Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

TAS Hepatology > Acute Liver Failure ✅ > Flashcards

Acute Liver Failure ✅ Flashcards

1
Q

What is acute liver failure defined as?

A

Development of hepatic necrosis in absence of pre-existing liver disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How might acute liver failure present in infancy?

A
  • Irritability
  • Sleepiness
  • Hypoglycaemia
  • Bruising, petechiae, or bleeding/oozing from venepuncture sites/umbilical stump
  • Jaundice
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the categories of causes of acute live failure in neonates/infants up to 6 months?

A
  • Infective
  • Metabolic
  • Poisoning
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the infective causes of acute liver failure in neonates/infants up to 6 months?

A
  • Septicaemia
  • Hepatitis B
  • Adenovirus
  • Echovirus
  • Coxsackie B
  • Herpes simplex
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the metabolic causes of acute liver failure in neonates/infants up to 6 months?

A
  • Neonatal haemchromotosis
  • Tyrosinaemia type 1
  • Mitochondrial disease
  • Fatty acid oxidation defects
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the categories of causes of acute liver failure in children >6 months?

A
  • Infection
  • Autoimmune
  • Drug
  • Metabolic
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the infectious causes of acute liver failure in over 6 month olds?

A
  • Hepatitis A/B/E
  • Epstein-Barr virus
  • Parvovirus B19
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the types of autoimmune disorders causing acute liver failure in children over 6 months?

A

Type I or II

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What drugs can cause acute liver failure in children over 6 months?

A
  • Paracetamol (in overdose)
  • Anti-epileptics, e.g. sodium valproate, carbamazepine
  • Isoniazid
  • Halothane
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What metabolic conditions can cause acute liver failure in over 6 months?

A
  • Wilson’s disease

- Alper’s disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is neonatal haemochromotosis?

A

Iron storage disorder

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

In what form does iron exist in the body?

A

As transferrin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is transferrin?

A

Iron bound to apotransferrin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Where is most of the iron used for haemoglobin production obtained from?

A

Haemoglobin breakdown of senescent red blood cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How is haemoglobin released from RBCs?

A

They are phagocytosed by macrophages. Proteolytic enzymes in the macrophages degrade the ingested cells, and release haem and globin molecules as separate entities

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Where are RBCs phagocytosed by macrophages?

A
  • Spleen
  • Liver
  • Bone marrow
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What happens to the globin molecules released from phagocytosed RBCs?

A

They are broken down into amino acids and used for protein production

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What happens to the haem molecules released from phagocytosed RBCs?

A

Iron is released from haem, leaving a porphyrin ring, which is converted to bilirubin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What can lead to accumulation of iron in the liver?

A

Failure of processes of RBC breakdown

Excessive iron absorption

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are the clinical features of iron accumulation in the liver?

A
  • Raised transaminases
  • Conjugated jaundice
  • Hypoalbuminaemia
  • Severe coagulopathy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What kind of disorder is neonatal haemochromatosis considered to be?

A

Alloimmune disorder

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is the pathological process behind neonatal haemochromotosis?

A

Mothers develop an abnormal immune response to fetal liver protein, which results in liver damage and a direct or indirect effect on fetal iron storage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

How does the number of pregnancies a mother has had affect the incidence of neonatal haemochromotosis?

A

It is rare in first pregnancies, but higher in subsequent

25
Q

What will be found on iron studies in neonatal haemochromotosis?

A
  • High serum iron
  • Hypersaturation of iron-binding capacity
  • Elevated ferritin
26
Describe the clinical course of neonatal haemochromotosis?
Progressive, with death from liver failure without transplantation
27
What is the diagnosis of neonatal haemochromotosis based on?
Demonstrating iron storage outside of the liver, e.g. salivary glands, pancreas, or brain on MRI
28
When does galactosaemia present?
Typically in early neonatal period
29
How does galactosaemia present?
- Sepsis - Liver failure - ‘Oil drop’ cataracts on opthalmoscopy
30
What is the inheritance of galactosaemia?
Autosomal recessive
31
What is the pathological process in galactosaemia?
Deficiency of galactose-1-phosphate uridyltransferase
32
How is a diagnosis of galactosaemia made?
- Suggested by detection of urinary reducing substances without glycosuria - Confirmed by demonstrating reduced enzyme activity in blood (Gal-1-PUT)
33
When might misleading results be obtained when testing for galactosaemia?
In presence of recent blood transfusion
34
What organs are particularly affected by mitochondrial disease?
Those that need large amounts of energy; - Brain - Heart - Liver - Renal tubules
35
How might neonates with mitochondrial disease present?
- Acute liver failure - Seizures - Cardiomyopathy - Renal failure
36
How might older children with mitochondrial disease present?
- Hepatic steatohepatitis - Cholestasis - Cirrhosis
37
What is a key feature of mitochondrial diseases?
Lactic acidaemia
38
Is liver transplasntation used in mitochondrial disease?
May be used if disease confined to liver, but contraindicated if multi-organ involvement
39
How might acute liver failure present in an older child?
- Jaundice - Encephalopathy - Coagulopathy
40
What complications may arise from acute liver failure?
- Cerebral oedema - Coagulopathy - Systemic inflammatory response syndrome - Hyponatraemia - Hypotension
41
What does cerebral oedema caused by liver failure lead to?
- Hepatic encephalopathy - Coma - Brain herniation - Death
42
How severe is hepatic encephalopathy?
Varies from subtle deficit in higher brain function, e.g. mood, concentration in grade I encephalopathy, to deep coma in grade IV encephalopathy
43
Which patients are at highest risk of developing cerebral oedema and grade IV encephalopathy?
Patients presenting with acute and hyperacute liver failure
44
What causes cerebral oedema and encephalopathy in acute liver failure?
Exact pathogenesis unclear, but likely to be consequence of; - Accumulation of toxins, e.g. ammonia, which affects neurotransmitter levels and neuroreceptor activation - Impairment of autoregulation of cerebral blood flow, resulting in anaerobic glycolysis and oxidative stress - Inflammatory mediatiators
45
Why do you get coagulopathy in acute liver failure?
Due to the livers role in production of coagulation factors
46
What measure of coagulation is used to monitor severity of hepatic injury?
PT time (prolonged in acute liver failure)
47
What happens to platelets in acute liver failure?
Platelet dysfunction, with loss of larger and more active platelets
48
In what % of patients with acute liver failure is systemic inflammatory response syndrome seen in?
Up to 60%
49
What does SIRS lead to?
Multi-organ failure
50
What causes SIRS in acute liver failure?
Impaired immune function, increasing the risk of sepsis
51
How is SIRS prevented in acute liver failure?
Patients are treated routinely with antibiotics and antifungals
52
How does acute liver failure cause hyponatraemia?
- Water retention in response to hypoalbuminaemia | - Shift in intracellular sodium transport from inhibition of Na/K-ATPase
53
What other electrolyte abnormalities are often seen in acute liver failure?
- Hypokalaemia - Hypophosphataemia - Metabolic alkalosis
54
Why is hypotension seen in acute liver failure?
- Peripheral vascular dilatation and reduced systemic vascular resistance - Tissue hypoxia and lactic acidosis
55
How is hypotension in acute liver failure compensated for?
Cardiac output increases
56
What are the indications for transplant in acute liver failure?
- Worsening coagulopathy, which is unsupported by blood products and not responsive to vitamin K - Worsening encephalopathy
57
What are the contraindications for transplant in acute liver failure?
- Multisystem disease - Irreversible brain damage - Pre-existing life-limiting disorders
58
What mutations have been identified as causing mitochondrial disease?
Mutations in nuclear genes such as SCO1, BCS1L, POLG, DGUOK, and MPV17, and deletion or rearrangement of mitochondrial DNA

TAS Hepatology (10 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions