back-pressure into the portal system
140
What are the 3 categories for portal hypertension? Give an example of each
Pre-hepatic: due to bloackage of portal vein before liver * Portal vein thrombosis Intrahepatic: resulting from distrortion of liver architecture * Cirrhosis (most common) * Sarcoidosis Post-hepatic: due to venous blockage outside of the liver * Right HF * IVC obstruction
141
What are the key presentations of portal hypertension? | Mnemonic
ABCDE - **A - A**scites - **B - B**leeding (haematemesis, piles) - **C - C**aput medusae - **D - D**iminished liver - **E - E**nlarged spleen
142
What are the signs and symptoms of portal hypertension?
Signs: * **Ascites ** * **Hepatic encephalopathy** * **Splenomegaly** * **Oesophago-gastric varices** * **Jaundice** * Signs + symptoms of encephalopathy Symptoms: * **Bleeding** from * **Oesophago-gastric varices**
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Ix for portal hypertension
* Paracentesis (SAAG) - determine if portal hypertension is cause of ascites * Upper endoscopy - diagnose/treat varices * CT/MRI scan - Ascites - Cirrhosis - Splenomegaly - FBC - LFTs
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How do you treat portal hypertension?
* **Beta-blockers** (decrease portal venous pressure) * Non-selective beta-blockers (prophylaxis) * Antibiotics (prophylaxis for SBP) * Diuretics + sodium restriction (for ascites)
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What complications can portal hypertension lead to?
* Oesophageal varices * Caput medusae (distension of abdominal wall veins) * Ascites (increased hydrostatic pressure + hypoalbuminaemia) * Spontaneous bacterial peritonitis * Anaemia, thrombocytopenia, leukopenia
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What are oesophageal varices?
Oesophageal varices are dilated collateral blood vessels that develop as a complication of portal hypertension, usually in the setting of cirrhosis. (Cirrhosis → Portal hypertension → oesophago-gastric varices)
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Where do oesphago-gastric varices tend to develop?
In the **lower oesophagus + gastric cardia**
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What is the cause of oesphago-gastric varices?
Portal hypertension (Rx: cirrhosis, alcoholism)
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Read: Pathology of oesophago-gastric varices
- As these vessels are thin and not meant to transport higher pressure blood, they can rupture - Rupture → haematemesis - Rupture → blood digested → melaena
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Signs and symptoms of oesophago-varices
* Haematemesis * Melaena * Abdominal pain * Rectal bleeding * Hypotension * Tachycardia * Ascites * Splenomegaly * Pallor * Signs of chronic liver damage (jaundice, easy bruising, ascites)
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A patient presents with haematemesis, abdominal pain. O/E find ascites, hypotension, and splenomegaly. You suspect oesphago-gastric varices. What Ix do you perform to confirm the diagnosis?
GI endoscopy
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How might someone with oesophago-gastric varices blood results look like?
- FBC - Microcytic anaemia - And/or thrombocytopenia - U&Es - Hyponatraemia - Due to fluid overload or use of diuretics may be present in patients with cirrhosis with ascites - LFTs - Elevated transaminases (with aspartate aminotransferase/alanine aminotransferase ratio ≥1) - Elevated alkaline phosphatase - Elevated bilirubin - Urea and creatinine - Elevated
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What are some DDx for oesophago-gastric varices?
* Peptic ulcer disease * Mallory-Weiss tear * Hiatal hernia
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What would someone's serum urea levels look like if they have a GI bleed?
**Elevated urea** (elevated without creatinine) (Result of breakdown of blood in the stomach in cases of acute bleeding)
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What is haemochromatosis?
* **Iron storage disorder** * Excessive total body iron * **Deposition of iron in tissues** **Excess iron deposition** due to high levels of **hepcidin**
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Haemochromatosis is caused by high levels of what?
Hepcidin
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What is the genetic mutation that causes haemochromatosis?
* Human Haemochromatosis protein (**HFE**) gene - on **chromsome 6** * **Autosomal recessive**
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Why does haemochromatosis present later in women?
Menstruation regulates iron elimination from the body
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A white male patient presents with skin pigmentation complaining joint pain and chronic tiredness. O/E you detect hepatomegaly. Possible diagnosis?
Haemochromatosis
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What are the signs and symptoms of haemochromatosis?
Signs: * Skin pigmentation (bronze/grey discolouration) * Hair loss * Erectile dysfunctiom * Hepatomegaly Symptoms: * Chronic tiredness * Arthralgias (joint pain) * Amenorrhoea * Cognitive symptoms (memory + mood disturbance)
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What investigations should you request for someone with haemochromatosis?
* **Transferrin saturation** (Iron overload → high transferrin saturation) * **Serum ferritin level** * **Genetic testing** (confirm diagnosis) Liver biopsy (Perl's stain used to establish iron conc in parenchymal cells) MRI (iron deposits in liver + heart)
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DDx for haemochromatosis
* Hepatitis B * Hepatitis C * Non-alcoholic fatty liver disease (NAFLD)
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Management for haemochromatosis
* **Venesection** - weekly * Monitoring serum ferritin * Avoid alcohol * **Genetic counselling**
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Complications of haemochromatosis?
* Type I diabetes (iron deposits in pancreas) * Liver damage/cirrhosis * Endocrine (iron deposits in pituitary glands) * Cardiomyopathy (iron deposits in the heart * Hypothyroidism (iron deposits in the thyroid)
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What is Wilson's disease?
**Autosomal recessive** disorder of **copper** **accumulation** + **toxicity** (ATP7B gene mutation)
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What happens in Wilson's disease (pathology)?
When **hepatic storage** is exceeded → copper is released into the circulation → **deposited into other organs**
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What are the key presentations (groups of Sx) for Wilson's disease?
* Kayser-Fleischer ring in cornea (brownish circles in eyes) * Hepatic problems * Neurological problems * Psychiatric problems
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A patient presents with brown circles around their pupils. What are these and what condition are they associated with?
* Kayser-Fleischer rings * Indication of Wilson's disease
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What are the signs of Wilson's disease?
* Kayser-Fleischer rings in cornea * Osteopenia * Haemolytic anaemia (RBCs being broken down too fast) * Renal tubular damage (leading to renal tubular acidosis)
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What are the neurological symptoms found in Wilson's disease?
- Concentration + coordination - Dysarthria (speech difficulties) - Dystonia (abnormal muscle tone) - Parkinsonism (copper deposition in the basal ganglia) - **Asymmetrical** in Wilson's disease (differentiates from Parkinson's)
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A patient presents with Parkinsonism Sx with Kayser-Fleischer rings in their eyes. Possible diagnosis?
Wilson's disease
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What is the first-line and gold standard Ix for Wilson's disease
First line: * Low serum caeruloplasmin (carries copper in the blood) Gold standard: * Liver biopsy (diagnostic) * 24-hr urine copper assay (elevated)
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What is the management for Wilson's disease
COPPER CHELATION * Penicillamine * Trientene
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Major complication of Wilson's disease?
Liver failure
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What genetic mutation causes Alpha-1 Antitrypsin Deficiency?
Autosomal recessive mutation in Alpha-1 antitrypsin Chromosme 14
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What is the alpha-1 antitrypsin enzyme responsible for?
Neutralising neutrophil elastase
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Who does Alpha-1 antitrypsin deficiency typically affect?
Men aged 32-41
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Pathology of alpha-1 antitrypsin deficiency
* Elastase = digests connective tissues (secreted by neutrophils) * Alpha-1-antitrypsin (A1AT) = mainly produced by the liver (travels around the body) * A1AT = inhibits neutrophil eastase enzyme → offering protection
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What are the 2 major organs affected by alpha-1-antitrypsin deficiency?
* **Liver**: Liver cirrhosis (after 50 years) * **Lungs**: Bronchiectasis + emphysema (after 30 years)
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A patient presents which jaundice, wheezing, productive cough and exertional dyspnoea. What condition(s) do they have?
* Liver cirrhosis * Bronchiectasis + emphysema ALPHA-1-ANTITRYPSIN DEFICIENCY
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What are the first line and gold standard testing for A1AT deficiency?
First line: * **Serum alpha-1-antitrypsin (low)** * **Liver biopsy** (cirrhosis, Acid-Schiff positive staining globules) * High resolution **CT thorax** (diagnoses bronchiectasis + emphysema) Gold-standard: * **Genetic testing for A1AT gene**
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DDx for Alpha-1-Antitrypsin deficiency
- Viral hepatitis - Alcoholic liver disease - Asthma - COPD - Bronchiectasis
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Mx for Alpha-1-antitrypsin deficiency
* Stop smoking (emphysema) * Symptomatic management * Organ transplant (for end-stage liver or lung disease) * Monitor for complications
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What is a complication of A1AT deficiency?
Granulomatosis with polyangiitis (Wegener's granulomatosis)
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What is Wernicke's encephalopathy?
Neurological emergency resulting from **thiamine** (vitamin B1) **deficiency** (Result from excessive alcohol)
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Which presents first; Wernicke's encephalopathy or Korsakoff's syndrome?
Wernicke's encephalopathy
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What can result in a thiamine deficiency?
* Decreased intake (oral or parenteral) * Relative deficiency - due to increased demands * Malabsorption in the GI tract
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How does Wernicke's encephalopathy present?
* Confusion * Occulomotor disturbances (disturbances in eye movements) * Ataxia (difficulties iin coordinated movement)
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Ix for Wernicke's encephalopathy
* Therapeutic trial of parenteral thiamine * FBC (infection, raised WBC count) * LFTs (elevated) * Blood alcohol level
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DDx for Wernicke's encephalopathy
* Alcohol intoxication * Alcohol withdrawal * Viral encephalitis
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Prevention + treatment of Wernicke's pathology?
Thiamine supplementation + abstaining from alcohol
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Name 2 complicatiosn of Wernicke's encephalopathy
* Korsakoff's psychosis * Hearing loss * Seizures
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Difference between Wernicke's encephalopathy and Korsakoff's syndrome?
**Features of Wernicke’s encephalopathy** - Confusion - Oculomotor disturbances (disturbances of eye movements) - Ataxia (difficulties with coordinated movements) **Features of Korsakoffs syndrome** - Memory impairment (retrograde and anterograde) - Behavioural changes (Memory + behaviour = difference)
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Difference between acute and chronic hepatits?
* Acute = liver inflammation 6 months or less * Chronic = more than 6 months * All cases are acute in the first instance → some go on to cause chronic hepatitis
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Which types of hepatitis are transmitted by the faecal-oral route and are linked to contaminated food + water?
HepA and HepE
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What is the treatment for HepA and HepE?
Supportive treatment as they are self-limiting
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Which forms of hepatitis are preventable by vaccine?
HepA and HepB
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Which forms of Hepatitis are blood-borne?
Hep B,C,D
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Which two forms of hepatitis can only be acute?
HepA and HepE
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Which is teh only form of hepatitis that is made of dsDNA?
Hepatitis B
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Which form of hepatitis does HepD need?
Hep D (Best BuDs)
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How is HepB transmitted?
3Bs: * Bodily fluids * Blood * Birth
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Is HepA a notifiable disease?
Yeah
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What are the causes of HepA in high-income countries?
* Travel * Sex (MSM) * Iv drug users
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What are some Rx for HepA?
* Shellfish * Travellers * Food handlers * Poor access to clean drinking water
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What are the phases of presentation hepatitis A?
* Incubation period (28 days) * Pre-icteric phase (non-specific symptoms + abdo pain) * Icteric phase (jaundice + hepatosplenomegaly) - Icteric = jaundice * 100% immunity after infection
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Ix for Hepatitis A
* Antibody tests (HAV antibodies) * LFTs (raised AST + ALT + bilirubin) * FBC (reduced WBC count) * Raised ESR
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What are the antibodies in the acute and chronic phase of HepA?
Acute: Anti-HAV IgM (non-specific) Chronic: Anti-HAV IgG (longer lasting)
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DDx causes of HepA
* Other causes of jaundice * Other types of viral + drug-induced hepatitis
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Mx for HepA
* SUPPORTIVE * Monitor liver function (INR, albumin, bilirubin, etc.) - Acute liver failure (’fulminant hepatitis’) in <1% - Liaise with hepatology/liver transplant centre - Management of close contacts (HNIG (human normal immunoglobulin), vaccine) - Primary prevention: Hep A vaccination - e.g. travellers, MSMs, IDUs
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Mx for HepA
* SUPPORTIVE * Monitor liver function (INR, albumin, bilirubin, etc.) - Acute liver failure (’fulminant hepatitis’) in <1% - Liaise with hepatology/liver transplant centre - Management of close contacts (HNIG (human normal immunoglobulin), vaccine) - Primary prevention: Hep A vaccination - e.g. travellers, MSMs, IDUs
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Complications for HepA
* Pancreatitis * Acalculous cholecytitis
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When can HepE become chronic?
In immunocompromised patients
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Which genotype of HepE is linked to undercooked meat?
G3&4
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Which type of hepatitis is linked to undercooked pork?
HepE (pork-EEEE)
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A patient presents with an acute neurological problem and abnormal LFTs. What is a possible viral diagnosis?
HepE
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What are the anitbodies found in the acute and chronic phases of HepE?
* Acute: Anti-HEV IgM * Chronic: Anti-HEV IgG
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Serology for HepE in immunocompromised patients is unreliable. What do you have to measure and monitor?
HEV RNA in serum +/- stool
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What anti-viral do you give in HepE cases?
Ribavirin
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Someone can have HepB and be co-infected with which other type of hepatitis?
HepD
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Rx or HepB
* Healthcare personnel * Emergency + rescue teams * KD/dialysis patients * Travellers * MSM * IV drug users
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What serology do you perform for HepB?
Acute: Anti-HB core IgM Chronic: Anti-HB core IgG - If they **have e-antigen (eAg)** → **high levels** of **infections** - **Presence** of **e-antibody (eAb)** → **lower** **levels** of **infection**
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What is the management for acute and chronic hepatitis B?
* Acute: Supportive treatment * Chronic: Pegylated interferon-alpha 2 - Neucloside analogues (tenofovir, entecavir)
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What do you check to check the vaccine response to HepB?
Anti-HB surface antibody (sAb)
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Preventation of HepB
- Check vaccine response with anti-HB surface antibody (sAb) - Antenatal screening (HBsAg testing) of pregnant mothers - HBV vaccination administered to baby at birth at 1,2,3,4,12 moths - Screening + immunisation - Sexual + household contacts - Blood products - Sterile equipment + universal precautions in healthcare - Immunisation of healthcare workers + higher risk groups (MSMs, IV drug users)
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Which hepatitis is common in Eastern Europe?
Hep D
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Ix for HepD
- Same as HepB - antibody test - Hep D antibody → if positive, test HDV RNA
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Management for HepD?
Pegylated interferon-alpha 2a
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Can you be re-infected with HepC?
Yes
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What is the serology process for HepC testing?
HCV antibody (can be negative in acute infections/immunocompromised patients) → HCV RNA (detected = current HCV) → HCV genotype (1a, 1b, 3)
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What medications do you use when treating HepC?
Direct acting ant-virals (DAAs) * Ribavirin + 2 DAAs (Glecaprevir/Pibrentasvir, Elbasvir/Grazoprevir) - ends in **-vir**
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Genetic risk factor for autoimmune hepatitis
HLA-DR3.DR4 (Young female)
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Signs and symptoms of autoimmune hepatitis?
* Fever * Jaundice * Hepatosplenomegaly (Wide spectrum: asymptomatic to cirrhosis + liver failure)
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Lab results for autoimmune hepatitis
↑↑ALT ↑AST ↓Albumin ↑Prothrombin time
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Antibodies present in type one autoimmune hepatitis?
Antinuclear antibodies (ANA)
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Do you perform a liver biopsy for autoimmune hepatitis?
Yes
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Mx for autoimmune hepatitis?
Immunosuppression: Prednisolone +/- azathoprine Liver transplant (if resistant to drug therapies)
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Complications of hepatitis?
* Acute liver failure * Chronic liver failure * Hepatocellualr carcinoma * Long-term suppression that can lead to malignancies
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What is the underlying pathology of hepatic encephalopathy?
As liver fails, nitrogenous waste e.g. **ammonia** builds up in circulation and passes to brain → permanent brain damage (ammonia is neurotoxic) - Ammonia = produced by intestinal bacteria - when they they break down proteins + absorbed in the gut - Ammonia builds up due to: - Functional impairment of liver cells = prevents the metabolising the ammonia - into harmless waste products - Collateral vessels between the portal + systemic circulation = mean that ammonia bypasses liver altogether → enters the systemic system directly
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Risk factors hepatic encephalopathy
* Hypovolaemia * Constipation * Diuretic overdose * Exces protein intake * Hypokalaemia + hyponatraemia
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How does hepatic encephalopathy present?
- Acute → reduced consciousness + confusion - Chronic → changes to personality, memory, mood Signs: - Hepatomegaly - Ascites - Jaundice - Peripheral oedema Symptoms: - Mood disturbances - Sleep disturbances - Motor disturbances - Advanced neurological deficits
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How is a diagnosis of hepatic encephalopathy diagnosed?
Based on: * Reported neurological deficits * Laboratory abnormalities showing severe liver dysfunction * LFTs (abnormal) - Coagulation profile (elevated prothrombin time)
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Mx for a patient with hepatic encephalopathy
* Laxative (lactulose) = help clear ammonia from the gut before it is absorbed * Antibiotics (rifaximin) = reduces the number of bacteria in the gut producing the ammonia * Nutritional support: Nasogastric feeding