Week 16 - Ascites, Cirrhosis, Haemochromotosis, Pancreatic Cancer, Wernickes

Question 1

gynaecomastia can be found in conditions that do what?

Answer 1

increase oestrogen

Question 2

what conditions increase oestrogen - 5

Answer 2

Obesity (aromatase is an enzyme found in adipose tissue that converts androgens to oestrogen)

Testicular cancer (oestrogen secretion from a Leydig cell tumour)

Liver cirrhosis and liver failure

Hyperthyroidism

Human chorionic gonadotrophin (hCG) secreting tumour, notably small cell lung cancer

Question 3

what steroid increases gynacomastia

Answer 3

Spironolactone (inhibits testosterone production and blocks testosterone receptors)

Question 4

what is ascites

Answer 4

fluid in the peritoneal cavity

Question 5

why do ascites form - 2 reasons

Answer 5

result from high pressure in certain veins of the liver - portal hypertension and low blood levels of albumin.

Question 6

what is the leading cause of ascites

Answer 6

liver cirrhosis

Question 7

what genetic liver diseases can cause ascites

Answer 7

haemochromatosis, Wilson’s disease and alpha-1 antitrypsin deficiency

Question 8

what is the most common complication of cirrhosis

Answer 8

ascites

Question 9

what are the symptoms of ascites

Answer 9

Swelling in your ankles.
Shortness of breath.
Digestive issues, such as bloating, abdominal pain, loss of appetite, indigestion and constipation.
Back pain.
Difficulty sitting.
Fatigue.

Question 10

what is the procedure used to remove fluid from ascites

Answer 10

Paracentesis: You’ll get a local anesthetic before this procedure. Your provider inserts a needle into your abdomen to remove fluid. The fluid is analyzed for signs of cancer, infection, portal hypertension and other conditions.

Question 11

what is crucial to treat ascites

Answer 11

limiting sodium in the diet.

taking diurectics

Question 12

what is TIPS

Answer 12

this is a procedure performed to treat fluid build up in the abdomen.

a wire stent is inserted into a vein in the liver. when inflated, the stent forms a channel to bypass the liver

TIPS may be appropriate if your ascites are not repsonding to other therapies, if you need several paracenteses per month and you are not a candidaste for a liver transplant

Question 13

what does the term haemochromatosis describe

Answer 13

a group of autosomal recessive disorders of iron metabolism.

it is sometimes called hereditary haemochromatosis (HHC)

Question 14

what is the most common mutation found in haemochromatosis

Answer 14

mutations in the HFE gene.

C282Y is by far the most common.

Question 15

what do these genetic defects seen in haemochromatosis result in

Answer 15

excessive iron absoprtion from the diet.

Question 16

what does this excess iron do in the body

Answer 16

Skin discolouration
Liver disease – these are usually the most serious complications
Cirrhosis
Liver carcinoma
Hepatocellular carcinoma
Diabetes (pancreatic involvement)
Joint involvement (arthiritis)
Pituitary – sexual dysfunction
Heart failure, cardiomyopathy and arrhythmia
Neurological signs
Impaired memory
Mood swings
Irritability
Depression

Question 17

what is the mainstay of treat ment in haemochromatosis

Answer 17

involves regular removal of blood from the patient (venesection)

Question 18

what does venesection, initially after diagnosis typically involve

Answer 18

removal of the blood every week until ferritin level falls below 100.

Question 19

after intial diagnosis, what is the treatment for haemochromatosis

Answer 19

patients typically may have 3-4 courses of venesection per year. typically volumes of about 500mls are removed.

Question 20

what is the typical presentation of haemochromatosis

Answer 20

Presents late in disease process

Typically age 30-50, later (usually post-menopause) in women

Usually begins with non-specific symptoms: lethargy, malaise, weakness, joint pains, erectile dysfunction

Later symptoms include: skin discolouration (darker), cirrhosis, hepatocellular carcinoma, diabetes, heart failure

Often diagnosed incidentally – when iron studies and / or LFTs are taken for another reason

Question 21

what is the most important investigation done for haemochromatosis

Answer 21

ferritin

Normal serum ferritin effectively rules out HHC as a differential

Raised ferritin is non-specific and can be due to acute inflammation, and many other liver diseases

Question 22

what is a late sign of haemochromatosis

Answer 22

deranged LFTs are a late sign

Question 23

what are the differentials for haemochromatosis

Answer 23

Iron overload anaemias – e.g. Thalassaemia, sideroblastic anaemia
Drugs
Haemodialysis
Chronic liver diseases
Hepatitis
Alcoholic liver disease
NAFLD
Cirrhosis
Myelodysplastic syndrome
Multiple blood transfusions

Question 24

what is the maintenance phase of haemochromatosis

Answer 24

Maintain ferritin in 50-100 mcg/L range
Typically this involves venesection 3-4x per year for men and 1-2x per year for menstruating women
Check ferritin every 6-12 months

Question 25

what kind of biopsy can be used to establish the iron concentration in the liver

Answer 25

liver biopsy with Perl’s stain can be used to establish the iron conc. in the liver.

Question 26

what is chondrocalcinosis and in what condition is this a complication of

Answer 26

(calcium pyrophosphate deposits in joints) causes arthritis

seen in haemochromatosis

Question 27

what kind of cancer is the vast majority of pancreatic cancers

Answer 27

vast majority are adenocarcinomas and most occur in the head of the pancreas

Question 28

what happens once a tumour in the head of the pancreas grows too large

Answer 28

it can compress the bile ducts, resulting in obstructive jaundice

Question 29

where do pancreatic cancers spread and metastitse to

Answer 29

Pancreatic cancers tend to spread and metastasise early, particularly to the liver, then to the peritoneum, lungs and bones

Question 30

what is the average survival when diagnosed with advanced pancreatic cancer

Answer 30

around 6 months

Question 31

what is the key presenting feature of pancreatic cancer

Answer 31

painless, obstructive jaundice.

Question 32

what is the key differential when thinking about pancreatic cancer

Answer 32

cholangiocarcinoma

Question 33

what does painless obstructive jaundice present with

Answer 33

Yellow skin and sclera
Pale stools
Dark urine
Generalised itching

Question 34

what else can be a sign of pancreatic cancer that is very important

Answer 34

new onset diabetes, or a rapid worsening of glycaemic control type 2 diabetes.

Question 35

what is the referral criteria for suspected pancreatic cancer according to the NICE guidlines

Answer 35

Over 40 with jaundice – referred on a 2 week wait referral

Over 60 with weight loss plus an additional symptom (see below) – referred for a direct access CT abdomen

Question 36

when do the NICE guidelines suggest a GP referral for a direct access CT abdomen to assess for pancreatic cancer

Answer 36

weight loss plus any of:

Diarrhoea
Back pain
Abdominal pain
Nausea
Vomiting
Constipation
New-onset diabetes

Question 37

what is the only scenario where a GP can refer directly for a CT scan

Answer 37

suspected pancreatic cancer is the only scenario where GPs can refer directly for a CT scan. Whenever guidelines and clinical practice have notable exceptions like this it is worth taking note of, as these make good facts for examiners to test your knowledge on.

Question 38

what is Courcoisier’s law

Answer 38

states that a palpable gallbladder along with jaundice is unlikely to be gallstones. the cause is usually cholangiocarcinoma or pancreatic cancer

Question 39

what is Trousseau’’s sign of malignancy

Answer 39

refers to migratory thrombophlebitis as a sign of malignancy.

Thrombophlebitis is where blood vessels become inflamed with an associated blood clot (thrombus) in that area. Migratory refers to the thrombophlebitis reoccurring in different locations over time.

Question 40

what is diagnosis of pancreatic cancer usually based on

Answer 40

Diagnosis is based on imaging (usually CT scan) plus histology from a biopsy.

Question 41

what does a staging CT scan for pancreatic cancer involve

Answer 41

Staging CT scan involves a full CT thorax, abdomen and pelvis (CT TAP). This is used to look for metastasis and other cancers.

Question 42

what tumour marker may be raised in pancreatic cancer

Answer 42

CA 19-9 (carbohydrate antigen) is a tumour marker that may be raised in pancreatic cancer. It is also raised in cholangiocarcinoma and a number of other malignant and non-malignant conditions.

Question 43

what can be used to assess the biliary system in detail to assess the obstruction

Answer 43

MRCP

Question 44

when is surgery considered in pancreatic cancer

Answer 44

small tumours isolated in the head of the pancreas (about 10% of cases).

Question 45

what are the surgical options depending on the location of the tumour

Answer 45

Total pancreatectomy
Distal pancreatectomy
Pylorus-preserving pancreaticoduodenectomy (PPPD) (modified Whipple procedure)
Radical pancreaticoduodenectomy (Whipple procedure)

Question 46

what are the palliative treatment options

Answer 46

Stents inserted to relieve the biliary obstruction
Surgery to improve symptoms (e.g., bypassing the biliary obstruction)
Palliative chemotherapy (to improve symptoms and extend life)
Palliative radiotherapy (to improve symptoms and extend life)
End of life care with symptom control

Question 47

what is the Whipple procedure

Answer 47

A Whipple procedure (pancreaticoduodenectomy) is a surgical operation to remove a tumour of the head of the pancreas that has not spread. A Whipple procedure is a massive operation so patients need to be in good baseline health. It involves the removal of the:

Head of the pancreas
Pylorus of the stomach
Duodenum
Gallbladder
Bile duct
Relevant lymph nodes

Question 48

what is a modified Whipple procedure

Answer 48

A modified Whipple procedure involves leaving the pylorus in place. It is also known as a pylorus-preserving pancreaticoduodenectomy (PPPD).

Question 49

what is Wernicke’es encephalopathy

Answer 49

a disorder that occurs as a result of inadequate levels of vitamin B1 (thiamine) in the body.

Question 50

what does Wernicke’s result in

Answer 50

Ataxia
Ophthalmoplegia
Nystagmus – may be horizontal and vertical
Conjugate gaze problems
Confusion
Short Term Memory Loss

Question 51

what is the pathology of Wernicke’s

Answer 51

Results from prolonged thiamine deficiency alongside continued carbohydrate ingestion
There is degradation of the thalamic nuclei, brainstem nuclei and cerebellum and other structures around the 3rd and 4th ventricles, and the aqueduct.
Changes are symmetrical

Question 52

what is the triad for Wernicke’s

Answer 52

Confusion
Nystagmus / opthalmoplegia – can be any eye sign
Ataxia

Question 53

why should treatment for Wernicke’s be urgent

Answer 53

to prevent the irreversible Korsakoff’s syndrome

Question 54

what is treatment

Answer 54

Give high dose IV thiamine (e.g. 500mg Thiamine TDS, or Pabrinex – 2-3 ampules IV every 8 hours over 10 minutes for 3-5 days)
- Then, continue with oral thiamine
- Risk of anaphylaxis (rare)

Question 55

what should you be wary of when treating Wernicke’s

Answer 55

WARNING – be wary of hypoglycaemia. if this also exists, then give thiamine before glucose as glucose can precipitate the symptoms of Wernicke’s in susceptible patients (due to the joint pathology of thiamine deficiency + carbohydrate ingestion).

Question 56

what is Korsakoff’s Syndrome & Korsakoff’s Psychosis

Answer 56

This is a complication of untreated Wernicke’s encephalopathy, although it can occur without prior symptoms of Wernicke’s

Question 57

what are the signs and symptoms of Korsakoff’s Syndrome & Korsakoff’s Psychosis

Answer 57

Amnesia – both antero and retrograde to varying degrees
Anterograde memory loss – inability to acquire new memories
Retrograde Memory loss – loss of old memories
Loss of orientation in time and space
Apathy and little response to events in the world around them
Mild euphoria
Confabulation– making up stories! These can often be very convincing, although usually have no basis in fact

Question 58

what is the prognosis and treatment of Korsakoff’s Syndrome & Korsakoff’s Psychosis

Answer 58

In thiamine deficiency, Korskoff’s is considered irreversible, however:
20% of patients will fully recover, but
25% of patients will require prolonged institutional care
Symptoms may improve up to 14 months after onset
Treatment is with thimaine and adequate hydration

In Korsakoff’s due to stroke or bleed, the prognosis is good, and symptoms will usually resolve after treatment of the underlying cause.