Gastroenterology Flashcards

1
Q

What are the causes of bloody diarrhoea?

A

Vascular: Ischaemic colitis
Infective: Campy, shigella, salmonella, E. coli,
Inflamm: UC/Crohns
Neoplastic

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2
Q

What is the most concerning complication of C. dif diarrhoea, and what are its further complications?

A

Pseudomembranous colitis (abdo pain, bloody diarrhoea, fever)

May lead to paralytic ileus, toxic megacolon and multi organ failure

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3
Q

What are the causes of constipation?

A

OPENED IT

Obstruction (mechanical or pseudo obstruction (post op ileus))
Pain - fissure
Endocrine(T4)/Electrolytes (low Ca/K)
Neuro - MS, Cauda Equina
Elderly
Diet/Dehydration
IBS
Toxins - Opiates, anti mACh
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4
Q

Give two osmotic laxatives

A

Lactulose

MgSO4 (fast acting)

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5
Q

Give four stimulant laxatives

A

Bisacodyl
Senna
Docusate
Sodium picosulphate (rapid)

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6
Q

What are the ROME criteria and what are they used for?

A

Diagnostic criteria for IBS

Abdo discomfort for >12 weeks + 2 of:

Urgency
Tenesmus
Bloating
PR mucous
Worse after food
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7
Q

What are the ROME criteria exclusion criteria?

A
Weight loss
Bloody stool
Anorexia
>40 years old
Diarrhoea at night
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8
Q

What are the causes of dysphagia?

A

Inflammatory, mechanical, motility

Inflamm: Tonsil/oseophagitis, candidiasis, aphthous ulcers

Mechanical: May be:
Luminal: FB
Mural: Benign web, oseophagitis, malignancy, pharyngeal pouch
Extramural: Lung Ca, hiatus, LNs, retrosternal goitre

Motility: May be:
Local: Achalasia, oesophageal spasm, (pseudo)bulbar palsy
Systemic: CREST, MG

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9
Q

Dx Dysphagisa for solids and liquids at the start

A

Motility disorder, commonly achalasia

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10
Q

Dx Dysphagia solids>liquids at start

A

Stricture

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11
Q

Dx Dysphagia difficulty making swallowing movement

A

(Pseudo)bulbar palsy

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12
Q

Dx Odynophagia

A

Ca, ulcer, spasm

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13
Q

Dx intermittent dysphagia

A

Oesophageal spasm

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14
Q

Dx constant worsening dysphagia

A

Malignant stricture

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15
Q

Dx dysphagia with neck bulging/gurgling on drinking

A

Pharyngeal pouch

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16
Q

What investigations might you do for dysphagia/

A
Bloods - FBC, UnE
CXR
OGD
Barium swallow +- video fluoroscopy
Oesophageal manometry
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17
Q

What are some secondary causes of achalasia?

A

Oesophageal cancer

Chagas disease - Trypanosoma cruzii

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18
Q

What is the management of achalasia?

A

Medical: CCBs, nitrates
Interventional: Endoscopic ballooning, botulinum
Surgical: Heller’s cardiomyotomy

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19
Q

What is Killian’s dehiscence?

A

The defect in the oesophagus which causes a pharyngeal pouch

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20
Q

What are the alarm symptoms associated with dyspepsia?

A

ALARMS

Anorexia
Loss of weight
Anaemia
Recent onset progression
Melaena/haematemesis
Swallowing difficulty
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21
Q

What is the management of new onset dyspepsia?

A
OGD if >55 or ALARMS
Conservative measures for 4 weeks
H. pyori test if no improvement
If +ve then eradication therapy (PPI, Amox, Clari for 7 days)
If -ve then 4 week PPI trial
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22
Q

What are some distinguishing features between gastric and duodenal ulcers?

A

Gastric ulcer pain is worsened on eating, and may also cause weight loss

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23
Q

What are some potential complications of peptic ulcer disease?

A

Haemorrhage
Perforation
Gastric outflow obstruction
Malignancy

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24
Q

What is the management approach to PUD?

A

Conservative: Wt loss, stop smoking, less EtOH, dietary stuff, stop NSAIDs, OTC antacids

Medical: PPIs, H pylori eradication, H2 antagonists

Surgical: Vagotomy/antrectomy

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25
Q

What is the cellular progression from GORD to cancer?

A

GORD -> Barrets oesophagus (squamous metaplasia to columnar epithelium) -> dysplasia -> adenocarcinoma

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26
Q

What is the surgical management for GORD?

A

Nissen fundoplication

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27
Q

Which type of hiatus hernia is more common?

A

Sliding (80%)

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28
Q

What are the differentials for haematemesis?

A

VINTAGE

Varices
Inflammation - PUD
Neoplasia
Trauma - Mallory Weiss/Boerhaave's 
Angiodysplasia
Generalised bleeding diatheses
Epistaxis
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29
Q

Rectal bleeding differentials?

A

DRIPING Arse

Diverticule
Rectal - haemorrhoids
Infection - Campy, shigella, E coli, C dif
Polyps
Inflammation - IBD
Neoplasia
Gastric bleeding
Angio - Ischaemic colitis, HHT
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30
Q

What is the Rockall score?

A

Prediction of re-bleed and mortality following upper GI bleed

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31
Q

What are the causes of portal hypertension?

A

Pre hepatic - Portal vein thrombosis
Hepatic - Cirrhosis
Post hepatic - Budd Chiari, RHF, pericarditis

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32
Q

What is the management approach for an upper GI bleed?

A

Resuscitate - Head down, O2, IV access + fluids, bloods
Blood products if still shocked
Variceal management: Terlipressin IV + Ciprofloxacin
Maintenance: Fluids, CVP, correct coagulopathy, thiamine if EtOH
Endoscopy if severe bleed

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33
Q

What are the pre-hepatic causes of jaundice?

A

Haemolytic anaemia

Ineffective erythropoiesis - e.g. thalassaemia

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34
Q

What are the hepatic causes of jaundice?

A

Unconjugated:
Contrast agents, CCF, Hypothyroid, Gilberts, C-N synd

Conjugated:
Hepatocellular dysfunction -
Congenital - Wilsons, a1atd

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35
Q

What are the post hepatic causes of jaundice?

A
Stones
Pancreatic cancer
Drugs
PBC/PSC
Biliary atresia
Choledochal cyst
Cholangiocarcinoma
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36
Q

What are the iatrogenic causes of jaundice?

A

Haemolysis - Antimalarials
Hepatitis - Paracetamol, valproate, statins
Cholestasis - Fluclox, coamox, COCP

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37
Q

What are the causes of liver failure?

A
Cirrhosis
Infection - Hep/CMV/EBV
Toxins - EtOH, Paracetamol, Isoniazid
Vascular - Budd Chiari
Obstetric - Eclampsia, acute fatty liver
Others - Wilsons, AIH
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38
Q

What are the signs of liver failure?

A
Jaundice
Oedema/ascites
Bruising
Encephalopathy (asterixis)
Fetor hepaticus
Spider naevi
JVP
Caput medusae
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39
Q

What blood results would you find in liver failure?

A
FBC - Infection, microcytosis if EtOH
UnE - Low urea (hepatic synthesis) with raised Cr (hepatorenal syndrome)
LFTs:
AST:ALT >2 = EtOH
AST:ALT <1 = Viral
Hypoalbuminaemia (chronic)
Raised PT (acute)
Clotting - INR raised
ABG - metabolic acidosis
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40
Q

What is the pathophysiology of hepatorenal syndrome and when is it seen?

A

Seen in advanced chronic liver failure, where cirrhosis causes splanchic vessel dilatation ->RAS activation -> Renal artery vasoconstriction. Persistent underfilling of the renal circulation leads to renal failure

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41
Q

What are the complications and preventative measures for each - of liver failure?

A

Bleeding - Vit K, platelets, FFP, blood
Sepsis - Tazocin
Ascites - Fluid + salt restrict, spiro, furosemide, ascitic tap, daily weights
Hypoglycaemia - regular BMs
Encephalopathy - Monitor, avoid sedatives, lactulose, rifamixin
Seizures - Lorazepam
Cerebral oedema - Mannitol

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42
Q

How does rifamixin work for hepatic encephalopathy?

A

Reduces ammonia production by gut flora

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43
Q

What are the examination findings in cirrhosis?

A

Hands - Dupuytrens contractures, clubbing, Terrys nails (white), palmar erythema, leukonychia (white flecks)

Face - Palor, Xanthelesma, parotid enlargement (EtOH)

Trunk - Spider naevi, gynaecomastia

Abdo - Striae, caput medisae, hepSplenomegaly, testicular atrophy

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44
Q

What are some possible complications of cirrhosis?

A

Decompensation - jaundice, encephalopathy, hypoalbuminaemia, coagulopathy, hypoglycaemia

Spontaneous bacterial peritonitis (ascitic fluid infection)

Portal hypertension - SAVE
Splenomegaly
Ascites
Varices
Encephalopathy

HCC

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45
Q

What investigations might you do to find the SPECIFIC cause of cirrhosis?

A
EtOH - FBC, LFT
NASH - hyperlipidaemia, hyperglycaemia
Infection - serology
Genetic - ferritin, a1at, caeruloplasmin (low in Wilson's)
AIH - SMA, ANA
PBC - AMA
PSC - ANCA, ANA
Cancer - alpha-fetoprotein
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46
Q

What specific management options are available for cirrhosis caused by:
HCV
PBC
Wilson’s

A

Interferon-alpha
Ursodeoxycholic acid
Penicillamine

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47
Q

What are the components of the Child Pugh score and what is it used for?

A

Used to assess prognosis in cirrhosis

Bilirubin
Albumin
INR
Ascites
Encephalopathy
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48
Q

What are the features of hepatic encephalopathy?

A

ACDCS

Asterixis, Ataxia
Confusion
Dysarthria
Constructional apraxia
Seizures
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49
Q

How would you differentiate ascites due to portal HTN from other causes?

A

Serum Ascites Albumin Gradient

> 1.1 = Portal HTN
< 1.1 = Other causes 
Neoplasia
Inflammation - pancreatitis
Nephrotic syndrome
Infection - TB peritonitis
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50
Q

What are some complications of chronic alcohol use?

A

Cirrhosis
GI - gastritis, PUD, varices, pancreatitis, carcinoma
Neuro - Wernickes, Korsakoffs, peripheral neuropathy
Cardio - arrhythmias, DCM, HTN
Blood - Microcytosis, folate def

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51
Q

What is the role of each medical management of alcoholism?

A

Baclofen - reduces cravings
Disulfaram - Hangover inducer
Acamprosate - reduces cravings

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52
Q

What are the features of the three phases of viral hepatitis?

A

Prodrome, Icteric, Chronic

Prodrome: Flu, malaise, arthralgia

Icteric - Acute jaundice, abdo pain, hepmegaly, cholestatic picture, rash

Chronic - Cirrhosis w. HCC risk

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53
Q

Which viral hepatitis has the poorest long term prognosis in terms of complications?

A

Hep C - 80% develop chronic disease and 20% get cirrhosis

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54
Q

What is the relationship between NASH and NAFLD?

A

NASH is the severe form of NASH

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55
Q

How might NAFLD present?

A

Commonly silent
Hepmegaly + RUQ pain
Metabolic syndrome (as it is a risk factor)

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56
Q

What are the causes of Budd Chiari syndrome?

A

Hypercoagulable states - myeloproliferative disorders, paroxysmal nocturnal haemoglobinuria, anti-phospholipid, OCP

Local tumour - commonly HCC

Congenital

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57
Q

What is the presentation of BC syndrome?

A

RUQ pain
Hepmegaly
Ascites (SAAG >1.1)
Jaundice

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58
Q

What is the pathophysiology of hereditary haemochromatosis?

A

Increased iron absorption leads to deposition in multiple organs

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59
Q

What are the features of hereditary haemochromatosis?

A

MEALS

Myocardial - DCM, arrhythmia
Endocrine - DM, hypogonadism, hypocalcaemia
Arthritis - big and small
Liver - CLD/cirrhosis/HCC, hepmegaly
Skin - slate grey
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60
Q

What are the blood findings in hereditary haemochromatosis (HH)?

A
Deranged LFTs
Raised ferritin
Raised Iron
Reduced TIBC
Hyperglycaemia
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61
Q

What is the management of HH

A

1st line:Venesection (aim Hct < 0.5)
2nd: Desferroxamine

General - Monitor DM, low Fe diet, screening

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62
Q

What are the two main features of A1At deficiency?

A

Cirrhosis

Emphysema

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63
Q

What are the features of Wilson’s disease?

A

CLANK

Cornea - Kayser Fleischer
Liver - Cirrhosis/fluminant necrosis
Arthritis
Neuro - Parkinsonism, psych, cerebellar syndrome
Kidney - Fanconi syndrome
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64
Q

What investigations would you do for Wilson’s disease and what are the results?

A

Blood - low Cu, low caeruloplasmin (acute phase reactant)
Raised 24hr urinary Cu
Raised hepatic Cu on biopsy

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65
Q

What (and when) is the presentation of autoimmune hepatitis?

A
Mostly teens and early twenties - 
Constitutional - fever, malaise, fatigue
Cushingoid - hirsute, acne, striae
Hepatitis
HSM
Polyarthritis

May also resent post-menopausally but more insidiously

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66
Q

What is the management and prognosis of AIH?

A

Pred + Azathioprine

Remission in 80% of patients

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67
Q

What is the difference between the pathophysiology of PSC and PBC?

A

PBC is intrahepatic bile duct destruction only, while PSC also affects extrahepatic ducts

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68
Q

True or false, PBC is more common in men and PSC more-so in women

A

False - PBC is 9x more common in women, while PSC is more commonly seen in men

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69
Q

What are the presenting features of PBC?

A

PPBBCCS

Pruritis
Pigmentation (facial)
Bones - osteomalacia+porosis
Big organs - HSM
Cirrhosis + coagulopathy
Cholesterol - Xanthelasma +xanthomata
Steatorrhoea
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70
Q

What are the features of PSC?

A

Obstructive jaundice, pruritis, abdo pain, HSM, Cholangiocarcinoma (^Risk)

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71
Q

Which is UC associated with? PSC or PBC

A

PSC - 3% of UC have PSC but 80% of PSC have UC

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72
Q

Which disorders are associated with PBC?

A

Thyroid disease
CTDs
Coeliac

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73
Q

Which antibodies are seen in PBC and PSC?

A

PBC - AMA (98%)

PSC - pANCA (80%)

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74
Q

What is the management of PBC and PSC?

A

Symptomatic:
Pruritis - colestyramine, naltrexone
Diarrhoea - Codeine
Osteoporosis (PBC) - Bisphosphonates

Specific:
ADEK vitamins
Ursodeoxycholic acid
Abx for PSC cholangitis

Liver transplant

Screening PSC - Cholangiocarcinoma (USS and Ca19-9) and Colorectal

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75
Q

What is the prognosis of PBC?

A

Once jaundice develops, survival is 2 years

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76
Q

What are the ERCP findings in PSC?

A

Beading

77
Q

What are the differences between PSC and PBC on liver biopsy?

A

PBC- non-caseating granulomatous inflammation

PSC- Fibrous, obliterative cholangitis

78
Q

What are the main LFT changes in PBC?

A

massive ALP, Massive GGT, also raised AST/ALT

79
Q

True or false; HCC is the commonest liver tumour.

A

False - Metastases are the commonest liver tumours. HCC is the commonest primary liver cancer

80
Q

What are the features of a liver cancer?

A
Commonly silent
Irregular hepatomegaly
Signs of CLD
Jaundice is a late sign
RUQ pain
81
Q

WHat are the causes of HCC?

A

Cirrhosis - EtOH, HH, PBC
Viral Hep
Aflatoxins

82
Q

Cholangiocarcinoma is a tumour of what structure?

A

Biliary tree

83
Q

What are some possible complications following liver transplant?

A
Acute rejection (50%)
Sepsis
Hepatic artery thrombosis
CMV infection
Chronic rejection
Recurrence
84
Q

Compare the epidemiology of UC and Crohn’s

A

UC more common
Crohns presents earlier
Smoking is protective for UC but a risk factor for Crohns

85
Q

Compare the pathology of Crohn’s and UC

A

Location - Crohns affects mouth to anus, esp the terminal ileum, while UC affects up to the caecum

Distribution - Crohns has skip lesions, UC is contiguous

Thickness - Crohns is transmural, UC is mucosal

Strictures - Crohn’s causes strictures, UC does not

86
Q

Which more commonly causes weight loss, Crohns or UC?

A

Crohns

87
Q

Which is more strongly associated with PR bleeding, Crohns or UC?

A

UC

88
Q

What are the GI features of Crohns?

A
Aphthous ulcers
Glossitis
Abdo tenderness
RIF mass
Perianal abscesses, fistulae, tags
Anal/rectal strictures
89
Q

What are the extra-GI features IBD?

A

Skin:
Clubbing
Erythema nodosum
Pyoderma gangrenosum (UC)

Eyes:
Ant uveitis
Episcleritis
Conjunctivitis

Joints:
Asymmetrical arthritis
Sacroilliitis
Ankylosing spondylitis

Hepatobiliary:
PSC + Cholangiocarcinoma (UC only)
Gallstones (esp Crohns)
Fatty liver

Other:
Amyloidosis
Oxalate renal calculi (Crohns)

90
Q

What complications are associated with UC?

A
Toxic megacolon (>6cm)
Bleeding 
Malignancy - colorectal and cholangiocarcinoma
Strictures -> obstruction
Venous thrombosis
91
Q

Which complications are associated with Crohn’s disease/

A
Fistulae
Strictures -> obstruction
Abscesses
Malabsorption:
fat ->steatorrhoea, gallstones
B12 -> anaemia
VitD -> Osteomalacia
protein -> oedema
92
Q

How would you investigate IBD and what would you find?

A

Bloods - FBC ( low hb high wcc), LFT (low alb), B12, CRP/ESR, cultures

Stool - MCnS (exclusion), C. dif toxin

Imaging - C+Axr (perforation + megacolon/wall thickening), CT, gastrograffin

93
Q

Which radiological features would you see in UC?

A

Lead piping
Thumbprinting
Pseudopolyps

94
Q

Which radialogical featuers would you see in Crohn’?

A

Skip lesions
Rose thorn ulcers
Cobblestoning
String sign of Kantor - narrowed terminal ileum

95
Q

What are the Truelove and Witts criteria/

A

Used to determine UC severity based on:

Bowel motions/day
PR bleed 
Temp
HR
Hb
ESR
96
Q

What is the management approach for an acute severe flare of UC?

A

Resus - NBM, IV fluids
Hydrocortisone IV
LMWH
Monitor - bloods, stools, AXR

97
Q

What are the guidelines for remission induction in UC?

A

1st: 5ASAs e.g. Sulfa/Mesalazine
2nd: Prednisolone

98
Q

What are the guidelines for remission maintenance in UC?

A

1st: Sulfa/Mesalazine
2nd: Azathioprine or 6-Mercaptopurine
3rd: Infliximab/Adalimumab

99
Q

What are the complications following surgery for UC?

A

Abdo - SBO, stricture, abscess, bleeding
Stoma - retraction, stenosis, prolapse, dermatitis
Pouch - pouchitis, leakage

100
Q

Metronidazole is recommended for flare up of which form of IBD?

A

Crohn’s

101
Q

What is the management for induction of remission in Crohns?

A

Supportive - high fibre diet, vitamin supplements

1st: Budesonide, Sulfasalazine
2nd: tapering Pred
3rd: Methotrexate
4th: inflix/Adalimumab

102
Q

What is the management for maintenance of remission in Crohns?

A

1st: Azathioprine, 6-Mercaptopurine
2nd: Methotrexate
3rd: Infliximab/Adalimumab

103
Q

What are the complications of bowel resection for Crohn’s?

A
As for UC
\+
Short gut - 
Steatorrhoea
ADEK + B12 malabsorption
Bile acide dep -> gallstones
Hyperoxaluria -> renal stones
104
Q

What are the features of Coeliac disease?

A

GLIAD

GI Malabsorption - carbs, fats, proteins, folate, iron, vitamins

Lyphoma and carcinoma - EATL, SB adenocarcinoma

Immune assocs - IgA def, T1DM, PBC

Anaemia - +hyposplenism

Det, - Dermatitis herpetiformis (symmetrical vesicles on extensor surfaces, v itchy), aphthous ulcers

105
Q

Which is the most specific antibody to test in Coeliac disease?

A

Anti-endomysial IgA

106
Q

What are the common causes of malabsorption in the UK?

A

Coeliac
Crohns
Chronic pancreatitis

107
Q

What are the risk factors for pancreatic cancer?

A

DINES

DM
Inflamm - chronic pancreatitis
Nutrition - high fat diet
EtOH
Smoking
108
Q

What is the most common type of cancer affecting the pancreas, and where is it usually found?

A

Adenocarcinoma, 60% in the head

109
Q

How might a cancer of the pancreatic body/tail present differently to one of the head/

A

Head cancers are the classic painless obstructive jaundice, while body/tail cancers cause epigastric pain which radiates to the back and is alleviated on sitting forward.

Both typically present in males over 60 with anorexia and weight loss

110
Q

How would you investigate a >Panc CA and what might you find?

A

Bloods - LFTs (cholestatic), Ca19, hypercalcaemia
USS - Pancreatic mass, dilated ducts +- hepatic mets
ERCP

111
Q

What is the management of pancreatic cancer?

A

If fit, with no mets and a small tumour - can perform Whipples pancreaticoduodenectomy

Otherwise palliate

112
Q

What is the commonest cause of chronic pancreatitis/

A

Alcohol

113
Q

How woudl you investigate and what would you find in chronic pancreatitis?

A

Bloods - hyperglycaemia
Reduced faecal elastase
USS - pseudocyst
AXR/CT - speckles pancreatic calcifications

114
Q

What are the complications of chronic pancreatitis?

A

DM
Pancreatic Ca
Biliary obstruction
Splenic vein thrombosis leading to splenomegaly

115
Q

What is the cell origin of carcinoid tumours, and what might they secrete/

A

Enterochromaffin cells

5-HT
VIP
Gastrin
Glucagon
Insulin
ACTH
116
Q

Which syndrome are carcinoid tumours assocaited with?

A

MEN1

117
Q

Where are carcinoid tumours commonly found?

A

Appendix
Ileum
Colorectum

118
Q

What are the clinical featuers of Carcinoid syndrome?

A

FIVE HT

Flushing - paroxysmal
Intestinal - diarrhoea
Valve fibrosis - TrRe, PuSt
E - Wheeze
Hepatic involvement
Tryptophan deficiency (B3 pellagra) - Diarrhoea, dermatitis, dementia
119
Q

How would you investigate a ?carcinoid tumour?

A

Urinary 5-HIAA
Plasma Chromogranin A
CT to find primary

120
Q

How would you manage Carcinoid syndrome?

A

Symptoms - octreotide, loperamide

Curative - resection

121
Q

What are the features of Vitamin A deficiency?

A

Dry conjunctivae
Corneal ulcerations
Night blindness

122
Q

What are the features of Vitamin B1 deficiency?

A

Wet: HF + oedema
Dry: Polyneuropathy
Wernickes/Korsakoffs

123
Q

What are the features of Vitamin B3 deficiency?

A

Pellagra -3Ds

Dementia
Dermatitis
Diarrhoea

124
Q

What are the features of Vitamin B6 deficiency?

A

Peripheral sensory neuropathy

125
Q

What are the features of Vitamin B12 deficiency?

A

Megaloblastic anaemia
Subacute combined degeneration of the cord (motor and sensory)
Peripheral sensory neuropathy
Glossitis

126
Q

What are the features of Vitamin C deficiency?

A

Scurvy

Gingivitis
Bleeding everywhere
Myalgia
Oedema

127
Q

Which factors are depleted in vitamin K deficiency?

A

2,7,9,10,C and S

128
Q

What are some possible examination finding indicating immunosuppression?

A

Cushingoid
Skin tumours (AKs, SCCs in particular)
Gingival hypertrophy 2 to ciclosporin

129
Q

What are the possible differentials for a Mercedes Benz scar/

A

Liver transplant
Segmental resection
Whipples pancreaticoduodenectomy

130
Q

Which immunosuppressants are commonly given following liver transplant?

A

Tacrolimus
Ciclosporin
Azathioprine
Prednisolone (withdraw after 3 months)

131
Q

What are the common and rare causes of hepatomegaly?

A

Common: Hepatitis, CLD, HF

Rarer:
Malignancy
Anatomical variations
Budd Chiari
Myeloproliferative disorders
Sarcoid/Amyloidosis
132
Q

How might you investigate an individual with hepatomegaly?

A

Bloods - FBC (anaemia, lymphocytosis), UnE (CCF -> renal impairment), LFTs, clotting, liver screen

Urine dip - Urobilinogen, proteinuria

Imaging - USS for anatomy, CT for cancer

Biopsy

133
Q

What are the causes of splenomegaly?

A
Myelo/lymphoproliferative disease
CLD
Infective endocarditis
Feltys syndrome
Malaria
Sarcoid
Amyloid
RA/SLE/Sjogrens
134
Q

What is Felty’s syndrome?

A

A triad of :

Rheumatoid arthritis
Splenomegaly
Neutropaenia

Characterised by repeated infections

135
Q

What symptoms might a patient with splenomegaly report?

A

Haem - fatigue, bruising, infections, bone pain
CLD - Viral exposure, FHx
Inflammatory arthritis

136
Q

What blood results might point you to an underlying diagnosis in splenomegaly?

A

CML - Massive WCC (PMNs)
Myelofibrosis - pancytopaenia
CLL - Lymphocytosis
Haemolysis - Microcytic anaemia

137
Q

What blood film results might point you to an underlying diagnosis in splenomegaly?

A

Myelofibrosis - Leukoerythroblastic teardrop poikiloctytes
CLL - Smear cells
Haemolysis - Smear cells, reticulocytosis
Malignancy - Uraemia
Malaria - thick and thin films

138
Q

What genetic mutations are seen in specific causes of splenomegaly?

A

CML - Philidelphia chromosome t(9;22) leading to BCR-ABL formation

Myelofibrosis - 50% are JAK2 pos

139
Q

What are the causes of massive splenomegaly

A
CML
Malaria
Myelofibrosis
Infectious Mononucleosis
Gaucher disease (lipid storage disorder)
140
Q

What are the features of CML?

A

Constitutional - Fever, lethargy, weight loss, night sweats
Massive HSM
Platelet dysfunction
Gout

141
Q

What is the pathophysiology of primary myelofibrosis?

A

Clonal proliferation of megakaryocytes results in extramedullary haematopoiesis in the liver and spleen due to resultant myelofibrosis

142
Q

What are the featuers of myelofibrosis?

A

Pancytopaenia
Constitutional
Massive HSM

143
Q

What are the common causes of hyposplenism?

A

Splenectomy
Coeliac
IBD
Sickle Cell disease

144
Q

What must be done to manage a splenectomy patient?

A

Immunisations - pneumovax, HiB, MenC, Flu
Daily Abs - Pen V
Alert card/bracelet

145
Q

What are the possible indications for splenectomy?

A

Trauma
Rupture (2 to EBV for exampls)
AIHA
ITP

146
Q

What are some complications of splenectomy?

A
Redistributive thrombocytosis
Gastric dilatation  - transient ileus
Left lower lobe atelectesis
Pancreatic ischaemia
bleeding
Infections (esp encapsulates)
147
Q

What are the important features of examining a patient with enlarged kidneys?

A

Inspection -
Nephrectomy scar, Rutherford Morrison scar, Tenchkhoff catheter/scar

Palpation - 
Kidneys
Liver
Spleen
Renal transplant (groin

Auscultation -
Renal bruits

Completion -
External genitalia
Urine dip
CV exam (MV prolapse)

148
Q

What might cause bilateral renal enlargement/

A

ADPKD
Bilateral RCC
Bilateral cysts - E.g. VHL
Amyloidosis

149
Q

What might cause unilateral renal enlargement?

A

Simple renal cysts
RCC
Compensatory hpertrophy
+ contralateral nephrectomy - ADPKD

150
Q

What are the features of ADPKD?

A
30-50yos
HTN
Recurrent UTIs
Loin pain
Haematuria
Hepatomegaly (hepatic cysts)
Berry aneurysms
MV prolapse (MS click w late systolic murmur)
151
Q

What investigations might point to the cause of renal enlargement?

A

Urine - Dip, cytology

Bloods -
Anaemia 2ary to ESRF
UnE
Renal osteodystrophy

Imaging; Abdo USS, CTMRI may show berry aneurysms

152
Q

What is the management of ADPKD?

A

General - lots of water, salt and caffeine restrict
Monitor BP and UnE
Genetic counselling
MRI screening

Medical - Aggressive HTN management (ACEi best), treat infections

Surgical - Nephrectomy

153
Q

What are the features of ARPKD?

A

Much rarer than AD form
Presents perinataly - oligohydramnios, HTN, CRF
Congenital hepatic fibrosis -> Portal HTN

154
Q

What are the features of a simple renal cyst?

A

Common - 1/3 of over 60s have one
May present as renal mass and haematuria
Solid components only
Main differential is RCC

155
Q

What are the renal manifestations of Tuberous Sclerosis?

A

Cysts, angiomyolipomas

156
Q

What are the risk factors for RCC?

A
Obesity
smoking
HTN
dialysis
Heritable - VHL
157
Q

What is the classic presenting triad of RCC?

A

Loin pain
Haematuria
Loin mass

158
Q

What paraneoplastic features may present as a result of RCC?

A
EPO - polycythaemia
PTHrP - hypercalcaemia
Renin - HTN
ACTH - Cushings syndrome
Amyloidosis
159
Q

What are the featuers of Von Hippel Lindau syndrome?

A
Bilateral renal cell carcinoma
Renal and pancreatic cysts
Cerebellar haemangioblastomas
Phaeochromocytoma
Islet cell tumours
160
Q

Where might you find a Rutherford Morrison scar and what does it indicate/

A

RIF

Renal transplant

161
Q

What are the commonest indications for renal transplant?

A

Diabetic nephropathy
Glomerulonephritis
Polycystic kidney disease
Hypertensive nephropathy

162
Q

What is the pathophysiology and presentation of hyperacute renal transplant rejection?

A

Path - ABO incompatability

Pres - Thrombosis and SIRS

163
Q

What is the pathophysiology and presentation of acute renal transplant rejection?

A

Path - Cell mediated
Pres - Fever, pain, low urine output, raised creatinine
Responsive to immunosuppression

164
Q

What is the pathophysiology and presentation of chronic renal transplant rejection?

A

Path - Interstitial fibrosis with tubular atrophy
Pres - Gradual rise in Cr and proteinuria
Not responsive to immunosuppression

165
Q

What are the common side effects of commonly used adjunctive immunosuppresants?

A

Ciclosporin - Nephrotoxic, gingival hypertrophy, hypertrichosis, hepatic dysfunction

Tacrolimus - Nephrotoxic, diabetogenic, cardiomyopathy, neurotoxic

Steroids - Cushings

166
Q

When would you consider commencing renal replacement therapy?

A

When GFR falls below 15 and the patient is symptomatic

167
Q

What are the complications of dialysis?

A
20% annual mortality
Cardiovascular disease
Malnutrition
Infections (2ary to uraemia)
Anmloidosis
Renal cysts -> RCC
168
Q

Compare the mechanism of each of the different types of renal replacement therapy

A

Haemodialysis - blood and dialysate flow in opposite directions separated by semipermeable membrane. Solution transfer by diffusion

Haemofiltration - Blood filtered under hydrostatic pressure - less haemodynamic instability

Peritoneal dialysis - Dialysate introduced into peritoneal cavity by Tenchkoff catheter with diffusion occuring across the peritoneum

169
Q

What are the complications of haemodialysis?

A

Disequibrilation syndrome - usually only on first go, rapid osmolarity change causes cerebral oedema
Fluid imbalance
Electrolyte imbalance
Aluminium toxicity -> dementia

170
Q

What are the complications of peritoneal dialysis/

A
Peritonitis
Infection
Catheter malfunction
Obesity (glucose in dialysate)
Hernias and back pain
171
Q

How would you examine an AV fistula/

A

Inspection
Swelling with scar over distal forearm/elbow
Needle marks
Evidence of infection

Palpation
Assess pain, tempearture, thrill

Auscultate for bruits

172
Q

What are the advantages and disadvantages of an AV fistula?

A

Ad: high flow rates, low recirculation, low infection rate, low stenosis rates

Dis: 6 weeks to arterialise, body image, must take care

173
Q

What are the possible complications of an AV fistula?

A
Thrombosis
Stenosis
Infection
Bleeding
Aneurysm
Steal syndrome - Distal tissue ischaemia which may necrose - Rx with banding
174
Q

Where does a tunnelled cuffed catheter typically sit?

A

Internal jugular vein

175
Q

What are the complications of a tunnelled cuffed catheter?

A

Insertion - e.g. pneumothorax
Infection
Blockage
Retraction

176
Q

What are the two commonest causes of CKD?

A

DM

HTN

177
Q

What are some other causes of CKD?

A
Renal artery stenosis
Glomerulonephritis
CKD
PKD
Drugs
Pyelonephritis
Myeloma
178
Q

What protein:creatinine may indicate CKD?

A

> 300

179
Q

What investigations might you do for CKD/

A

Urine
Dip - protein, haemat, glucosuria
PCR - >300 indictes nephrotic syndrome
Bence jones proteins - MM

Bloods
Anaemia
Low eGFR
Bone profile
DM bloods
ESR
Antibodies
Viral serology
Imaging
CXR - pulmonary oedema
Renal USS
Bone Xray - renal osteodystrophy
CT KUB - cortical scarring from pyelonephritis
180
Q

What are the complications of CKD?

A

CRF HEALS

Cardiovascular disease
Renal osteodystrophy
Fluid (oedema)
HTN
Electrolyte disturbances
Anaemia
Leg restlessness
Sensory neuropathy
181
Q

What are the features of renal osteodystrophy?

A
Osteoporosis
Osteomalacia
2/3ry hyperparathyroidism
Osteosclerosis
Band keratopathy (corneal calcifications)
182
Q

What is the mechanism of development of renal osteodystrophy

A

Renal impairment causes reduction in 1alpha hydroxylase -> less vit D activation -> low Ca -> high PTH -> Phosphate retention further increases PTH -> osteoclast and osteoblast activation and acidosis which also contributes to bone resorption

183
Q

What is the management approach to CKD?

A
General
Treat reversible causes
Stop nephrotoxic
Electrolyte and fluid restriction
Optimise CV risk

Specifics
HTN - If DM give ACEi/ARB
Oedema - furosemide
Bone disease - Phosphate binders, Vit D analogues, Ca
Anaemia - Exclude other causes and give Epo
Restless legs - Clonazepam

184
Q

How does diabetes lead to CKD?

A

Hyperglycaemia leads to renal hypertrophy and ROS production -> glomerulosclerosis and nephron loss
Nephron loss results in RAS activation and thus hypertension

185
Q

Which screening programme aims to prevent T2DMs from developing CKD?

A

Microalbuminuria

186
Q

What are the causes of renal artery stenosis?

A

Atherosclerosis (80%)
Fibromuscular dysplasia
Thromboembolism
External compression

187
Q

How might renal artery stenosis present?

A
Refractory hypertension
Renal artery bruits
Woresning renal function in response to ACEi/ARBs
Flasu pulmonary oedema
Other PVD signs
188
Q

Which medications must be avoided in renal artery stenosis?

A

ACEi/ARBs