Gastro

Question 1

What is Eosinophilic Oesophagitis?

Answer 1

Eosinophilic oesophagitis is characterised by an allergic inflammation of the oesophagus. An oesophageal biopsy will show dense infiltrate of eosinophils in the epithelium. Although this disease is relatively poorly understood, it is thought to be caused by an allergic reaction to ingested food. The resulting oesophageal inflammation results in pain and dysphagia, amongst other symptoms.

Question 2

Which gender and age group is Eosinophilic oesophagitis common in?

Answer 2

3:1 male:female ratio
Average age at diagnosis is 30-50 years old

Question 3

what are the risk factors for developing eosinophilic oesophagitis?

Answer 3

Allergies/ asthma: suffering from food/ environmental allergies or atopic dermatitis and asthma increases the risk of diagnosis
Male sex
Family history of eosinophilic oesophagitis or allergies
Caucasian race
Age between 30-50
Coexisting autoimmune disease e.g. coeliac disease

Question 4

what are the symptoms and signs of eosinophilic oesophagitis?

Answer 4

Patients typically present with a subacute onset of:
In children, disease presents with failure to thrive due to food refusal
Adults often experience dysphagia, strictures/ fibrosis (56%), food impaction (55%), regurgitation/ vomiting, anorexia

Signs:
Signs are minimal and suspicion of this diagnosis relies mainly on the reported symptoms, past medical history and exclusion of other differential diagnoses e.g. GORD
Weight loss

Question 5

What are the investigations of eosinophilic oesophagits?

Answer 5

Endoscopy - histological analysis.
There must be more than 15 eosinophils per high power microscopy field to diagnose the condition. Other findings on endoscopy include reduced vasculature, thick mucosa, mucosal furrows, strictures and laryngeal oedema. Histologically, the diagnosis is made more likely in the presence of epithelial desquamation, eosinophilic microabscesses, and abnormally long papillae
PPI trial: persistence of eosinophilia and no improvement of symptoms after trialling a proton pump inhibitor. This can help the clinician differentiate between eosinophilic oesophagitis and GORD, which can be a tricky task

Question 6

How is eosinophilic oesophagitis managed?

Answer 6

dietary modification
There are three methods available to begin excluding food from the diet. The elemental diet (involves taking an amino acid mixture for six weeks), exclusion of six food groups (involves avoiding foods commonly associated with allergy e.g. nuts, soy, egg, seafood), and the targeted elimination diet (involves excluding foods that have been identified as allergy-triggering during allergy testing).

Topical steroids e.g. fluticasone and budesonide are options when dietary modification fails. This requires the patient to swallow solutions of the steroid to line the oesophagus. This should be done for eight weeks before being reassessed
Oesophageal dilatation: 56% of patients require this at some point in their treatment to reduce the symptoms associated with oesophageal strictures

Question 7

what are the complications of eosinophilic oesophagitis

Answer 7

Strictures of the oesophagus (56%)
Impaction: 55% of patients experience this, and 38% of these require endoscopic removal of the impaction
Mallory-Weiss tears

Question 8

what is ocreotide and when is it used?

Answer 8

A synthetic somatostatin analogue that is licensed to treat symptoms associated with carcinoid tumours with features of carcinoid syndrome, particularly flushing and diarrhoea. It reduces the secretion of serotonin which is responsible for these symptoms. This peptide hormone is naturally produced by D (delta) cells in the pancreas and stomach and is thought to act to reduce acid secretion from gastric parietal cells.

Can also be used for:
acute treatment of variceal haemorrhage
acromegaly
carcinoid syndrome
prevent complications following pancreatic surgery
VIPomas
refractory diarrhoea

Question 9

what are the hormones involved in food digestion?

Answer 9

Gastrin, CCK, Secretin, VIP, somatostatin,

Question 10

Where is gastrin produced, what it the stimulus and what are its actions?

Answer 10

Produced - G cells in the antrum of the stomach
Stimulus - distension of the stomach, vagus nerves (mediated by gastrin releasing peptide), luminal peptides/amino acids. Inhibited by low antral pH, somatostatin.
Actions - increases acid secretion by gastric parietal cells, pepsinogen and IF secretion, increases gastric motility, stimulates parietal cell maturation

Question 11

Where is CCK produced, what it the stimulus and what are its actions?

Answer 11

Produced - I cells in the upper small intestines
Stimulus - patirally digested proteins and triglycerides
Actions - increases secretion of enzyme rich fluid from the pacreas, contraction of the gallbladder and relaxation of sphincter of Oddi, decreases gastric emptying, trophic effect on the pancreatic acinar cells, induces satiety.

Question 12

Where is Secretin produced, what it the stimulus and what are its actions?

Answer 12

Source: S cells in upper small intestine
Stimulus - acidic chyme, fatty acids
Actions - Increases secretion of bicarbonate-rich fluid from pancreas and hepatic duct cells, decreases gastric acid secretion, trophic effect on pancreatic acinar cells

Question 13

Where is VIP produced, what it the stimulus and what are its actions?

Answer 13

Source: small intestine, pancreas
Stimulus: Neural
Actions: stimulates secretion by pancreas and intestines, inhibits acid production

Question 14

Where is Somatostatin produced, what it the stimulus and what are its actions?

Answer 14

Produced - D Cells in the pancreas & Stomach
Stimulus - Fat, bile salts and glucose in the intestinal lumen
Action: Decreases acid and pepsin secretion, decreases gastrin secretion, decreases pancreatic enzyme secretion, decreases insulin and glucagon secretion
inhibits trophic effects of gastrin, stimulates gastric mucous production

Question 15

How is Wilson’s disease inherited?

Answer 15

AR

Question 16

what is Wilson’s disease?

Answer 16

characterized by excessive copper deposition in the tissues
Metabolic abnormalities include increased copper absorption from the small intestine and decreased hepatic copper excretion

The onset of symptoms is usually between 10 - 25 years. Children usually present with liver disease whereas the first sign of disease in young adults is often neurological disease

Question 17

What gene and chromosome is the defect in Wilsons disease?

Answer 17

Wilson’s disease is caused by a defect in the ATP7B gene located on chromosome 13.

Question 18

what are the features of wilsons disease:

Answer 18

Features result from excessive copper deposition in the tissues, especially the brain, liver and cornea:]

liver: hepatitis, cirrhosis

neurological:
basal ganglia degeneration: in the brain, most copper is deposited in the basal ganglia, particularly in the putamen and globus pallidus
speech, behavioural and psychiatric problems are often the first manifestations
also: asterixis, chorea, dementia, parkinsonism

Kayser-Fleischer rings
green-brown rings in the periphery of the iris
due to copper accumulation in Descemet membrane
present in around 50% of patients with isolated hepatic Wilson’s disease and 90% who have neurological involvement

renal tubular acidosis (esp. Fanconi syndrome)
haemolysis
blue nails

Question 19

what are the investigations for Wilson’s disease?

Answer 19

slit lamp examination for Kayser-Fleischer rings
reduced serum caeruloplasmin
reduced total serum copper (counter-intuitive, but 95% of plasma copper is carried by ceruloplasmin)
free (non-ceruloplasmin-bound) serum copper is increased
increased 24hr urinary copper excretion
the diagnosis is confirmed by genetic analysis of the ATP7B gene

Question 20

What is the management of Wilson’s disease?

Answer 20

penicillamine (chelates copper) has been the traditional first-line treatment
trientine hydrochloride is an alternative chelating agent which may become first-line treatment in the future
tetrathiomolybdate is a newer agent that is currently under investigation

Question 21

what is Purtscher retinopathy?

Answer 21

A condition that may be seen following head trauma and in conditions such a acute pancreatitis, fat embolization, amniotic fluid embolization and vasculitic disease.
Cotton wool spots are seen on fundoscopy

Question 22

What are the scoring systems to identify the cases of severe acute pancreatitis?

Answer 22

Ranson score, Glasgow score and APACHE II.

Common factors in these scoring systems include:
age > 55 years
hypocalcaemia
hyperglycaemia
hypoxia
neutrophilia
elevated LDH and AST

Question 23

How is drug induced liver disease devided?

Answer 23

hepatocellular, cholestatic or mixed.

Question 24

what drugs cause a hepatocellular liver injury?

Answer 24

paracetamol
sodium valproate, phenytoin
MAOIs
halothane
anti-tuberculosis: isoniazid, rifampicin, pyrazinamide
statins
alcohol
amiodarone
methyldopa
nitrofurantoin

Question 25

what drugs cause cholestasis +/- hepatitis?

Answer 25

combined oral contraceptive pill antibiotics: flucloxacillin, co-amoxiclav, erythromycin* anabolic steroids, testosterones phenothiazines: chlorpromazine, prochlorperazine sulphonylureas fibrates rare reported causes: nifedipine

Question 26

what drugs cause liver cirrhosis

Answer 26

methotrexate methyldopa amiodarone

Question 27

what is the most common type of inherited colorectal cancer?

Answer 27

Hereditary non-polyposis colorectal carcinoma (HNPCC), also known as Lynch syndrome.

Question 28

what are the three types of colon cancer?

Answer 28

sporadic (95%) hereditary non-polyposis colorectal carcinoma (HNPCC, 5%) familial adenomatous polyposis (FAP, <1%)

Question 29

what is sporadic colon cancer due to?

Answer 29

Studies have shown that sporadic colon cancer may be due to a series of genetic mutations. For example, more than half of colon cancers show allelic loss of the APC gene. It is believed a further series of gene abnormalities e.g. activation of the K-ras oncogene, deletion of p53 and DCC tumour suppressor genes lead to invasive carcinoma.

Question 30

What in Lynch syndrome?

Answer 30

AD condition patients usually develop cancers in the proximal colon, which are poorly differentiated and highly aggressive. The most common genes involved are MSH2 (60% of cases) MLH1 (30%)

Question 31

What is the criteria used to aid the diagnosis of Hereditary non-polyposis colorectal carcinoma

Answer 31

The Amsterdam criteria are sometimes used to aid diagnosis: at least 3 family members with colon cancer the cases span at least two generations at least one case diagnosed before the age of 50 years

Question 32

What is FAP?

Answer 32

Familial adenomatous polyposis AD condition hundreds of polyps form by the age of 30-40. Patients inevitably develop a carcinoma due to a mutation in a tumour suppressor gene celled adenomatous polyposis coli gene (APC), located on chromosome 5. Genetic testing can be done by analysing DNA from a patient's white blood cells

Question 33

How is FAP managed?

Answer 33

Patients generally have a total proctocolectomy with ileal pouch anal anastomosis (IPAA) formation in their twenties. However they are also at risk from duodenal tumours

Question 34

What is the management for FAP

Answer 34

Patients generally have a total proctocolectomy with ileal pouch anal anastomosis (IPAA) formation in their twenties.

Question 35

How can ascites be devided?

Answer 35

The causes of ascites can be grouped into those with a <11 g/L or a gradient >11g/

Question 36

What does anascitic fluid with a SAAG >11g/L indicates

Answer 36

portal hypertension

Question 37

what causes acites with a SAAG > 11g/L

Answer 37

Liver disorders are the most common cause cirrhosis/alcoholic liver disease acute liver failure liver metastases Cardiac right heart failure constrictive pericarditis Other causes Budd-Chiari syndrome portal vein thrombosis veno-occlusive disease myxoedema

Question 38

What causes ascites with a SAAG < 11g/L

Answer 38

Hypoalbuminaemia nephrotic syndrome severe malnutrition (e.g. Kwashiorkor) Malignancy peritoneal carcinomatosis Infections tuberculous peritonitis Other causes pancreatitis bowel obstruction biliary ascites postoperative lymphatic leak serositis in connective tissue diseases

Question 39

How are ascites managed?

Answer 39

reducing dietary sodium fluid restriction is sometimes recommended if the sodium is < 125 mmol/L aldosterone antagonists: e.g. spironolactone loop diuretics are often added. Some authorities only add loop diuretics in patients who don't respond to aldosterone antagonists whereas other authorities suggest starting both types of diuretic on the first presentation of ascites drainage if tense ascites (therapeutic abdominal paracentesis) large-volume paracentesis for the treatment of ascites requires albumin 'cover'. Evidence suggests this reduces paracentesis-induced circulatory dysfunction and mortality paracentesis induced circulatory dysfunction can occur due to large volume paracentesis (> 5 litres). It is associated with a high rate of ascites recurrence, development of hepatorenal syndrome, dilutional hyponatraemia, and high mortality rate prophylactic antibiotics to reduce the risk of spontaneous bacterial peritonitis. NICE recommend: 'Offer prophylactic oral ciprofloxacin or norfloxacin for people with cirrhosis and ascites with an ascitic protein of 15 g/litre or less, until the ascites has resolved' a transjugular intrahepatic portosystemic shunt (TIPS) may be considered in some patients

Question 40

How is the severity of UC classified?

Answer 40

mild: < 4 stools/day, only a small amount of blood moderate: 4-6 stools/day, varying amounts of blood, no systemic upset severe: >6 bloody stools per day + features of systemic upset (pyrexia, tachycardia, anaemia, raised inflammatory markers)

Question 41

How do you induce remission in mild-moderate UC?

Answer 41

proctitis topical (rectal) aminosalicylate: for distal colitis rectal mesalazine has been shown to be superior to rectal steroids and oral aminosalicylates if remission is not achieved within 4 weeks, add an oral aminosalicylate if remission still not achieved add topical or oral corticosteroid proctosigmoiditis and left-sided ulcerative colitis topical (rectal) aminosalicylate if remission is not achieved within 4 weeks, add a high-dose oral aminosalicylate OR switch to a high-dose oral aminosalicylate and a topical corticosteroid if remission still not achieved stop topical treatments and offer an oral aminosalicylate and an oral corticosteroid extensive disease topical (rectal) aminosalicylate and a high-dose oral aminosalicylate: if remission is not achieved within 4 weeks, stop topical treatments and offer a high-dose oral aminosalicylate and an oral corticosteroid

Question 42

how do you induce remission in severe UC?

Answer 42

should be treated in hospital IV steroids are usually given first-line IV ciclosporin may be used if steroids are contraindicated if after 72 hours there has been no improvement, consider adding IV ciclosporin to IV corticosteroids or consider surgery

Question 43

How do you maintain remission in UC?

Answer 43

Following a mild-to-moderate ulcerative colitis flare proctitis and proctosigmoiditis topical (rectal) aminosalicylate alone (daily or intermittent) or an oral aminosalicylate plus a topical (rectal) aminosalicylate (daily or intermittent) or an oral aminosalicylate by itself: this may not be as effective as the other two options left-sided and extensive ulcerative colitis low maintenance dose of an oral aminosalicylate Following a severe relapse or >=2 exacerbations in the past year oral azathioprine or oral mercaptopurine

Question 44

what kind of bacteria is c-diff

Answer 44

Clostridioides difficile is a Gram positive rod

Question 45

How is C-diff severity classified?

Answer 45

Mild -normal WCC Moderate - ↑ WCC ( < 15 x 109/L) Typically 3-5 loose stools per day Severe - ↑ WCC ( > 15 x 109/L) or an acutely ↑ creatinine (> 50% above baseline) or a temperature > 38.5°C or evidence of severe colitis(abdominal or radiological signs) Life-threatening - Hypotension Partial or complete ileus Toxic megacolon, or CT evidence of severe disease

Question 46

How is C.diff diagnosed

Answer 46

is made by detecting C. difficile toxin (CDT) in the stool C. difficile antigen positivity only shows exposure to the bacteria, rather than current infection

Question 47

How is c.diff managed?

Answer 47

First episode of C. difficile infection first-line therapy is oral vancomycin for 10 days second-line therapy: oral fidaxomicin third-line therapy: oral vancomycin +/- IV metronidazole Recurrent episode recurrent infection occurs in around 20% of patients, increasing to 50% after their second episode within 12 weeks of symptom resolution: oral fidaxomicin after 12 weeks of symptom resolution: oral vancomycin OR fidaxomicin Life-threatening C. difficile infection oral vancomycin AND IV metronidazole specialist advice - surgery may be considered Other therapies bezlotoxumab is a monoclonal antibody which targets C. difficile toxin B NICE do not currently support its use to prevent recurrences as it is not cost-effective faecal microbiota transplant may be considered for patients who've had 2 or more previous episodes

Question 48

what are the features of hepatic encephalopathy?

Answer 48

confusion, altered GCS (see below) asterixis: 'liver flap', arrhythmic negative myoclonus with a frequency of 3-5 Hz constructional apraxia: inability to draw a 5-pointed star triphasic slow waves on EEG raised ammonia level (not commonly measured anymore)

Question 49

what is the grading of hepatic encephalopathy?

Answer 49

Grade I: Irritability Grade II: Confusion, inappropriate behaviour Grade III: Incoherent, restless Grade IV: Coma

Question 50

what are the precipitating factors for hepatic encephalopathy?

Answer 50

infection e.g. spontaneous bacterial peritonitis GI bleed post transjugular intrahepatic portosystemic shunt constipation drugs: sedatives, diuretics hypokalaemia renal failure increased dietary protein (uncommon)

Question 51

How is hepatic encephalopathy managed?

Answer 51

treat any underlying precipitating cause NICE recommend lactulose first-line, with the addition of rifaximin for the secondary prophylaxis of hepatic encephalopathy lactulose is thought to work by promoting the excretion of ammonia and increasing the metabolism of ammonia by gut bacteria antibiotics such as rifaximin are thought to modulate the gut flora resulting in decreased ammonia production other options include embolisation of portosystemic shunts and liver transplantation in selected patients

Question 52

What are the features of auto-immune hepatitis?

Answer 52

commonly seen in young females may present with signs of chronic liver disease acute hepatitis: fever, jaundice etc (only 25% present in this way) amenorrhoea (common) ANA/SMA/LKM1 antibodies, raised IgG levels liver biopsy: inflammation extending beyond limiting plate 'piecemeal necrosis', bridging necrosis

Question 53

what are the 3 types of autoimmune hepatitis?

Answer 53

Type 1 - Anti-nuclear antibodies (ANA) and/or anti-smooth muscle antibodies (SMA) Affects both adults and children Type II - Anti-liver/kidney microsomal type 1 antibodies (LKM1) Affects children only Type III - Soluble liver-kidney antigen Affects adults in middle-age

Question 54

How do you manage auto-immune hepatitis?

Answer 54

steroids, other immunosuppressants e.g. azathioprine liver transplantation

Question 55

what are the features of Crohn's disease?

Answer 55

presentation may be non-specific symptoms such as weight loss and lethargy diarrhoea the most prominent symptom in adults Crohn's colitis may cause bloody diarrhoea abdominal pain: the most prominent symptom in children perianal disease: e.g. Skin tags or ulcers extra-intestinal features are more common in patients with colitis or perianal disease

Question 56

what are exta-intestinal features of Crohn's?

Answer 56

Related to disease activity Arthritis: pauciarticular, asymmetric Erythema nodosum Episcleritis Osteoporosis Unrelated to disease activity Arthritis: polyarticular, symmetric Uveitis Pyoderma gangrenosum Clubbing Primary sclerosing cholangitis

Question 57

What does pigment laden macrophages on biopsy of colon suggest ?

Answer 57

pigment-laden macrophages in the colon, often referred to as 'pseudomelanosis coli', is indicative of chronic laxative use. Anthraquinone laxatives such as senna and cascara are culprits, leading to a brown-black discolouration of the colonic mucosa due to the accumulation of lipofuscin pigment in macrophages within the lamina propria.

Question 58

what would you see on biopsy on intestinal melanoma?

Answer 58

atypical melanocytes and the presence of melanin pigment within these cells. Melanomas can metastasize to the gastrointestinal tract but primary intestinal melanomas are extremely rare.

Question 59

what would you see on biopsy of UC?

Answer 59

s crypt abscesses, goblet cell depletion and continuous inflammation starting from the rectum

Question 60

What is Barrett's oesophagus?

Answer 60

metaplasia of the lower oesophageal mucosa with the usual squamous epithelium being replaced by columnar epithelium.

Question 61

what are this histological features of Barrett's oesophagus?

Answer 61

the columnar epithelium may resemble that of either the cardiac region of the stomach or that of the small intestine (e.g. with goblet cells, brush border)

Question 62

What are the risk factors for Barrett's

Answer 62

gastro-oesophageal reflux disease (GORD) is the single strongest risk factor male gender (7:1 ratio) smoking central obesity

Question 63

How do you manage Barrett's oesophagus?

Answer 63

high dose PPI Endoscopic surveillance with biopsies If dysplasia of any grade is indented - endoscopic intervention is offered - radio frequency ablation , endoscopic mucosal resection

Question 64

causes of pancreatitis?

Answer 64

Popular mnemonic is GET SMASHED Gallstones Ethanol Trauma Steroids Mumps (other viruses include Coxsackie B) Autoimmune (e.g. polyarteritis nodosa), Ascaris infection Scorpion venom Hypertriglyceridaemia, Hyperchylomicronaemia, Hypercalcaemia, Hypothermia ERCP Drugs (azathioprine, mesalazine*, didanosine, bendroflumethiazide, furosemide, pentamidine, steroids, sodium valproate)

Question 65

How is Haemochromatosis inherited?

Answer 65

Autosomal recessive

Question 66

what is Haemochromatosis ?

Answer 66

disorder of iron absorption and metabolism resulting in iron accumulation. It is caused by inheritance of mutations in the HFE gene on both copies of chromosome 6

Question 67

What are the presenting features of Haemochromatosis?

Answer 67

early symptoms include fatigue, erectile dysfunction and arthralgia (often of the hands) 'bronze' skin pigmentation diabetes mellitus liver: stigmata of chronic liver disease, hepatomegaly, cirrhosis, hepatocellular deposition) cardiac failure (2nd to dilated cardiomyopathy) hypogonadism (2nd to cirrhosis and pituitary dysfunction - hypogonadotrophic hypogonadism) arthritis (especially of the hands)

Question 68

How is remission induced in Crohn's ?

Answer 68

Glucocorticoids (oral, topical IV) Enteral feeding with an elemental diet 5-ASA drugs - second line to steroids Azathioprine or mercaptopurine may be used as an add on medication to induce remission (but not used as a mono therapy) Infliximab in refractory cases and fistulating disease (patients will usually continue azathioprine or mercaptopurine Metronidazole is often used for isolated peri-anal disease

Question 69

How do you maintain remission in Crohn's disease?

Answer 69

azathioprine or mercaptopurine is used first-line to maintain remission +TPMT activity should be assessed before starting methotrexate is used second-line

Question 70

when is surgery indicated in Crohn's disease?

Answer 70

80% of patients will eventually have surgery structuring terminal ileal disease --> ileocaecal resection segmental bowel resection Perianal fistulae or tract formation (if they are symptomatic give metronidazole) perianal abscess - may require drainage

Question 71

What are the complications of Crohn's disease

Answer 71

small bowel cancer (standard incidence ratio = 40) colorectal cancer (standard incidence ratio = 2, i.e. less than the risk associated with ulcerative colitis) osteoporosis

Question 72

What is carcinoid syndrome?

Answer 72

usually occurs when metastases are present in the liver and release serotonin into the systemic circulation may also occur with lung carcinoid as mediators are not 'cleared' by the liver

Question 73

what are the features of carcinoid tumours?

Answer 73

lushing (often the earliest symptom) diarrhoea bronchospasm hypotension right heart valvular stenosis (left heart can be affected in bronchial carcinoid) other molecules such as ACTH and GHRH may also be secreted resulting in, for example, Cushing's syndrome pellagra can rarely develop as dietary tryptophan is diverted to serotonin by the tumour

Question 74

What are the investigations for carcinoid tumours?

Answer 74

urinary 5-HIAA plasma chromogranin A y

Question 75

How are carcinoid tumour managed?

Answer 75

somatostatin analogues e.g. octreotide diarrhoea: cyproheptadine may help

Question 76

what are the scoring systems to classify severity of liver cirrhosis?

Answer 76

Child Pugh (used to classify severity) Meld (used to predict survival

Question 77

what is included in the child Pugh and the meld scoring systems?

Answer 77

Child Pugh Bilirubin, albumin, PT, encephalopathy, ascites MELD - bilirubin, creatinine, INR

Question 78

what is Zollinger -Ellison syndrome ?

Answer 78

Zollinger-Ellison syndrome is a condition characterised by excessive levels of gastrin secondary to a gastrin-secreting tumour. The majority of these tumours are found in the first part of the duodenum, with the second most common location being the pancreas. Around 30% of gastrinomas occur as part of MEN type I syndrome.

Question 79

What are the features of Zollinger-Ellison syndrome?

Answer 79

multiple gastroduodenal ulcers diarrhoea malabsorption

Question 80

How is Zollinger-Ellison syndrome diagnosed?

Answer 80

fasting gastrin levels: the single best screen test secretin stimulation test

Question 81

what is primary biliary cholangitis?

Answer 81

Is also known as primary biliary cirrhosis - a chronic liver disorder typically seen in middle aged females. Thought to be auto-immune Interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis which may eventually progress to cirrhosis. The classic presentation is itching in a middle-aged woman

Question 82

What conditions are associated with primary biliary cholangitis?

Answer 82

Sjogren's syndrome (seen in up to 80% of patients) rheumatoid arthritis systemic sclerosis thyroid disease

Question 83

What are the clinical features of PBC?

Answer 83

asymptomatic fatigue pruritus Cholestatic jaundice hyperpigmentation around 10% of patients will have RUQ pain xanthelasmas, xanthomata clubbing, hepatosplenomegaly

Question 84

how do you diagnose primary binary cholangitis ?

Answer 84

AMA, raised IgM Imaging - MRCP

Question 85

what is the management of primary biliary cholangitis?

Answer 85

1st line - ursodeoxycholic acid (slows progression and improves symptoms) Pruritus - cholestyramine fat soluble vitamin supplements Liver transplant - if bilirubin > 100

Question 86

what are the complications of PBC?

Answer 86

cirrhosis → portal hypertension → ascites, variceal haemorrhage osteomalacia and osteoporosis significantly increased risk of hepatocellular carcinoma (20-fold increased risk)

Question 87

what are smooth muscle antibodies associated with ?

Answer 87

autoimmune hepatitis

Question 88

which clotting factor increases in liver disease?

Answer 88

Factor VIII This is because factor VIII is synthesised in endothelial cells throughout the body, unlike the other clotting factors which are synthesised purely in hepatic endothelial cells. Furthermore, whilst activated factor VIII is usually rapidly cleared from the blood stream, good hepatic function is required for this to occur, further leading to increases in circulating factor VIII

Question 89

what is budd-chiari syndrome

Answer 89

hepatic vein thrombosis usually seen in the context of underlying haematological disease or another procoagulant

Question 90

why is there increased risk of thrombosis in liver disease?

Answer 90

increase in clotting factor VIII reduced synthesis of the purely hepatic derived natural anticoagulants protein c and protein s (vitamin k dependent), and anti-thrombin (non-vitamin k dependent)

Question 91

what are the causes of Budd-Chiari syndrome?

Answer 91

polycythaemia rubra vera thrombophilia: activated protein C resistance, antithrombin III deficiency, protein C & S deficiencies pregnancy combined oral contraceptive pill: accounts for around 20% of cases

Question 92

What are the features of Budd-Chiari syndrome?

Answer 92

abdominal pain: sudden onset, severe ascites → abdominal distension tender hepatomegaly

Question 93

how do you investigate Budd-Chiari syndrome?

Answer 93

USS Doppler

Question 94

which laxative is carcinogenic?

Answer 94

Co-danthramer

Question 95

what are osmotic laxatives?

Answer 95

lactulose, macrogols and rectal phosphates

Question 96

what are examples of stimulant laxatives?

Answer 96

senna, docusate, bisacodyl and glycerol

Question 97

what are examples of bulk forming laxatives?

Answer 97

ispaghula husk and methylcellulose

Question 98

What is Gilberts syndrome?

Answer 98

Autosomal recessive condition of defective bilirubin conjugation due to a deficiency of UPD glucuronosyltransferase

Question 99

what are the features of Gilbert's syndrome?

Answer 99

Unconjugated hyperbilirubinaemia (i.e. not in urine) jaundice may only be seen during an intercurrent illness, exercise or fasting

Question 100

What are the investigations + management for Glbert's syndrome

Answer 100

Investigation and management investigation: rise in bilirubin following prolonged fasting or IV nicotinic acid no treatment required

Question 101

What is Dublin-Johnson syndrome?

Answer 101

Dubin-Johnson syndrome is a benign autosomal recessive disorder resulting in hyperbilirubinaemia (conjugated, therefore present in urine). It is due to a defect in the canillicular multispecific organic anion transporter (cMOAT) protein. This causes defective hepatic bilirubin excretion

Question 102

What is Rotor syndrome

Answer 102

autosomal recessive - defect in hepatic uptake and storage of bilirubin benign a conjugated bilirubinaemia

Question 103

What is the most common type of anal cancer?

Answer 103

squamous cell carcinoma (80%)

Question 104

What is the most common cause of anal cancer?

Answer 104

HPV

Question 105

How does anal cancer present?

Answer 105

Perianal pain, perianal bleeding A palpable lesion Faecal incontinence A neglected tumour in a female may present with a rectovaginal fistula.

Question 106

what medication is used as prophylaxis to reduce the risk of variceal bleeding?

Answer 106

A non-cardioselective B-blocker (NSBB) is used for the prophylaxis of oesophageal bleeding e.g. propranolol endoscopic vatical band ligation may be used - NICE who recommends: 'Offer endoscopic variceal band ligation for the primary prevention of bleeding for people with cirrhosis who have medium to large oesophageal varices.'

Question 107

How is variceal haemorrhage managed?

Answer 107

resuscitate prior to endoscopy transfusion correct clotting - FFP, vitamin K, platelets Vasoactive agents - terlipressin Prophylactic abx have been shown to reduce mortality endoscopy and ligation Sengstaken-Blakemore tube if uncontrolled haemorrhage Transjugular Intrahepatic Portosystemic Shunt (TIPSS) if above measures fail connects the hepatic vein to the portal vein exacerbation of hepatic encephalopathy is a common complication

Question 108

What is intrahepatic cholestasis of pregnancy?

Answer 108

Intrahepatic cholestasis of pregnancy (also known as obstetric cholestasis) occurs in around 1% of pregnancies and is generally seen in the third trimester. It is the most common liver disease of pregnancy.

Question 109

what are the features of obstetric cholestasis and what is the management?

Answer 109

Features pruritus, often in the palms and soles no rash (although skin changes may be seen due to scratching) raised bilirubin Management ursodeoxycholic acid is used for symptomatic relief weekly liver function tests women are typically induced at 37 weeks Complications include an increased rate of stillbirth. It is not generally associated with increased maternal morbidity

Question 110

when may acute fatty liver of pregnancy happen?

Answer 110

Acute fatty liver of pregnancy is rare complication which may occur in the third trimester or the period immediately following delivery. potentially fatal complication

Question 111

what are the features of acute fatty liver of pregnancy?

Answer 111

abdominal pain nausea & vomiting headache jaundice hypoglycaemia severe disease may result in pre-eclampsia

Question 112

what investigations for acute fatty liver of pregnancy ?

Answer 112

ALT is typically elevate > 600

Question 113

How is acute fatty liver of pregnancy managed?

Answer 113

supportive care once stabilised - delivery is the definitive management

Question 114

what kind of bacteria is C.diff

Answer 114

gram positive rod , spore-forming, toxin-producing bacillus

Question 115

how is C.diff diagnosed?

Answer 115

is made by detecting C. difficile toxin (CDT) in the stool C. difficile antigen positivity only shows exposure to the bacteria, rather than current infection

Question 116

How is c.diff managed?

Answer 116

1st line - oral vancomycin 2nd line - oral fidaxomicin 3rd line - oral vancomycin +/- IV metronidazole Life-threatening C. difficile infection oral vancomycin AND IV metronidazole specialist advice - surgery may be considered bezlotoxumab is a monoclonal antibody which targets C. difficile toxin B NICE do not currently support its use to prevent recurrences as it is not cost-effective faecal microbiota transplant may be considered for patients who've had 2 or more previous episodes

Question 117

what are the adverse effects of PPI ?

Answer 117

hyponatraemia, hypomagnasaemia osteoporosis → increased risk of fractures microscopic colitis increased risk of C. difficile infections

Question 118

which hormone causes contraction of the gall bladder?

Answer 118

CCK is released in response to a fatty meal and is the major hormonal stimulator of biliary contraction. CCK is secreted from I cells in the upper small intestine

Question 119

Histology of Crohn's

Answer 119

skip lesions Inflammation in all layers from mucosa to serosa increased goblet cells granulomas

Question 120

Histology of UC?

Answer 120

continuous disease No inflammation beyond submucosa (unless fulminant disease) - inflammatory cell infiltrate in lamina propria neutrophils migrate through the walls of glands to form crypt abscesses depletion of goblet cells and mucin from gland epithelium granulomas are infrequent

Question 121

what are the types of bariatric surgery

Answer 121

Primarily restrictive operations laparoscopic-adjustable gastric banding (LAGB) - it is normally the first-line intervention in patients with a BMI of 30-39kg/m^2 - produces less weight loss than malabsorptive or mixed procedures but as it has fewer complications sleeve gastrectomy - stomach is reduced to about 15% of its original size intragastric balloon - the balloon can be left in the stomach for a maximum of 6 months Primarily malabsorptive operations biliopancreatic diversion with duodenal switch usually reserved for very obese patients (e.g. BMI > 60 kg/m^2) Biliopancreatic diversion with duodenal switch is a primarily malabsorptive procedure and reserved for patients who are very obese. Mixed operations Roux-en-Y gastric bypass surgery is both restrictive and malabsorptive in action

Question 122

what can be used for ongoing diarrhoea in a Crohn's patient post resection with normal CRP?

Answer 122

the procedure leads to malabsorption of bile acid. The increased load of bile acid in the colon results in increased secretion of salt and water - appropriate to manage with cholestyramine.

Question 123

What is primary sclerosis cholangitis?

Answer 123

a biliary disease of unknown aetiology characterised by inflammation and fibrosis of intra and extra-hepatic bile ducts.

Question 124

What are the associations with primary sclerosis cholangitis?

Answer 124

cholestasis jaundice, pruritus raised bilirubin + ALP right upper quadrant pain fatigue

Question 125

what are the investigations for primary sclerosis cholangitis?

Answer 125

endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance cholangiopancreatography (MRCP) are the standard diagnostic investigations, showing multiple biliary strictures giving a 'beaded' appearance p-ANCA may be positive there is a limited role for liver biopsy, which may show fibrous, obliterative cholangitis often described as 'onion skin'

Question 126

what are the complications of primary sclerosis cholangitis?

Answer 126

cholangiocarcinoma (in 10%) increased risk of colorectal cancer

Question 127

What is achalasia?

Answer 127

Failure of oesophageal peristalsis and of relaxation of the lower oesophageal sphincter (LOS) due to degenerative loss of ganglia from Auerbach's plexus i.e. LOS contracted, oesophagus above dilated. Achalasia typically presents in middle-age and is equally common in men and women.

Question 128

What are the clinical features of Achalasia ?

Answer 128

dysphagia of BOTH liquids and solids typically variation in severity of symptoms heartburn regurgitation of food may lead to cough, aspiration pneumonia etc malignant change in small number of patients

Question 129

what are the investigations for Achalasia ?

Answer 129

oesophageal manometry - excessive LOS tone which doesn't relax on swallowing barium swallow - shows grossly expanded oesophagus, fluid level CXR - wide mediastinum, fluid level

Question 130

How is achalasia managed>

Answer 130

penumaitic balloon dilation surgical intervention - Heller cardiomyotomy intra-sphincteric injection of botulinum toxin is sometimes used in patients who are a high surgical risk drug therapy (e.g. nitrates, calcium channel blockers) has a role but is limited by side-effects

Question 131

what is angiodysplasia ?

Answer 131

Angiodysplasia is a vascular deformity of the gastrointestinal tract which predisposes to bleeding and iron deficiency anaemia. There is thought to be an association with aortic stenosis, although this is debated. Angiodysplasia is generally seen in elderly patients

Question 132

what may cause hyperchylomicronaemia and what may in predispose to?

Answer 132

Hyperchylomicronaemia may be caused by hereditary lipoprotein lipase deficiency and apolipoprotein CII deficiency. It predisposes to recurrent attacks of acute pancreatitis

Question 133

what are the features of angiodysplasia?

Answer 133

anaemia gastrointestinal (GI) bleeding if upper GI then may be melena if lower GI then may present as brisk, fresh red PR bleeding

Question 134

How is angiodysplasia diagnosed and managed?

Answer 134

Diagnosis colonoscopy mesenteric angiography if acutely bleeding Management endoscopic cautery or argon plasma coagulation antifibrinolytics e.g. Tranexamic acid oestrogens may also be used

Question 134

What is small bowel bacterial overgrowth syndrome?

Answer 134

Small bowel bacterial overgrowth syndrome (SBBOS) is a disorder characterised by excessive amounts of bacteria in the small bowel resulting in gastrointestinal symptoms.

Question 135

What are the risk factors for small bowel bacterial overgrowth syndrome ?

Answer 135

neonates with congenital gastrointestinal abnormalities scleroderma diabetes mellitus

Question 136

What are the features of small bowel bacterial overgrowth syndrome?

Answer 136

chronic diarrhoea bloating, flatulence abdominal pain

Question 137

How is small bowel bacterial overgrowth syndrome diagnosed and managed?

Answer 137

Diagnosis hydrogen breath test small bowel aspiration and culture: this is used less often as invasive and results are often difficult to reproduce clinicians may sometimes give a course of antibiotics as a diagnostic trial Management correction of the underlying disorder antibiotic therapy:rifaximin is now the treatment of choice due to relatively low resistance. Co-amoxiclav or metronidazole are also effective in the majority of patients.

Question 138

how is Haemochromatosis diagnosed?

Answer 138

general population- transferrin saturation is considered the most useful marker, ferritin should also be measured but is not usually abnormal in the early stages of iron accumulation testing family members- genetic testing for HFE mutation Further tests liver function tests molecular genetic testing for the C282Y and H63D mutations MRI is generally used to quantify liver and/or cardiac iron liver biopsy is now generally only used if suspected hepatic cirrhosis

Question 139

What are the typical iron study profiles in haemochromatosis?

Answer 139

Typical iron study profile in patient with haemochromatosis transferrin saturation > 55% in men or > 50% in women raised ferritin (e.g. > 500 ug/l) and iron low TIBC

Question 140

How is Haemochromatosis managed?

Answer 140

Venesection is the first line - TSAT should be kept below 50 and serum ferritin concentration below 50 ug/l desferrioxamine may be used second-line

Question 141

how should a baby be managed when born to a mum who has chronic hep B or hep B during pregnancy?

Answer 141

babies born to mothers who are chronically infected with hepatitis B or to mothers who've had acute hepatitis B during pregnancy should receive a complete course of vaccination + hepatitis B immunoglobulin ** hep B can't be transferred via breast feeding

Question 143

Most common cause for pyogenic liver abscess?

Answer 143

The most common organisms found in pyogenic liver abscesses are Staphylococcus aureus in children and Escherichia coli in adults.

Question 144

What can octreotide be used for?

Answer 144

acute treatment of variceal haemorrhage acromegaly carcinoid syndrome prevent complications following pancreatic surgery VIPomas refractory diarrhoea