Liver et al Flashcards

1
Q

Main 4 points in acute liver failure

A

Hepatic encephalopahty
Jaundice
Coagulopathy
With no evidence of serious prior liver disease.

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2
Q

Time scale and classification of acute liver disease

A
Hyperacute = within 7days 
Acute = 8-29 days
Subacute = 4 to 12 weeks 

Also Fulminant = within 2 weeks and subfulminant within 2-12weeks.

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3
Q

Risk factors for acute liver failure

A

Chronic alcohol use
Poor nutrition
Older age (over 40)
Narcotic use

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4
Q

Causes of acute liver failure

A
Paracetamol toxicity
Viral hepatitis - HBV and HAV
Budd-Chiari syndrome
CMV, EBV
Autoimmune liver disease
Wilson's disease
Alpha-1 antitrypsin deficiency.
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5
Q

S+S of acute liver failure

A

Jaundice
Symptoms of hepatic encephalopathy - altered mood, sleep disturbance, slurred speech, confused.
Abdo pain
Nausea and vomiting

O/E:
Signs of hepatic encephalopathy - apraxia, liver flap, stupor.
Jaundice
Hepatomegaly

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6
Q

How to test for apraxia

A

Copy a 5 point star

Show me how to brush your teeth, use a hammer, comb your hair etc etc

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7
Q

Ix for acute liver failure

A
Laboratory:
FBC
U+E
LFT
Clotting and synthetic liver function
BM
Paracetamol levels
Viral hepatitis antibodies
Copper studies, alpha1-anti trypsin levels etc etc
Blood culture, urine culture, ascitic tap mc+s
Imaging:
Abdo USS
CT/MRI of liver
CT head
Doppler USS of liver and portal vein.
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8
Q

Management of acute liver failure

A

In ITU 🌈
A-E including urinary catheter, NG tube, frequent BMs.
Treat cause.

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9
Q

Treating complications of acute liver failure

1) Cerebal oedema
2) Ascites
3) Hypoglycaemia
4) Encephalopathy

A

1) 20% Mannitol IV
2) Fluid and salt restriction, diuretic e.g spironolactone.
3) 10% glucose IV
4) Lactulose + Rifaximin.

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10
Q

List 5 hepatotoxic drugs

A
Pyrazinamide, Isoniazid (TB)
Paracetamol
Methotrexate
Azothioprine
Infliximab
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11
Q

Liver function test:
Which are markers of hepatocellular damage?
Which are markers of the liver’s synthetic function

A

Damage = ALT, AST, ALP, gamma-GT.

Synthetic liver function = PT, Albumin and bilirubin.

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12
Q

Interpreting ALT and ALP

A
ALT = mostly found within hepatocytes so if serum levels high = injury to hepatocytes.
ALP = high concentrations in liver, bile duct and bone tissue, if serum levels high = cholestasis.

Therefore:
a >10X rise in ALT and a <3X rise in ALP = hepatocellular injury.
a >3X rise in ALP and a <10X rise in ALT = cholestasis.
And can have a mixed picture too.

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13
Q

List some functions of the liver

A

Bile production - stored in gallbladder.
Metabolise fat soluble vitamins e.g. vitamin D hydroxylation.
Drug metabolism using CYP450 enzymes.
Conjugation and elimination bilrubin
Synthesis of plasma proteins e.g. albumin
Synthesis of clotting factors
Gluconeogenesis

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14
Q

Pathophysiology of cirrhosis

A

Fibrosis and loss of normal liver architecture to abnormal, regenerative nodules.

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15
Q

Causes of cirrhosis

A
Chronic HBV and HCV
Non-alcohol fatty liver diease
Chronic alcohol
Alpha1-antitrypsin deficiency
Wilson's disease
Budd-Chiari syndrome
Drugs = methotrexate
Primary biliary cholangitis
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16
Q

Presentation of liver cirrhosis

A
Abdo distension
Jaundice
Itch
Easy bruising
Poor memory
Fatigue and weakness
Peripheral oedema

O/E: LOTS!!!
Hands = Leuconychia (white nails), clubbing, palmar erythema, spider naevi, Dupuyren’s contracture.
Face = telangiectasia, xanthelasma, yellowing of sclera in eyes (jaundice)
Abdo = gynaecomastia, hepatomegaly, visible collateral vessel sin the abdo wall. Ascites + shifting dullness and fluid thrill on percussion.
Other = muscle wasting, loss of pubic hair, testicular atrophy on men.

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17
Q

Investigating cirrhosis and expected results

A
Laboratory:
LFT - raised liver enzymes.
Gamma-GT - raised.
Albumin - low
PT and INR - high
FBC 
Ascites tap mc+s
Find cause - viral hepatitis antibodies serology, ferritin, alpha1-antitrypsin levels.
Imaging:
Abdo US + duplex
Abdo CT/MRI
Liver biopsy
Transient elastography
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18
Q

What will you see in the histology of a liver biopsy in cirrhosis

A

Loss of hepatic architecture.
Bridging fibrosis.
Nodular regeneration.

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19
Q

Classification of liver cirrhosis

A

Child-Pugh-Turcotte Score.

Based on: (BAAIN mnemonic)
Bilirubin
Ascities
Albumin low
INR high
Encephalopathy

Class = level of cirrhosis and determines life expectancy.

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20
Q

End-stage liver disease score

A

Model of End-Stage Liver Disease score uses bilirubin, sodium, creatinine and INR/PT to assess liver disease.
Used in liver transplant list.

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21
Q

Management of cirrhosis

A

Lifestyle advice = avoid alcohol, exercise to avoid muscle wasting, good nutrition, avoid hepatotoxic drugs (NSAIDs).
Management of ascites = fluid restriction, low salt diet, spironolactone +/- furesomide, daily weight.
Liver transplant list appropriateness.

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22
Q

Fluid restriction level in ascites and target weight loss with spironolactone

A

Max 1.5L per day.

Aim for up to 0.5g/day of weight loss.

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23
Q

Complications of cirrhosis

A
Hepaticellular carcinoma
Coagulopathy
Encephalopathy
Sepsis
Spontaneous bacterial peritonitis
Hypoglycaemia
Oesophageal varicies (and GI haemorrhage)
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24
Q

Spontaneous bacterial peritonitis

A

Ascites + sudden deterioration.
Commonly E.coli, Klebsiella
Rx = Cefotaxime or Tazosin
Can give prophylactic Ciprofloxacin.

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25
Portal triad and another thing which helps bile flow
Portal vein Hepatic artery Bile duct Found in a corner of each liver lobular. Bile canaliculi.
26
Decompensated cirrhosis
Acute deterioration in a patient with chronic liver disease. Precipitated by: infection (Spont bac peritonitis), high alcohol intake, drugs (NSAIDS, opiates), portal vein thrombus. S+S: jaundice, increasing ascites, hepatic encephalopathy, AKI, oesophageal varices haemorrhage/GI bleed. Ix: Vital signs, FBC, U+E, LFT, coagulation, blood glucose, blood cultures, ascitic tap MC+S, urine dipstick, CXR and AXR. Alcohol intake history. Rx: A to E assessment, treat cause (ABx, FFP/transfusion, lactulose + Rifaxmin)
27
Grading of hepatic encephalopathy
West-Haven criteria
28
Compound involved in hepatic encephalopathy
Serum ammonia build up causing glutamine excess in brain which draws fluid in = cerebral oedema.
29
Name 3 metabolic liver diseases and how are they all inherited
Hereditary haemochromatosis, Chr 6 Wilson's disease, Chr 13 Alpha-1 antitrypsin deficiency, Chr 14 All autosomal recessive 🧬
30
Pathophysiology of hereditary haemochromatosis
Autosomal recessive. Inherited on chromosome 6, HFE gene. Lack of hormone HEPCIDIN which regulates iron levels. Increased iron absorption from intestine. Deposits of iron in liver, heart, skin, joints and pancreas. More common in males as females loose blood and hence iron during menses.
31
S+S of hereditary haemochromatosis
Early in life = asymptomatic, tiredness, arthralgia, low libido. Mid-life = slate-grey tinged skin pigmentations, arthralgia, DM, fatigue and weakness, hepatomegaly, signs of cirrhosis and chronic liver disease, cardiac arrythmias.
32
Where is the arthralgia common in HH
2nd and 3rd metacarpophalangeal in hands.
33
Investigating hereditary haemochromatosis
Iron studies: Serum transferrin saturation = >45% (specific marker). Serum ferritin = raised. Serim TIBC = low/normal ``` LFT HFE gene testing. MRI Liver biopsy + Perl's stain = iron loading and fibrosis. Liver FibroScan ``` Ix extra-hepatic manifestations: echocardiogram, BM/HbA1c/OGTT.
34
Management of hereditary haemochromatosis
Weekly venesection - target ferritin <50mcg/L. Monitor for DM and arrhythmias. Consider for transplant.
35
Pathophysiology of Wilson's disease
Autosomal recessive. Mutation in chromosome 13. Copper retention in liver and basal ganglia due to impaired excretion in the liver.
36
Presentation of Wilson's disease
Early in life = Liver disease signs (hepatitis, cirrhosis). Young adults = CNS signs (tremor, dysarthria, dystonia, dementia, ataxia). Psych = depression, change in libido, personality change, poor memory. O/E: Kayser-Fleischer rings from copper in iris. Blue nails Hypermobile joints Grey tinged skin Hepatomegaly and signs of cirrhosis (spider naevi, jaundice, ascites, bruising).
37
Investigating Wilson's disease
Copper studies: 24hr urinary copper excretion = high Serum copper = low Serum caeruloplasmin = low ``` LFT = raised enzymes. Hepatic biopsy = high copper levels. Slit-lamp exam = Kayser-Fleischer rings. Head MRI = degeneration of basal ganglia. Genetic testing for Wilson's disease ```
38
Management of Wilson's disease
Lifestyle advice = avoid copper rich foods (chocolate, mushrooms, liver meat). Avoid alcohol and hepatotoxic drugs. Chelation agent to bind to copper and excrete in urine = penicillamine (oral) Prevent copper absorption = zinc compounds. Liver transplantation appropriateness. Screen family!
39
Drug for chelation of copper in Wilson's disease
Penicillamine | S/Es = rash, nausea, anaemia, haematuria etc etc.
40
Pathophysiology of alpha1-antitrypsin deficiency
Autosome recessive. Chromosome 14, SERPINA1 gene. Loss of protease inhibitor involved in inflammatory cascade causing serpinopathy. Loss of neutrophil elastase enzyme action.
41
S+S of alpha1-antitrypsin deficiency
Mostly males. Symptoms fo COPD in under 40yrs e.g. productive cough, SOBOE, wheeze. Symptoms of cirrhosis e.g. ascites, jaundice, confusion, hepatomegaly, cholestasis.
42
Investigating alpha1-antitrypsin deficiency
Serum alpha1-antitrypsin levels = low --> genetic testing/phenotyping. Spirometry = obstructive pattern. CXR LFTs - raised enzymes. Liver biopsy = diastase-resistant globules, +ve for Periodic acid Schiff
43
Management of alpha1-antitrypsin deficiency
Lifestyle advice - smoking cessation, annual influenza vaccine. Treat as for COPD Monitor LFT, screen for HCC.
44
Drug to help pruritus in liver disease
Colestyramine.
45
Pathophysiology behind autoimmune hepatitis
Autoantibodies against hepatocyte surface antigen cause ***inflammation and necrosis***. T-cell mediated attack. Associated with HLA-DR3
46
Common patients with autoimmune hepatitis
Females. Not usually that old, under 40yrs.
47
Presentation of autoimmune hepatitis
Acute = 40% present acutely. Fever, jaundice, abdo pain. Subacute = gradual onset jaundice, pruritus, hepatomegaly, abdo discomfort, signs of cirrhosis (confusion, ascites) CFx of other autoimmune diseases e.g. arthralgia, bowel dysfunction (UC).
48
Investigating autoimmune hepatitis
LFT - raised transaminases, ALT/AST >1.5X. FBC - anaemia, low WCC, low platelets esp if splenic dysfunction too. Criteria: Serum protein electrophoresis for autoantibodies - ANA, ASMA, LKM1. Hypergammaglobulinaemia esp IgG Liver biopsy - mononuclear infiltrates of portal and periportal areas, piecemeal necrosis, fibrosis, cirrhosis.
49
What is seen in liver biopsy of autoimmune hepatitis
Mononuclear infiltrates in portal and periportal areas = interface hepatitis. Piecemeal necrosis Fibrosis.
50
Management of autoimmune hepatitis
1) Prednisolone - high dose then reduce after 1 month for maintenance. 2) If corticosteroids CI can use Azathioprine or if not responsive to mono therapy use in combo. Liver transplant
51
Definition of portal hypertension
Increase in portal venous pressure due to increase in hepatic vascular resistance and hepatic blood flow. Commonly caused by chronic end-stage liver disease. The raised pressure opens up venous collaterals to connect the portal and system circulation. Clinically significant if pressure gradient is >10mmHg.
52
Causes of portal HTN
Prehepatic - before portal vein in liver: - Congenital atresia - Portal venous thrombus - Extrinsic compression by tumour. Hepatic: - Cirrhosis - Chronic hepatitis - Sarcoid granulomata. Post-hepatic - block in hepatic vein: - Budd-Chiari syndrome - Constrictive pericarditis - Right heart failure
53
Sites for portal-systemic circulation collaterals
Gastro-oesophageal junction = oesophageal varices. Anterior abdo wall = visible as caput medusae around umbilical area. Ano-rectal junction Retroperitoneal viscera.
54
Effects of portal HTN on systemic circulation
``` Produce hyperdynamic circulation Bounding pulse Hypotension Increases cardiac output Salt and water retention = hypervolaemia and hypernatraemia. ```
55
Presentation of portal HTN
Presents as chronic liver disease mostly - jaundice, alcohol consumption, ascites, hepatomelgay, splenomegaly, hepatic encephalopathy, visible collaterals on abdo wall, spider naevi, gynaecomastia, liver flap, oesophageal varicies bleed. Vital signs = bounding pulse, low BP but warm peripheries.
56
Investigating portal HTN
``` Abdo USS. Doppler US of portal vessels. Spiral CT Endoscopy for oesophageal varices. hepatic venous pressure gradient (>10mmHg) ``` Bloods: LFT, U+E, FBC, clotting profile, PT. Find cause - iron studies, copper studies, hepatitis serology etc etc
57
Management of portal HTN
Reduce BP = non-selective beta blockers (propranolol), nitrates. Surgical = transjugular intrahepatic bypass shunt. Treat or manage cause - diuretics, salt and fluid restriction,
58
Hepatopulmonary syndrome
Complication of portal HTN | Hepatic dysfunction, hyperaemia, extreme vasodilation.
59
Common cause of haemoperitoneum
Trauma + HCC
60
Common cancers in the liver
Most common are due to metastasis from breast, bronchus and GI tract. Primary liver tumours = hepatocellullar carcinomas.
61
Risk factors/Causes for hepatocellular carcinoma
``` MALE Hepatitis B and C virus. Heavy, chronic alcohol consumption. Metabolic syndrome and obesity. Non-alcohol fatty liver disease. ```
62
S+S of HCC
``` Fatigue Anorexia RUQ pain Loss of weight Bruit over liver area on auscultation. ``` S+S of cirrhosis e.g. Itch, bleeding oesophageal varices, ascites, jaundice, hepatomegaly,collateral veins on chest, liver flap.
63
Ix for HCC
CT = DIAGNOSTIC, then MRI and BIOPSY. Lab: FBC, LFT, U+E, PT, viral hepatitis serology, **alpha fetoprotein**. Screening via ultrasound scans.
64
Management of HCC
Resection of tumour. Ablation therapy Liver transplant list. Advanced/untreated = Lenvatinib or Sorafenib. Not great prognosis, 6months from diagnosis.
65
Prevention measures of HCC
High-risk group annual ultrasound scan screening and monitor AFP (HBV patients). HBV vaccine programme. Needle exchanges, safer sex education.
66
Another name for Budd-Chiari Syndrome
Hepatic venous outflow obstruction - obstruction anywhere from inferior vena cava to small hepatic veins.
67
Triad in Budd-Chiari
Abdo pain, ascites and hepatomegaly (quite rapid onset).
68
Causes of hepatic venous outflow obstruction/ B-C syndrome
Vascular - right heart failure, constricitve pericarditis, protein C and protein D deficiency and other thrombophilic disoders. Infective - syphilis, amoebic abscess Trauma Autoimmune - SLE, Sjogren's, IBD Iatrogenic - COCP Neoplastic - HCC, Wilm's tumour Congenital - congenital anatomical abnormality in vena cava.
69
Presentation of hepatic venous outflow obstruction
Sudden abdo pain Ascites Hepatomegaly AKI rare = jaundice.
70
Ix and Mx of hepatic venous outflow obstruction
Doppler USS Other = LFT, PT, ascitic fluid mc+s, CT or MRI of liver, US of hepatic vessels. Liver biopsy. Treat cause.
71
Pathophyisology behind ascites
Decreased oncotic pressure due to hypoalbuminaemia and increased hydrostatic pressure form portal hypertension
72
3 stages of alcohol liver disease
Steatosis (fatty), mainly triglycerides. Hepatitis (inflammation and necrosis) Cirrhosis (regenerative nodules, fibrosis, loss of normal architecture)
73
Investigating alcohol liver disease
``` LFT Bilirubin Albumin gamma-GT PT FBC, U+E. ``` Hepatic USS Liver biopsy - depends on extent of disease on what you will see. Steatosis -->. hepatitis --> cirrhosis signs.
74
Treatment of alcohol liver disease
Lifestyle = REDUCE alcohol consumption. Avoid smoking, reduce weight, nutritional advice. Pharma = corticosteroids e.g prednisolone. Assess appropriate for transplant?
75
Other causes/RFx of non alcohol fatty liver disease
``` Metabolic syndrome/Obesity T2DM PCOS OSA Hypothyroidism Drugs e.g. methotrexate, corticosteroids ```
76
Signs and symptoms of alcohol and non alcohol related fatty liver disease (steatosis/steatohepatitis)
``` Asymptomatic Fatigue Right upper quadrant pain. Hepatomegaly Persistent elevation in LFTs! ``` S+S of cirrhosis: Pruritus, jaundice, nausea, bruising, gynaecomastia, spider naevi, ascites etc etc
77
LFT in steatosis
ALT x3 and are higher than AST levels. | Although AST:ALT ratio is <1
78
LFT in acute alcohol hepatitis
AST:ALT ratio >2
79
Management of non alcohol related fatty liver disease
LIFESTYLE: avoid alcohol, loose weight, control co-morbidities e.g. DM, HTN. Secondary care Rx = vitamin E
80
Causes of liver abscesses
``` Pyogenic = bacteria e.g. E.coli, Klebsiella. Complications of appendicitis, cholangitis, biliary stones. Amoebic = complication of amoebiasis - Entamoeba histolytica. ``` RFx = DM, immunocompromised, local malignancies, visit to amoebiasis area.
81
S+S of liver abscess
``` Fever Fatigue Nausea and vomiting RUQ tenderness Hepatomegaly ```
82
Ix for liver abscess
``` FBC LFT Synthetic function - albumin and PT Blood cultures CRP and ESR Serology for E.histolytica. ``` Abdo USS --> CT. Guided needle aspiration for mc+s
83
Mx of liver abscess
Drainage | ABx - Tazosin
84
Acute pancreatitis causes
``` Causes: Gall stones Ethanol/alcohol Trauma Surgery Malignancy Autoimmune conditions e.g. SLE Scorpion venom Hyperlipidaemia, Hypercalcaemia Post-endoscopic retrograde cholangiopancreatography. EMV, mumps and other infections Drugs e.g. thiazide diuretics ```
85
Complications of acute pancreatitis
Haemorrhagic pancreatitis Necrosis of pancreas and decline in pancreatic function and infected pancreatic necrosis. Pseudocyst (disruption in pancreatic ducts lead to collections of pancreatic fluid). Pancreatic abscess Fistula e.g. to abdo cavity causing ascites, pericardial cavity causing effusions.
86
Presentation of acute pancreatitis
Severe, sudden onset epigastric pain, radiating to back. (**intensity and location of pain not correlate with severity**) Worse on movement and alleviated by fetal position. Nausea and vomiting Anorexia ``` O/E: tachycardia Hypotension Abdo tenderness in upper right quadrant, rebound and guarding. Abdo distension. Cullen's sign Grey-Turners sign ```
87
Cullen's sign and Grey-Turners sign
Present in haemorrhage pancreatitis. Cullens = bluish discolouration around umbilicus. Grey-Turner's = bluish bruising-like discolouration on flanks.
88
Ix and Mx for acute pancreatitis
Resusciation - oxygen, fluids, analgesia, assess feeding ability/NG tube, Abx e.g. Ciprofloxacin. Ix to prioritise - Lipase, amylase, haematocrit (sign of necrosis), ABG. Transabdo USS in resus bay, CT of abdomen. Analgesia - not morphine as can spasm Sphincter of Oddi, use buprenorphine ± intravenous (IV) benzodiazepines.
89
Criteria for acute pancreatitis prognosis
Glasgow criteria Uses info from first 48hrs of admission to predict prognosis. Score greater than 2 = severe pancreatitis likely. ``` Parameters = PANCREAS PaO2 Age (>55) Neutrophils/WCC Calcium Renal function (urea) Enzymes (Lactate dehydroengase) Albumin Sugar (blood glucose) ```
90
Criteria for alcohol induced pancreatitis
Ranson score
91
Causes of chronic pancreatitis
``` ALCOHOL Smoking Autoimmune e.g. Sjogren's, primary biliary cirrhosis. Drugs - oestrogen, thiazide diuretics. Tropical Idiopathic ```
92
S+S of chronic pancreatitis
Recurrent, chronic upper epigastric pain. Can radiate to back. Described as deep, severe. Alleviated on sitting forward. Nausea Anorexia Hx of alcohol misuse Hx of recurrent acute pancreatitis. Exocrine dysfunction = weight loss (malabsorption), statorrhoea. Endocrine dysfunction = DM and impaired glucose regulation. O/E: Jaundice Abdo distension
93
2 pathological mechanisms in chronic pancreatitis
Endocrine dysfunction from islets of langerhans, failure to produce insulin. Exocrine dysfunction from acini cells, failure to produce digestive enzymes.
94
Ix chronic pancreatitis
``` Lab: FBC Faecal elastase U+E Creatinine Amylase Calcium HbA1c LFT ``` Imaging: Abdominal USS CT scan
95
Management of chronic pancreatitis
Lifestyle advice - avoid smoking and alcohol. Diet advice - low fat, high protein, high calorie diet Analgesia - paracetamol, ibuprofen. Nutritional - replace pancreatic enzymes with Lipase, Creon. Supplement fat soluble vitamins. Screen for DM Screen for osteoporosis (malabsorption complication)
96
Dietary and nutritional support in chronic pancreatitis
Low fat, high protein, high calorie diet. Supplement fat soluble vitamins Pancreatic enzyme supplements.
97
Main complication of chronic pancreatitis
Diabetes mellitus.
98
Functions of pancreas
Endocrine - Islets of Langerhans produce insulin Exocrine - acinar cells produce pancreatic fluid Pancreatic fluid = Lipase, amylase, proteases, bicarbonate and water etc etc
99
Histology of pancreatic cancers
Most cancers are exocrine tumours - adenocarcinomas esp infiltrating ductal adenocarcinoma of head/neck/uncinate. Endocrine cancers = pancreatic neuroendocrine tumours (PET).
100
Risk factors for pancreatic exocrine tumours
``` Smoking FHx Obesity Chronic pancreatitis Peutz-Jegher's syndrome ```
101
Presentation of pancreatic exocrine tumours
Epigastric pain, radiating to back. Easing on sitting forward. Back pain. Painless, progressive, obstructive jaundice Weight loss Anorexia Steatorrhoea and malabsorption O/E: Palpable gallbladder = Courvoisier's sign.
102
Courvoisier's sign
Palpable gallbladder with painless jaundice. | Cause unlikely to be gallstones therefore Ix for neoplasm.
103
Investigating pancreatic cancer
Lab: FBC, LFT, glucose. TUMOUR MARKER = CA19-9. Imaging: Pancreatic protocol CT scan or PET-CT Endoscopic US with tissue sampling --> Biopsy histology and cytology.
104
Management of pancreatic cancer
Nutritional support - pancreatin Resection - Whipple's procedure Chemotherapy Pallative care
105
Example of a pancreatic neuroendocrine tumour
Insulinoma | Vipoma
106
Biliary colic
Right upper quadrant pain, may radiate to intra-scapular region. Impact of a stone in cystic duct or ampulla of water.
107
Acute cholecystitis
RUQ pain + fever/raised WCC Distension of the gallbladder causing ischaemia. +ve Murphy's sign.
108
Ascending cholangitis
MEDICAL EMERGENCY!! Stasis of bile due to obstruction leads to infection (E.coli, Klebsiella). RUQ pain + fever/raised WCC + Jaundice = CHARCOT'S TRIAD
109
What is Charcot's triad and when does it occur
RUQ pain + fever/raised WCC + jaundice. | In ascending cholangitis (Infection of bile duct)
110
Types of gallstones
Cholesterol stones (most common) Pigment - black or brown. Mixed
111
What is in bile?
Cholesterol, bile pigments (broken down Hb), phospholipids.
112
Cholelithiasis
Presence of solid stones in gallbladder
113
Presentation of gallstones
Most are asymptomatic. Biliary colic (most common presentation) - post-prandial, nausea. Acute cholecystitis Ascending cholangitis = jaundice.
114
Who gets gallstones // Risk factors
Family history, fat, around 40, females. Obesity, DM,
115
Investigating gallstones
FBC, LFT, lipase and amylase. Abdominal USS shows thickened gallbladder wall, dilated CBD. ERCP MRCP (magnetic resonance cholangiopancreatography).
116
Managing gallstones
Watch and wait. Analgesia - pethidine Laparoscopic cholecystectomy +/- bile duct clearance.
117
Murphy's sign - what is it and when is it seen?
2 fingers over RUQ and patient inspires = pain and pt will stop breathing in. Repeat in left upper quad and no pain. Seen in acute cholecystitis.
118
Ix, Mx and complications for ascending cholangitis
ABC (if all there make diagnosis if only A+ B or C suspected it) A = systemic inflammation (fever, elevated WCC or CRP) B = Cholestasis (Jaundice abnormal LFTs) C = Imaging KUB XR/USS or CT or MRCP (biliary dilation, evidence of gallstones) Other Ix = U+E, amylase, blood cultures. Mx = Resuscitation. Broad spec ABx (metronidazole + ceftriaxone), emergency biliary drainage. Cx = sepsis/septic shock, AKI, liver abscess, liver failure.
119
What conditions are associated with primary sclerosis cholangitis
Inflammatory bowel disease (UC and Crohn's) Hepato-biliary malignancies e.g. HCC Colorectal cancer.
120
Pathophysiology behind primary sclerosis cholangitis
Chronic, cholestatic liver disease due to inflammation and fibrosis of intrahepatic and extra hepatic bile ducts. Leads to bile duct stricture formation. Progressive disorder which can cause development of cirrhosis, portal hypertension and hepatic decompensation.
121
Presentation of primary sclerosis cholangitis
Hx of IBD RUQ pain Jaundice Weight loss, fatigue. O/E: Hepatomegaly Jaundice (yellowing of sclera)
122
Ix for primary sclerosis cholangitis
``` LFT - high ALP, ALT. Immunoglobulins - high IgG Autoantibodies e.g. p-ANCA Imaging: Liver USS, MRCP - shows multifocal strictures and dilations of bile ducts. Biopsy to stage. ```
123
Mx of primary sclerosis cholangitis
Lifestyle advice = avoid alcohol. Supplementation for fat soluble vitamins Pruritus = Colestyramine. Bile acid analogue = Ursodeoxycholic acid, improve LFT not life expectancy. Annual colonoscopy for colorectal cancer. Liver transplant
124
Another name for primary biliary cirrhosis
Primary biliary cholangitis
125
Pathophysiology behind biliary cholangitis
Chronic autoimmune granulomatous inflammation damages interlobular bile ducts (canals of Hering) leading to cholestasis, fibrosis, cirrhosis and portal hypertension.
126
Which autoantibody is most highly associated with primary biliary cholangitis
Antimitochondrial antibodies (AMA)
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Sterotypical patient presenting with primary biliary cholangitis
50yr old woman who's sister has got similar symptoms.
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S+S of primary biliary cholangitis
Fatigue Pruritus RUQ pain O/E: jaundice Xanthelasma hepatomegaly
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Most common autoimmune conditions associated with primary biliary cholangitis
Sjorgren's syndrome - dry eyes, dry mouth. | Thyroid disease
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Criteria to diagnose primary biliary cholangitis
Biochemical evidence fo cholestasis (raised ALP). AMA +ve Biopsy shows evidence of interlobular bile duct destruction.
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Ix for primary biliary cholangitis
``` Biochemical: LFT - raised ALP, raised gamma-GT Autoantibodies - AMA + Thyroid function test Immunoglobulins - raised IgM Cholesterol raised In later disease - raised bilirubin ``` Imaging USS to exclude other causes of cholestasis. MRCP/cholangiography. Biopsy
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Mx for primary biliary cholangitis
Pruritus = cholestyramine Bile acid analogue = Ursodeoxycholic acid Immunosuppression = Prednisolone Curative = transplant
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Differences between primary sclerosis cholangitis and primary biliary cholangitis
PSC = male, sclerosis, intrahepatic and extrahepatic ducts, associated with p-ANCA autoantibodies, common in UC and colorectal cancer patients. PBC = female, interlobular hepatic bile duct involvement, associated with AMA autoantibodies, common in Sjorgren's and thyroid disease patients.
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Name a cancer of the biliary tree 🌲 and list some facts about it.
Cholangiocarcinoma / Klatskin's tumour: - Ductal adenocarcinomas - Associated with liver flukes so more common in SE Asia. - Jaundice, RUQ pain, pruritus, hepatomegaly, weight loss, cholestasis (raised ALP). - CA19-9 and CEA raised. - Rx = resection.
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Where is bile made and where is stored
Made in liver, stored and concetrated in gall bladder.
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What is the poo and wee like in cholestasis?
Dark urine, pale stools - conjugated hyperbilirubinaemia. | Conjugated bilirubin can't be excreted in faeces so reflexed back into systemic circulation and excreted in urine.
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Blood test results in cholestasis
``` Raised ALP (x3) Raised serum bilirubin ```
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Main symptoms of cholestasis and their treatment
Jaundice Dark urine, pale stools Pruritus - cholestyramine
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2 types of causes for jaundice
Unconjugated hyperbilirubinaemia - unconjugated bilirubin is water insoluble. Caused by: over production from haemolysis, poor hepatic uptake from ischaemic hepatitis, Gilbert's syndrome. Conjugated hyperbilirubinaemia - conjugated bilirubin is soluble so dark urine. Caused by: hepatocellular dysfunction from cirrhosis, haemachromatosis, hepatitis, Budd-Chiari and cholestasis from PBC, PSC, pancreatic cancer.
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What type of bilirubin is found in urine and faeces
``` Urine = urobilinogen Faeces = stercobilinogen. ```
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Describe hepatorenal syndrome
- Renal failure with severe liver disease. - Poor prognosis. - Renal vasoconstriction, portal hypertension. - Diagnosis must exclude cause of renal failure. - Mx = terlipression, dialysis, TIPS
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Causes of ascites
Cirrhosis and liver disease Heart failure Nephrotic syndrome Ovarian malignancy
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2 signs O/E for ascites
Fluid thrill | Shifting dullness
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Complications of ascites
Spontaneous bacterial peritonitis | Hepatorenal syndrome
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Why would someone with chronic liver disease get prophylactic ABx
For spontaneous bacterial peritonitis - which has a high mortality rate.
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CFx, common organisms and Mx for SBP
CFx = abdo pain, fever, vomiting. O/E: guarding, rebound tenderness, pain on palpation diffusely over abdo. Ix = paracentesis (cloudy appearance, high neutrophils, gram-staining and culture.. Mx = ABx e.g. ceftriaxone Common organisms = E.coli, S.aureus, Klebsiella.
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Ix for Budd-Chiari
Doppler USS
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Biopsy of steatohepatitis
Fat inclusions and cells are swollen | if alcohol related = mallory bodies
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Complications of HBV
``` Chronic = HCC, Cirrhosis. Acute = Fulminant hepatic necrosis - increasing INR, need transplant! ```
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Autoimmune hepatitis and associated antibodies
Type 1 = most common. ANA (antinuclear antibody) +ve, ASM (anti smooth muscle) +ve. Type 2 = anti-LKM1, rapidly progressive. Type 3 = anti-SLA
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Differentials for acute pancreatitis
``` Abdo aorta aneurysm PUD Intestinal obstruction Cholangitis Mesenteric ischaemia ```
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What is TIPS
Transjugular intrahepatic portosystemic shunt. | Shunt form hepatic to portal vein to relieve pressure.
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Surgical/endoscopic interventions for a variceal upper GI bleed
band ligation balloon tamponade sclerotherapy injection with adhesives/glue
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Criteria for HCC and components of score
CLIP (Cancer of the liver Italian programme) | Scores for Child-Pugh stage, Tumour morphology, Extent/presence of portal vein thrombosis, Serum alpha fetoprotein.