Week 16 - Ascites, Cirrhosis, Haemochromotosis, Pancreatic Cancer, Wernickes Flashcards
gynaecomastia can be found in conditions that do what?
increase oestrogen
what conditions increase oestrogen - 5
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
what steroid increases gynacomastia
Spironolactone (inhibits testosterone production and blocks testosterone receptors)
what is ascites
fluid in the peritoneal cavity
why do ascites form - 2 reasons
result from high pressure in certain veins of the liver - portal hypertension and low blood levels of albumin.
what is the leading cause of ascites
liver cirrhosis
what genetic liver diseases can cause ascites
haemochromatosis, Wilson’s disease and alpha-1 antitrypsin deficiency
what is the most common complication of cirrhosis
ascites
what are the symptoms of ascites
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.
what is the procedure used to remove fluid from ascites
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.
what is crucial to treat ascites
limiting sodium in the diet.
taking diurectics
what is TIPS
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
what does the term haemochromatosis describe
a group of autosomal recessive disorders of iron metabolism.
it is sometimes called hereditary haemochromatosis (HHC)
what is the most common mutation found in haemochromatosis
mutations in the HFE gene.
C282Y is by far the most common.
what do these genetic defects seen in haemochromatosis result in
excessive iron absoprtion from the diet.
what does this excess iron do in the body
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
what is the mainstay of treat ment in haemochromatosis
involves regular removal of blood from the patient (venesection)
what does venesection, initially after diagnosis typically involve
removal of the blood every week until ferritin level falls below 100.
after intial diagnosis, what is the treatment for haemochromatosis
patients typically may have 3-4 courses of venesection per year. typically volumes of about 500mls are removed.
what is the typical presentation of haemochromatosis
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
what is the most important investigation done for haemochromatosis
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
what is a late sign of haemochromatosis
deranged LFTs are a late sign
what are the differentials for haemochromatosis
Iron overload anaemias – e.g. Thalassaemia, sideroblastic anaemia
Drugs
Haemodialysis
Chronic liver diseases
Hepatitis
Alcoholic liver disease
NAFLD
Cirrhosis
Myelodysplastic syndrome
Multiple blood transfusions
what is the maintenance phase of haemochromatosis
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
what kind of biopsy can be used to establish the iron concentration in the liver
liver biopsy with Perl’s stain can be used to establish the iron conc. in the liver.
what is chondrocalcinosis and in what condition is this a complication of
(calcium pyrophosphate deposits in joints) causes arthritis
seen in haemochromatosis
what kind of cancer is the vast majority of pancreatic cancers
vast majority are adenocarcinomas and most occur in the head of the pancreas
what happens once a tumour in the head of the pancreas grows too large
it can compress the bile ducts, resulting in obstructive jaundice
where do pancreatic cancers spread and metastitse to
Pancreatic cancers tend to spread and metastasise early, particularly to the liver, then to the peritoneum, lungs and bones
what is the average survival when diagnosed with advanced pancreatic cancer
around 6 months
what is the key presenting feature of pancreatic cancer
painless, obstructive jaundice.
what is the key differential when thinking about pancreatic cancer
cholangiocarcinoma
what does painless obstructive jaundice present with
Yellow skin and sclera
Pale stools
Dark urine
Generalised itching
what else can be a sign of pancreatic cancer that is very important
new onset diabetes, or a rapid worsening of glycaemic control type 2 diabetes.
what is the referral criteria for suspected pancreatic cancer according to the NICE guidlines
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
when do the NICE guidelines suggest a GP referral for a direct access CT abdomen to assess for pancreatic cancer
weight loss plus any of:
Diarrhoea
Back pain
Abdominal pain
Nausea
Vomiting
Constipation
New-onset diabetes
what is the only scenario where a GP can refer directly for a CT scan
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.
what is Courcoisier’s law
states that a palpable gallbladder along with jaundice is unlikely to be gallstones. the cause is usually cholangiocarcinoma or pancreatic cancer
what is Trousseau’’s sign of malignancy
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.
what is diagnosis of pancreatic cancer usually based on
Diagnosis is based on imaging (usually CT scan) plus histology from a biopsy.
what does a staging CT scan for pancreatic cancer involve
Staging CT scan involves a full CT thorax, abdomen and pelvis (CT TAP). This is used to look for metastasis and other cancers.
what tumour marker may be raised in pancreatic cancer
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.
what can be used to assess the biliary system in detail to assess the obstruction
MRCP
when is surgery considered in pancreatic cancer
small tumours isolated in the head of the pancreas (about 10% of cases).
what are the surgical options depending on the location of the tumour
Total pancreatectomy
Distal pancreatectomy
Pylorus-preserving pancreaticoduodenectomy (PPPD) (modified Whipple procedure)
Radical pancreaticoduodenectomy (Whipple procedure)
what are the palliative treatment options
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
what is the Whipple procedure
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
what is a modified Whipple procedure
A modified Whipple procedure involves leaving the pylorus in place. It is also known as a pylorus-preserving pancreaticoduodenectomy (PPPD).
what is Wernicke’es encephalopathy
a disorder that occurs as a result of inadequate levels of vitamin B1 (thiamine) in the body.
what does Wernicke’s result in
Ataxia
Ophthalmoplegia
Nystagmus – may be horizontal and vertical
Conjugate gaze problems
Confusion
Short Term Memory Loss
what is the pathology of Wernicke’s
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
what is the triad for Wernicke’s
Confusion
Nystagmus / opthalmoplegia – can be any eye sign
Ataxia
why should treatment for Wernicke’s be urgent
to prevent the irreversible Korsakoff’s syndrome
what is treatment
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)
what should you be wary of when treating Wernicke’s
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).
what is Korsakoff’s Syndrome & Korsakoff’s Psychosis
This is a complication of untreated Wernicke’s encephalopathy, although it can occur without prior symptoms of Wernicke’s
what are the signs and symptoms of Korsakoff’s Syndrome & Korsakoff’s Psychosis
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
what is the prognosis and treatment of Korsakoff’s Syndrome & Korsakoff’s Psychosis
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.
