Gastro Flashcards

Question

what type of organism is c. diff? -what syndrome does this cause?

Answer 1

gram +ve rod It produces an exotoxin which causes intestinal damage leading to a syndrome called pseudomembranous colitis.

Answer 2

Clindamycin is historically associated with causing Clostridium difficile but the aetiology has evolved significantly over the past 10 years. Second and third generation cephalosporins (e.g ciprofloxacin) are now the leading cause.

Answer 3

Features: • Diarrhea • Abdominal pain • If severe, toxic dilatation • Sometimes seen in nosocomial outbreaks

Answer 4

Diagnosis is made by detecting Clostridium difficile TOXIN (CDT) in the stool

Answer 5

Management: • ORAL metronidazole for 10-14 days • If severe or not responding to metronidazole then ORAL vancomycin may be used. • For life-threatening infections a combination of oral vancomycin and intravenous metronidazole should be used fidaxomicin may also be used for patients who are not responding , particularly those with multiple co-morbidities Other therapies bezlotoxumab is a monoclonal antibody which targets Clostridium difficile toxin B - it is not in widespread use

Answer 6

Small bowel bacterial overgrowth syndrome (SBBOS) is a disorder characterised by excessive amounts of bacteria in the small bowel resulting in gastrointestinal symptoms. It should be noted that many of the features overlap with irritable bowel syndrome: chronic diarrhoea bloating, flatulence abdominal pain

Answer 7

Risk factors for SBBOS -neonates with congenital gastrointestinal abnormalities -scleroderma -diabetes mellitus Important association: Systemic sclerosis, diverticulae and blind loop.

Answer 8

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

Answer 9

Diphtheria toxin commonly causes a 'diphtheric membrane' on tonsils caused by necrotic mucosal cells. Systemic distribution may produce necrosis of myocardial, neural and renal tissue.

Answer 10

Staph. aureus exotoxins lead to acute gastroenteritis, toxic shock syndrome and Staphylococcal scalded skin syndrome

Answer 11

Management correction of 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.

Answer 12

Lockjaw is caused by Clostridium tetani neurotoxin (tetanospasmin)

Answer 13

Cholera toxin causes activation of adenylate cyclase leading to ↑ in cAMP levels, which in turn leads to ↑ chloride secretion.

Answer 14

Acute treatment of variceal hemorrhage • ABC: patients should ideally be resuscitated prior to endoscopy • Correct clotting: FFP, vitamin K • Vasoactive agents: terlipressin is currently the only licensed vasoactive agent. It has been shown to be of benefit in initial hemostasis and preventing rebleeding. It acts by Constriction of the splanchnic vessels (contraindicated in IHD, use Octreotide as alternative) • Prophylactic antibiotics have been shown in multiple meta-analyses to ↓ mortality in patients with liver cirrhosis • Endoscopy: endoscopic variceal band ligation is superior to endoscopic sclerotherapy • Sengstaken-blakemore tube if uncontrolled hemorrhage (in urgent setting when endoscopy is not ready) • Transjugular intrahepatic portosystemic shunt (TIPSS) if above measures fail

Answer 15

Prophylaxis of variceal hemorrhage • Propranolol: ↓ rebleeding and mortality compared to placebo • Endoscopic variceal band ligation (EVL) is superior to endoscopic sclerotherapy. It should be performed at two-weekly intervals until all varices have been eradicated. Proton pump inhibitor cover is given to prevent EVL-induced ulceration

Answer 16

Barrett's Esophagus refers to the metaplasia of the lower esophageal mucosa, with the usual squamous epithelium being replaced by columnar epithelium. There is ↑ risk of esophageal adenocarcinoma, estimated at 50-100 folds. Barrett's can be subdivided into short (<3cm) and long (>3cm). The length of the affected segment correlates strongly with the chances of identifying metaplasia.

Answer 17

Histological features: 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)

Answer 18

Management • Endoscopic surveillance with biopsies -for patients with metaplasia (but not dysplasia) endoscopy is recommended every 3-5 years If dysplasia of any grade is identified endoscopic intervention is offered. Options include: endoscopic mucosal resection radiofrequency ablation

Answer 19

Indications for upper GI endoscopy: • Age > 55 years • Symptoms > 4 weeks or persistent symptoms despite treatment • Dysphagia • Relapsing symptoms • Weight loss Usually there is poor correlation between symptoms and endoscopy appearance

Answer 20

24hr esophageal pH monitoring is gold standard investigation in GORD If endoscopy is negative consider 24-hr oesophageal pH monitoring (the gold standard test for diagnosis)

Answer 21

inhaled steroid therapy

Answer 22

-associated with weight loss, anorexia or vomiting during eating -Past history may include Barrett's esophagus, GERD, excessive smoking or alcohol use

Answer 23

-May be history of heartburn -Odynophagia but no weight loss and systemically well

Answer 24

There may be a history of HIV or other risk factors such as steroid inhaler use

Answer 25

-Dysphagia of both liquids and solids from the start -Heartburn -Regurgitation of food - may lead to cough, aspiration pneumonia etc

Answer 26

More common in older men -Represents a posteromedial herniation between thyropharyngeus and cricopharyngeus muscles -Usually not seen but if large then a midline lump in the neck that gurgles on palpation -Typical symptoms are dysphagia, regurgitation, aspiration and chronic cough. Halitosis may occasionally be seen

Answer 27

Other features of CREST syndrome may be present, namely Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia

Answer 28

Other symptoms may include extraocular muscle weakness or ptosis Dysphagia with liquids as well as solids Globus hystericus

Answer 29

May be history of anxiety Symptoms are often intermittent

Answer 30

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

Answer 31

• 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 • 7% ↑ in risk of squamous cell carcinoma

Answer 32

The gold standard test for achalasia is esophageal manometry • Barium swallow shows grossly expanded esophagus, fluid level • CXR: wide mediastinum, fluid leve

Answer 33

Loss of peristalsis in distal esophagus, failure of LOS to relax during swallowing and (i.e ↑ residual relaxing pressure

Answer 34

Loss of peristalsis in distal esophagus BUT ↓ resting LOS pressure

Answer 35

• Intra-sphincteric injection of botulinum toxin • Heller cardiomyotomy • Balloon dilation • Drug therapy has a role but is limited by side-effects

Answer 36

Complete transmural (full-thickness) laceration or perforation of the esophagus, distinct from Mallory-Weiss syndrome, a nontransmural esophageal tear also associated with vomiting.

Answer 37

• Perforation is almost always on Left side of Lower esophagus. • Gastric contents enter the mediastinum and pleural cavity, if one were to perform a pleural fluid aspirate; one is likely to aspirate gastric contents! • ♂ > ♀ and typically between 50-70 years old • Other clinical features that may suggest the diagnosis include odynophagia and surgical emphysema in the neck

Answer 38

• Vomiting (against a closed glottis) in eating disorders such as bulimia • Rarely: Extremely forceful coughing - Obstruction by food Iatrogenic perforation, accounts for 85-90% of cases of esophageal rupture, typically as a complication of an endoscopic procedure, feeding tube, or unrelated surgery.

Answer 39

• Radiographs show mediastinal gas, effusion, and later pneumothorax. Esophagram is used to confirm leak, first with water-soluble contrast, then barium if no leak demonstrated.

Answer 40

Early operation after appropriate resuscitation offers the best chance of survival.

Answer 41

Causes • NSAIDs • Bisphosphonates • Steroids The following drugs may cause reflux by reducing lower esophageal sphincter (LOS) pressure • Calcium channel blockers* • Nitrates* • Theophyllines *calcium channel blockers and nitrates are occasionally used in the management of achalasia, itself a cause of dyspepsia, because of their effect on the LOS.

Answer 42

• Chronic gastrointestinal bleeding • Progressive unintentional weight loss • Progressive difficulty swallowing • Persistent vomiting • Iron deficiency anemia • Epigastric mass • Suspicious barium meal

Answer 43

Urgent referral (within 2 weeks) is indicated for patients with any alarm signs irrespective of age. Routine endoscopic investigation of patients of any age, presenting with dyspepsia and without alarm signs is not necessary, however patients aged 55 years and over should be referred urgently for endoscopy if dyspepsia symptoms are: • Recent in onset rather than recurrent and • Unexplained (e.g. New symptoms which cannot be explained by precipitants such as NSAIDs) and • Persistent: continuing beyond a period that would normally be associated with self-limiting problems (e.g. Up to four to six weeks, depending on the severity of signs and symptoms)

Answer 44

This can be summarised at a step-wise approach • Review medications for possible causes of dyspepsia • Lifestyle advice • Trial of full-dose PPI for one month* • 'Test and treat' using carbon-13 urea breath test *it is unclear from studies whether a trial of a PPI or a 'test and treat' should be used first

Answer 45

Helicobacter pylori is a Gram negative bacteria

Answer 46

Associations • Peptic ulcer disease (95% of duodenal ulcers, 75% of gastric ulcers) • Gastric cancer • B cell lymphoma of MALT tissue (eradication of H pylori 80% causes regression) • Atrophic gastritis

Answer 47

The role of H pylori in Gastresophageal reflux disease (GERD) is unclear - there is currently no role of eradication of H pylori in GERD.

Answer 48

-Urea breath test -Rapid urease test (e.g. CLO test) -Serum antibody -Culture of gastric biopsy -Gastric biopsy -Stool antigen test

Answer 49

• Patients consume a drink containing carbon isotope 13 (13C) enriched urea • Urea is broken down by H. pylori urease • After 30 mins patient exhale into a glass tube • Mass spectrometry analysis calculates the amount of 13C CO2 • Sensitivity 95-98%, specificity 97-98% • Used to confirm eradication • Should not be performed within 4 weeks of treatment with an antibacterial or within 2 weeks of an antisecretory drug (e.g. a proton pump inhibitor)

Answer 50

• Biopsy sample is mixed with urea and pH indicator • Color change if H pylori urease activity • Sensitivity 90-95%, specificity 95-98%

Answer 51

• Remains positive after eradication • Sensitivity 85%, specificity 80%

Answer 52

• Provide information on antibiotic sensitivity • Sensitivity 70%, specificity 100%

Answer 53

• Histological evaluation alone, no culture • Sensitivity 95-99%, specificity 95-99%

Answer 54

• Sensitivity 90%, specificity 95%

Answer 55

Peutz-Jeghers syndrome is an autosomal dominant condition characterized by numerous hamartomatous polyps in the gastrointestinal tract.

Answer 56

Around 50% of patients will have died from a gastrointestinal tract cancer by the age of 60 years.

Answer 57

• Autosomal dominant • Responsible gene encodes serine threonine kinase LKB1 or STK11

Answer 58

• Hamartomatous polyps in GI tract (mainly small bowel) • Pigmented lesions on lips, oral mucosa, face, palms and soles • classical histological appearance of smooth muscle “arborisation” • Intestinal obstruction e.g. Intussusception • Gastrointestinal bleeding

Answer 59

• Conservative unless complications develop

Answer 60

Until recent time esophageal cancer was most commonly due to a squamous cell carcinoma but the incidence of adenocarcinoma is rising rapidly. Adenocarcinoma is now (since 2010) the most common type of oesophageal cancer and is more likely to develop in patients with a history of gastro-esophageal reflux disease (GERD) or Barrett's. The majority of tumours are in the middle third of the esophagus.

Answer 61

Helicobacter pylori may actually be protective against esophageal cancer

Answer 62

• Smoking • Alcohol • Achalasia • Plummer-vinson syndrome • Rare: coeliac disease, scleroderma • Sensetive to radiotherapy

Answer 63

• GERD • Barrett's esophagus

Answer 64

signet ring cells signet ring cells may be seen in gastric cancer. They contain a large vacuole of mucin which displaces the nucleus to one side. Higher numbers of signet ring cells are associated with a worse prognosis

Answer 65

pidemiology • Overall incidence is decreasing, but incidence of tumors arising from the cardia is increasing • Peak age = 70-80 years • More common in Japan, China, Finland and Columbia than the west • More common in ♂s, 2:1

Answer 66

• H. pylori infection (although it is protective against esophageal cancer) • Blood group A: gAstric cAncer • Gastric adenomatous polyps • Pernicious anemia • Smoking • Diet: salty, spicy, nitrates • May be negatively associated with duodenal ulcer

Answer 67

• Diagnosis: endoscopy with biopsy • Staging: -CT or endoscopic ultrasound - endoscopic ultrasound has recently been shown to be superior to CT (CT scanning of the chest abdomen and pelvis is the routine first line staging investigation in most centres. Laparoscopy to identify occult peritoneal disease PET CT (particularly for junctional tumours))

Answer 68

dyspepsia nausea and vomiting anorexia and weight loss dysphagia

Answer 69

Type 1 True oesophageal cancers and may be associated with Barrett's oesophagus. Type 2 Carcinoma of the cardia, arising from cardiac type epithelium or short segments with intestinal metaplasia at the oesophagogastric junction. Type 3 Sub cardial cancers that spread across the junction. Involve similar nodal stations to gastric cancer.

Answer 70

Proximally sited disease greater than 5-10cm from the OG junction may be treated by sub total gastrectomy Total gastrectomy if tumour is <5cm from OG junction For type 2 junctional tumours (extending into oesophagus) oesophagogastrectomy is usual Endoscopic sub mucosal resection may play a role in early gastric cancer confined to the mucosa and perhaps the sub mucosa (this is debated) Lymphadenectomy should be performed. A D2 lymphadenectomy is widely advocated by the Japanese, the survival advantages of extended lymphadenectomy have been debated. However, the overall recommendation is that a D2 nodal dissection be undertaken. Most patients will receive chemotherapy either pre or post operatively.

Answer 71

Screening: • Most cancers develop from adenomatous polyps therefore a screening program could theoretically ↓ mortality • Main techniques being evaluated are faecal occult blood (FOB) testing, sigmoidoscopy and colonoscopy • Fecal occult blood testing is the only method to have been proven to ↓ mortality (by about 20%) in trials. Sensitivity can be ↑ by DNA analysis for the APC gene • Trials looking at screening using flexible sigmoidoscopy are currently underway • Carcinoembryonic antigen may be used to monitor for recurrence in patients post- operatively or to assess response to treatment in patients with metastatic disease. The CEA-scan study is a nuclear medicine procedure — that uses a small dose of radioactive isotope to image tumors sometimes invisible to other diagnostic tests. That isotope is guided to tumors via antibody fragments engineered to seek out and attach to any tissue that expresses carcinoembryonic antigen (CEA), a protein found on virtually all colorectal tumors. CEA blood tests attempt to detect this same protein in blood, but often fail to do so, due to lack of sensitivity.

Answer 72

Causes for a positive fecal occult blood testing are: • 2-10%: cancer (colorectal cancer, gastric cancer) • 20-30% adenoma or polyps • Bleeding peptic ulcer • Angiodysplasia of the colon

Answer 73

-the NHS now has a national screening programme offering screening every 2 years to all men and women aged 60 to 74 years in England, 50 to 74 years in Scotland. Patients aged over 74 years may request screening -eligible patients are sent Faecal Immunochemical Test (FIT) tests through the post -a type of faecal occult blood (FOB) test which uses antibodies that specifically recognise human haemoglobin (Hb) -used to detect, and can quantify, the amount of human blood in a single stool sample -advantages over conventional FOB tests is that it only detects human haemoglobin, as opposed to animal haemoglobin ingested through diet -only one faecal sample is needed compared to the 2-3 for conventional FOB tests -whilst a numerical value is generated, this is not reported to the patient or GP, who will instead be informed if the test is normal or abnormal -patients with abnormal results are offered a colonoscopy

Answer 74

5 out of 10 patients will have a normal exam 4 out of 10 patients will be found to have polyps which may be removed due to their premalignant potential 1 out of 10 patients will be found to have cancer

Answer 75

screening for bowel cancer using sigmoidoscopy is being rolled out as part of the NHS screening program the aim (other than to detect asymptomatic cancers) is to allow the detection and treatment of polyps, reducing the future risk of colorectal cancer this is being offered to people who are 55-years-old NHS patient information leaflets refer to this as 'bowel scope screening' patients can self-refer for bowel screening with sigmoidoscopy up to the age of 60, if the offer of routine one-off screening at age 55 had not been taken up

Answer 76

• The Dukes staging system is widely employed for classifying colorectal cancers and is a useful predictor of survival. Tumour grade and depth of penetration are also important: • Duke A (Stage I) defines a tumour confined to the bowel wall (i.e. mucosa and submucosa). • Duke B (Stage II) invades through the muscle wall. • Duke C (Stage III) involves lymph nodes. • After this the patient presents with metastatic disease at distant sites (Stage IV).

Answer 77

• Poorly differentiated histological type. • Tumour adherence to adjacent organs. • Bowel perforation. • Colonic obstruction at the time of diagnosis. • Venous invasion by the tumour.

Answer 78

• Surgical excision of a colonic carcinoma is the main treatment • Adjuvant chemotherapy (5-fluorouracil and folinic acid) is warranted in high-risk stage II colonic carcinomas and all stage III colonic carcinomas. • The addition of oxaliplatin has been shown to improve survival in these patients in a large multicentre trial (MOSAIC study), but the additional drug can cause a severe peripheral neuropathy. • Adjuvant radiotherapy is used in rectal carcinomas. This is combined with chemotherapy in stage II and III rectal carcinomas to reduce the risk of local as well as metastatic relapse.

Answer 79

• Sporadic (95%) • Hereditary non-polyposis colorectal carcinoma (HNPCC, 5%) • Familial adenomatous polyposis (FAP, <1%)

Answer 80

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 adenomatous polyposis coli (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

Answer 81

HNPCC, an autosomal dominant condition, is the most common form of inherited colon cancer. Around 90% of patients develop cancers, often of the proximal colon, which are often poorly differentiated and highly aggressive. Currently four gene mutations have been identified (including in the hMLH1 and hMSH2 genes).

Answer 82

Amsterdam criteria for HNPCC • 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

Answer 83

HNPCC screening for ↑ risk group: • Colonoscopy every 2 years from 20 to 40 years age then annually • Check mutation in DNA or mismatched repair gene.

Answer 84

Familial Adenomatous Polyposis (FAP) is a rare autosomal dominant condition which leads to the formation of hundreds of polyps by the age of 30-40 years. Patients inevitably develop carcinoma. Patients with FAP are also at risk from duodenal tumours which is important cause of death.

Answer 85

It is due to a mutation in a tumour suppressor gene called adenomatous polyposis coli gene (APC), located on chromosome 5. Genetic testing can be done by analysing DNA from a patient’s white blood cell.

Answer 86

Patients generally have a total colectomy with ileo-anal pouch formation in their twenties.

Answer 87

A variant of FAP called Gardner's syndrome can also feature osteomas of the skull and mandible, can be identified based on oral findings, including multiple impacted and supernumerary teeth, multiple jaw osteomas which give a "cotton-wool" appearance to the jaws, as well as multiple odontomas, Congenital Hypertrophy of the Retinal Pigment Epithelium (CHRPE), in addition to multiple adenomatous polyps of the colon, thyroid carcinoma and epidermoid cysts on the skin.

Answer 88

• Patients > 40 years old, reporting rectal bleeding with a change of bowel habit towards looser stools and/or ↑ stool frequency persisting for 6 weeks or more • Patients > 60 years old, with rectal bleeding persisting for 6 weeks or more without a change in bowel habit and without anal symptoms • Patients > 60 years old, with a change in bowel habit to looser stools and/or more frequent stools persisting for 6 weeks or more without rectal bleeding • Any patient presenting with a right lower abdominal mass consistent with involvement of the large bowel • Any patient with a palpable rectal mass • Unexplained iron deficiency anemia in men or non-menstruating women (Hb < 11 g/dl in men, < 10 g/dl in women)

Answer 89

is condition characterized by excessive levels of gastrin, usually from a gastrin secreting tumour usually of the duodenum or pancreas. Around 30% occur as part of MEN type I syndrome

Answer 90

Zollinger-Ellison syndrome typically presents with multiple gastroduodenal ulcers causing abdominal pain, diarrhea and malabsorption. High-dose proton pump inhibitors are needed to control the symptoms.

Answer 91

• Fasting gastrin levels, the single best screen test: done in 3 different days as the gastrin secretion is pulsatile • Secretin stimulation test: considered +ve if there is ↑ in gastrin >200 pg/mL after secretin injection (Normally Secretin supresses gastrin, but in ZE, it simply shows that the source of gastrin is not the stomach and it is somewhere else like pancresae)

Answer 92

• If not mets, surgical resection is the cure • Octreotide can be used to alleviate symptoms with interferon and chemotherapy to attempt cure non respectable tumor • PPI is used to control symptoms in acute stages

Answer 93

• Associated with H. pylori infection in 95% of cases • Good prognosis • If low grade then 80% respond to H. pylori eradication • Paraproteinemia may be present

Answer 94

is a vascular deformity of the gastrointestinal tract which predisposes to bleeding and iron deficiency anemia

Answer 95

aortic stenosis The association between angiodysplasia and aortic stenosis is thought to be caused by von Willebrand factor (vWF) being proteolysed in the turbulent blood flow around the aortic valve. vWF is most active in vascular beds with high shear stress, such as angiodysplasia, and deficiency of vWF increases the bleeding risk from such lesions

Answer 96

Diagnosis • Colonoscopy • Mesenteric angiography if acutely bleeding

Answer 97

Management • Endoscopic cautery or argon plasma coagulation • Antifibrinolytics e.g. Tranexamic acid • Estrogens may also be used

Answer 98

• Gallstones • Ethanol • Trauma • Steroids • Mumps (other viruses include Coxsackie B) • Autoimmune (e.g. Polyarteritis nodosa), Ascaris infection • Scorpion venom • Hypertriglyceridemia, Hyperchylomicronemia**, Hypercalcemia, Hypothermia • ERCP • Drugs (azathioprine, mesalazine*, didanosine, bendroflumethiazide, furosemide, pentamidine, steroids, sodium valproate) • ↑ Degree burn of large skin area with significant inhalation injury

Answer 99

• Ischemic (Purtscher) retinopathy (cotton wool spots seen on fundoscopy) - may cause temporary or permanent blindness. This condition may be seen following head trauma and in conditions such as acute pancreatitis, fat embolisation, amniotic fluid embolisation, and vasculitic diseases.

Answer 100

• Age > 55 • WBC > 16 • Urea > 16 • Glucose > 11 • Alb < 30 • ALT > 250 • Ca+ < 1 • LDH > 350 • PO2 < 8 • Hematocrit ↓ >10% • Base deficit > 4 • Fluid loss > 6L

Answer 101

Clinical impression of severity Body mass index >30 Pleural effusion APACHE score >8

Answer 102

Clinical impression of severity APACHE II >8 Glasgow score of 3 or more Persisting multiple organ failure CRP>150

Answer 103

Glasgow Score of >3 CRP >150 Persisting or progressive organ failure

Answer 104

PaO2< 7.9kPa Age > 55 years Neutrophils (WBC > 15) Calcium < 2 mmol/L Renal function: Urea > 16 mmol/L Enzymes LDH > 600IU/L Albumin < 32g/L (serum) Sugar (blood glucose) > 10 mmol/L

Answer 105

Pancreatitis in the context of HIV infection may be secondary to anti- retroviral treatment (especially didanosine) or by opportunistic infections e.g. CMV

Answer 106

Acute pancreatitis Pancreatic pseudocyst Mesenteric infarct Perforated viscus Acute cholecystitis Diabetic ketoacidosis

Answer 107

Traditionally hyperamylasaemia has been utlilised with amylase being elevated three times the normal range. However, amylase may give both false positive and negative results. Serum lipase is both more sensitive and specific than serum amylase. It also has a longer half life. Serum amylase levels do not correlate with disease severity

Answer 108

Nutrition -There is reasonable evidence to suggest that the use of enteral nutrition does not worsen the outcome in pancreatitis -Most trials to date were underpowered to demonstrate a conclusive benefit. -The rationale behind feeding is that it helps to prevent bacterial translocation from the gut, thereby contributing to the development of infected pancreatic necrosis. Use of antibiotic therapy -Many UK surgeons administer antibiotics to patients with acute pancreatitis. -A recent Cochrane review highlights the potential benefits of administering Imipenem to patients with established pancreatic necrosis in the hope of averting the progression to infection. -There are concerns that the administration of antibiotics in mild attacks of pancreatitis will not affect outcome and may contribute to antibiotic resistance and increase the risks of antibiotic associated diarrhoea. Surgery -Patients with acute pancreatitis due to gallstones should undergo early cholecystectomy. -Patients with obstructed biliary system due to stones should undergo early ERCP. -Patients who fail to settle with necrosis and have worsening organ dysfunction may require debridement, fine needle aspiration is still used by some. -Patients with infected necrosis should undergo either radiological drainage or surgical necrosectomy. The choice of procedure depends upon local expertise.

Answer 109

Chronic Pancreatitis is an inflammatory condition which can ultimately affect both the exocrine and endocrine functions of the pancreas. Around 80% of cases are due to alcohol excess with up to 20% of cases being unexplained.

Answer 110

• Pain is typically worse 15 to 30 minutes following a meal • Steatorrhoea: symptoms of pancreatic insufficiency usually develop between 5 and 25 years after the onset of pain • Diabetes mellitus develops in the majority of patients. It typically occurs more than 20 years after symptom begin

Answer 111

• Abdominal x-ray shows pancreatic calcification in 30% of cases • CT is more sensitive at detecting pancreatic calcification • Functional tests: pancreolauryl and Lundh tests may be used to assess exocrine function if imaging inconclusive

Answer 112

Management • Pancreatic enzyme supplements • Analgesia • Antioxidants: limited evidence base - one study suggests benefit in early disease

Answer 113

Peripancreatic fluid collections Pseudocysts Pancreatic necrosis Pancreatic abscess Haemorrhage

Answer 114

Occur in 25% cases Located in or near the pancreas and lack a wall of granulation or fibrous tissue May resolve or develop into pseudocysts or abscesses Since most resolve aspiration and drainage is best avoided as it may precipitate infection

Answer 115

-In acute pancreatitis result from organisation of peripancreatic fluid collection. They may or may not communicate with the ductal system. -The collection is walled by fibrous or granulation tissue and typically occurs 4 weeks or more after an attack of acute pancreatitis -Most are retrogastric -75% are associated with persistent mild elevation of amylase -Investigation is with CT, ERCP and MRI or endoscopic USS -They cannot be diagnosed until more than 6 weeks after the acute attack -Symptomatic cases may be observed for 12 weeks as up to 50% resolve -Treatment is either with endoscopic or surgical cystogastrostomy or aspiration

Answer 116

Pancreatic necrosis may involve both the pancreatic parenchyma and surrounding fat Complications are directly linked to extent of parenchymal necrosis and extent of necrosis overall Early necrosectomy is associated with a high mortality rate (and should be avoided unless compelling indications for surgery exist) Sterile necrosis should be managed conservatively (at least initially) Some centres will perform fine-needle aspiration sampling of necrotic tissue if infection is suspected. False negatives may occur and the extent of sepsis and organ dysfunction may be a better guide to surgery

Answer 117

Intraabdominal collection of pus associated with pancreas but in the absence of necrosis Typically occur as a result of infected pseudocyst Transgastric drainage is one method of treatment, endoscopic drainage is an alternative

Answer 118

Infected necrosis may involve vascular structures with resultant haemorrhage that may occur de novo or as a result of surgical necrosectomy. When retroperitoneal haemorrhage occurs Grey Turner's sign may be identified

Answer 119

Acute respiratory distress syndrome associated with a high-mortality rate of around 20%

Answer 120

• Abdominal pain or a mass. • Fever • Persistently raised amylase and liver function tests

Answer 121

• Serous cystadenoma: benign and remain benign. • Mucinous cystadenoma: this may be benign but has the potential to become malignant.

Answer 122

• CT or MRI can distinguish between the two. • Aspiration of the cyst for cytology and carcinogenic embryonic antigen

Answer 123

• Mucinous neoplasm: most patients undergo limited surgery of the pancreatic cyst. • Serous cystadenoma or aymptomatic pseudocyst: can also be associated with polycystic kidney disease and von Hippel–Lindau disease. Considerable debate exists as to whether follow up is necessary; in younger patients it may not be. Anyhow, guidelines generally recommend annual review for a period of around 4 years.

Answer 124

• Incidence in the West: 9 cases per 100 000 and it’s increasing over the last 20 years. • 60% are ♂ • 5-year survival rate: 2% • K-ras is the most common oncogene in this condition • Insulinomas are the commonest form of endocrine tumours of the pancreas • Majority of cases occur in patients over the age of 60

Answer 125

• Smoking (with a twofold increase in incidence) • Diabetes • Chronic pancreatitis • Hereditary pancreatitis • Hereditary non-polyposis colorectal carcinoma • Multiple endocrine neoplasia • Peutz-jeghers syndrome • BRCA2 • Dysplastic naevus syndrome

Answer 126

• Contrast CT is used for diagnosis and assessment of invasion • ERCP restricted to palliative care • CA 19-9 • U/S abdomen is less reliable when the tumor is in the body or tail

Answer 127

• Surgical intervention represents the only chance of long-term survival with < 20% suitable for surgery at diagnosis. Eligibility for resection depend on: " Tumor size < 4cm " Invasion of superior mesenteric artery or portal vein " Presence of mets • Radio and chemotherapy are ineffective

Answer 128

Cowden’s syndrome is an inherited condition resulting from a defect in the PTEN tumour suppressor gene.

Answer 129

Hamartomatous polyps of the GI tract are often the first manifestation along with characteristic muco-cuteneous lesions such as oral mucosal papillomas, palmoplantar keratoses and trichilemmomas (benign tumours of hair follicles). The syndrome is important to diagnose early because of the high risk of malignancy, particularly of the breast and thyroid. Thyroid dysfunction is common even in the absence of cancer.

Answer 130

Familial juvenile polyposis also results in multiple polyps in the colon identical to those found in Cowden’s syndrome but the associated oral lesions are absent.

Answer 131

• Coeliac disease • Crohn's disease • Tropical sprue • Whipple's • Giardiasis • Brush border enzyme deficiencies (e.g. ↓ Lactase)

Answer 132

• Chronic pancreatitis • Cystic fibrosis • Pancreatic cancer

Answer 133

• Biliary obstruction • Primary biliary cirrhosis

Answer 134

malabsorption

Answer 135

• Risk of colorectal cancer high in UC than CD

Answer 136

• Inflammation always starts at rectum and never spreads beyond ileocaecal valve • Continuous disease • Associated with pANCA antigen

Answer 137

• Lesions may be seen anywhere from the mouth to anus • Skip lesions may be present • Associated with ASCA (Anti- Saccharomyces Cerevisiae Antibodies

Answer 138

• 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

Answer 139

• Inflammation in all layers from mucosa to serosa • goblet cells • granulomas

Answer 140

• Widespread ulceration with preservation of adjacent mucosa which has the appearance of polyps ('pseudopolyps')

Answer 141

Deep ulcers, skip lesions

Answer 142

Barium enema • loss of haustrations • superficial ulceration, 'pseudopolyps' • long standing disease: colon is narrow and short -'drainpipe colon'

Answer 143

Small bowel enema • high sensitivity and specificity for examination of the terminal ileum • strictures: 'Kantor's string sign' • proximal bowel dilation • 'rose thorn' ulcers • fistulae

Answer 144

Inducing remission Maintaining remission

Answer 145

• Treatment depends on the extent and severity of disease • Rectal aminosalicylates or steroids: for distal colitis rectal mesalazine has been shown to be superior to rectal steroids • Oral aminosalicylates or steroids • Severe colitis should be referred to hospital In severe ulcerative colitis, patients should be treated with intravenous treatments including steroids, fluids, subcutaneous heparin and elemental diet. The patient should also have daily examination and monitoring including review by surgeons, blood tests, stool chart, heart rate, temperature and abdominal X-rays. intravenous steroids are usually given first-line (intravenous ciclosporin may be used if steroid are contraindicated if after 72 hours there has been no improvement, consider adding intravenous ciclosporin to intravenous corticosteroids or consider surgery) DON’T GIVE ANTI-DIARRHEAL Rx FOR ACUTE COLLITIS → TOXIC MEGACOLON

Answer 146

(Truelove and Witt criteria) Severe ulcerative colitis = 30% risk of colectomy >6 bloody stools/24 hour + 1 or more of -Fever (>37.8°C) -Tachycardia (>90/min) -Anaemia (Haemoglobin <10.5g/dl) -Elevated ESR (>30mm/hr) The severity of UC is usually classified as being mild, moderate or severe: 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)

Answer 147

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 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

Answer 148

Patients with concomitant UC and PSC are at ↑ risk of developing colonic cancer, and it is recommended that they be screened annually with colonoscopy. Patients with extensive UC should be screened 8 to10 years from the onset of symptoms. Colonoscopy should be performed: • 3-yearly in the second decade • 2-yearly in the third decade • Yearly by the fourth decade. Patients post-liver transplant for PSC still should have yearly screening. Each case should be considered for the benefits and risks of the colonoscopy.

Answer 149

Arthritis: pauciarticular, asymmetric Erythema nodosum Episcleritis Osteoporosis Arthritis is the most common extra-intestinal feature in both CD and UC Episcleritis is more common in CD

Answer 150

Arthritis: polyarticular, symmetric Uveitis Pyoderma gangrenosum Clubbing Primary sclerosing cholangitis Primary sclerosing cholangitis is much more common in UC Uveitis is more common in UC

Answer 151

• Disease duration > 10 years • Patients with pancolitis • Onset before 15 years old • Unremitting disease • Poor compliance to treatment

Answer 152

C-reactive protein correlates well with disease activity

Answer 153

o Colonoscopy is the investigation of choice o Features suggest of crohn's include deep ulcers, skip lesions

Answer 154

• Patients should be strongly advised to stop smoking • Some studies suggest an ↑ risk of relapse secondary to NSAIDs and the combined oral contraceptive pill but the evidence is patchy

Answer 155

• Mesalazine: whilst evidence base is limited widely used in active disease • Steroids (oral, topical or intravenous) • Azathioprine is used as a second-line treatment in active disease • Mercaptopurine (also called 6-mercaptopurine, 6-MP) • Methotrexate is used in patients intolerant of azathioprine or refractory disease. Usually given intramuscularly, but not effective in maintaining remission. Significant anemia is a contra- indication for methotrexate. • Infliximab is useful in refractory disease and fistulating crohn's. Patients typically continue on azathioprine or methotrexate

Answer 156

*assess thiopurine methyltransferase (TPMT) activity before offering azathioprine or mercaptopurine

Answer 157

Perianal disease: Metronidazole is first-line

Answer 158

Whipple's disease is a rare multi-system disorder caused by Tropheryma whippelii infection. It is more common in those who are HLA-B27 positive and in middle-aged men

Answer 159

• Malabsorption: diarrhea, weight loss • Large-joint arthralgia (seronegative arthropathy) • Lymphadenopathy • Skin: hyperpigmentation and photosensitivity • Pleurisy, pericarditis • Neurological symptoms (rare): ophthalmoplegia, dementia, seizures, ataxia, myoclonus

Answer 160

• Jejunal biopsy shows deposition of macrophages containing Periodic acid-Schiff (PAS) granules • ↑ CRP and ESR • Hypoalbuminemia

Answer 161

Management • Varies e.g. IV penicillin then oral co-trimoxazole for a year

Answer 162

Microscopic colitis: is defined by the triad of: 1. Watery diarrhea 2. Normal colonoscopy 3. ↑ Inflamation of the lamina propria of the colon. This disease includes collagenous and lymphocytic colitis. It is an uncommon disease with an incidence of 5/100,000 per year and frequents the elderly (mean age 55-68) and women.

Answer 163

• NSAIDs • Omeprazole, Lansoprazole • Ticlopidine • Cimetidine

Answer 164

Treatment: includes just stopping the offending medication. Corticosteroids, cholestyramine and now bismuth have been used as treatment with some effect. Recurrence however, does occur.

Answer 165

The diagnosis of IBS should be considered if the patient has had the following for at least 6 months: • Abdominal pain, and/or • Bloating, and/or • Change in bowel habit

Answer 166

A positive diagnosis of IBS should be made is the patient has abdominal pain relieved by defecation or associated with altered bowel frequency stool form, in addition to 2 of the following 4 symptoms: • Altered stool passage (straining, urgency, incomplete evacuation) • Abdominal bloating (more common in women than men), distension, tension or hardness • Symptoms made worse by eating • Passage of mucus Features such as lethargy, nausea, backache and bladder symptoms may also support the diagnosis

Answer 167

Red flag features should be enquired about: • Rectal bleeding • Unexplained/unintentional weight loss • Family history of bowel or ovarian cancer • Onset after 60 years of age

Answer 168

Suggested primary care investigations are: • Full blood count • ESR • CRP • Coeliac disease screen (antiendomysial antibodies or tissue transglutaminase)

Answer 169

First-line pharmacological treatment - according to predominant symptom • Pain: antispasmodic agents • Constipation: laxatives but avoid lactulose • Diarrhea: loperamide is first-line

Answer 170

Second-line pharmacological treatment • low-dose tricyclic antidepressants (e.g. amitriptyline 5-10 mg) are used in preference to selective serotonin reuptake inhibitors

Answer 171

Other management options • Psychological interventions - if symptoms do not respond to pharmacological treatments after 12 months and who develop a continuing symptom profile (refractory IBS), consider referring for cognitive behavioural therapy, hypnotherapy or psychological therapy • Complementary and alternative medicines: 'do not encourage use of acupuncture or reflexology for the treatment of IBS'

Answer 172

• Have regular meals and take time to eat • Avoid missing meals or leaving long gaps between eating • Drink at least 8 cups of fluid per day, especially water or other non-caffeinated drinks such as herbal teas • Restrict tea and coffee to 3 cups per day • ↓ intake of alcohol and fizzy drinks • Consider limiting intake of high-fibre food (for example, wholemeal or high-fibre flour and breads, cereals high in bran, and whole grains such as brown rice) • ↓ intake of ‘resistant starch’ often found in processed foods • Limit fresh fruit to 3 portions per day • For diarrhea, avoid sorbitol • For wind and bloating consider increasing intake of oats (for example, oat-based breakfast cereal or porridge) and linseeds (up to one tablespoon per day).

Answer 173

Coeliac Disease: is caused by sensitivity to the protein gluten. Repeated exposure leads to villous atrophy which in turn causes malabsorption.

Answer 174

Conditions associated with coeliac disease include dermatitis herpetiformis (a vesicular, pruritic skin eruption) and autoimmune disorders (type 1 diabetes mellitus and autoimmune hepatitis). It is strongly associated with HLA-DQ2 (95% of patients) and HLA-B8 (80%) as well as HLA-DR3 and HLA-DR7

Answer 175

The management of coeliac disease involves a gluten-free diet. Gluten containing cereals include: • Wheat: bread, pasta, pastry • Barley*: beer • Rye • Oats** Some notable foods which are gluten-free include: • Rice • Potatoes • Corn (maize)

Answer 176

? Chronic or intermittent diarrhea ? Failure to thrive or faltering growth (in children) ? Persistent or unexplained gastrointestinal symptoms including nausea and vomiting ? Prolonged fatigue (‘tired all the time’) ? Recurrent abdominal pain, cramping or distension ? Sudden or unexpected weight loss ? Unexplained iron-deficiency anemia, or other unspecified anemia ? Recurrent mouth ulcers

Answer 177

? Autoimmune thyroid disease ? Dermatitis herpetiformis ? Irritable bowel syndrome ? Type 1 diabetes ? First-degree relatives (parents, siblings or children) with coeliac disea

Answer 178

• Anemia: iron, folate and vitamin B12 deficiency (folate deficiency is more common than vitamin B12 deficiency in coeliac disease) • Hyposplenism • Osteoporosis • Lactose intolerance • Enteropathy-associated T-cell lymphoma of small intestine • Subfertility, unfavourable pregnancy outcomes • Rare: esophageal cancer, other malignancies

Answer 179

Immunological testing and jejunal biopsy NICE issued guidelines on the investigation of coeliac disease in 2009. If patients are already taking a gluten-free diet they should be asked, if possible, to reintroduce gluten for at least 6 weeks prior to testing

Answer 180

• Tissue transglutaminase (TTG) antibodies (IgA) are first-choice according to NICE • Endomyseal antibody (IgA) • Anti-gliadin antibody (IgA or IgG) tests are NOT recommended by NICE • Anti-casein antibodies are also found in some patients

Answer 181

• Villous atrophy • Crypt hyperplasia • ↑ in intraepithelial lymphocytes • Lamina propria infiltration with lymphocytes

Answer 182

Tropical Sprue: this disease is most common in the Carribbean and the Far-East. It is characterized by a picture of small intestinal malabsorption and the cause is thought to be infectious in origin

Answer 183

Diagnosis: • Jejunal biopsy reveals: o Mild villous atrophy o ↑ villous crypts o Mononuclear cellular infiltrates o Enlarged epithelial cells o Large nuclei caused by folate and/or vitamin B12 deficiency.

Answer 184

Treatment: • Tetracyclines 250mg qds up to 6 months • Ampicillin may be used as an alternative in patients who are intolerant of tetracyclines. • Folate and B12 deficiencies should also be corrected • Complete recovery is possible with appropriate therapy.

Answer 185

• Coeliac disease - most common • Tropical sprue • Hypogammaglobulinemia • Gastrointestinal lymphoma • Whipple's disease • Cow's milk intolerance

Answer 186

Toxic Megacolon: usually ssociated with severe colitis (criteria mentioned above). The colon is dilated when it is measured > 5.5cm

Answer 187

• Toxic dilatation can be treated medically for 12-24 hours with IV and rectal hydrocortisone but if there is then no improvement in the triad of o Pulse o Stool frequency o Colon dilatation • Patient should undergo an urgent colectomy. Otherwise, there is an increased risk of perforation, which has a mortality of 30%. • In addition to steroids, the acutely unwell patient should receive: o K+ supplementation o Thromboembolism prophylaxis. • Antibiotics have not been demonstrated to help

Answer 188

Short Bowel Syndrome: in patients who have removal of more than one-half of the small intestine, malabsorption syndrome may result. It may occur in patients with Crohn’s disease or those with ischemic bowel who have undergone significant bowel resection.

Answer 189

Presentation: • Diarrhoea • Steatorrhoea • Malabsorption manifestation: Weight loss, anemia, osteoporosis/osteomalacia, electrolyte disturbances and hypovolemia.

Answer 190

Management: • In patients with > 100cm of jejunum, oral intake is still possible. Diarrhoea may reduce with lactose exclusion or with a course of metronidazole to eradicate bacterial overgrowth. • In patients with < 100cm of jejunum, total parenteral nutrition is recommended.

Answer 191

Melanosis coli is a disorder of pigmentation of the bowel wall. Histology demonstrates pigment- laden macrophages. It is associated with laxative abuse, especially anthraquinone compounds such as senna

Answer 192

Diagnosis • Paracentesis: neutrophil count > 250 cells/ul

Answer 193

Management • Intravenous cefotaxime is usually given • Patients who have had an episode of SBP should be on prophylactic antibiotics

Answer 194

Alcoholic liver disease is a marker of poor prognosis in SBP.

Answer 195

Transudate (protein < 30g/l) Exudate (protein > 30g/l)

Answer 196

• Cirrhosis and portal hypertension • Nephrotic syndrome • Cardiac failure • Budd–Chiari syndrome • Myxodema

Answer 197

• Intra-abdominal tuberculosis • Pancreatitis. • Hepatic or peritoneal malignancy

Answer 198

However the serum-ascites albumin gradient (SAAG) has now largely replaced this concept and is the best single test for classifying ascites into portal hypertensive (SAAG >1.1 g/dL) and non–portal hypertensive (SAAG <1.1 g/dL) causes. The terms high-albumin gradient and low-albumin gradient should replace the terms transudative and exudative in the description of ascites

Answer 199

• Cirrhosis • Alcoholic hepatitis • Schistosomiasis • Fulminant hepatic failure • Budd–Chiari syndrome • Acute or chronic portal vein obstruction • Cardiac diseases • Spontaneous bacterial peritonitis secondary to cirrhosis.

Answer 200

• • • • • • Nephrotic syndrome Protein losing enteropathy Peritoneal carcinomatosis Tuberculous peritonitis Pancreatic duct leak Biliary ascites.

Answer 201

Bilirubin: o Bilirubin is derived from the breakdown of heme in the red blood cells within the reticuloendothelial system. o The unconjugated bilirubin then binds albumin and is taken up by the liver. o In the liver it is conjugated which then makes it water-soluble and thus allows it to be excreted into the urine. o Normally total serum bilirubin is measured; however, the unconjugated (indirect bilirubin) and conjugated (direct bilirubin) portions can be.

Answer 202

Albumin - sensitive marker of hepatic function, but not useful in the acute stages as it has a long half life (20 days).

Answer 203

Transferases - usually either alanine aminotransferase (ALT) or aspartate aminotransferase (AST); rarely does a laboratory routinely provide both: o These enzymes normally reside inside cells (in cytoplasm) so raised levels usually represent hepatocellular damage. ALT is more specific to the Liver, as AST is also found in cardiac and skeletal muscle and red blood cells. o Very high levels (>1000 IU/L) suggest drug-induced hepatitis (e.g. paracetamol), acute viral hepatitis (A or B), ischemic, or rarely, autoimmune hepatitis. o The ratio of AST to ALT can give some extra clues as to the cause: $ In chronic Liver disease ALT >AST, once cirrhosis established AST >ALT $ The extremes of the ratio of AST:ALT can also be helpful: ! >2 suggests alcoholic liver disease. ! <1.0 suggests nonalcoholic liver disease.

Answer 204

• Gamma-glutamyltransferase (GGT) - also related to the bile ducts. Typically elevated in cholestasis (with elevated ALP) but, if ALP normal, suggests induction of hepatic metabolic enzymes (e.g alcohol or enzyme-inducing drugs).

Answer 205

• Alkaline phosphatase (ALP) - comes mainly from the cells lining bile ducts but also in bone. Marked elevation is typical of cholestasis (often with elevated GGT) or bone disorders (usually normal GGT). Isoenzyme analysis may help identify source. It is physiologically increased when there is increased bone turnover (e.g. adolescence) and is elevated in the third trimester of pregnancy (produced by the placenta).

Answer 206

When Bilirubin is elevated and it’s mentioned that no bilirubin in uring dipstick ↓ Unconjugated Bilirubin

Answer 207

• Cirrhosis: if early disease, but later liver ↓ in size. Associated with a non-tender, firm liver

Answer 208

• Malignancy: metastatic spread or primary hepatoma. Associated with a hard, irregular. Liver edge

Answer 209

Right heart failure: firm, smooth, tender liver edge. May be pulsatile

Answer 210

alcoholic liver disease

Answer 211

• Chronic liver disease* with portal hypertension • Infections: glandular fever, malaria, hepatitis • Lymphoproliferative disorders • Myeloproliferative disorders e.g. CML • Amyloidosis *the latter stages of cirrhosis are associated with a small liver

Answer 212

is chronic liver disorder typically seen in middle-aged ♀s (♀:♂ ratio of 9:1). The aetiology is not fully understood although it is thought to be an autoimmune condition, with HLA DR3 association. 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

Answer 213

primary biliary cirrhosis

Answer 214

• Early: may be asymptomatic (e.g. Raised ALP on routine LFTs) or fatigue, pruritus • Cholestatic jaundice • Hyperpigmentation, especially over pressure points • Xanthelasmas, xanthomata • Also: clubbing, hepatosplenomegaly • Late: may progress to liver failure

Answer 215

Diagnosis • Anti-mitochondrial antibodies (AMA) M2 subtype in 98% of patients and are highly specific. • Smooth muscle antibodies in 30% of patients • Raised serum IgM

Answer 216

Management • Median survival is 7-10 yrs, but ↓ to 2 yrs if jaundice is present • Pruritus: cholestyramine • Fat-soluble vitamin supplementation • Ursodeoxycholic acid • Liver transplantation e.g. If bilirubin > 100 (PBC is a major indication) - recurrence in graft can occur but is not usually a problem. 5 yr survival post transplant 80%

Answer 217

Primary biliary cirrhosis - the M rule • IgM • Anti-Mitochondrial antibodies, M2 subtype • Middle aged ♀s

Answer 218

Complications • Malabsorption: osteomalacia, coagulopathy • Sicca syndrome (Sjögren's syndrome) occurs in 70% of cases • Portal hypertension: ascites, variceal hemorrhage • Hepatocellular cancer (20-fold ↑ risk)

Answer 219

Primary sclerosing cholangitis is a biliary disease of unknown aetiology characterized by inflammation and fibrosis of intra and extra-hepatic bile ducts

Answer 220

Associations • Ulcerative colitis: 4% of patients with UC have PSC, 80% of patients with PSC have UC • Crohn's (much less common association than UC) • HIV

Answer 221

Features • Cholestasis • Hepatomegaly • Intermittent jaundice

Answer 222

Investigation • ERCP is the standard diagnostic tool, showing multiple biliary strictures giving a 'beaded' appearance • MRCP is superior to ERCP • ANCA may be positive • There is a limited role for liver biopsy, which may show fibrous, obliterative cholangitis often described as ‘onion skin’

Answer 223

Complications • Cholangiocarcinoma (in 10%) • ↑ risk of colorectal cancer

Answer 224

Management: • Liver transplantation, survival post transplant is 90% although rejection is high. Indication for transplantation: o Bilirubin > 100 μmol/l o Recurrent bacterial cholangitis o Ascitis o Refractoryitching o Cholangiocarcinoma is a contraindication for transplantation

Answer 225

The most common cause of biliary disease in patients with HIV is sclerosing cholangitis due to infections such as CMV, Cryptosporidium and Microsporidia

Answer 226

Wilson's Disease is an autosomal recessive disorder characterized by excessive copper deposition in the tissues. Metabolic abnormalities include ↑ copper absorption from the small intestine and ↓ hepatic copper excretion. Wilson's disease is caused by a defect in the ATP7B gene located on chromosome 13.

Answer 227

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

Answer 228

Features result from excessive copper deposition in the tissues, especially the brain, liver and cornea: • Liver: hepatitis, cirrhosis • Neurological: speech and behavioral problems are often the first manifestations. Also: tremor, chorea • Kayser-fleischer rings • Renal tubular acidosis (esp. Fanconi syndrome) • Hemolysis • Blue nails

Answer 229

Wilson's disease

Answer 230

Diagnosis • ↓ serum ceruloplasmin • ↑ 24hr urinary copper excretion • Slit lamp: Kayser-Fleischer ring

Answer 231

Management • D-penicillamine: chelates copper 1.5-2g/day in divided dose then 0.75-1.5g/day maintenance • Trientine • Zinc

Answer 232

Gilbert's syndrome is an autosomal recessive condition of defective bilirubin conjugation due to a deficiency of UDP glucuronyl transferase. The prevalence is approximately 1-2% in the general population. Viral infections are common triggers for a rise in the bilirubin in patients with Gilbert's

Answer 233

Features • Isolated hyperbilirubinemia • Unconjugated hyperbilirubinemia (i.e. Not in urine) • Jaundice may only be seen during an intercurrent illness

Answer 234

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

Answer 235

Hyperbilirubinemia Normal dipsticks urinalysis excludes Dubin-Johnson and Rotor syndrome as these both produce a conjugated bilirubinemia i.e. bilirubin in urine

Answer 236

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

Answer 237

Features: • ↑ of conjugated • NO elevation of liver enzymes (ALT, AST). • Usually asymptomatic but may be diagnosed in early infancy based on laboratory tests. • Liver biopsy shows dark granules in the hepatocytes (thought to be due to melanin deposition) • Prognosis is good, and treatment of this syndrome is usually unnecessary. Most patients are asymptomatic and have normal life spans.

Answer 238

Rotor syndrome: is a rare, relatively benign autosomal recessive bilirubin disorder of unknown origin

Answer 239

R - Normal DJS - Liver has black pigmentation

Answer 240

Rotor - ↑ with <70% being isomer 1 DJS - Normal with >80% being isomer 1 (Normal urine contains more of isomer 3 than isomer 1)

Answer 241

Crigler-Najjar Syndrome: (CNS) is a rare disorder affecting the metabolism of bilirubin, which results in high levels of unconjugated bilirubin and often leads to brain damage in infants. This syndrome is divided type I and type II, with the latter sometimes called Arias syndrome. Type I is usually fatal while only type II can survive to adult life. The syndrome is treated by liver transplantation.

Answer 242

Gilberts syndrome Rotor syndrome DJS Crigler-najjar type I Crigler-najjar type 2

Answer 243

• Type 1: Bile acid malabsorption, secondary to ileal resection, or ileal inflammation (e.g. in Crohn's disease) • Type 2: Idiopathic bile acid malabsorption, Primary bile acid diarrhea • Type 3: Secondary to various gastrointestinal diseases including cholecystectomy, vagotomy, small intestinal bacterial overgrowth, radiation enteropathy, celiac disease, chronic pancreatitis, etc.

Answer 244

• SeHCAT test (selenium homocholic acid taurine or tauroselcholic acid): nuclear test involves two scans a week apart and only very limited radiation exposure. Retention of SeHCAT at 7 days is normally above 15%. Values less than 15% predict a response to bile acid sequestrants. The SeHCAT test measures multiple cycles of bile acid excretion and reabsorption over 7 days. This test is not licensed in the USA, and is underutilized even where it is available • Fecal bile acid quantfication or the 14C-glycocholic breath test are no longer in routine clinical use.

Answer 245

Treatment with bile acid sequestrants is often effective e.g. cholestyramine.

Answer 246

• Incubation period (weeks) = 6-20 • Double-stranded DNA virus • Spread: body fluids + vertical • Chronic disease in 5-10% • Vaccination available

Answer 247

• Contains HBsAg adsorbed onto aluminum hydroxide adjuvant and is prepared from yeast cells using recombinant DNA technology • Most schedules give 3 doses of the vaccine with a recommendation for a one-off booster 5 years following the initial primary vaccination

Answer 248

• At risk groups who should be vaccinated include: healthcare workers, intravenous drug users, sex workers, close family contacts of an individual with hepatitis B, individuals receiving blood transfusions regularly, chronic kidney disease patients who may soon require renal replacement therapy, prisoners, chronic liver disease patients

Answer 249

• Around 10-15% of adults fail to respond or respond poorly to 3 doses of the vaccine. Risk factors include age over 40 years, obesity, smoking, alcohol excess and immunosuppression

Answer 250

Testing for anti-HBs is only recommended for those at risk of occupational exposure (i.e. Healthcare workers) and patients with chronic kidney disease. In these patients anti-HBs levels should be checked 1-4 months after primary immunization. The table below shows how to interpret anti-HBs levels: Anti-HBs level (mIU/ml) Response > 100 Indicates adequate response, no further testing required. Should still receive booster at 5 years 10 - 100 Suboptimal response - one additional vaccine dose should be given. If immunocompetent no further testing is required < 10 Non-responder. Test for past or previous infection. Give further vaccine course (i.e. 3 doses again) with testing following. If still fails to respond then HBIG would be required for protection if exposed to the virus

Answer 251

• Chronic infection (5-10%) • Fulminant liver failure (1%) • Hepatocellular carcinoma • GBS • Glomerulonephritis (memberanoproliferative) • Polyarteritis nodosa • Cryoglobulinemia (more with Hepa C than Hepa B)

Answer 252

• Pegylated interferon-alpha used to be the only treatment available. It reduces viral replication in up to 30% of chronic carriers. A better response is predicted by being ♀ < 50 years old, low HBV DNA levels, non-Asian, HIV negative, high degree of inflammation on liver biopsy • However due to the side-effects of pegylated interferon it is now used less commonly in clinical practice. Oral antiviral medication is increasingly used with an aim to suppress viral replication (not in the dissimilar way to treating HIV patients) • Examples include lamivudine, tenofovir and entecavir

Answer 253

Treatment of Hep B is based on: • Abnormal ALT in at least 1 occasion • Liver biopsy shows fibrosis • Hepa B viremia >105 HBV DNA copies per mL

Answer 254

• Surface antigen (HBsAg) is the first marker to appear and causes the production of anti-HBs • HBsAg normally implies acute disease (present for 1-6 months) • If HBsAg is present for > 6 months then this implies chronic disease • Anti-HBs implies immunity (either exposure or immunization) • Anti-HBc implies previous (or current) infection. IgM anti-HBc appears during acute or recent hepatitis B infection and is present for about 6 months (c=current or recent) Anti-HBc implies previous (or current) infection. IgG anti-HBc persists. • HBeAg results from breakdown of core antigen from infected liver cells as is therefore a marker of infectivity (e=infectivity)

Answer 255

Anti-HBs positive, all others negative

Answer 256

anti-HBc positive, HBsAg negative

Answer 257

anti-HBc positive, HBsAg positive

Answer 258

• All pregnant women are offered screening for hepatitis B • 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 • Studies are currently evaluating the role of oral antiviral treatment (e.g. Lamivudine) in the latter part of pregnancy • There is little evidence to suggest caesarean section reduces vertical transmission rates • Hepatitis B cannot be transmitted via breastfeeding (in contrast to HIV)

Answer 259

Hepatitis C is likely to become a significant public health problem in the UK in the next decade. It is thought around 200,000 people are chronically infected with the virus. At risk groups include intravenous drug users and patients who received a blood transfusion prior to 1991 (e.g. hemophiliacs). Hepa C is an RNA virus.

Answer 260

Transmission • Risk of transmission during a needle stick injury is about 2% • The risk of transmitting the virus during sexual intercourse is probably less than 5% • Vertical transmission rate from mother to child is about 6% (high viral load at delivery ↑ risk) • Breast feeding is not contraindicated in mothers with Hepatitis C

Answer 261

Features • After exposure to the Hepatitis C virus less than 20% of patients develop an acute hepatitis

Answer 262

Complications • Chronic infection (80-85%) - only 15-20% of patients will clear the virus and will hence the majority will develop chronic hepatitis C • Cirrhosis (20-30% of those with chronic disease) • Hepatocellular cancer • Cryoglobulinemia

Answer 263

• Currently a combination of interferon-α (S/C) and ribavirin (PO) are used • Amantadine is a useful alternative for ribavirin and interferon in case if adverse effects. • Genotype 2 and 3 respond to Rx while genotype 4 has less responsiveness. • Genotype 3a on PCR is indication for chronic infection; it is more likely to respond to Rx

Answer 264

Ribavirin - side-effects: hemolytic anemia, cough. Women should not become pregnant within 6 months of stopping ribavirin as it is teratogenic

Answer 265

Interferon α - side-effects: flu-like symptoms, depression, fatigue, leucopenia, thrombocytopenia

Answer 266

Autoimmune hepatitis is condition of unknown etiology which is most commonly seen in young ♀s.

Answer 267

Recognized associations include other autoimmune disorders, hypergammaglobulinemia and HLA B8, DR3.

Answer 268

anti-nuclear antib ANA and/or anti-smooth muscle antib SMA Affects both adults and children

Answer 269

Anti-liver/kidney microsomal type 1 antibodies (LKM1) Affects children only

Answer 270

Soluble liver-kidney antigen affects middle-aged adult > 60% remission with steroids but relapse is 80% Azathioprine is an alternative

Answer 271

autoimmune hepatitis

Answer 272

• May present with signs of chronic liver disease • Acute hepatitis: fever, jaundice etc (only 25% present in this way) • Amenorrhea (common) • ANA/SMA/LKM1 antibodies, raised IgG levels • Liver biopsy: inflammation extending beyond limiting plate 'piecemeal necrosis', bridging necrosis

Answer 273

Management • Steroids, other immunosuppressants e.g. Azathioprine • Liver transplantation

Answer 274

Alcoholic Hepatitis: commonly occurs after a prolonged period of alcohol abuse or return to alcohol after period of abstinence

Answer 275

Features: • Increased serum transaminase • Macrocytosis • Thrombocytopenia • Poor hepatic synthetic function • Leucocytosis irrespective of infection • Increased acute phase reactants. • AST/ALT is usually > 2. • Liver enzymes do not exceed 500.

Answer 276

Malnutrition, jaundice, fluid retention and encephalopathy are associated with poor outlook

Answer 277

The discriminant function (DF) can be used to identify patients for treatment. Those who have DF > 32 have 1 month mortality rate of 50%, they may benefit from corticosteroids or pentoxyfilline. Histology can help to confirm diagnosis. DF= 4.6 x (PT – control) + bilirubin (mg/dl)

Answer 278

Zieve’s Syndrome: occurs in patients with excessive alcohol consumption with hemolysis and severe hyperlipidemia, abdominal pain, transient mildly raised bilirubin. It usually occurs in males and resolves once alcohol consumption is stopped.

Answer 279

Non-alcoholic fatty liver disease (NAFLD) is now the most common cause of liver disease in the developed world. It is largely caused by obesity and describes a spectrum of disease ranging from: • Steatosis - fat in the liver • Steatohepatitis - fat with inflammation, non-alcoholic steatohepatitis (NASH), see below • Progressive disease may cause fibrosis and liver cirrhosis

Answer 280

NAFLD is thought to represent the hepatic manifestation of the metabolic syndrome and hence insulin resistance is thought to be the key mechanism leading to steatosis

Answer 281

Non-alcoholic steatohepatitis (NASH) is a term used to describe liver changes similar to those seen in alcoholic hepatitis in the absence of a history of alcohol abuse. It is relatively common and though to affect around 3-4% of the general population. The progression of disease in patients with NASH may be responsible for a proportion of patients previously labeled as cryptogenic cirrhosis.

Answer 282

Associated factors • Obesity • Hyperlipidemia • T2DM • Jejunoileal bypass • Sudden weight loss/starvation

Answer 283

Features • Usually asymptomatic • Hepatomegaly • ALT is typically greater than AST • Increased echogenicity on ultrasound

Answer 284

• The mainstay of treatment is lifestyle changes (particularly weight loss) and monitoring • There is ongoing research into the role of gastric banding and insulin-sensitizing drugs (e.g. Metformin

Answer 285

Micronodular cirrhosis is associated with alcohol liver disease

Answer 286

Macronodular cirrhosis is associated with chronic hepatitis

Answer 287

Granuloma formation is not classically seen in cirrhosis.

Answer 288

The Child-Pugh classification is a scoring system to assess the severity of liver cirrhosis Bilirubin (μmol/l) Normal: 5-17 1: <34 2: 34-50 3: >50 Albumin (g/l) N: 36-52 1: >35 2: 28-35 3: <28 Prothrombin time, prolonged by (s) N: 12-15 1: <4 2: 4-6 3: >6 Encephalopathy 1: none 2: mild 3: marked Ascites 1: none 2: mild 3: marked • <7 = A • 7-9 = B • >9 =C

Answer 289

Hepatocellular carcinoma (HCC) is the third most common cause of cancer worldwide. Chronic hepatitis B is the most common cause of HCC worldwide with chronic hepatitis C being the most common cause in Europe.

Answer 290

The main risk factor for developing HCC is liver cirrhosis, for example secondary* to hepatitis B & C, alcohol, hemochromatosis and primary biliary cirrhosis. Other risk factors include: • Alpha-1 antitrypsin deficiency • Hereditary tyrosinosis • Glycogen storage disease • Aflatoxin • Drugs: oral contraceptive pill, anabolic steroids • Porphyria cutanea tarda • ♂ sex • Diabetes mellitus, metabolic syndrome *Wilson's disease is an exception

Answer 291

Features • Tends to present late • Features of liver cirrhosis or failure may be seen: jaundice, ascites, RUQ pain, hepatomegaly, pruritus, splenomegaly • Possible presentation is decompensation in a patient with chronic liver disease

Answer 292

Screening with ultrasound (+/- alpha-fetoprotein) should be considered for high risk groups such as: • Patients liver cirrhosis secondary to hepatitis B & C or hemochromatosis • Men with liver cirrhosis secondary to alcohol

Answer 293

Management options • Early disease: surgical resection • Liver transplantation • Radiofrequency ablation • Transarterial chemoembolisation • Sorafenib: a multikinase inhibitor

Answer 294

• Deranged clotting (e.g. INR > 1.4) • Low platelets (e.g. < 60 * 109/l) • Anemia • Bile duct obstruction • Hydatid cyst • Hemoangioma • Uncooperative patient • Ascites

Answer 295

Management • Drainage (needle aspiration or catheter) should always be performed • Amoxicillin + ciprofloxacin + metronidazole • If penicillin allergic: ciprofloxacin + clindamycin

Answer 296

Hepatorenal Syndrome (HRS): refers to the development of acute renal failure in a patient who has advanced liver disease, who has no identifiable cause of intrinsic renal disease. It usually represents the end-stage of a sequence of reductions in renal perfusion induced by increasingly severe hepatic injury. Splanchnic vasodilatation appears to play an important role in the decline in renal function in hepatic disease.

Answer 297

Diagnostic criteria have been proposed for the hepatorenal syndrome: 1. Chronic or acute hepatic disease with advanced hepatic failure and portal hypertension 2. Creatinine > 133 mmol/l that progresses over days to weeks 3. Absence of any other apparent cause for the renal disease, including shock, active sepsis, current nephrotoxic drugs, and the absence of ultrasonographic evidence of obstruction or parenchymal renal disease. It is particularly important to exclude spontaneous bacterial peritonitis, which is complicated with acute renal failure 4. Urine sodium < 10 meq/l (off diuretics) and protein excretion < 500 mg/day 5. Lack of improvement in renal function after volume expansion with 1.5litres of isotonic saline.

Answer 298

Type 1: • Rapidly progressive • Doubling of serum creatinine to > 221 μmol/L or a halving of the creatinine clearance to less than 20 ml/min over a period of less than 2 weeks • Very poor prognosis Type 2: •Slowly progressive •Prognosis poor, but patients may live for longer

Answer 299

Management: is notoriously difficult. The ideal treatment is liver transplantation but patients are often too unwell to have surgery and there is a shortage of donors. Management options: • Vasopressin analogues, for example terlipressin, have a growing evidence base supporting their use. They work by causing vasoconstriction of the splanchnic circulation • Volume expansion with 20% albumin • Transjugular intrahepatic portosystemic shunt

Answer 300

Anal fissures are longitudinal or elliptical tears of the squamous lining of the distal anal canal. If present for less than 6 weeks they are defined as acute, and chronic if present for more than 6 weeks. Around 90% of anal fissures occur on the posterior midline

Answer 301

Management of an acute anal fissure (< 6 weeks) • Dietary advice: high-fiber diet with high fluid intake • Bulk-forming laxatives are first line - if not tolerated then lactulose should be tried • Lubricants such as petroleum jelly may be tried before defecation • Topical anesthetics • Sits baths: hip baths in hot water for 2–5 minutes followed by cold water for 1 minute • Topical steroids do not provide significant relief

Answer 302

Management of a chronic anal fissure (> 6 weeks) • The above techniques should be continued • Topical glyceryl trinitrate (GTN) is first line treatment for a chronic anal fissure • If topical GTN is not effective after 8 weeks then secondary referral should be considered for surgery or botulinum toxin

Answer 303

Mesenteric ischemia is primarily caused by arterial embolism resulting in infarction of the colon. It is more likely to occur in areas such as the splenic flexure that are located at the borders of the territory supplied by the superior and inferior mesenteric arteries.

Answer 304

Predisposing factors • Increasing age • Atrial fibrillation • Other causes of emboli: endocarditis • Cardiovascular disease risk factors: smoking, hypertension, diabetes

Answer 305

Features • Abdominal pain • Rectal bleeding • Diarrhea • Fever • Bloods typically show an elevated WBC associated with acidosis (low HCO3)

Answer 306

Meckel's Diverticulum: • Presents with intermittent melena and anemia • Diagnosed with Meckel's Scan [Technetium-99m (99mTc)] or Video Capsule Endoscopy

Answer 307

Management • Mesenteric angiography is the diagnostic tool • Supportive care • Laparotomy and bowel resection

Answer 308

Ischemic Colitis: Although uncommon in the general population, ischemic colitis occurs with greater frequency in the elderly, and is the most common form of bowel ischemia. Causes of the reduced blood flow can include ↓BP or local factors such as constriction of blood vessels or atheroma in the mesenteric vessels (most common).

Answer 309

Diagnosis: • Barium enema results tend to be abnormal in 90% showing thumbprinting, an indicative of mucosal edema • CT is the single best test after plain radiography • Colonoscopy is the diagnostic tool of choice: it shows petechiae, pallor, hyperemia and necrosis

Answer 310

Lower back pain is the most common presenting feature

Answer 311

Associations • Riedel's thyroiditis • Previous radiotherapy • Sarcoidosis • Inflammatory abdominal aortic aneurysm • Drugs: methysergide

Answer 312

Hepatitis D terminology: Co-infection: Hepatitis B and Hepatitis D infection at the same time. Superinfection: A hepatitis B surface antigen positive patient subsequently develops a hepatitis D infection.

Answer 313

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

Answer 314

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

Answer 315

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

Answer 316

they have a BMI of 40 kg/m^2 or more, or between 35 kg/m^2 and 40 kg/m^2 and other significant disease (for example, type 2 diabetes mellitus, hypertension) that could be improved if they lost weight all appropriate non-surgical measures have failed to achieve or maintain adequate clinically beneficial weight loss for at least 6 months they are receiving or will receive intensive specialist management they are generally fit for anaesthesia and surgery they commit to the need for long-term follow-up Consider surgery as a first-line option for adults with a BMI of more than 50 kg/m2 in whom surgical intervention is considered appropriate; consider orlistat before surgery if the waiting time is long

Answer 317

primarily restrictive: laparoscopic-adjustable gastric banding (LAGB) or sleeve gastrectomy primarily malabsorptive: classic biliopancreatic diversion (BPD) has now largely been replaced by biliopancreatic diversion with duodenal switch mixed: Roux-en-Y gastric bypass surgery

Answer 318

LAGB produces less weight loss than malabsorptive or mixed procedures but as it has fewer complications it is normally the first-line intervention in patients with a BMI of 30-39kg/m^2

Answer 319

patients with a BMI > 40 kg/m^2 may be considered for a gastric bypass or sleeve gastrectomy. The latter may be done as a sole procedure or as an initial procedure prior to bypass

Answer 320

primarily malabsorptive procedures are usually reserved for very obese patients (e.g. BMI > 60 kg/m^2)

Answer 321

confusion, altered GCS (see below) asterix: '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)

Answer 322

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

Answer 323

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

Answer 324

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

Answer 325

hypophosphataemia hypokalaemia hypomagnesaemia: may predispose to torsades de pointes abnormal fluid balance

Answer 326

Patients are considered high-risk if one or more of the following: BMI < 16 kg/m2 unintentional weight loss >15% over 3-6 months little nutritional intake > 10 days hypokalaemia, hypophosphataemia or hypomagnesaemia prior to feeding (unless high) or 2 or more of the following BMI < 18.5 kg/m2 unintentional weight loss > 10% over 3-6 months little nutritional intake > 5 days history of: alcohol abuse, drug therapy including insulin, chemotherapy, diuretics and antacids

Answer 327

NICE recommend that if a patient hasn't eaten for > 5 days, aim to re-feed at no more than 50% of requirements for the first 2 days.

Answer 328

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

Answer 329

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

Answer 330

methotrexate methyldopa amiodarone

Answer 331

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.

Answer 332

Features pruritus, often in the palms and soles no rash (although skin changes may be seen due to scratching) raised bilirubin

Answer 333

Management ursodeoxycholic acid is used for symptomatic relief weekly liver function tests women are typically induced at 37 weeks

Answer 334

Complications include an increased rate of stillbirth. It is not generally associated with increased maternal morbidity

Answer 335

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

Answer 336

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

Answer 337

Investigations ALT is typically elevated e.g. 500 u/l

Answer 338

Management support care once stabilised delivery is the definitive managemen

Answer 339

Budd-Chiari syndrome - a triad of abdominal pain, hepatomegaly and ascites. It is occlusion of the hepatic veins that drain the liver that causes a painful distension of the liver capsule and backflow portal hypertension - which will lead to the ascites.

Answer 340

ultrasound with Doppler flow studies is very sensitive and should be the initial radiological investigation

Answer 341

Charcot's triad (right upper quadrant pain, fever and jaundice), which is classically linked to ascending cholangitis.

Answer 342

Management intravenous cefotaxime is usually given Antibiotic prophylaxis should be given to patients with ascites if: patients who have had an episode of SBP patients with fluid protein <15 g/l and either Child-Pugh score of at least 9 or hepatorenal syndrome 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'

Answer 343

Diarrhoea + hypokalaemia → villous adenoma Villous adenomas are colonic polyps with the potential for malignant transformation. They characteristically secrete large amounts of mucous, potentially resulting in electrolyte disturbances. The vast majority are asymptomatic. Possible features: non-specific lower gastrointestinal symptoms secretory diarrhoea may occur microcytic anaemia hypokalaemia

Answer 344

Triad of: dysphagia (secondary to oesophageal webs) glossitis iron-deficiency anaemia Treatment includes iron supplementation and dilation of the webs

Answer 345

This patient has typical symptoms of haemochromatosis: lethargy arthralgia, with evidence of chrondrocalcinosis diabetes mellitus (polyuria and polydipsia)

Answer 346

• Travel should be avoided for 10 days following abdominal surgery • Laparoscopic surgery: after 24 hours • Colonoscopy: after 24 hours • Following the application of a plaster cast, the majority of airlines restrict flying for 24 hours on flights of less than 2 hours or 48 hours for longer flights

Answer 347

Reye's syndrome: is a severe, progressive encephalopathy affecting children that is accompanied by fatty infiltration of the liver, kidneys and pancreas. The aetiology of Reye's syndrome is not fully understood although there is a known association with aspirin use and a viral cause has been postulated.

Answer 348

The peak incidence is 2 years of age, features include: • May be history of preceding viral illness • Encephalopathy: confusion, seizures, cerebral oedema, coma • Fatty infiltration of the liver, kidneys and pancreas • Hypoglycaemia

Answer 349

Management is supportive Prognosis is poor - 30-40% mortality