Liver + Friends Flashcards
WHAT IS LIVER CIRRHOSIS?
Cirrhosis is a condition that occurs as a response to liver damage
What is some causes of cirrhosis of the liver?
Alcoholic liver disease
Viral hepatitis - types B, C and delta
Metabolic:
Haemochromatosis - primary and secondary
Wilson’s disease
Alpha-1 anti-trypsin deficiency
Primary biliary cirrhosis; secondary biliary cirrhosis
Toxins/ drugs e.g. methrotrexate, amiodarone, bush tea
What are the symptoms of liver cirrhosis?
Symptoms:
Lethargy
Itch, especially in primary biliary cirrhosis
Fever
Weight loss
Swelling of abdomen and ankles.
Signs:
Jaundice
Finger clubbing
Leuconychia
Palmar erythema
Bruising
Spider naevi
Splenomegaly
What are some investigations for liver cirrhosis?
Investigations aimed at finding cause:
- History of alcohol abuse
- Mean cell volume, gamma GTP, serum ferritin, alpha-1 antitrypsin, ceruloplasmin
- Viral titres and antibodies
Investigations aimed at determining severity:
- Plasma biochemistry - clotting studies, bilirubin, AST, albumin, U+Es, glucose
- Platelets
- Ultrasound (for liver, spleen and ascites)
- Liver biopsy
What is the treatment for liver cirrhosis?
There is no treatment that reverses liver cirrhosis.
There are treatments that may slow down the development of various types of liver cirrhosis, for example
- Alcohol abstinence for alcoholic hepatitis
- Venesection for haemochromatosis
- Steroids for autoimmune chronic active hepatitis
WHAT IS PORTAL HYPERTENSION?
The normal pressure in the venous portal system is 7-14 mm Hg.
In portal hypertension, levels may rise to 20-50 mm Hg.
In the UK, the most common cause is liver cirrhosis; worldwide, schistosomiasis is more likely.
What are the causes of portal hypertension?
https://www.youtube.com/watch?v=Cox6Z5pqMBo
Pre-hepatic
Portal vein thrombosis
Splenic vein thrombosis
Cancer compression
Hepatic
Pre-sinusoidal
Alcoholic hepatitis
Congenital hepatic fibrosis
Sinusoidal
Cirrhosis - Specific types include
Alcoholic
Viral
Post-sinusoidal
Budd-Chiari syndrome - hepatic vein thrombosis
Veno-occlusive disease
Constrictive pericarditis
What are the symptoms of portal hypertension?
Often symptomless
- Haematemesis or melaena - due to rupture of gastro - oesophageal varices
- Oedema - Peripheral or Ascites - with low plasma albumin
- Hepatic encephalopathy
- Porto-systemic shunts - e.g. caput Medusae
Where do varices most commly occur?
Gastro-oesophageal junciton
Ileocaecal junction
Rectum
Abdomen
ODG
What are the investivations for portal vein hypertension?
Low platelet
Low albumin
High bilirubin
Prolonged PT
USS abdo = nodular outline of the liver, fatty liver, reduced portal vein flow, splenomegaly ascites
Venogram - GOLD STANDARD
What is the management of portal hypertension?
Management of the bleeding
Prevention of recurrence of bleeding
Prophylaxis to prevent haemorrhage
How are varices formed from cirrhosis and portal hypertension?
Cirrhosis occurs which means less blow flow can get into liver
Pressure builds up in the veins and causes portal hypertension
Anastomosing vien take the path of lowest resistance
Flow towards the heart straight away rather than portal system
Smaller vein dilate causing varices
HOW DOES PORTAL HYPERTENSION CAUSE OESOPHAGAEL VARICES?
Oesophageal varices are varicosities of branches of the azygos vein
which anastomose with tributaries of the portal vein in the lower oesophagus, due to portal hypertension in conditions such as cirrhosis of the liver
What are the symptoms of oesophagael varices caused by cirhosis?
Haematemesis
If the varices bleed slowly then the patient may present with melaena or anaemia
What are the investigations for oesophagael varices?
Endoscopy which permits identification of the site of bleeding as well as enabling treatment, e.g. endoscopic sclerotherapy.
What is the management of oesophagael variceal bleeding?
- *Management of variceal bleeding**
1. Terlipressin - to suspected variceal bleeding at presentation. - Stop treatment after definitive haemostasis has been achieved
2. Prophylactic antibiotics - *Oesophageal varices**
1. Band ligation
2. Transjugular intrahepatic portosystemic shunts (TIPS) if not controlled by band ligation
WHAT IS PRIMARY BILIARY CIRRHOSIS?
https://www.youtube.com/watch?v=CQtHOMzLzwU&t=1s
Primary biliary cholangitis (previously known as primary bilary cirrhosis) cirrhosis is an autoimmune disease characterised by chronic, progressive, destruction of intrahepatic bile ducts, resulting in chronic cholestasis, portal inflammation, and fibrosis which will eventually lead to cirrhosis and liver failure
What is the cause of PBC?
Unknown environmental triggers
+ Genetic predisposition (IL12A)
Leading to loss of immune tolerance to self-mitochondrial proteins.
What are the symptoms for primary biliary cirrhosis?
Mainly asymptomatic
Symptomatic
Fatigue
Pruritus
What are the investigations for primary biliary cirrhosis?
Liver function tests
Serum alkaline phosphatase ALP RAISED x3 or x4
Immunology
Anti-mitochondrial antibodies
Liver biopsy
Confirms the diagnosis but not mandatory to make the diagnosis
Imaging
MRI or endoscopic retrograde cholangiography – to exclude primary sclerosing cholangitis or other disorders that might lead to chronic cholestasis
What is needed for the diagnosis of primary bilary cholangitis?
When 2 of the 3 are met:
- Biochemical evidence of cholestasis based mainly on alkaline phosphatase elevation of at least 1.5 times the upper limit of normal for more than 24 weeks
- Presence of AMA at titres of 1:40 or higher
- Compatible histologic evidence - nonsuppurative destructive cholangitis and destruction of interlobular bile ducts
What is the treatment of primary biliary cirrhosis?
Symptomatic treatment
Pruritus - Cholestyramine
Fatigue doesn’t really respond to treatment
**Disease-modifying therapy** Ursodeoxycholic acid (UDCA) - slows progression
Liver transplantation
WHAT IS ALCOHOLIC LIVER DISEASE?
Liver manifestations of alcohol overconsumption, including
Fatty liver
Alcoholic hepatitis
Fibrosis or cirrhosis
What are the risk factors for alcoholic liver disease?
Drinking pattern
Sex - Women
Genetic
Nutrition
What are the symptoms of alcoholic liver disease?
Anorexia
Morning nausea with dry retching
Darrhoea
Vague right upper quadrant abdominal pain.
What are the investigations for alcoholic liver disease?
FBC
May reveal macrocytosis
LFTs:
Raised gamma GT indicates possible alcohol abuse
Abnormal ALT reflects hepatocellular damage
An AST:ALT ratio that is greater than 2 suggests alcoholic damage
Elevated serum IgA; anti-smooth muscle antibodies may be found
Liver biopsy
Reveals extent of liver damage and suggests prognosis
What is the treatment of alcoholic liver disease?
-
Alcoholic Steatosis
Reversible with abstinence -
Alcoholic Hepatitis
Stop drinking
Corticosteroids to suppress immune system
Lorazapam for alcohol withdrawal/Delierium tremens -
Liver Cirrhosis
Irreversible
Treat complications
Transplant
WHAT IS HAEMOCHROMATOSIS?
https://www.youtube.com/watch?v=T7ybRVFXRD0
Increased intestinal iron absorption
Iron deposition in joints, liver, heart, pancreas, pituitary, adrenals and skin.
What is the cause of haemochromatosis?
Autosomal recessive
HFE gene
Chromosome 6
What does the excess iron cause to happen in haemochromatosis?
Produces ROS (reactive oxygen species) which damage the cells they are in
What are the symptoms of haemochromatosis?
Often none until after 40 years
Then non-specific e.g.
Tiredness
Arthralgia
Weight loss
In chronic disease:
Diabetes - ‘Bronze diabetes’ from iron deposition in pancreas
Dilated cardiomyopathy
Slate-grey skin pigmentation
What are the investigations for haemochromatosis?
Transferrin saturation - is the proportion of the iron transport protein transferrin that is saturated with iron
Increased
Serum ferritin
Increased
HFE genotyping
Liver biopsy
What is the treatment for haemochromatosis?
- Venesect (removing blood)
- Iron chelation therapy
Deferoxamine OR defarasirox
Binds iron and excretes in urine - Low iron diet
WHAT IS ALPHA-1 ANTITRYPSIN DEFICIENCY?
Genetic condition when alpha-1 antitryspin is absent
What is the genetics of alpha-1(4)-antitrypsin?
Austosomal recessive / co-dominant
Chromosome 14
What is the pathology of alpha-1-antitrypsin deficiency?
Misfolded alpha-1 antitrypsin builds up in hepatocytes
Leading to cirrhosis
Results in inability to export alpha1-antitrypsin from liver
What are the symptoms of alpha-1 antitrypsin?
Jaundice
Cirrhosis
Inability to make coagulation factors
Buildup of toxins
What are the investigations for alpha-antitrypsin deficiency?
Serum alpha1-antitrypsin (1AT) levels lower
Liver biopsy
Periodic acid Schi (PAS) +ve
Diastaise resistant
Liver ultrasound
What is the treatment for anti-1-antitrypsin deficiency?
Danazol therapy - increases alpha-1-antitrypsin levels
Liver transplant
WHAT IS WILSON’S DISEASE?
https://www.youtube.com/watch?v=Cr8R_bnKAtk
Too much copper (Cu) in liver and CNS
What type of gene disease is Wilson’s disease?
Autosomal recessive
Chromosome 13
Codes for a copper transporting ATPase
What is the pathology of Wilson’s disease?
In the liver, copper is incorporated into caeruloplasmin.
Copper incorporation into caeruloplasmin in hepatocytes
and its excretion into bile are impaired.
Therefore, copper accumulates in liver, and later in other organs.
ROS made and damage liver
What are the symptoms of Wilson’s disease?
Kayser–Fleischer (KF) rings
Copper in iris
Neurological signs - parkinsonian tremor, dysarthria
Due to copper in CNS
Liver failure
Hepatosplenogmegaly
What are the investigations for Wilson’s disease?
Urine Copper + Free Copper
HIGH
Total serum copper + serum caeruloplasmin
LOW
Molecular genetic testing can confirm the diagnosis.
Liver biopsy
Increase Hepatic copper
MRI
Degeneration of brain
What is the management of Wilson’s disease?
Penicillamine
Bind copper, easier to excrete
Zinc
Decrease copper reabsorption
Liver transplantation
If severe
WHAT IS ASCITES?
Abnormal accumulation of fluid in the abdominal (peritoneal) cavity
What are the causes of ascites?
Liver cirrhosis
Intra-abdominal malignancy
Nephrotic syndrome
Constrictive pericarditis
Meig’s syndrome
Budd-Chiari syndrome
Tuberculous peritonitis
What are the symptoms of ascties?
Rapid weight gain
Abdominal swelling
Sacral oedema
Ankle swelling
What are the differential diagnosis of ascites?
5 F’s
Fat
Feaces
Flatus
Fetus
Fliipin big tumour
What are the investigations for ascites?
Ultrasound
Diagnostic paracentesis - in which 30 to 50 ml of fluid is withdrawn. This will enable identification of:
Protein content: albumin and total protein
Malignant cells
Bacteria
White blood cells
Glucose
What is the managent of ascites?
Tense ascites
Therapeutic paracentesis of 4-6 litres
Plasma volume expansion with albumin is used by many at the same time as therapeutic paracentesis
Non-tense ascites
Treat cause
Limit dietary sodium intake
Diuretics - Spironolactone
Therapeutic paracentesis
Surgical shunts
WHAT IS APPENDICITIS?
https://www.youtube.com/watch?v=r9amif1DQMc
Inflammation of the appendix
Where is the appendix?
Connected to the cecum
Also known as veriform appendix (worm shaped)
What are the causes of appendicits?
Obstruction
From
Feacalith
Undigested seeds
Pinworm infection
Lymphoid follicle growth
Collection of lymphocytes become maximum size in adolesence
Viral infection caues follicle growth
What bacteria become trapped and what happens as a result?
E.Coli
Bacteriodes fragilis
Immune cells calls WBC
Pus builds up in appendix