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Systems: Paediatrics AB > Liver Problems > Flashcards

Liver Problems Flashcards

1
Q

What are the functions of the liver?

A
  • Factory for producing essential proteins including albumin
  • Produces clotting factors
  • Essential role in absorption and metabolism of fats and other food stuffs
  • Storage of glycogen, minerals and other substrates
  • Metabolism and excretion of toxic products in the body
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2
Q

What is included in LFTs?

A

-Bilirubin (total and split)
-ALT/AST (alanine aminotransferase/aspartate aminotransferase)
-Alkaline phosphatase
Gamma glutamyl transferase (GGT)

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3
Q

When is ALT/AST elevated?

A

In hepatocellular damage (hepatitis)

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4
Q

When are alkaline phosphatase and GGT elevated?

A

Biliary disease

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5
Q

What tests are used to assess the function of the liver?

A
  • Coagualtion (prothrombin time (PT)/INR, APTT)
  • Albumin
  • Bilirubin
  • Blood glucose
  • Ammonia
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6
Q

How can paediatric liver disease manifest?

A
  • JAUNDICE
  • Incidental finding of abnormal blood test
  • Symptoms/signs of chronic liver disease
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7
Q

What are the signs of chronic liver disease in children?

A
  • Encephalopathy
  • Jaundice
  • Epistaxis
  • Cholestasis
  • Ascites
  • Hypotonia
  • Peripheral neuropathy
  • Rickets secondary to vit D deficiency
  • Varices with portal hypertension
  • Spider naevi
  • Muscle wasting
  • Bruising and petechiae
  • Spleomegaly
  • Hypersplenism
  • Hepatorenal failure
  • Liver palms
  • Clubbing
  • Loss of fat stores
  • Growth failure
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8
Q

What is jaundice?

A
  • Yellow discolouration of skin and tissues due to accumulation of bilirubin
  • Usually most obvious in sclera
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9
Q

When is jaundice usually visible?

A

Total bilirubin >40-50umol/l

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10
Q

What is diagnosis of infant jaundice dependent on?

A
  • Bilirubin metabolism

- Age

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11
Q

How is bilirubin metabolised?

A
  • Post mature RBC broken down in the reticuloendothelial system to bilveridan
  • Converted to unconjugated bilirubin
  • Bound to albumin and conjugated in the liver
  • Mixes with bile in gallbladder and enters small intestine
  • Coverted to urobilinogen and excreted by the kidneys as urine or in faeces
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12
Q

What is the solubility of conjugated bilirubin?

A
  • Water soluble

- Fat insoluble

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13
Q

What is the solubility of unconjugated bilirubin?

A
  • Fat soluble

- Water insoluble

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14
Q

When does pre-hepatic jaundice occur?

A

Excess of unconjugated birlirubin

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15
Q

When does intrahepatic jaundice occur?

A

-Excess of conjugated and unconjugated bilirubin jaundice due to issue within the liver

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16
Q

When does post-hepatic jaundice occur?

A

Excess of conjugated bilirubin normally due obstruction

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17
Q

What is early neonatal jaundice?

A
  • <24 hours old

- ALWAYS PATHOLOGICAL

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18
Q

What can cause early neonatal jaundice?

A
  • Haemolysis

- Sepsis

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19
Q

What is intermediate neonatal jaundice?

A

Occurs between 24hours and 2 weeks of age

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20
Q

What can cause intermediate neonatal jaundice?

A
  • Physiological
  • Breast milk
  • Sepsis
  • Haemolysis
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21
Q

What is prolonged neonatal jaundice?

A

Occurs after 2 weeks of age

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22
Q

What can cause prolonged neonatal jaundice?

A
  • Extrahepatic obstruction
  • Neonatal hepatitis
  • Hypothyroidism
  • Breast milk
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23
Q

Why does physiological jaundice occur?

A
  • Shorter RBC life span in infants (80-90 days)
  • Relative polycythaemia
  • Relative immaturity of liver function
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24
Q

What type of jaundice is physiological jaundice?

A

Unconjugated

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25
Q

When does physiological jaundice occur?

A

After the 1st day of life as it takes time for RBC to break down

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26
Q

Why does jaundice occur with breast fed babies?

A
  • Exact reason for prolongation of jaundice in breastfed infants unclear
  • Inhibition of UDP by progesterone metabolite?
  • Increased enterohepatic circulation?
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27
Q

What type of jaundice is breast milk jaundice?

A

Unconjugated

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28
Q

How long can breast milk jaundice persist?

A

Up to 12 weeks from birth

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29
Q

Apart from breast milk and physiological what other causes of unconjugated infant jaundice are there?

A
  • Sepsis
  • Haemolysis (excessive)
  • Abnormal conjugation
30
Q

Why might there be excessive haemolysis in a baby leading to jaundice?

A
  • ABO incompatibility
  • Rhesus disease
  • Bruising/cephalhaematoma
  • Red cell membrane defects (e.g. spherocytosis)
  • Red cell enzyme defects (e.g. G6PD)
31
Q

What causes of abnormal conjugation are there?

A
  • Gilbert’s disease (common, mild)

- Crigler-Najjar syndrome (very rare but sever)

32
Q

What investigation would you do for sepsis causing jaundice?

A
  • Urine culture
  • Blood culture
  • TORCH screen
33
Q

What investigation would you do for ABO incompatibility causing jaundice?

A
  • Blood group

- DCT

34
Q

What investigation would you do for Rhesus disease causing jaundice?

A
  • Blood group

- DCT

35
Q

What investigation would you do for bruising/cephalhaematoma causing jaundice?

A

Clinical examination

36
Q

What investigation would you do for red cell membrane defects causing jaundice?

A

Blood film

37
Q

What investigation would you do for red cell enzyme defects causing jaundice?

A

G6PD assay

38
Q

What investigation would you do for Gilbert’s disease causing jaundice?

A

Genotype/phenotype

39
Q

What investigation would you do for Crigler-Najjar syndorme causing jaundice?

A

Genotype/phenotype

40
Q

What is a possible complication of unconjugated jaundice?

A

Kernicterus

41
Q

Why does kernicterus occur?

A
  • Unconjugated bilirubin is fat-soluble (water insoluble) so can cross blood-brain barrier
  • It is neurotoxic and deposits in brain (particularly the basal ganglia)
42
Q

What are the early signs of kernicterus?

A
  • Encephalopathy
  • Poor feeding
  • Lethargy
  • Seizures
43
Q

What are the late signs of kernicterus?

A
  • Severe choreoathetoid cerebral palsy
  • Learning difficulties
  • Sensorineural deafness
44
Q

How is unconjugated jaundice treated?

A

Phototherapy

  • Visible light (450nm wavelength) (not UV) converts bilirubin to water soluble isomer (photoisomerisation)
  • Threshold for phototherapy in infants guided by charts
45
Q

What is prolonged infant jaundice?

A

Jaundice persisting beyond 2 weeks of life or 3 weeks of life in preterms

46
Q

What are the conjugated causes of prolonged infant jaundice?

A
  • Anatomical (biliary obstruction)

- Neonatal hepatitis

47
Q

What are the unconjugated causes of prolonged infant jaundice?

A
  • Hypothyroidism

- Breast-milk jaundice

48
Q

What causes of biliary obstruction can lead to prolonged jaundice?

A
  • Biliary atresia
  • Choledochal cyst
  • Alagille syndrome
49
Q

What is biliary atresia characterised by?

A

Conjugated jaundice with pale stools

50
Q

What is choledochal cyst characterised by?

A

Conjugated jaundice with pale stools

51
Q

What is Alagille syndrome characterised by?

A
  • Intrahepatic cholestasis
  • Dysmorphism
  • Congenital cardiac disease
52
Q

What is biliary atresia?

A

Congenital fibro-inflammatory disease of the bile ducts leading to destruction of extra-hepatic bile ducts

53
Q

How does biliary atresia present?

A
  • Prolonged conjugated jaundice

- Pale stools and dark urine

54
Q

What can biliary atresia progress to?

A

Liver failure if not identified and treated (can occur within months of life)

55
Q

What is the most common indication for liver transplant in children?

A

Biliary atresia

56
Q

What is the treatment for biliary atresia?

A

Kasai portoenterostomy

  • Success rate diminishes rapidly with age
  • Best results if performed before 60 days (<9 weeks)
  • Small bowel divided and extrahepatic ducts removed. Liver bile ducts plugged directly into the small bowel
57
Q

What investigations are done for biliary atresia?

A
  • Split bilirubin
  • Stool colour
  • Ultrasound
  • liver biopsy
58
Q

What investigations are done fro choledochal cyst?

A
  • Split bilirubin
  • Stool colour
  • Ultrasound
59
Q

What investigations are done for Alagille syndrome?

A
  • Dysmorphism

- Genotype

60
Q

What causes of neonatal hepatitis are there?

A
  • Alpha-1-antitrypsin deficiency
  • Galactosaemia
  • Tyrosinaemia
  • Urea cycle defects
  • Haemochromatosis
  • Glycogen storage disorders
  • Hypothyroidism
  • Viral hepatitis
  • Parenteral nutrition
61
Q

How is alpha-1 antitrypsin deficiency investigated?

A

Phenotype/level

62
Q

How is galactosaemia investigated?

A

GAL-1-PUT

63
Q

How is tyrosinaemia investigated?

A

Amino acid profile

64
Q

How are urea cycle defects investigated?

A

Ammonia levels

65
Q

How is haemochromotosis investigated?

A
  • Iron studies

- Liver biopsy

66
Q

How are glycogen storage disorders investigated?

A

Biopsy

67
Q

How is hypothyroidism investigated?

A

TFTs

68
Q

How is viral hepatitis investigated?

A
  • Serology

- PCR

69
Q

How is parenteral nutrition investigated?

A

History

70
Q

What should you always ask about with prolonged infant jaundice?

A

Stool colour

71
Q

What is the most important test fro prolonged infant jaundice?

A

Split bilirubin to determine if it is conjugated or unconjugated in nature

72
Q

What is the main diagnosis to exclude with conjugate prolonged jaundice?

A

Biliary atresia

Systems: Paediatrics AB (31 decks)

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