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