Paediatrics > Liver Problems COPY > Flashcards

Liver Problems COPY Flashcards

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1
Q

What are the functions of the liver?

A
  • Produces essential proteins including albumin and clotting factor
  • Absorption and metabolism of fat, carbahydrate and protein
  • Storage of glycogen, vitamins and minerals
  • 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
  • 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 - best test for liver function
    • prothrombin time (PT) / INR
    • APTT
  • Albumin
  • Bilirubin
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6
Q

How can paediatric liver disease manifest?

A
  • Jaundicece - usually most obvious in sclera
  • 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
  • Cholestasis
  • Ascites
  • Rickets secondary to vit D deficiency
  • Varices with portal hypertension
  • Spleomegaly
  • Finger clubbing
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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 >40-50 micromol/l

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10
Q

What is infant jaundice classification dependent on?

A
  • Bilirubin metabolism
    • Pre-hepatic, hepatic, post hepatic
  • Age
    • Early (<24 hours), Intermediate (24 hours to 2 weeks), Prolonged (>2 weeks)
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11
Q

What is the solubility of conjugated and unconjugated bilirubin?

A
  • Water soluble
  • Fat insoluble
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12
Q

When does pre-hepatic jaundice occur?

A

Excess of unconjugated birlirubin

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13
Q

When does intrahepatic jaundice occur?

A

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

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14
Q

When does post-hepatic jaundice occur?

A

Excess of conjugated bilirubin normally due obstruction

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15
Q

What is early neonatal jaundice?

A

-<24 hours old -ALWAYS PATHOLOGICAL

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16
Q

What can cause early neonatal jaundice?

A

-Haemolysis -Sepsis

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17
Q

What is intermediate neonatal jaundice?

A

Occurs between 24hours and 2 weeks of age

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18
Q

What can cause intermediate neonatal jaundice?

A

-Physiological -Breast milk -Sepsis -Haemolysis

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19
Q

What is prolonged neonatal jaundice?

A

Occurs after 2 weeks of age

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20
Q

What can cause prolonged neonatal jaundice?

A

-Extrahepatic obstruction -Neonatal hepatitis -Hypothyroidism -Breast milk

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21
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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22
Q

What type of jaundice is physiological jaundice?

A

Unconjugated

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23
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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24
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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25
What type of jaundice is breast milk jaundice?
Unconjugated
26
How long can breast milk jaundice persist?
Up to 12 weeks from birth
27
Apart from breast milk and physiological what other causes of unconjugated infant jaundice are there?
-Sepsis -Haemolysis (excessive) -Abnormal conjugation
28
Why might there be excessive haemolysis in a baby leading to jaundice?
-ABO incompatibility -Rhesus disease -Bruising/cephalhaematoma -Red cell membrane defects (e.g. spherocytosis) -Red cell enzyme defects (e.g. G6PD)
29
What causes of abnormal conjugation are there?
-Gilbert's disease (common, mild) -Crigler-Najjar syndrome (very rare but sever)
30
What investigation would you do for sepsis causing jaundice?
-Urine culture -Blood culture -TORCH screen
31
What investigation would you do for ABO incompatibility causing jaundice?
-Blood group -DCT
32
What investigation would you do for Rhesus disease causing jaundice?
-Blood group -DCT
33
What investigation would you do for bruising/cephalhaematoma causing jaundice?
Clinical examination
34
What investigation would you do for red cell membrane defects causing jaundice?
Blood film
35
What investigation would you do for red cell enzyme defects causing jaundice?
G6PD assay
36
What investigation would you do for Gilbert's disease causing jaundice?
Genotype/phenotype
37
What investigation would you do for Crigler-Najjar syndorme causing jaundice?
Genotype/phenotype
38
What is a possible complication of unconjugated jaundice?
Kernicterus
39
Why does kernicterus occur?
-Unconjugated bilirubin is fat-soluble (water insoluble) so can cross blood-brain barrier -It is neurotoxic and deposits in brain (particularly the basal ganglia)
40
What are the early signs of kernicterus?
-Encephalopathy -Poor feeding -Lethargy -Seizures
41
What are the late signs of kernicterus?
-Severe choreoathetoid cerebral palsy -Learning difficulties -Sensorineural deafness
42
How is unconjugated jaundice treated?
Phototherapy -Visible light (450nm wavelength) (not UV) converts bilirubin to water soluble isomer (photoisomerisation) -Threshold for phototherapy in infants guided by charts
43
What is prolonged infant jaundice?
Jaundice persisting beyond 2 weeks of life or 3 weeks of life in preterms
44
What are the conjugated causes of prolonged infant jaundice?
-Anatomical (biliary obstruction) -Neonatal hepatitis
45
What are the unconjugated causes of prolonged infant jaundice?
-Hypothyroidism -Breast-milk jaundice
46
What causes of biliary obstruction can lead to prolonged jaundice?
-Biliary atresia -Choledochal cyst -Alagille syndrome
47
What is biliary atresia characterised by?
Conjugated jaundice with pale stools
48
What is choledochal cyst characterised by?
Conjugated jaundice with pale stools
49
What is Alagille syndrome characterised by?
-Intrahepatic cholestasis -Dysmorphism -Congenital cardiac disease
50
What is biliary atresia?
Congenital fibro-inflammatory disease of the bile ducts leading to destruction of extra-hepatic bile ducts
51
How does biliary atresia present?
-Prolonged conjugated jaundice -Pale stools and dark urine
52
What can biliary atresia progress to?
Liver failure if not identified and treated (can occur within months of life)
53
What is the most common indication for liver transplant in children?
Biliary atresia
54
What is the treatment for biliary atresia?
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
55
What investigations are done for biliary atresia?
-Split bilirubin -Stool colour -Ultrasound -liver biopsy
56
What investigations are done fro choledochal cyst?
-Split bilirubin -Stool colour -Ultrasound
57
What investigations are done for Alagille syndrome?
-Dysmorphism -Genotype
58
What causes of neonatal hepatitis are there?
-Alpha-1-antitrypsin deficiency -Galactosaemia -Tyrosinaemia -Urea cycle defects -Haemochromatosis -Glycogen storage disorders -Hypothyroidism -Viral hepatitis -Parenteral nutrition
59
How is alpha-1 antitrypsin deficiency investigated?
Phenotype/level
60
How is galactosaemia investigated?
GAL-1-PUT
61
How is tyrosinaemia investigated?
Amino acid profile
62
How are urea cycle defects investigated?
Ammonia levels
63
How is haemochromotosis investigated?
-Iron studies -Liver biopsy
64
How are glycogen storage disorders investigated?
Biopsy
65
How is hypothyroidism investigated?
TFTs
66
How is viral hepatitis investigated?
-Serology -PCR
67
How is parenteral nutrition investigated?
History
68
What should you always ask about with prolonged infant jaundice?
Stool colour
69
What is the most important test fro prolonged infant jaundice?
Split bilirubin to determine if it is conjugated or unconjugated in nature
70
What is the main diagnosis to exclude with conjugate prolonged jaundice?
Biliary atresia
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