GASTROENTEROLOGY Flashcards
A patient attends your clinic with suspected IBD. Their presenting complaint is having a change in bowel habit. What might you ask in your history to assess this change in bowel habit?
How often are they going to the toilet?
Has this changed from their usual?
Has the form of the stool changed?
Are they waking overnight to open their bowels?
Is there any blood in the motion?
Do they have tenesmus?
Do they have fecal urgency or incontinence?
Do the motions flush away easily?
What are the two conditions involved in IBD?
Chron’s disease and UC
What features distinguish Chron’s from UC?
Format for below: Chron’s vs UC
Affects anywhere from mouth to anus vs always affects rectum and extends proximally.
Skip lesions vs continuous
Transmural inflammation vs mucosa and submucosal inflammation only
Fissuring ulcers vs crypt absecesses
Increased incidence in smokers vs decreased incidence in smokers
Name two features specific to the microscopic appearance of Chron’s
Lymphoid and neutrophil aggregates
Non caseating granulomas
Name 3 investigations would you do for a patient who presents with a change in bowel habits
Blood tests - FBC, U&Es, CRP
Stool tests - stool cultures, faecal calprotectin
Simple imaging - AXR
Endoscopy - flexible sigmoidoscopy, colonoscopy, capsule endoscopy
Cross sectional imaging - CT abdomen, MRI enterography, MRI recutum.
What are the causes of an upper GI bleed?
Oesophageal varices
Mallory-Weiss tear
Peptic ulcers
Cancers of stomach or duodenum
A patient presents with a GI bleed, what do you need to ascertain from their PMH?
Hx of varices or chronic liver disease
Any stigmata of (chronic) liver disease
use of :NSAIDs,Anti-platelets,Anti-coagulants
What are the 2 scoring systems used in GI bleeding and what do they score?
Rockall- for patients that have or are going to have endoscopy, their risk of dying
Blatchford- establishes the risk of patient who you ?GI bleed is a GI bleed, used to determine whether should intervene
What parameters does the Blatchford score take into account?
Drop in Hb
Rise in Urea
Blood pressure
Heart rate
Malaena
Syncope
What do you need to establish if a patient has a GI bleed?
Is it variceal?
What is the initial management for patients with GI bleeding
Used mneumonic ABATED
A- A-E assessment
B- Bloods
A-Access- IV access - if pt haemodynamically compromised, resus fluids and then transfuse
T- Transfuse
E- Endoscopy
D- Drugs, stop any NSAIDs or Anticoagulants
What bloods do you need to do for a patient with GI bleeding and WHY?
FBC and Platelets- Check if Hb is dropping and thrombocytopenia can indicate chronic liver disease. Platelets need to be replaced if lost
U&Es- rising urea supports diagnosis of GI bleed
LFTs- check liver function, may show impaired function/liver disease
VBG- quick Hb reading
Coag screening - are they bleeding due to a clotting disorder?
Crossmatch/group and save- crossmatch if patient is haemodynamically unstable
You have started initial management for a patient with GI bleeding, the cause of this is suspected ruptured varices, what additional steps would you add in your management?
IV terlipressin
IV broad spec antibiotics
Endoscopic banding to stop the bleeding
If this fails- Linton tube or TIPSS (trans jugular intrahepatic porto systemic shunt)
What is the most common cause of non-variceal GI bleeding?
Peptic ulcer disease
What is dieulafoys?
An abnormally large artery in the lining of GI tract, most commonly the stomach
What are the risk factors for peptic ulcer disease?
Long term Steroid use
Long term NSAID use
H.pylori
Alcohol
Stress
Spicy food
Caffeine
Smoking
How would a patient present with peptic ulcer disease?
Epigastric pain
Dyspepsia
Nausea and vomitting
Bleeding- malaena, coffee ground vomit or haematemesis
Iron deficiency anaemia
If duodenal ulcer (more common) = have epigastric pain when hungry, relieved by eating.
If gastric ulcer = epigastric pain worsened by eating
How would you treat a patient that presents with peptic ulcer disease?
If actively bleeding see ABATED mnemonic in Z2F
Rapid urease test to check for H.pylori- treat with amoxicillin and clarithromycin for 7 days +PPI
PPIs is the mainstay of treatment
What the complications of peptic ulcer disease?
Bleeding
Perforation leading to acute abdomen and/or peritonitis
Scarring/ strictures leading to pyloric stenosis
Alcoholic liver disease has 3 stages of liver damage. What are they?
- Fatty liver (steatosis)
- Alcoholic hepatitis (inflammation and necrosis)
- Alcoholic liver cirrhosis
What risk factors may be present in a patient attending your clinic with alcoholic liver disease?
Prolonged heavy alcohol consumption
Hep C
Female
How may a patient with alcoholic liver disease present? (as if you were taking a Hx)
(Question made after talking to Reg in ward round about common presentations)
PC: Right upper quadrant abdominal pain. Sudden onset (as asymptomatic to start) Nauseous. Loss of appetite. Jaundice in eyes and skin. Haematemesis, jaundice.
PMH: previous admissions with alcohol related problem. Hepatitis C.
DH: previous use of diazepam, lorazepam, disulfiram, use of thiamine.
FH: alcohol misuse in family is a potential RF. Hepatitis in family.
SH: alcohol binging, live alone, smoker (occasionally).
What may you find on examination of a patient with alcoholic liver disease?
Hepatomegaly.
Obvious distension to abdomen - ascites.
Discomfort in RUQ.
Engorged para-umbilical veins
Splenomegaly
Jaundice of sclera and skin
Palmar erythema
Spider naevi i.e Cutaneous telangiectasia (trunk, face, UL)
Asterixis - i.e. liver flap
Caput medusae
Signs of malnutrition - wasting and anorexia
Confusion
What are functions of the liver?
Stores glycogen, releases glucose, absorbs fats, fat soluble vitamins and iron, makes cholesterol.
Bile salts dissolve dietary fats
Haemaglobin breakdown into bilirubin.
Produces most clotting factors
Has Kupfer cells to engulff antigens
Excretes drugs and breaks down alcohol
Produces important proteins - albumin and binding proteins
How does acute liver disease contrast to chronic liver disease?
Acute = no pre-existing liver disease. Chronic = starts with acute liver disease which may be asymptomatic.
Acute = resolves in 6 months. Chronic = Ongoing beyond 6 months.
Which conditions can cause acute liver disease?
Hepatits A, E, cytomegalovirus, Epstein-Barr virus, Drug induced liver injury
if present - then need to look for acute liver failure
Which conditions can lead to chronic liver disease?
Most common: Alcoholic liver disease, non-alcoholic steatohepatitis, viral hepatitis (B+C)
Less common but important:
- in women = AI hepatitis, PBC
- in men = PSC associated to IBD
- younger men = haemochromatosis
- adolescents and young adults = Wilson’s disease and anti-LKM AI hepatitis
Which 3 conditions are part of autoimmune liver disease?
Primary biliary cirrhosis, primary sclerosing cholangitis, autoimmune hepatitis.
What are some causes of liver cirrhosis?
Alcohol.
Non-alcoholic fatty liver/non-alcoholic steatohepatitis
Hep B, Hep C
Alpha-1-antitrypsin deficiency,
Methotrexate use
Haemachromatosis
Wilson’s disease
PBC
PSC
Z2F said to remember common 4:
Alcoholic liver disease, NAFLD, Hep B, Hep C
How does liver cirrhosis present?
Asymptomatic with abnormal LFTs
Tiredness,
Itching
Arthralgia
Jaundice
Ascites
Upper GI bleed
Confusion or drowsy
What are RF for liver cirrhosis/disease?
Blood transfusion before 1990.
IVDU
Operations or vaccines with non-sterile equipment
Sexual exposure
Medications
Fix of liver disease
Obesity, metabolic syndrome
Alcohol
Foreign travel
Name 4 visible characteristics of chronic liver disease
Any from:
Spider nave, leukonychia, clubbing, Dupuytren’s contracture, parotid swelling, testicular atrophy, cachexia, para-umbilical vein engorgement, mild splenomegaly.
What are complications of liver cirrhosis?
Portal HTN
Splenomegaly
Oedema,
Ascites with shifting dullness,
R sided pleural effusion, hepatic flap aka asterisks, jaundice
Hepatorenal syndrome
A patient has liver cirrhosis. Over the phone, the radiologist says they have a R sided pleural effusion over 500ml. On their CXR, what typical sign may you see with this volume of effusion?
Hepatic hydrothorax
What is NAFLD, and how does this differ to NASH?
NAFLD = non-alcoholic fatty liver disease. This is where you get deposition of fat in the liver.
NASH = is where you have deposition of fat in the liver (aka accumulation of triglycerides in the hepatocytes) AND have inflammation present as a result of this = non-alcoholic steatohepatitis.
Risk factors of Non Alcoholic fatty liver disease?
DM, Obesity, Metabolic syndrome, Familial hyperlipidaemia
What may you prescribe to a patient at risk of non alcoholic liver disease?
Oral hypoglycaemic agents e.g. Pioglitazone
social prescription - walking, lifestyle modifications.
You are a foundation doctor working in a Gastroenterology rotation. A medical student presents the following history:
A 39-year-old man presents for the third time in 2 years with an intermittent productive cough and increasing dyspnoea on exertion. He has a 15 pack-year smoking history, reports thick, yellow phlegm at times. His medical history reveals mild intermittent asthma controlled with a salbutamol inhaler. His symptoms have persisted despite stopping smoking, with some attacks being unresponsive to salbutamol. Physical examination reveals a generally healthy-looking male apart from fatigue. He has hepatomegaly and ascites. Spirometry shows FEV1 of 40% of predicted value. I
What are your differentials and give reasoning why? Maximum of 3 so be selective !
Top differential = Alpha-1 antitrypsin deficiency - productive cough, cigarette smoker, hepatomegaly, ascites. A-1-antitrypsin= inherited, causes lung and liver problems. (Why is this relevant? Can cause inflammation and cirrhosis of liver!)
COPD - long period of smoking. Spirometry results.
Bronchiectasis - daily sputum.
Where can varies form as a result of portal hypertension?
Oesophageal
Anorectal
Umbilical
Describe the pathophysiology of hepatorenal syndrome. (Clue: this is the development of AKI in presence of cirrhosis - from GI module with Hannah Bonfield)
- Get portal hypertension
- This causes arterial vasodilation (splanchnic)
- This activates RAAS
- So get renal artery vasoconstriction = reduced blood flow to the kidney
Define UC
An IBD characterised by diffuse inflammation of the colonic mucosa
Characteristically, where is UC found in bowel?
Rectum and extends proximally.
On endoscopy, what would you see in Chron’s?
Apthous ulcers (look at pic in slides on BB on UC)
On endoscopy, what would you see in UC?
Ulcerative proctitis (look at pic in slides on BB on UC)
In UC, the inflammation of mucosa can spread proximally by different amounts. What are the three main subcategories of this spread?
- distal (proctitis)
- left sided (inflammation up to the splenic flexure)
- can be extensive = beyond splenic flexure.
- (also have pancolitis = whole colon)
Who does UC usually affect?
Young adults
2nd peak in later life
Patients can have a relapse of colitis. What is a common reason for this?
Pathogen causing gastroenteritis
What are cardinal symptoms of UC?
Bloody diarrhoea
Urgency (Including waking at night needing to open bowels)
Tenesmus
What investigations would you do for patient with suspected IBD?
Blood:
FBC (WCC - infection?, platelets - bleeding?), CRP (inflammation), U&E (may have hypokalaemia from diarrhoea)
Stool:
MC&S, C diff toxin
Radiological:
AXR - toxic megacolon, to see extent of inflammation, to see if they have proximal constipation (seen in left sided disease).
Endoscopic:
Flexible sigmoidoscopy, biopsy of bowel, colonoscopy at later date for bowel cancer.
Note - Dr Rogers said to give one from each group to ensure marks in exam. And even though it may say list investigations, justify why.
Name the three categories of extra-intestinal manifestations you would cover in a Hx of a pt with IBD
1) Those defo related to disease activity (present when bowels are bad)
2) Those usually related to disease activity
3) Those which are unrelated to disease activity (present regardless of bowel disease severity, can even present before diagnosis of IBD or after curative surgery)
What are extra-intestinal manifestations of IBD? 2 marks - so name 4!
1) Those defo related to disease activity
- erythema nodosum - usually on shins
- apathos ulcers
- episcleritis - one form of red eye
- acute arthropathy - a bit like RA but pain and stiffness which gets better when bowels get better
2) Those usually related to disease activity
- pyoderma gangrenous - ulcerative skin condition
- anterior uveitis - another form of red eye.
3) Those which are unrelated to disease activity
- sacroileitis - pain and inflammation in sacroiliac joint
- ankylosing spondylitis - bamboo spine
- primary sclerosing cholangitis - beaded appearance of bile ducts in imaging
If a patient has UC and PSC, why is it important to have bowel screening annually?
Both of these individually increase risk of bowel cancer. Together = further increase in bowel cancer risk!!! Can come about in between surveillances too.
What are aims of treatment for IBD?
Induce remission in acute disease
Maintain remission
Improve QofL
Decrease risk of colorectal cancer
Do IBD flares lead to an antithrombotic or prothrombotic state?
IBD patients are v v v PROTHROMBOTIC - some patients develop DVTs and PEs. Therefore pts should be on LWMH ! Prevents microvascular occlusions seen in IBD too so beneficial in more than one way
What is first line of treatment in UC? And why?
Steroids. Why? These induce remission.
Note - these do not maintain remission
What must be given alongside steroids for IBD?
Bone protection - bisphosphonates, or calcium and vit d in younger pts.
What is given for long term treatment of UC?
5-ASAs - Mesalazine
Why is Mesalazine given as a topical preparation to apply PR or modified in treatment of UC?
Mesalazine is absorbed from the jejunum - not ideal when we want it to reach the colonic mucosa in UC.
Because of this property, it has to be applied PR or linked to a molecule that is enzymatically cleaved in the colon - to ensure it reaches there. Can also be given as suppositories or enema
What are side effects of mesalazine
These are uncommon but good to know:
- diarrhoea
- headache
- nausea
- rash
v rare: interstitial nephritis, nephritic syndrome
Why do U&Es need to be monitored in patients taking mesalazine?
Can cause interstitial nephritis and nephritic syndrome
A patient has UC. They are unable to take steroids as they are intolerant. What would you use to manage them?
Azathioprine - these are steroid sparing agents
What are ADRs of azathioprine?
Flu like symptoms, GI upset, leucopenia, hepatitis, pancreatitis, rash and infections
What is a disadvantage of Azathioprine?
Immunosuppresnts- so cause Flu like symptoms, GI upset, leucopenia, hepatitis, pancreatitis, rash and infections
Increase risk of skin cancer
Onset of action takes at least 6 weeks (Dr Rogers said usually 12 weeks)
A patient has a flare of UC but steroids do not seem to be helping. She still has severe bloody diarrhoea, tenesmus and urgency. This is termed Severe refractory colitis (where steroids and 5ASAs have failed to work in UC).
What might you do next in your management plan?
Ciclosporin. A salvage therapy in severe refractory colitis.
What are benefits of ciclosporin in severe refractory UC?
Rapid onset - starts IV then given orally.
A good drug to use while azathioprine is being taken but hasn’t reached onset of action (6 weeks)
Why do patients with UC need to have their proximal constipation relieved?
Necessary to allow for UC to improve, and induce remission - as once evacuated, no stool there to irritate already inflamed mucosa.
Why do patients with left sided UC have proximal constipation?
Colonic motility is affected by inflammation with rapid transit occurring in inflamed part of colon.
In left sided disease, distal transit is rapid, but the proximal transit is slowed (in non inflamed part of bowel) - this causes proximal constipation.
Note: This is a protective mecahanism by the body.
How is proximal constipation relieved in UC?
Give laxatives
When is emergency colectomy done in patients with PMH of UC?
They have toxic megacolon and acute colitis that isn’t responding to medical therapy
When may elective surgery be planned for a pt with PMH of UC?
When they:
- are steroid dependent
- want surgery
- have a high grade dysplasia or cancer found in screening
You see a 26M, 2wk Hx of bloody D-. Saw GP who gave him codeine and loperamide as well as ORT.
No one else at home has loose motions.
Feels tired and lethargic.
Most recent bloods show raised CRP.
What are Ddx?
UC - main differential
Chron’s
Infective colitis
Define decompensated liver disease
Liver disease = damage to the liver which affects structure. Structure becomes distorted and get nodules and fibrosis. Synthetic, metabolic and excretory functions are affected.
Decompensated = Liver damage is so advanced that organ can not function, and clinical complications (e.g. jaundice and ascites) are present that can not be overcome.
What does the Child-Pugh score assess?
Assess prognosis/severity of cirrhosis
What features are included in Child-Pugh score?
Bilirubin, albumin, INR, ascites, encephalopathy - see pg76 Z2F
What is the MELD score used for?
Used every 6months in pts with compensated cirrhosis.. Helps guide referral for liver transplant and percentage estimated 3 month mortality.
Why may AST and ALT levels be normal in patient with liver cirrhosis?
Not enough healthy tissue to release elevated quantities of these enzymes, so appear normal
What is hepatitis?
Inflammation of the liver
What are causes of hepatitis?
Alcoholic hepatitis - alcohol
NAFLD
Viral hepatitis - viruses, CMV, EBV
Autoimmune
Drug induced
Name 3 functions of the liver
Nutrition/metabolic
Bile salts
Protein synthesis
Clotting factors
Bilirubin
Detoxification
Immune function
What things is it important to ask when taking a history from a patient with suspected liver disease?
Did they have any blood transfusions in the UK before 1990?
IVDU?
Operations and vaccinations, any with dubious sterility
Sexual exposure
Medications
Fhx of liver disease, diabetes, IBD
Obesity/other features of metabolic syndrome
Alchohol (?dependency)
Foreign travel
Acute vs Chronic liver disease
Acute:
Resolves within 6 months
Hep A,E, CMV, EBV
Drug induced liver injury
Chronic:
Usually starts as acute- usually asymptomatic
Still effects after 6 months
Can lead to cirrhosis
Alcohol
Hep C
Non-alcoholic steatohepatitis
Autoimmune
What is the significance of ALT and ALP
ALT- released from hepatocytes
ALP- released from the ducts
A patient has an ALT between 100-200, what are your differentials?
Non-alcoholic hepatitis
Chronic viral hepatitis
Autoimmune hepatitis
Drug induced liver injury
A patient has an ALT>500, what are your ddx?
Viral
Ischaemia
Toxic- any drug but most commonly paracetamol
Autoimmune
What are your ddx in patients with a higher ALP than ALT?
Cholestatic- dilated ducts:
Gallstone
Malignancy
Non-dilated ducts:
Alcoholic hep
Cirrhosis due to PSC, PBC or Alcohol
Drug induced liver injury e.g. antibiotics
What is the stepwise progression of alcoholic liver disease?
1) Alcohol relate fatty liver- reversible
2) Alcoholic hepatitis- if mild may be reversible
3) Alcoholic cirrhosis- where normal tissue replaced with scar tissue- not reversible, but can slow progression if stop drinking, however poor prognosis if keep drinking
What are some complications from alcohol consumption?
Increased risk of cancer
Pancreatitis
Alcoholic cirrhosis and complications from that
Hepatocellular carcinoma
Dependence and withdrawal
Alcoholic liver cirrhosis
What are the causes for pancreatitis?
Idiopathic
Gallstones
Ethanol
Trauma
Steroids
Mumps/malignancy
Autoimmune
Scorpion stings
Hypertriglyridaemia/hypercalcaemia
ERCP
Drugs e.g. thiazides
What are the signs of liver disease?
Jaundice
Sceleral icterus
Bruising
Ascites
Palmar erythema
Spider naevi
Gynacomastia
Hepatomegaly
Caput medusa
Flapping tremor (in decompensated)
What blood tests would you in a patient with suspected liver disease?
FBC- Increased MCV
LFTs- Increased ALT and AST, increased ALP in cirrhosis, Increased bilirubin in cirrhosis, decreased albumin
Coagulation Screening- Prothrombin time is increase
U&Es to check for hepatorenal syndrome
What is hepatorenal syndrome?
Happens in cirrhosis
Portal hypertension leads to back pressure of blood, leading to vasodilators being released
This leads to arterial vasodilation which leads to a drop in pressure
This leads to RAAS being activated and so you get vasoconstriction of the renal arteries, leading to reduced blood flow to the kidneys
What imaging would you use in a patient with liver disease?
USS- to detect any fatty changes and detect any changes related to cirrhosis
Fibroscan- elasticity of the liver and measures the degree of cirrhosis
CT/MRI- check for fatty infiltration, hepatocellular carcinoma, hepatosplenomegaly
When would a biopsy be indicated in a patient with liver disease?
When considering starting steroids
What is the general management in a patient with alcoholic liver disease?
Stop drinking
Thiamine and high protein diet
Consider a detox regime
Treat complications of cirrhosis
Steroids- short term (1 month) in severe alcoholic hepatitis
Liver transplant, but patient has to be sober for 3-6 months
How does alcohol withdrawal present?
6-12hrs- craving, anxiety, sweats, headache
12-24hrs- hallucinations
24-48hrs-seizures
48-72hrs- delirium tremens
What is the pathophysiology of delirium tremens?
In alcohol dependency GABA receptors gets upregulated and glutamate receptors gets down regulated, so when you stop, you have under functioning of GABA and over functioning of Glutamate leading to increased excitability, causing delirium tremens
What are the symptoms of delirium tremens?
Severe agitation
Confusion
Delirium and hallucinations
Tachycardia
hypertension
hyperthermia
Ataxia
How you manage alcohol withdrawal?
Chlordiazepoxide (benzo)- reducing regime 10-40mg, 1-4hrs depending on patients needs
Pabrinex- high dose IV B vitamins and then followed by thiamine (oral)
Lactulose- to remove ammonia
Why is thiamine so important to replace in alcohol dependency?
To prevent Wernicke-Korsakoff syndrome
What is Wernicke Korsakoff Syndrome?
Made up of Wernicke encephalopathy which comes first- confusion, oculomotor disturbances, ataxia- medical emergency
Korsakoffs syndrome- comes after
Anterograde and retrograde memory loss
Behaviour changes
Irreversible and patient needs to be institutional care
What would you see on histology in UC?
Crypt abcesses and decreased goblet cells
What is the most common patient presentation of a paracetamol overdose?
Initially, most patients are asymptomatic or have mild GI symptoms. W/o treatment patients may have varying degrees of liver injury
Rarely, in massive overdose, patient may be in coma and have severe metabolic acidosis
What is the recommended dose of paracetamol in adults?
4g in 24 hours
What are the different types of paracetamol overdose?
Acute overdose- excessive amount of paracetamol taken in 1 hour or less, usually context of self harm
Staggered overdose- excessive amount of paracetamol taken in over 1 hour, usually in context of self harm
Therapeutic excess- Excessive paracetamol taken with intent to treat pain/fever no self harm intent. Ingested at a dose greater than licensed daily dose AND more than or equal to 75mg/kg/24hours
Aside from being asymptomatic, what other features may a patient with a paracetamol overdose present with?
N&V
RUQ pain
jaundice
hepatomegaly
loin pain
altered conscious
What are the risk factors for paracetamol overdose?
History of self harm
Hx of frequent or repeated use of medications for pain relief
Glutathione deficiency
Long term treatment with CYP450 inducers
What are risk factors for glutathione deficiency?
Malnourishment
Eating disorders
Psychiatric disorders
Chronic illness
Cachexia
Alcohol-use disorder
What are some examples of CYP450 inducers?
Carbamazepine
Phenytoin
Rifampicin
St John’s wort
What investigations should you consider in a paracetamol overdose?
Serum paracetamol conc
LFTs- ALT may be raised in liver injury
Prothrombin time and INR- PT may be increased and INR may be raised
Blood glucose- hypoglycaemia may indicate acute liver injury
U&Es- creatine may be elevated-> indicative of AKI or acute liver injury (heptorenal syndrome)
VBG/ABG- may show lactic acidosis
FBC- may show leukocytosis, anaemia or thrombocytopenia
How do you treat an acute single overdose of paracetamol?
<8hrs since the overdose: supportive care and If more than 150mg/kg of paracetamol activated charcoal
8-24hrs since overdose- supportive care and (consider) acetylcysteine
> 24hrs since ingestion- supportive care and (consider) acetylcysteine
How you treat a staggered overdose of paracetamol?
Supportive care and acetylcysteine
How do you treat a therapeutic overdose of paracetamol?
Supportive care and acetylcysteine
How is acetylcysteine given?
3 sequential infusions over 1hr, 4 hrs and 16hrs
What is Haemochromatosis?
Iron storage disorder, results in excess iron in the body and causing it to be stored in the tissues
What mutation is responsible for Haemochromatosis?
Human haemochromatosis protein (HFE) on chromosome 6, in most cases.
However, can be caused by other gene mutations
What is the pattern of inheritance for Haemochromatosis?
Autosomal recessive
When does Haemochromatosis present?
Usually over 40. Present later in women as menstruation helps remove iron from the body
How does Haemochromatosis present?
Joint pain
Tiredness
Bronze colour
Hair loss
Erectile dysfunction
Amenorrhoea
Memory and mood disturbances
How do you investigate potential haemochromatosis?
Bedside:
* obs - HR, BP, Temp, Sats, RR
* ECG - as complications include cardiac failure and cardiomyopathy
* Blood glucose - can present with DM
Bloods:
* Serum ferritin and transferrin saturation- if both high, genetic testing to check for Haemochromatosis
* FBC - presents with fatigue - so check Hb and also WCC, platelets etc
* U+Es - to do for baseline and before interventiosn are started
* LFTs - can cause chronic liver disease, hepatomegaly, cirrhosis.
* CRP - check for any inflam.
Imaging
* MRI of heart and brain - to look at any iron deposition
* CT abdo - can show attenuation of the liver
Procedures:
* Liver biopsy- confirm increased iron stores (usually done after genetic testing if needed)
* genetic testing of HFE mutation, C282Y and H63D mutations
What are the complications of Haemochromatosis?
Type 1 diabetes
Liver cirrhosis
Iron deposits in pituitary and gonads–> endocrine and sexual problems
Cardiomyopathy
Hepatocellular carcinoma
Hypothryoidism
Chrondocalcinosis - aka pseudo gout with calcium pyrophosphate crystals
What is the treatment of Haemochromatosis?
Venesection
Monitoring serum ferritin
Monitoring and treating complications
Refeeding syndrome occurs when ___A____ is introduced after a period of prolonged starvation.
It is characterised by __B___, __C___, __D___ and __E___ dysfunction
The biochemical marker is ___F____.
It can be avoided by recognition of those at risk and __G____
Refeeding syndrome occurs when ___CARBOHYDRATE___ is introduced after a period of prolonged starvation.
It is characterised by __HEART FAILURE__, __OEDEMA__, RESP and NEURO dysfunction
The biochemical marker is __HYPOPHOSPHATEMIA__
It can be avoided by recognition of those at risk and __PREVENTATIVE MEASURES__
Who is at risk of refeeding syndrome?
1 or more:
BMI <16 kg/m2
little or no nutritional intake >10 days
low phosphate / magnesium before feeding
unintentional weight loss >15% in 3-6 months
2 or more:
BMI <18.5 kg/m2
unintentional weight loss >10% over 3-6 months
little or no nutritional >5 days
Hx of alcohol abuse or drugs (incl. insulin, chemo, antiacids, diuretics)
Before commencing re-feeding what is it essential to start first?
What else is helpful to prescribe as it is a co-factor in carb metabolism?
Essential to replenish phosphate stores with an IV phosphate infusion before starting refeeding.
Also give parenteral multivitamins as they are an important co-factor in carbohydrate metabolism.
What should be checked rigorously until full feeding has been established
Phosphate
Magnesium
Potassium levels
Re-introduction of diet must be slow, give an example of caloric regime?
may be as low as 5-10kcal/kg/day
What is the Enhanced liver fibrosis blood test?
First line recommend investigation for investigating non-alcoholic fatty liver disease
What happens when reintroduce carbohydrate to the starved state?
Insulin is released in response causing:
Increased glucose uptake
Increased uptake of magnesium, potassium, phosphate and water into cells
increased thiamine use.
results in:
Hypophosphateaemia
Hypokalaemia
Hypomagnesaemia
Thiamine deficiency
Sodium and water retention
Explain why re-feeding syndrome causes fluid shifts, reduced tissue oxygenation and impaired cardiac function?
Uptake of phosphate, potassium, magnesium and water into cells. Sodium is pushed out of cells and the phosphate stores are depleted causing fluid shift which causes oedema.
Hypophosphataemia reduces the production of ATP and impairs function of cardiac muscle.
In addition 2,3-DGP is reduced in red cells and this decreases the ability of red cells to deliver oxygen to tissues.
The combined effect of fluid shifts can cause acute congestive cardiac failure
Biochemical hallmark of refeeding syndrome?
Low: phosphate, magnesium, potassium
What is parenteral nutrition?
Nutrition which is delivered to a patient without accessing or utilising the gastrointestinal tract. By definition, PN is delivered intravenously
When is PN used?
PN is used when the gut is:
Inaccessible
Blocked
Failing.
The gut may inaccessible due to an oesophageal tumour, blocked due to small bowel obstruction or failing following massive intestinal resection
How do you work out calories in a bag of Parenteral nutrition ?
- Convert the amount Nitrogen on the bag to protein calories by multiplying the grams of Nitrogen given on the bag by 25.
- Add this to the lipid and glucose calories given on the bag
e.g. Nitrogen = 12 g
Lipid = 550 Kcal
Glucose 1000 kcal
so:
12g x 25 = 300 (protein calories)
+ 550 (lipid)
+ 1000 (glucose
= 1850kcal in total
When is PN feeding used?
PN is used when the gut is:
Inaccessible - e.g. oesophageal tumour
Blocked - e.g. small bowel obstruction
Failing - e.g. following massive intestinal resection
Can someone be on both Parenteral and Enteral nutrition?
In many situations a patient may be receiving both as a bridge to full enteral nutrition. This is why the term ‘Total Parenteral Nutrition’ or ‘TPN’ is inappropriate as PN is not always ‘total’
How is Parenteral nutrition given? over what time frame?
Prolonged infusion over 12-24 hours via a central venous catheter which feeds directly into central vein e.g. Subclavian vein, SVC, RA
Either by:
PICC line (longer as it is peripherally inserted through vein in the arm via basillic or cephalic vein )
or
Hickman Line (inserted into chest vein e.g. jugular vein or subclavian vein)
Why is a central venous catheter (CVC) used for Parenteral nutrition?
it needs to be a large vein to prevent thrombosis and damage to vessel wall that could be caused if inserted in a small peripheral vein.
CVC is a long flexible cannula that sits with its tip in a large vein
What is in Parenteral nutrition solution?
Why must it be kept covered?
PN is a solution that contains water, glucose, nitrogen, lipid, electrolytes, vitamins and trace elements- nourishes and hydrates.
Must be kept covered as sensitive to daylight can be degraded by sunlight
can be cloudy (if lipid added) or clear (if no lipid added)
What are complications of PN ?
- Biochemical
- Mechanical
- Infectious
- Biochemical:
Electrolyte disturbance
fluid overload
hyperglycaemia
abnormal liver function (carb overload) - Mechanical:
Thrombosis
Line fracture
Line occlusion
Pneumothorax (on CVC insertion)
Air embolus (if line left open)
3 Infectious:
CVC related bacteraemia
CVC related sepsis
What is the MUST tool?
‘MUST’ is a five-step screening tool to identify adults, who are malnourished, at risk of malnutrition
(undernutrition), or obese.
It also includes management guidelines which can be used to develop
a care plan.
- Outline the MUST tool steps .
Malnutrition Universal screening tool
Step 1: Measure height and weight to get BMI
Step 2: Note % unplanned weight loss and score using table
Step 3: Establish acute disease effect and score (if patient acutely ill and there is likely to be no nutritional intake for > 5 days = 2)
Step 4 : add scores to see risk
Step 5: see guidelines to develop care plan
- Outline the management guidelines based on the MUST tool score
Score 0 - low risk
Repeat screening
e.g. weekly in hospital, annually in community for >75.
Score 1 - Medium risk
Observe and document dietary intake for 3 days
Repeat screening
Score 2 or higher
Refer to dietician / nutritional support team
set goals and increase intake
All risk categories:
Treat underlying condition
Advise on food choices, eating and drinking when
necessary.
Record malnutrition risk category.
Record need for special diets
Characteristic findings in alcoholic hepatitis ?
AST/ALT ratio is 2:1 in alcoholic hepatitis
e.g. AST - 790
ALT- 375
+ Macrocytic anaemia (raised MCV)
+ Raised GGT
Present: RUQ pain, jaundice, and signs of liver failure
What is liver cirrhosis?
Result of chronic inflammation of the liver–> scar tissue and nodules. Causes resistance in the vessels going to the liver, resulting in back pressure
What are the most common causes of liver cirrhosis?
Alcoholic liver disease
Non-alcoholic liver disease
Hep B
Hep C
What are the less common causes of liver cirrhosis?
Autoimmune hepatits
Primary biliary cirrhosis
Haemochromatosis
Wilsons disease
Alpha-1-antitrypsin deficiency
CF
Drugs e.g. amiodarone, methotrexate and sodium valproate
What are the signs of liver cirrhosis?
Jaundice- due to high bilirubin
Hepatomegaly - however may be smaller the more cirrhotic it gets
Splenomegaly- due to portal hypertension
Spider naevi
Palmar erythema- caused by hyper dynamic circulation
Gynaecomastia and testicular atrophy in males due to endocrine dysfunction
Bruising- due to abnormal clotting
Ascites
Caput medusa-due to portal hypertension
Flapping tremor
What investigations would you do for liver cirrhosis?
LFTs- often normal but in decompensated cirrhosis, they’re often deranged
Albumin- drop
Prothrombin time- increases
Both useful for synthetic function of the liver
Hyponatraemia- fluid retention in severe disease
Urea and creatinine become deranged in hepatorenal syndrome
Further bloods can help establish the cause
Alpha-fetoprotein- tumour marker for hepatocellular carcinoma, can be checked every 6 months in patients with cirrhosis as a screening tool
How do you interpret the results of the Enhanced liver fibrosis blood test?
<7.7 -none to mild fibrosis
> or equal to7.7-9.8- moderate fibrosis
> or equal to 9.8- severe fibrosis
What would an USS show in a pt with liver cirrhosis?
Nodularity of liver surface
Corkscrew appearance to hepatic arteries
Enlarged portal vein with reduced blood flow
Ascites
Splenomegaly
What does fibroscan measure?
Liver elasticity, therefore helps assess degree of cirrhosis
Done every 2 years in high risk pts:
Hep C
Heavy alcohol drinkers
Diagnosed alcoholic liver disease
What is the general management of liver cirrhosis?
USS and alpha-fetoprotein every 6 months to screen for heptocellular carcinoma
Endoscopy every 3 years for pt with known varices
High protein, low sodium diet
MELD score every 6 months
Consider liver transplant
Manage complications
Why does liver cirrhosis relate to malnutrition
Increase use of muscle tissue as fuel and reduces amount of protein available in body for muscle growth .
Disrupts ability of liver to store glucose as glycogen and release it when required
= body using muscle tissue as fuel–> muscle wasting and weight loss
Management of malnutrition secondary to cirrhosis?
Meals every 2-3 hours
Low Na diet–>minimise fluid retention
High protein and high calorie diet
Avoid alcohol
Sites of varices?
Gastro-oesophageal junction
Ileocaecal junction
Rectum
Ant abdo wall via umbilical vein (caput medusa)
Treatment of stable varices?
Propranolol reduces protein hypertension by acting as a non-selective B blocker
Elastic band ligation of varices
Injection of sclerosant (less effective than band ligation)
What is TIPS?
Trans intra-hepatic portalsystemic shunt
X-ray guided wire into jugular vein–>vena cava–>liver via hepatic vein.
Connection between hepatic vein and portal vein and place stent–> relieves pressure in portal system
When other treatments fail or bleeding cannot be controlled
What is ascites?
Fluid in peritoneal cavity
Increased pressure in portal system forces fluid out into peritoneal cavity–> drop circulating volume–> reduced BP–> RAAS activated–> aldosterone leads to increased fluid and sodium reabsorption–> fluid and sodium overload
Management of ascites?
Anti-aldosterone diuretics–> Spironolactone
Paracentesis if tense
Low Na diet
Prophylactic antibiotics agains SBP–> ciprofloxacin or norfloxacin in pts with less than 15g/L of protein
Consider TIPS in refractory ascites
Consider liver transplant in refractory ascites
Prophylatic antibiotic in liver cirrhosis against SBP?
ciprofloxacin or norfloxacin
Presentation of SBP?
Can be asymptomatic
Fever
Abdo pain
Raised WCC, CRP, creatinine or metabolic acidosis
Ileus
hypotension
Most common organisms in SBP?
Escherichia Coli
Klebsiella pneumoniae
Gram positive cocci- staphylococcus or enterococcus
Management of SBP?
Ascitic culture before giving antibiotics
IV cephalosporin e.g. cefotaxime
What is hepatic encephalopathy?
Build up of toxins that affect the brain e.g. ammonia
Why do you get build up of ammonia in liver cirrhosis?
Builds up due to liver unable to metabolise the ammonia and collateral vessels between portal and systemic circulation mean that the ammonia bypasses liver all together
Precipitating factors of hepatic encephalopathy?
Constipation
Electrolyte disturbances
Infection
GI bleeding
high protein diet
Medications
Presentation of hepatic encephalopathy?
Reduced consciousness and confusion
Chronically–> personality changes, memory and mood
Management of hepatic encephalopathy?
Laxatives e.g. lactulose, dose appropriately so patient has 2-3 motions a day
Rifaximin- antibiotic–> reduces ammonia producing bacteria
Nurtrional support
Investigations fo NAFLD?
1st line-Enhanced liver fibrosis blood test
2nd line- NAFLD fibrosis score
3rd line- Fibroscan
Management for NAFLD?
Weight loss
Exercise
Stop smoking
Control of diabetes, BP and cholesterol
Avoid alcohol
Presentation of hepatitis?
Asymptomatic or vague symptoms
Abdominal pain
Fatigue
Pruritis
Muscle and joint aches
Nausea and vomitting
Jaundice
Fever (viral hepatits)
Epidemiology of Hep A?
Most common hep worldwide but relatively rare in UK
Transmission of Hep A?
Faecal oral route usually in contaminated water?
What type of virus is Hep A?
RNA
Presentation of Hep A?
Nausea, vomitting, jaundice and anorexia
Cholestasis- dark urine and pale stools
Moderate hepatomegaly
Management of Hep A?
Basic analgesia
Resolves without treatment 1-3 months
Prevention of Hep A?
Vaccination and it is a notifiable disease
What type of virus is Hep B?
DNA
Transmission of Hep B?
Direct contact with blood or bodily fluids, sharing household items e.g. toothbrush
Vertical transmission
Prognosis of Hep B?
Most people fully recover in 1-2 months
10-15% become chronic hepatitis B carriers–> virus DNA integrated into their own DNA
Viral markers and their relevance in Hep B?
Surface antigen (HBsAg)- active infection
E Antigen (HBeAg)- marker of replication–> high infectivity
Core antibodies (HBcAb)- past or current infection
Surface antibody (HBsAb)- vaccination or past or correct infection
Hep B virus DNA(HBV DNA)- direct count of viral load
How can Core antibodies in hep B determine past or current infection?
Look at IgM or IgG.
IgM–> high in acute infection and low in chronic infection
IgG–> past infection where HBsAg is negative
Outline the Hep B vaccine?
Inject HBsAg
3 doses at different intervals
Check of HBsAb
Included as past of UK routine
Management of Hep B?
Low threshold for screening at risk patients
Screen for other blood borne viruses and other sexual transmitted disease
Reder to GI/hepatology or infectious disease for specialist management
Notify public health England
Stop smoking and alcohol
Education- transmission and at risk contacts
Test for complications
Antiviral medication to slow disease progression and reduce infectivity
Liver transplant for end stage liver disease
What type of virus is Hep C?
RNA
Transmission of Hep C?
Blood and bodily fluids
Disease course of Hep C?
1 in 4–> full recovery
3 in 4–> chronic
Complications of Hep C?
Liver cirrhosis
Hepatocellular carcinoma
How do you test for Hep C?
Hep C antibody screening test
Hep C RNA testing used to confirm the diagnosis of Hep C, calculate viral load and assess for the individual genotype
Management of Hep C?
direct acting antivirals- tailored to specific genotype–> successful in over 90% patients, take 8-12 weeks
Low threshold for screening at risk patients
Screen for other blood borne viruses and other sexual transmitted disease
Reder to GI/hepatology or infectious disease for specialist management
Notify public health England
Stop smoking and alcohol
Education- transmission and at risk contacts
Test for complications
Liver transplant for end stage
What are the two types of Autoimmune hepatitis?
Type 1: occurs in adults
Type 2: occurs in children
How does type 1 autoimmune hepatitis present? Who does it often affect?
Women around 40s-50s or around the menopause with fatigue and features of disease on examination
How does type 2 autoimmune hepatitis present?
Teenage years or early 20s with high transaminases and jaundice
Type 1 autoimmune hep antibodies?
Anti nuclease antibodies- ANA
Anti- smooth muscle antibodies- Anti- actin
Anti-soluble liver antigen- anti-SLA/LP
Type 2 autoimmune hep antibodies?
Anti-liver kidney microsomes-1 (anti-LKM1)
Anti-liver cytosol antigen type 1 (Anti-LC1)
Parameters in MELD score?
A patient’s bilirubin, creatinine, and the international normalized ratio (INR) to predict survival.
Define acute liver failure
Rapid onset of hepatocellular dysfunction. Leads to a variety of systemic complications.
Common causes of acute liver failure
Paracetamol overdose
Alcohol
Viral hepatitis - usually Hep A or Hep B
Acute fatty liver of pregnanvy
Features of acute liver failure?
Jaundice
Hypoalbuminaemia
Hepatic encephalopathy
Renal failure is common - hepatorenal syndrome
Investigations for acute liver failure (ALF) and why?
1) LFTs: would be raised:
Bilirubin = Jaundice is a defining feature of acute liver failure - so look for hyperbilirubinaemia
AST and ALT = high in paracetamol hepatotoxicity
2) INR = coagulopathy is a defining feature of ALF
3) U+Es = renal failure common in ALF
4) FBC - high WCC for infection.Thrombocytopenia common. Anaemia common
5) Cross match and G+S = may need blood transfusion
6) ABG = metabolic acidosis in paracetamol overdose
Immediate management for acute liver failure?
- Intensive care management - if hepatic encephalopathy is present
plus the following if required:
- liver transplant assessment
- neurological status monitoring
- blood glucose (every 1-2hrs), electrolytes (2x a day) and cultures monitoring. Correct if needed.
- acetylcysteine if paracetamol overdose
Compare AST : ALT liver enzyme results in:
1. NAFLD
2. Alcoholic liver disease
3 Acute alcoholic hepatitis
4. Liver cirrhosis
- NAFLD : ALT will be higher than AST
- ALD: AST >2 x higher than ALT
- Acute alcoholic hepatitis: AST>3 x higher than ALT
- Cirrhosis: AST > 2.5 x higher than ALT
In chronic hepatitis, you would expect AST to be 10x raised.
What are the parameters of the Child-Pugh Score ? the 5 things it look at ?
Bilirubin
Albumin
INR
Ascites
Hepatic Encephalopathy?
What are some risk factors for variceal haemorrhage?
High portal pressures (>12mmHg)
Large varices
Abnormal variceal wall at endoscopy (eg haematocystic spots)
High Child-Pugh score
What are some complications of liver biopsy?
Shoulder tip pain
Bowel perforation
Renal laceration
pnuemothorax
billiary peritonitis
In what groups of people might liver biopsy be contraindicated and why?
High risk of bleeding in pts with:
Large ascites
Disordered platelets / clotting
Not co-operative
Severe cirrhosis
WIth these ppl can do transjugular or laparoscopic routes
Treatment of chronic liver disease?
Remove underlying aetiology - e.g. stop drinking (if alcohol related), weight loss (if non-alcoholic liver steatohepatitis), antivirals (if hep B or hep C), venesection (if haemochromatosis)
Why is it important to treat chronic liver disease?
Prevent further liver damage and prevent progression to cirrhosis
Benefits of using a fibroscan for liver cirrhosis over other imaging?
Quicker
More specific
Can be performed in clinic room
What complications should you screen for in patients with liver cirrhosis? How would you screen for these?
- DEXA scan = screen for osteoporosis
- USS of liver and AFP = screen for hepatocellular carcinoma
Pt has ascites. What investigation should be done for all pts with ascites?
Diagnostic ascitic tap (cell count and MC&S) - look for SBP.
Initial blood tests to do for cirrhosis and reasoning?
1) LFTs - albumin, bilirubin, liver enzymes = assess liver function
2) FBC - WCC look for infective cause, thrombocytopenia = chronic liver disease, Hb for anaemia = normocytic in Wilson’s disease, oesophageal bleed or macrocytic if folate/b12 deficiency in alcohol excess
3)Urea and electrolytes to establish baseline renal function and to look for hepato-renal syndrome or any electrolyte abnormalities
4) INR - for coagulpathy
5) Others - autoantibodies - for AI hepatitis, alpha 1 antitrypsin, iron studies in haemochromatosis etc
What is the primary mechanism causing gynecomastia in liver cirrhosis?
Disordered metabolism of sex steroids, which leads to excess levels of oestrogen
What are the cardinal features of decompensation in liver disease?
Jaundice
Ascites
Hepatic flap
Altered mental status
If a pt drinks around 2 litres of cider (ABV 5%) a day. How many units is that a week?
Alcohol units = volume (ml) * ABV / 1,000
2000 ml x 5% = 10,000
Divide this figure by 1,000 to get the number of units = 10,000/1,000 = 10 units/day
10 units/day x 7 days = 70 units
What LFT results would you expect in a patient with autoimmune hepatitis?
Raised ALT and bilirubin
Normal/mildly raised ALP.
May be IgG predominant hypergammaglobulinemia.
Treatment for autoimmune hepatitis?
Immunosuppressants e.g. azathioprine
Extra-intestinal features of IBD that are related to disease activity?
Arthritis - affects a few joints, asymmetrical
Erythema nodosum
Espiscleritis
Osteoporosis
Extra-intestinal features of IBD that are unrelated to disease activity?
Arthritis - polyarticular, symmetrical
Uveitis
Pyoderma gangrenosum
Clubbing
Primary sclerosing cholangitis
Presentation of PBC (primary biliary cirrhosis)?
Xanthoma, xanthelasma
Fatigue
Pruritis
GI disturbance, abdo pain
Jaundice
Pale, greasy stools
Signs of cirrhosis, liver failure —> ascites, hepatomegaly, splenomegaly, spider naevi
Investigations for PBC?
LFTs - ALP raised early on, ALT and bilirubin raised later.
Autoantibodies - Anti-mitochon (most specific), ANA
CRP, ESR, IgM
Liver biopsy to diagnose and stage
How is PBC treated?
Immunosuppresion - azathioprine or steriods
Liver transplant
Ursodeoxycholic acid = reduce intestinal absorption of cholesterol
Colestyramine = sequesters bile acid (bile acid = a cause of itching in PBC) so reduces this symptoms
Complications of PBC?
Advanced liver cirrhosis
Portal HTN
Fatigue - TATT
Distal renal tubular acidosis
HypoThyroidism
Osteoporosis
Hepatocellular carcinoma
Fatty, greasy stools = steatorrhea
RF for PSC? aka primary sclerosing cholangitis
Male
30-40
UC in PMH
FHx
Presentation of PSC?
Jaundice
Fatigue
Chronic RUQ pain
Pruritus
Hepatomegaly
Inv and corresponding results for PSC?
LFTs - ALP v high, raised bilirubin, ALT and AST also raised but more common as disease progresses
Autoantibody screen - p-ANCA, ANA and aCL
MRCP = gold standard for Dx!! - show bile duct lesions or strictures.
Complications of PSC?
Acute bacterial cholangitis
Cholangiocarcinoma
Colorectal cancer (relation to UC)
Cirrhosis, liver failure
Biliary strictures
Fat sol vitamin deficiency - ADEK
Management of PSC?
Liver transplant
ERCP to dilate and stent strictures
Ursodeoxycholic acid
Colestyramine
Monitor for complications - cirrhosis, cholangiocarcinoma, oesophageal varices
Imaging of choice in PSC?
ERCP/MRCP
How do you differentiate between an upper GI bleed vs lower GI bleed based on blood results?
Upper GI bleed more likely to have high urea
What is seen on duodenal biopsy in coeliac disease?
Villous atrophy
Crypt hyperplasia
Increase in intraepithelial lymphocytes
What are the drug causes of pancreatitis?
Azathioprine
Sulfasalazine
Valproate
Statins
ACE inhibitors
Oral contraceptives
What is ischaemic hepatitis?
diffuse hepatic injury resulting from acute hypoperfusion (sometimes known as ‘shock liver’). It is not an inflammatory process. It is diagnosed in the presence of an inciting event (e.g. a cardiac arrest) and marked increases in aminotransferase levels (exceeding 1000 international unit/L or 50 times the upper limit of normal).
When to suspect ischaemic hepatitis?
- if the ALT is high: exceeding 1000 international unit/L or 50 times the upper limit of normal).AND
- the pt just had a cardiac arrest
- low BP
- Any acute hypoperfusion
- Often, it will occur in conjunction with acute kidney injury (tubular necrosis) or other end-organ dysfunction.
