GASTROENTEROLOGY Flashcards
1
Q
What is Acute Pancreatitis?
A
Leakage of pancreatic enzymes that auto digest the pancreas
2
Q
What are the causes of acute pancreatitis?
A
I GET SMASHED
Idiopathic Gallstones Ethanol Trauma Steroids Mumps virus (also Coxsackie B) AI (e.g. polyarteritis nodosa) Scorpion stings Hypertryglyceridaemia/Hypercalcaemia/Hypothermia ERCP Drugs Azothiprine Mesalazine Bendroflumethiazide Furosemide Valproate GLP-1 agonists: -tides
OR
PANCREATITIS
Posterior peptic ulcer rupture Alcohol Neoplasm Cholesthiasis, cholecystectomy, increased calcium Renal disease ERCP Anorexia Toxins Incineration Trauma Infections Scorpion stings
3
Q
What are the symptoms of Acute Pancreatitis?
A
Severe epigastric pain: Steady Boring Radiates to back Relieved by sitting forward Nausea and vomiting Weakness Low-grade fever Shock due to loss of fluid in peripancreatic third space
4
Q
What are the signs of Acute Pancreatitis?
A
Low grade fever
Tachycardia
Hypovolaemia due to oedema and fluid shifting-shock
Abdominal tenderness (no guarding or rebound)
Diminished sounds from a localised ileus
Haemorrhage:
Grey turner’s: bleeding into the peritoneum
Cullen’s: methemalbumin formed from digested blood tracks around the abdomen from the inflamed pancreas
Jaundice is rare
Can rarely cause ischaemic (Purtscher) retinopathy causing temporary of permanent blindness
5
Q
What is the sequalae of pancreatitis?
A
- Pancreatic fluid collections
- Psuedocysts
- Pancreatic abscess
- Pancreatic necrosis
- Haemorrhage
6
Q
What are pancreatic fluid collections?
A
Located on or near the pancreas and lack a wall of granulation or fibrous tissue
May resolve or develop into pseudocysts or abscesses
Aspiration should be avoided in case of infection
7
Q
What are pancreatic psuedocysts?
A
Result from organisation of peripancreatic fluid collection, may or may not communicate with ductal system
Usually retrogastric
Walled by fibrous or granulation tissue
Within 4 weeks or more
8
Q
What are pancreatic abscesses?
A
Intra abdominal collection of pus in the absence of necrosis
Result of an infected pseudocyst
9
Q
What is pancreatic necrosis?
A
May involve parenchyma and surrounding fat
Complications directly linked to the extent
May degrade into vasculatur and cause haemorrhage
10
Q
What are the investigations for Acute Pancreatitis?
A
Elevated (3x normal range or >1000u/ml) SERUM AMYLASE during first 24 hours
Initially increases 2-6 hours after onset of pain, does not correlate to disease severity
Elevated LIPASE is more specific and sensitive
Abdominal X-RAY
SENTINEL loop: isolated, dilated loop of bowel caused by irritation of the adjacent bowel
COLON CUTOFF: gaseous distention in proximal colon due to narrowing at the splenic fracture
Chest x-ray: left sided exudative pleural effusion
CT and USS: Peripancreatic fluid
Pancreatic calcifications
11
Q
What is are the severity scores for Acute Pancreatitis?
A
Glasgow score:
PaO2: <8kPa Age: >55yr Nuetrophilia: >15x10’/L Calcium (hypocalcaemia): <2mmol/L Renal function: Urea >16 mmol/L Enzymes: LDH >600, AST >200 Albumin: <32g/L Sugar: Blood glucose >10mmol/L
12
Q
How is Acute Pancreatitis managed?
A
IV fluids Bowel rest ?Parentral nutrition NG decompression Abx (controversial) Analgesia Surgical debridement if peripancreatic fluid Surgery/ERCP for gallstones If infected necrosis Radiological drainage Surgical necrosectomy
13
Q
What are the complications of acute pancreatitis?
A
Pulmonary complications:
- Pleural effusions
- Atelectasis
- Mediastinal abscess
- ARDS
Psuedocyst: circumscribed collection of fluid rich in pancreatic enzymes, blood and necrotic tissue located in the lesser sac of the abdomen.
Chronic pancreatitis
Splenic vein thrombosis
Pancreatic cancer if multiple episodes
14
Q
What is Chronic Pancreatitis?
A
Chronic inflammation of the pancreas affecting endocrine and exocrine function
15
Q
What causes Chronic Pancreatitis?
A
80% is alcoholism
16
Q
What are the symptoms of Chronic Pancreatitis?
A
- Recurrent epigastric pain
- Constipation
- Steatorrhoea (symptoms of pancreatic insufficiency usually develop 5-25 years after pain)
- Flatulence
- Weight loss
- Vitamins ADEK deficiency
17
Q
What are the investigations for Chronic Pancreatitis?
A
Mildly elevated amylase and lipase (although can be normal) STOOL ELASTASE (<200mcg/g) Serum TRYPSINOGEN (<20ng/mL) Glycosuria Abdominal X-RAY MRCP or endoscopic USS CT for pancreatic calcification
18
Q
How is Chronic Pancreatitis managed?
A
Alcohol cessation Low fat diet Oral pancreatic enzymes: Lipase before, during and after meals PPIs Vitamins ADEK and B12 if alcoholic Analgesia Steroids if AI
19
Q
What are the complications of Chronic Pancreatitis?
A
Diabetes mellitus-usually after >20 years
Analgesia addiction
Exocrine and endocrine insufficiency
Ductal obstruction
Pseudocyst
Increased risk of pancreatic cancer
Gastric varices
20
Q
What is the MC Pancreatic Cancer?
A
85% are pancreatic adenocarcinoma
21
Q
What are the causes of Pancreatic Cancer?
A
Age Smoking Diabetes Chronic pancreatitis (not alcohol) Hereditary non-polyposis colorectal carcinoma MEN BRCA 2 gene
22
Q
What are the symptoms of Pancreatic Cancer?
A
Anorexia
Weight loss
VOMITING AFTER MEALS due to GASTRIC OUTLET OBSTRUCTION
Epigastric PAIN and atypical back pain-body and tail tumours
STEATORRHOEA (loss of exocrine function)
23
Q
What are the signs of Pancreatic Cancer?
A
Painless jaundice-Head of pancreas tumours TROUSSEAU'S sign: migratory thrombophlebitis (vessel inflammation due to blood clot) Diabetes Palpable gallbladder Epigastric mass Hepatosplenomegaly Lymphadenopathy Ascites
24
Q
What are the investigations for Pancreatic Cancer?
A
USS
High-res CT
show pancreatic pass with dilated biliary tree and hepatic metastases
25
How is Pancreatic Cancer managed?
<20% are suitable for surgery at diagnosis
Whipple’s resection: PANCREATICODUODENECTOMY is performed for resectable lesions in the head of the pancreas
Adjuvant chemotherapy
ERCP with stent palliative
26
What is an Insulinoma?
90% are benign pancreatic B islet cell tumours
27
What causes Insulinomas?
Sporadic
| MEN-1
28
What are the signs of Insulinomas?
Whipple’s triad:
- Symptoms associated with fasting or exercise
- Recorded hypoglycaemia with symptoms
- Symptoms relieved by glucose
29
What are the investigations for Insulinomas?
Screening test: HYPOGLYCAEMIA and INCREASED PLASMA INSULIN
Suppressive tests: give INSULIN and measure C-PEPTIDE, NO SUPPRESSION of c-peptide from exogenous insulin
CT/MRI + endoscopic USS
30
How are Insulinomas managed?
Excision
31
What are Glucagonomas?
Rare tumour of alpha cells of the pancreas, usually CANCEROUS and METASTATIC
32
What causes Glucagonomas?
Sporadic
| MEN-1
33
What are the signs of Glucagonomas?
- Overproduction of glucagon leading to INCREASED BLOOD GLUCOSE levels including gluconeogenesis and lipolysis
- NECROLYTIC MIGRATORY ERYTHEMA (NME) is presenting problem in 70% of cases:
Erythmatous blisters and swelling across friction areas (e.g. abdomen, buttocks, perineum and groin).
- DIABETES MELLITUS
34
How are Glucagonomas managed?
Treat octerotide, somatostatin analog which inhibits glucagon release
Doxorubicin and streptozotocin (chemotherapies) selectively damage alpha cells
Surgical resection
35
What is Zollinger-Ellison syndrome?
Excessive levels of GASTRIN resulting from a gastrin secreting ADENOMA (GASTRINOMA) of the pancreas (or duodenum)
60% are malignant
36
What causes Zollinger-Ellison?
MEN-1
| Sporadic
37
What are the signs of Zollinger-Ellison Syndrome?
Multiple gastroducodenal ulcers
- Abdominal pain
- Dyspepsia
Chronic diarrhoea due to inactivation of pancreatic enzymes
Malabsorption
38
What are the investigations for Zollinger-Ellison?
Fasting gastric levels
| Secretin stimulation test
39
How is Zollinger-Ellison managed?
High-dose PPIs e.g. Omeprazole 60mg/d
Octreotide: synthetic somatostatin
40
What is Diverticulosis?
HERNIATION of colonic MUCOSA through the muscle wall at a TAENIA COLI (where vessels pierce the muscle to supply the mucosa) often due to LACK OF DIETRY FIBRE
95% are in the sigmoid colon and the rectum is spare as it doesn't have Taenia Coli
41
What is Diverticular disease?
Symptomatic diverticulosis
- Colicky L sided pain
- Change in bowel habits
- Bleeding
treat with high fibre
42
What is Diverticulitis?
When a diverticular becomes inflamed and infected
- LIF pain and tenderness
- Anorexia
- N+V+D+Flatulence
- Infection: pyrexia, raised CRP and WCC
43
How is Diverticulitis managed?
Mild: Oral Abx
Severe: IV Abx (Cephalosporin C. Dif and metronidazole)
Haartmann's if perforated: temporary colonostomy and partial colectomy
44
What are the complications of Diverticulitis?
Haemorrhage
Fistula:
- Enterocolic
- Colovaginal
- Colovesicle
Abscess: swinging fever, boggy rectal mass, drain rectally
Obstruction-strictures
Jejunal diverticulosis is a cause of malnutrition
Perforation: ileus, shock, peritonitis
45
What investigations should be done in suspected Diverticulitis?
- Abdominal CT
- Colonoscopy
- Barium enema
46
What is the Hinchey Classification?
Describes colonic perforation due to diverticulitis
I- Para-colonic abscess
II- Pelvic abscess
III- Purulent peritonitis
IV- Faecal peritonitis
Laproscopic washout and drainage
47
What are the causes of Mallory-Weiss tears?
```
Retching
Vomiting
Coughing
Straining
Chronic hiccoughs
Brunt
NG tube
```
Increased abdominal pressure, large transient increase in transmural pressure across the gastro-oesophageal junction
Acute distention (non-distensible distal oesophagus)
Shearing forcs > transmural pressure gradient, M-W tear and rupture of SUBMUCOSAL ARTERIES
48
What are Mallory-Weiss tears?
Superficial mucosal tear at GOJ or proximal stomach causing RUPTURE OF SUBMUCOSAL ARTERIES
49
How do Mallory-Weiss tears present?
Blood oxidased:
- Coffee-ground emesis
- Malena
Systemic Blood loss:
- Tachycardia
- HoTN
- Anaemia
- Presyncope/syncooe
Rapid non-oxidised blood loss:
- Haematemesis
- Haemachetzia
50
What is Boorhaave's syndrome?
Perforation: of full-thickness rupture posterolateral oesophagus just above the diagraphm
51
What are the causes of Boorhaave's?
Iatrogenic from endoscopy
| Severe vomiting
52
How does Boorhaave's syndrome present?
Mackler's triad:
- Vomit
- Subcutaneous emphesema
- Chest pain
Severe retrosternal pain
Respiratory distress
53
How is Boorhaave's syndrome managed?
- Group and save
- CXR- pneumomediastinum
- CT will show contrast leaking into mediastinum
Surgery if unstable/spontaneous to decompress oesophagus, stop the leak and eradicate pleural and mediastinal contamination
- Give Abx and antifungals
- NBM 1-2 weeks insertion of NG tube, TPN or feeding jejunostomy
- Large-bore chest pain
54
Where are bowel cancers found?
What surgical intervention is done for them?
15% Ascending colon and caecum
Right hemicolectomy with ileo-colic anastomosis
10% Transverse colon
R/L hemicolectomy
5% Descending colon
Left hemicolectomy
colo-colon anastamosis
30% Sigmod colon
High anterior resection + TME (Total Mucosal Excision)
Colorectal anastamosis
40% Rectum
Low anterior resection + low TME
Colorectal anastamosis
Anal Verge
abdominal-perianal excision of rectum with no anastamosis
55
What are the criteria to refer via the 2 week wait?
>40 + unexplained weight loss + abdominal pain
<50 with rectal bleeding plus any of:
- abdominal pain
- change in bowel habits
- weight loss
- iron deficiency anaemia
>50 unexplained rectal bleeding
>60 iron deficiency anaemia + change in bowel habit
occult blood in faeces (screen everyone 60-74)
rectal abdominal mass
unexplained anal mass or ulcer
56
What is Duke's staging?
A Tumour confined to mucosa
B Invaded bowel wall
C Lymph metastases
D Distant metastases
(T1+T2/N0 requires no radiation, T4 long corse of R+C)
57
What are the risk factors for Gastric Cancer?
```
Blood group A
Gastric adenomatous POLYPS
HY. PYLORI Ulceration (-vely associated with duodenal ulcers)
PERNICIOUS anaemia
SMOKING
Diet: salt, spice, nitrates
```
M>F ~70-80y/o
58
How does Gastric Cancer present?
- N&V
- Dyspepsia
- Anorexia and weight loss
- Dysphagia
59
How is Gastric Cancer investigated?
U.GI endoscopy with Biopsy
MOST COMMONLY IN THE CARDIA
SIGNET RING CELLS- CONATIN LARGE VACUOLE OF MUCIN WHICH DISPLACES NUCLEUS TO ONE SIDE
CT to stage
60
What is Hepatic Encephalopathy?
Due to excess AMMONIA and GLUTAMINE in bacterial break down in the gut
Usually Acute
61
What are the causes of Hepatic Encephalopathy?
GI bleed
Infection (SBP)
Constipation
Renal failure
High protein
Low K+
Drugs:
- sedatives
DIURETICS
62
What are the features of Hepatic Encephalopathy?
Confusion, reduced GCS
Asterixis Flap (Arrhytmic -ve myoclonus 2-5Hz)
Constructional Apraxia (can't draw 5pt star)
Triphasic slow waves EEG
63
What are the grades of Hepatic Encephalopathy?
I-Irratible
II-Confusion and inappropriate behaviour
III-Incoherence and restless
IV-Coma
64
How is Hepatic Encephalopathy managed?
Give Lactulose which increases ammonia secretion and metabolism
Liver transplant
Porto-systemic shunt
65
What can cause Small Bowel Obstruction?
```
Adhesions from past surgery
Barbed structures
Hernias
Congenital issues
Gallstone ileus
Caecal volvulus
```
66
How does Small Bowel Obstruction present?
Vomiting then constipation
Central and mid abdominal pain
Shorter spasms of pain
67
What is seen on AXR in Small Bowel Obstruction?
- Central gas shadows
- Valvulae commitantes completely cross the lumen
- Gas absent in large bowel
Oral gastrografin can detect partial obstructions
68
What can cause Small and Large Bowel Obstruction?
Foreign bodies
Intusseception
Crohn's strictures
TB
69
How does Bowel Obstruction present?
Vomiting
- Faeculent
Colicky pain
Distention-progresses
Constipation
'Tinkling' bowel sounds
70
What can cause Large Bowel obstruction?
Colorectal carcinoma
Diverticular strictures
Constipation
Volvulus
71
How does Large Bowel Obstruction present?
Less likely to vomit, can be faecal
Lower abdominal pain with longer spasms that are more constant
Constipation starts earlier
72
What is seen on AXR in Large Bowel Obstruction?
- Peripheral gas shadows proximal to the obstruction but none in the rectum
- Hasutra do not completely cross the lumen
- Large, dilated loop with air-fluid levels: coffee bean sign
73
What is an Ileus?
FUNCTIONAL obstruction due to reduced motility
NO PAIN
NO BOWEL SOUNDS
74
What is a volvulus?
TORSION of the colon around MESENTERIC axis
Can cause:
ISCHAEMIA
CLOSED-LOOP OBSTRUCTION
80% are in the sigmoid
Older, chronic, Chagas, Neuromuscular e.g. Parkinson's or Duchenne's
Large bowel: rigid sigmoidoscopy and rectul tube insertion
20% Caecum, failure of fixation of the proximal bowel in utero
All ages, adhesions, pregnancy
Small bowel: R hemicolectomy
75
What are Simple Obstructions?
one obstructing point with no vascular compromise
76
What are Closed-Loop Obstructions?
obstruction at two points (e.g. a sigmoid volvulus, distention with competent ileoceacal valve) forming a loop of grossly distended bowel
Risks perforating >12cm
77
What are Strangualted Obstructions?
MESENTERIC ISCHAEMIA
blood supply is compromised and patient is very ill
Sharp, constant, localised pain: peritonism
78
What is Paralytic Ileus?
adynamic bowel due to the absence of normal peristalsis
due to:
- abdo surgery
- pancreatitis
- localised peritonitis
- spinal injury
- TCAs
79
What is Psuedo Obstruction?
Presents as mechanical obstruction but with no cause found
Acute: Oglivie's syndrome
After pelvic surgery, trama or carcinoma
Treat with neostagmine
80
How is Bowel Obstruction Managed?
```
Surgery:
- Strangulated
- Closed loop
- Large bowel
Remove or stent
```
'Drip and Suck'
- NBM
- Aspirate with NG tube
- IV fluids
81
What is Oesophagitis and what are the causes?
Inflammation of intraabdominal epithelia
```
GORD
Infection- Candida Albicans, Herpes, CMV
Drugs-Bisphosphonates
Radiotherapy
Crohn's
Alcohol
```
82
What is the gradning classification for Oesophagitis?
LA
A- One or more mucosal breaks < 5mm
B - Mucosal breaks > 5mm but no continuity across mucosal folds
C- spans across 2 folds but <75% of cicumference
D- mucosal breaks >75% of circumference
83
What are the boundaries of the epiploic foramen of Wisnlow?
Communication between peritoneal pouches: opening of lesser sac inter greater sac
Anterior: free border of lesser omentum (with hepatic artery, portal vein and bile duct)
Posterior: IVC
Superior: Caudate process of Liver
Inferior: 1st part of duodenum
84
What is Primary Sclerosing Cholangitis?
Progressive cholestasis with bile duct inflammation and strictures (fibrosis)
ASSOCIATED WITH UC: P-ANCA
HLA-DR3
- Jaundice
- Fatigue
- Pruritis
- RUQ pain
ERCP
Increased risk of cholangiocarcinoma and colorectal cancer
85
What are the side effects of prophylactic antibiotics?
Diarrhoea
Psuedomembranous colitis
Thrombocytopaenia
86
What is Primary Biliary Cirrhosis?
Interlobular bile ducts are damaged by chronic AI granulomatous inflammation causing obstruction
Progressive cholestasis -> fibrosis -> cirrhosis -> portal HTN
87
How doe PBC present?
```
Jaundice
Pruritis
Xanthelasma
Hepatosplenomegaly -> clubbing
Fatigue
Malabsorption
Bilirubin
ALP
IGM
```
Also have renal tubular acidosis -> hyperchloraemic metabolic acidosis
88
What are the antibodies present in PBC?
AMA Anti-Mitochondrial Antibodies
SmM antibodies
89
How is PBC managed?
Cholestyramine
Fat soluble vitamin supplements
Ursodeoxycolic acid
Liver transplant
90
What are the 3 types of bowel polyp?
HAMARTOMATOUS: foetal malfomation resembling neoplasm in normal cells
- Juvenile polyps
- Peutx-Jeghers syndrome
NEOPLASTIC
- Tubular
- Villous Adenoma
- Familial Adenomatous Polyposis and Gardner's syndrome
INFLAMMATORY
- UC - Psuedopolyps
- Crohn;s
- Lymphoid hyperplasia
91
What is Peutz-Jegher's syndrome?
AD mutation of tumour suppressor gene
GI Polyps in SI:
- Obstruction
- Intesusseption
- Bleeds
- 15x risk of GI cancer
Pigmented lesions:
- Face
- Lips
- Oral mucosa
- Palms of hands and soles of feet
92
What is Villous Adeonma?
Have the potential for malignant transformation
Secrete large amounts of mucous:
- Secretory diarrhoea
- Microcytic Anaemia
- Electrolyte imbalance-hypokalaemia
93
What is FAD?
Familial Adenomatous Polyposis
AD- mutation of polyposis coli onco gene
Hundreds of polyps by 30-40 years
Inevitably develop carcinoma - 1% of carcinomas
Risk of duodenal ulcers
94
What is Gardner's syndrome?
FAD with
- osteomas of skull and mandible
- retinal pigmentation
- thyroid carcinoma
- epidermal cysts on skin
95
What is Hereditary Non-Polyposis Colorectal Cancer?
AD 7 mutations in DNA mismatch repair genes
90% end up with colon cancer, usually proximal
Poorly differentiated and highly aggressive
Associated with endometrial caner
Amsterdam criteria:
- at least 3 FHx
- at least 3 generations
- at least 1 <50 y/o
96
What are the intestinal features of Crohn's disease?
- Non-bloody diarrhoea
- Weight loss
- Abdominal pain
- Mouth to anus most commonly in termial ileum
- Skip lesions
- 'Cobble-stone'
- Abdominal mass in RIF
- Megacolon - colonic dilation: proximal bowel dilation
- 'Kantor's string sign' - long narrowed ileum
- Bowel obstruction and adhesion
- Perianal disease: ulcers and fistulae
97
What are the histologic features of Crohn's disease?
- Inflammation through all layers: mucosa to serosa
- Increased goblet cells
- Granulomas
- Rose-thorn ulcers (barium enema)
98
What are the extra-intestinal features of Crohn's disease?
- Gallstones secondary to reduced bile acid reabsorption
- Oxalate renal stones as more Calcium lost in bile and it usually binds Oxalate
- Arthritis
- Erethema Nodosum on shins
- Pyoderma Gangrenosum
- Osteoporosis
99
What is the antibody present in Crohn's?
Anti-S Cerevisiae Antibody
| ASCA
100
What are the pharmacological options for Crohn's?
```
Aminosalicylates
Steroids
Immunosuppressants
TNF inhibitors
Abx
Supplements
```
101
Surgery for Crohn's
Not curative but symptom relief
Indications: fistuals abscesses and strictures
Careful of short bowel syndrome
102
How are mild flare-ups of Crohn's managed?
Symptomatic but systemically well
Prednosolone 30mg/d PO for 1 week then 20mg/d for 4 weeks
103
How are severe flare-ups of Crohn's managed?
IV steroids (Hydrocortisone) and NBM and fluids
Metronidazole
If improving put on oral prednisolone
If not infliximub or adalimumab
104
What are the intestinal features of UC?
- Ileocaecal valve to rectum
- Bloody Diarrhoea
- LLQ Pain
- Tenesmus
- Loss of haustra
- Short and narrow colon: drain-pipe
- Continuous disease
- Toxic mega colon
105
What are the histological features of UC?
- Contained to submucosa
Inflammatory cell infilitrate in lamino propria
- Crypt abscesses formed by migrating neutrophils
- Goblet cells depleted and reduced mucin
- Psuedopolyps
- Red, friable mucosa
106
What are the extra-intestinal features of UC?
- Primary sclerosing cholangitis
- Uveitis
- Arthritis
- Erythema nodosum
- Pyoderma gangrenosum
- Osteoporosis
107
What is the antibody present in UC?
P-ANCA
108
What are the complications of UC?
Higher risk of colorectal cancer
109
How is UC managed?
```
1st line:
Topical (suppository) aminosalicylate
Topical + oral aminosalicylate
Oral aminosalicylate
- mesalazine
- sulfasalazine
```
2nd line:
Steroids: prednisolone
oral/topical
3rd line:
Infliximab-monoclonal antibody against TNF-a
110
What is Hereditary Haemochromatosis?
A.R Increased intestinal absorption of iron which builds up in the body and is deposited in joints, the liver, heart, pancreas, adrenal glands and skin
111
What are the features of Hereditary Haemochromatosis?
'Bronze Diabetes'-hyperpigmentation due to melanin deposition
Grey skin
Cardiomyopathy
Diabetes Mellitus Type 2
Chronic liver disease: cirrhosis and hepatomegaly
Gonadal failure: erections, hypogynadism
Arthralgia: 2nd and 3rd MCP
Knee psuedogout: joint xrays show chondrocalcinosis
Hypoaldosteronism
112
How is Hereditary Haemochromatosis managed?
Venesect ~1unit/1-3 weeks until ferritin <50ug
Low iron diet
113
How is Hereditary Haemochromatosis investigated?
Transferrin saturation
>55% in males
>50% in females
114
What is the HLA association in Hereditary Haemochromatosis?
HLA-A3
115
What is Wilson's disease?
A.R
Accumulation of Copper in Liver and Organs
Ceruloplasmin is low and so reduced excretion into bile
116
What are the features of Wilson's disease?
- Liver disease
- Kayser-Fleischer rigns-Cu ring in iris
- Haemolysis -> haemolytic anaemia
- Blue nails
- Renal Tubular Acidosis-Faconi syndrome
117
What are the neurological features of Wilson's disease?
```
Build up in Basal Ganglia:
- Tremor
- Dysarthia
- Dysphagia
- Dyskinesia
- Dystonias
Affects mood and cognition
```
118
How is Wilson's disease managed?
Penicillamin (may cause membranous nephropathy)
Trietine hydrochloride
119
What is a-1 antitrypsin deficiency?
a-1 antitrypsin is protective against elastase (e.g. from neutrophils)
Affects lung and liver
120
What are the features of a-1 antitrypsin deficiency?
- Dyspnoea
- Cirrhosis and hepatocellular CA
- Cholestatic jaundice in children
121
How is a-1 antitrypsin deficiency managed?
Transplant, volume reduction
IV a1-ATP concentrates
Physio and bronchodialtors
122
What are the Pre-Hepatic causes of Jaundice?
Acquired
Inherited
Congenital
123
What are the Acquired causes of Pre-Hepatic jaundice?
- Mechanical
- Immune
- Drugs
- Acquired membrane defects
- Infection
- Burns
124
What are the Inherited causes of Pre-Hepatic jaundice?
- Hb abnormalities
- Metabolic defects
- RBC membrane defects
Spherocytosis
Eliptocytosis
125
What are the Congenital causes of Pre-Hepatic jaundice?
Dubin Johnson
Gilbert's
Crigler-Najar
126
What is Dubin Johnson syndrome?
A.R MRP2 defect resulting in defective hepatic extraction of blirubin
Black, glossy liver
127
What is Gilbert's syndrome?
A.R
UDP-Glucuronyl transferase deficiency leading to defective conjugation of bilirubin
Bilirubin increases on fasting
128
What is Crigler Najar syndrome?
Type 1
Type 2
129
What are the causes of Hepatocellular jaundice?
```
Congenital (Gilbert's, Crigler Najar)
Tumours: metastases/hepatocellular
Inflammation
Cirrhosis
Drugs
```
130
What are the Congenital causes of Hepatocellular jaundice?
(Gilbert's, Crigler Najar)
Rotor syndrome
A.R, defect in storage and uptake of bilirubin
131
What are the Inflamamtory causes of Hepatocellular jaundice?
```
AI Hepatitis
Viral:
- Hepatitis ABCDE
- EBV
Alcohol
Wilson's
Hereditary haemochromatosis
a1-antitrypsin deficiency
```
132
What are the Cirrhotic causes of Hepatocellular jaundice?
Alcohol
| Metabolic
133
What are the causes of Post-Hepatic jaundice?
Intrahepatic
| Extrahepatic
134
What are the causes of Intrahepatic Post-Hepatic jaundice?
(Drugs, Cirrhosis, Hepatitis)
Primary Billiary Cirrhosis
135
What are the causes of Entrahepatic Post-Hepatic jaundice?
- Pancreatitis
- Biliary stricture
- Gallstones
- Primary Sclerosing cholangitis
- Carcinoma
Head of pancreas, Liver mets, Porta-hepatic lymoh nodes, Cholangiocarcinoma, Ampulla
136
What is Budd-Chiari syndrome?
Hepatic vein thrombosis due to underlying haematological/pro-coagulant condition
Severe abdominal pain of sudden onset with ascites and tender hepatomegaly
Polycythemia Rubra vera: bone marrow neoplasm
Thrombophilia: Protein C+S deficiency, protein C resistance, anti-thrombin II deficiency
Pregnancy
COCP
137
What are the causes of Cirrhosis?
```
V: Budd Chiari
I: Hep B and C
T
A: Hepatitis, PSC, PBC
M: Non-Alcoholic Fatty Liver
I:
N
D
I
C
G: Hereditary Haemochromatosis, a1 anti, Wilson's
```
138
What is Liver Cirrhosis?
Liver necrosis leading to nodular fibrosis and regeneration which is irreversible
139
What are the 3 main impacts of Liver Cirrhosis?
- Increased resistance to blood flow causing PORTAL HYPERTENSION
- REDUCED liver FUNCTION
- Hepatocellular CARCINOMA
140
What is the impact of Portal Hypertension?
Blood backs up in the venous system
- Oesophageal varices -> U GI BLEED
- Enlarged Periumbilical veins -> CAPUT MEDUSAE
- Congested and enlagred spleen -> SPLENOMEGALY -> PANCYTOPENIA
- Increased hydrostatic pressure in the abdominal capillaries causes increase in vasodilators causing splanchnic vasodialtion which reduces effective volume in the kidneys activating RAAS further increasing the hydrostatic pressure (HEPATORENAL SYNDROME)
This forces fluid out of the capillaries into the body (OEDEMA) and peritoneal cavity ASCITES
141
What is the impact of Reduced Liver function?
- Reduced albumin synthesis reduces oncotic pressure and fluid leaks into tissues causing ASCITES, ODEMA and LEUCONYCIA (white nails)
- Reduced clotting factors leads to BRUISING
- Reduced toxin removal causes NH3 build up causing ENCEPHALOPATHY
- Reduced Bilirubin conjugation, reduced secretion into bile so bilirubin >40-50 causing JAUNDICE AND SCLERA ICTERUS
142
What is the Child-Pugh score?
Prognosis of Cirrhosis
- Bilirubin
- Albumin
- PTT
- Ascites
- Encephalopathy
143
What is the Model for End-Stage Liver Disease?
- Bilirubin
- Creatinine
- INR
144
What are the two types of Hepatorenal syndrome?
Type 1:
- Rapid
- Poor prognosis (< 2 weeks)
- Doubling of serum creatinine >221
- Halving of Creatinine clearance
Give TERLIPRESSIN
Type 2:
- Slow
- Poor prognosis but live longer (<6 mo)
Make TRANSJUGUALAR INTRAHEPATIC PORTOSYSTEMIC SHUNT
145
What is Non-Alcoholic Fatty Liver Disease?
Caused by obesity, hyperlipidaemia, DMT2, sudden starvation
Spectrum from STEATOSIS (fat in liver) to STEATOHEPATITIS (fat and inflammation) to FIBROSIS+CIRRHOSIS
Features are Hepatomegaly and ALT>AST but usually asymptomatic and found incidentally
To test for it to ENHANCED LIVER FIBROSIS BLOOD TEST
- Hyaluronic acid
- Procollagen III
- Tissue inhibitor of metalloproteinase
146
What is the fluid composition of Ascites?
TRANSUDATE
147
What are the Primary Liver Tumours?
MALIGNANT:
- Hepatocellular Carcinoma (most common)
- Angiosarcoma
- Hepatoblastoma
BENIGN:
- Cysts
- Haemangioma
- Adenoma common in anabolic steroids, preg and ocp
- Fibrma
148
What are the causes of Hepatocellular Carcinoma?
Cirrhosis (causes of)
Male
Diabetes
149
How does Hepatocellular Carcinoma present?
- Jaundice
- Ascites
- RUQ pain
- Hepatomegaly-irregular edge
- Pruritis
- Splenomegaly
- Anorexia
USS
150
What is the tumour marker for Hepatocellular Carcinoma?
Alpha-fetoprotein
151
How is Hepatocellular Carcinoma managed?
- Surgically resect
| - Transplant
152
What are the Secondary Liver Tumours?
90% of liver cancers
Metastasised from
- Stomach
- Lung
- Colon
- Breast
- Uterus
153
What is a Carcinoid Tumour of the Liver?
LIVER METS that release SEROTONIN (also lung)
- Flushing
- Diarrhoea
- Bronchospasm
- Hypotension
- R valve stenosis
- Niacin B3 deficiency: Pallagra
Also may secrete ACTH (Cushing's) or GHRH
154
What are the Liver Abscesses/Cysts?
Pyogenic
Amoebic
Cysts
155
What are the causes and management of Pyogenic Liver Abscess?
Staph aureus in children
E. coli in adults
Give AMOC+CIPRO+METRON
(or CLIND if PA)
156
What are the causes and management of Amoebic Liver Abscess?
Single mass in the R lobe - ANCHOVY SAUCE PUS
157
How do Liver Abscesses present?
- swinging fever
- sweats
- RUQ pain
- increased WCC
- USS/CT/Aspirate
158
What causes Liver cysts?
- Simple-sporadic
- Polysistic Liver Disease
Hyatid cysts caused by Echinoccus Granulosus (tapweowrm parasite)
Outer capsule with multiple small daughter cysts
Causes Hypersensitivity Type I reaction
159
Cholestasis
obstruction of bile flow along biliary tract from lvier to duodenum
160
Cholelithiasis
Presence of gallstones in the gallbladder
PIGMENT <10%: small, friable and irregular due to haemolysis
CHOLSTEROL- large, solitary due to age and obesity
MIXED: CALCIUM slats +pigment + cholesterol
>60% have 1 or more deranged LFT
161
Choleodocholithiasis
Gallstones in the Billiary tract
162
Biliary Colic
Intermittent obstruction of the cystic duct by gallstones causing steading, colicky, crescendoing, RUQ
Worse after eating fatty foods when CHOLECYSTOKININ levels are high
USS + LapChole
163
Ascending Cholangitis
inflammation or infection of the biliary tree mostly due to gallstones obstructing the tract DISTAL to the cystic duct
Severely septic and unwell
CHARCOT'S TRIAD: Jaundiced, RUQ, Fever
Fluid resuss, BS Abx, Early ERCP
164
Acute Cholecystitis
Inflammation of the gallbladder WALL due to GALLSTONE TRAUMA and BLOCKED CYSTIC DUCT
ESCHERICHIA
bile builds up and irritates
- RUQ
- Fever
- Murphy's sign
Mildly deranged LFTs
USS + Lapchole
165
Acalculous Cholecystits
Inflammation of the gallbladder with no gallstone obstruction
High fever
Systemic illness e.g. DM
Lapchole
166
Gallstone Ileus
Obstruction of the small bowel by an impacted gallstone >2.5cm
Travelled through a CHOLEO-ENTERIC FISTULA
Known gallstones + small bowel obstruction
Laparotomy-remove the stone proximal to the obstruction
167
Gallbladder abscess
Prodromal illness + RUQ
- Swinging pyrexia
- Systemically
NO generalised peritonism
Surgery with subtotal Chole if Calot's triangle is hostile
Percutaneous drainage if unfit for surgery
168
Mirizzi's syndrome
Gallbladder presses on the common hepatic or common bile duct causing jaundice
May also cause fistula
169
Mucocele/Empyema
overdistended gallbladder filled with mucoid or clear and watery content. Usually noninflammatory, it results from outlet obstruction of the gallbladder and is commonly caused by an impacted stone in the neck of the gallbladder or in the cystic duct
Infected: empyema
170
Cholangiocarcinoma
Symptoms of biliary colic + jaundice
COURVOISIER'S SIGN; palpable mass in ruq
Periumbilical lymphadenopathy: SILVER MARY JOSEPH NODES
Virchow's node
171
What are the mechanical causes of Dysphagia?
- Malignancy
- Strictures
- External compression
- PHARYNGEAL POUCH
- OESOPHAGEAL WEB
- OESOPHAGITIS
- CREST
- Hypertensive LOS
- Foreign body
172
What are the Neuromuscular causes of Dysphagia?
- ACHALASIA
- DIFFUSE OESOPHAGEAL SPASM
- Stroke liquids worse
- Parkinson's
- MS liquids and solids
- Myesthenia gravis
173
What are Oesophageal webs?
Thin membranes covering along the oesophagus
SOLID>LIQUIDS
Plummer-vinsens syndrome: associated with iron deficiency aneamia
GLOSITIS
DYSPHAGIA
SPLENOMEGALY
Barium swallow
Endoscopic Dilation
174
What is a Pharyngeal Pouch?
ZENICKER'S DIVERTICULUM
Posteriomedial diverricul through KILLIAN'S DEHISCIENCE (TRIANGLE) between the thyropharyngeus and cricpharyngeus muscles AT C5-C6
M>F
```
Dysphagia
Hallitosis
Regurgitation (risk of asp. pneumonia)
Aspirtion
Neckswelling gurgles on palpation
```
175
What is Achalasia?
Primary motility disorder of the oesophagus where the smooth muscle fails to relax (progressive destruction of the ganglion cells in the myenteric plexus)
Oesophagus fails to relax and so does LOS, LOS pressure increases and food gets stuck leading to progressive dilation of the oesophagus
Progressive dysphagia ofsolid and liquid with regurgitation, chect pain and weight loss
Do Oesophageal manometry
Barium swallow shows bird beak
Baloon dilation
Laproscopic hellar myotomy
176
What is Diffuse Oesophageal Spasm?
Multi focal high amplitude contractions of the oesophagus due to dysfunction of the inhibitory nerves
Severe dysphagia of solids and liquids
severe chest pain
Manometry shows repetative contractions
Cork screw on barium swallow
Relax SmM with nitrates or CCB
Pnuematic dilatation
Myotomy if severe
May progress to achalasia
177
What is a hiatus hernia?
Protrusion of an organ (stomach) from abdo cavity into thorax via OESOPHAGEAL HIATUS
Sliding, whole thing goes up
Rolling/Para-oesophageal FUNDUS rises up to lie alongside lOS
178
How are pepto-duodenal ulcers managed?
Trple therapy
PPI
Clarith
Metronidazole or Amoxicillin
