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Sydney Child Health Program > 4.1 Liver Disease - Paeds > Flashcards

4.1 Liver Disease - Paeds Flashcards

1
Q

Jaundiced neonate approach?

A 
Family history
Examine baby
Urine and stools for colour
-pale stools
-dipstick for bilirubin
Check newborn screen
FBE+film,(haemolysis), TFTs
Urine culture
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2
Q

What are the new born screen diseases that can cause jaundice?

A

CF
Galactosaemia
Phenylketonuria
Hypothyroidism

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3
Q

Prolonged unconjugated tested hyperbilirubinaemia in neonate definition?

A

Persistence of jaundice beyond 2nd weeks of life

  • total bilirubin raised
  • direct fraction <30umol/l, <20% total
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4
Q

Prolonged unconjugated tested hyperbilirubinaemia in neonate 2 main causes

A

Increased production

Decreased excretion

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5
Q

Prolonged unconjugated tested hyperbilirubinaemia in neonate causes of increased production

A

Haemolysis
-thesis, aBO, G6PD, -Pyruvate kinase—thalassemia
Extra vascular blood: cephalohematoma
Polycythemia

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6
Q

Prolonged unconjugated tested hyperbilirubinaemia in neonate kinds of reduced excretion?

A 
-hypothyroidism
UTI
Dehydration
Delayed meconium
Familial
Critter-Najarro syndrome
Breast-milk jaundice
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7
Q

Breast milk jaundice, how to diagnose?

A

Diagnosis of exclusion

  • usually serum bilirubin is not >340
  • reassure
  • no role of ceasing breast feeding or phenobarbitone
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8
Q

Conjugated bilirubinaemia/cholestasis is dangerous?

A

Always pathological

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9
Q

Cholestasis in the newborn, causes?

A

Infections
Metabolic diseases
Biliary diseases
Neonatal/giant cell hepatitis

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10
Q

Cholestasis in the newborn, which infections?

A 
TORCHS
Toxoplasmosis
Rubella
CMV
Herpes
Syphilis

Echo, Coxsackie
UTI
Septicaemia
Hep B,C(rare)

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11
Q

2 Most common metabolic disorders causing Cholestasis in the newborn?

A

Alpha-1-antitrypsin deficiency

Galactosaemia

CF
Neonatal iron storage disease

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12
Q

Biliary diseases causing Cholestasis in the newborn?

A
  • Biliary Atresia
  • Alagille’s syndrome (intrahepatic biliary hypoplasia)
  • Choledochal cyst
  • Pigment stone obstruction
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13
Q

Cholestasis workup?

A 
LFTs
U/S
DISIDA scan (for biliary atresia)
Exclude infectious/metabolic causes
Cholangiogram
Liver biopsy
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14
Q

Biliary atresia cause?

A

Unknown, not inherited

?environmental/infective

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15
Q

Epidemiology of biliary atresia

A

1 in 10 000

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16
Q

Treatment for biliary atresia

A
  • Porto-enterostormy (Kasai procedure)

- liver transplant

17
Q

Prognosis with Kasia procedure?

A

1/3 do well
1/3 well but complications
1/3 need liver transplantation

18
Q

Jaundice in order child, what to ask for in history?

A 
Viral? Contact Hx
Fix
Shellfish, oysters/hep A
Recent blood produces, six, injections
Recent ABx
Wild mushrooms?
19
Q

DDx of hepatitis in child?

A

-Infections
-Drugs: sulphur, antiepileptics, halothane, ecstasy, +++paracetamol
-Toxins: amanita phylloides
-Wilson’s
Autoimmune chronic hepatitis
-budd-chiari

20
Q

Infectious causes of hepatitis?

A 
Hep, A,B,C,E
EBV
CMV
HHV6
VZV, HSV
Echo, coxsackie
21
Q

Worry about Hep E where?

A

Pregnant women

22
Q

How to test of a child is in acute liver failure? Best:

A

Coags

23
Q

What do you do if child in liver failure and coags are abnormal?

A

Give Vit. K
Reassess
?transplantation

24
Q

What do you do if child in liver failure and coags are normal?

A

Viral serology

U/S - choledochal cyst

25
Q

What do you do if child in liver failure and coags are normal and viral serology is negative? 2 things

A 
Wilsons’s disease
-copper - caerulospamin
-KF rings
Urinary Cu
-biopsy

Autoimmune hepatitis

26
Q

Common causes of chronic liver disease in paeds?

A 
Alpha-1-antitrypsin deficiency
Wilson’s
Chronic hep B,C
Autoimmune
Sclerosing cholangitis
Biliary obstruction
CF
Budd chiari
Haemochromatosis(rare)
27
Q

Management of chronic liver disease in paeds?

A
  • Portal HT: varicella bleeding, asictes, hyperslenism
  • nutrition: vit, ADEK, albumin
  • sepsis, immunocompromised
  • renal, encephalopathy, pulmonary
  • itch
28
Q

Most common cause of liver transplantation in paeds?

A

50% biliary atresia

Chronic liver disease
Acute liver failure
Enzyme replacement

29
Q

What is the syndrome that can cause benign mild jaundice?

A

Gilbert’s Syndrome: 3% of pop

-partial deficient to of UDP glucuronyl transferase

30
Q

DDx of Gilbert syndrome?

A

Haemolysis

Sydney Child Health Program (113 decks)

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