Liver Failure Flashcards
How is Alpha-1 Antitrypsin Deficiency inherited
AUTOSOMAL RECESSIVE
On what chromosome is alpha-1antitrypsin gene located
14
What is alpha-1-antitrypsin’s main function
PROTECT the lungs from neutrophil elastase which can disrupt its connective tissues
What two organs does alpha-1 antitrypsin effect
Lung and liver
What disease does Alpha-1 Antitrypsin Deficiency cause in the lungs
emphysema
What is emphysema
A condition sin which the alveoli enlarged - making it hard to breathe
What diseases does Alpha-1 Antitrypsin Deficiency cause in the liver
CIRRHOSIS
HEPATOCELLULAR CARCINOMA
What is the phenotype of symptomatic patients with Alpha-1 Antitrypsin Deficiency
PiZZ phenotype
Clinical presentation of Alpha-1 Antitrypsin Deficiency in children
Present as liver disease (hepatitis, cirrhosis)
Clinical presentation of Alpha-1 Antitrypsin Deficiency in adults
Presents as respiratory problems (dyspnoea)
When do adults develop cirrhosis from Alpha-1 Antitrypsin Deficiency
Over the age of 50
How do we diagnose Alpha-1 Antitrypsin Deficiency
Serum alpha-1 antiitrypsin levels are LOW
How is Alpha-1 Antitrypsin Deficiency treated
- NO treatment
- Treat complications of liver disease
- Stop smoking
- Manage emphysema
- Liver transplant
What is hepatic failure
When liver loses ability to regenerate or repair so that decompensation occurs
What is ACUTE hepatic failure
Acute liver injury with encephalopathy and deranged coagulation in a patient with a perviously normal liver
What is Acute-on-chronic hepatic failure
Liver failure as a result of decompensation of chronic liver disease
Signs of hepatic encephalopathy caused by hepatic failure
- Confusion
- Coma
- Liver flap
- Drowsiness
- Jaundice
- Ascites
How does hepatic failure cause hepatic encephalopathy
- As liver fails, nitrogenous waste builds up in circulation and passes across BBB = brain damage due to ammonia (halts krebs’ cycle)
NEURAL CELL DEATH
How does hepatic failure cause cerebral oedema
Astrocytes try to clear the ammonia (using a process involving glutamate -> glutamine), excess glutamine causes osmotic imbalance and a shift of fluid into these cells causing cerebral oedema
What is Fulminant hepatic failure
- Massive necrosis of liver cells leading to severe impairment of liver function
What is hyper acute fulminant hepatic failure
Encephalopathy within 7 days of jaundice onset
What is acute Fulminant hepatic failure
Encephalopathy within 8-28 days of jaundice onset
What is subacute Fulminant hepatic failure
within 5-26 weeks
What disease usually causes fulminant hepatic failure
Acute Hepatitis
What is the most common cause for Fulminant hepatic failure
Paracetamol overdose
Histological aspect of Fulminant hepatic failure
Multiacinar necrosis involving a substantial part of the liver
Main causes of Fulminant hepatic failure
- Virus (Hep A,B,D,E - RARELY C)
- Cytomegalovirus
- EBV
- Heroes simplex virus
- PARACETAMOL
- Alcohol
- Amitriptyline
- NSAIDS
- ECSTACY
- HEPATOCELLULAR CARCINOMA
- Wilson’s Disease or Alpha-1-antitrypsin deficiency
- Acute fatty liver or pregnancy
Clinical presentation of Fulminant hepatic failure
- Jaundiced
- Small liver
- Signs of hepatic encephalopathy
- Fetor hepaticas
- Cerebral Oedema
- Signs of chronic liver disease
- Fever, vomiting, hypertension
Grading of HEPATIC ENCEPHALOPATY
I - Altered mood/Behaviour, sleep disturbance, dyspraxia
II - Increasing drowsiness, confusion, slurred speech +/- liver flap, inappropriate behaviour/personality change
III - Incoherent, restless, liver flap, stupor
IV - Coma
Sign of fetor hepaticus
Patient smells like pear drops
What are the signs of chronic liver disease
- Bruising
- Clubbing
- Dupuytren’s contracture
- Ascites (RARE)
- Fever, committing and hypertension
What would neurological examination show in liver failure
Spasticity
Hyper-reflexia
Plantar responses remain flexor
Differential diagnosis in liver failure
- Space occupying lesions in th ebrian
- Cerebral infection
- Drug or alcohol intoxication
- Hypoglycaemia, electrolyte imbalance or hypoxia
Blood test in liver failure
- Hyperbilirubinaemia
- High serum ALT + AST
- Low levels of coagulation factors and raising PTT
- Low glucose (glycogen is no longer being stored)
- Ammonia levels high
Name some imaging diagnostics of liver failure
EEG
Ultrasound
CXR
Dopper ultrasound
What will an EEG be used for
Grading encephalopathy
What will an ultrasound be used for
Define liver size
What does the doppler ultrasound show
Hepatic vein potency
Why would we be looking at blood cultures, urine cultures and ascitic taps for liver failure
Rule out infections by pathogens