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Flashcards in Liver disease Deck (80)
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1

What is the most common presentation of liver disease in neonatal period?

Prolonged aka persistent jaundice

2

What is the definition of prolonged neonatal jaundice

Jaundice after 2 weeks in term babies or 3 weeks in pre-term babies

3

What is the cause of normal neonatal jaundice

Unconjugated hyperbilirubinaemia as fetal haemoglobin replaced with adult haemoglobin - resolves spontaneously

4

What sort of jaundice is caused by liver disease?

Raised conjugated bilirubin

5

Signs of neonatal liver disease

Pale stools, dark urine, bleeding tendency and failure to thrive

6

What is biliary atresia?

Progressive disease with destruction or absence of extrahepatic biliary tree and intrahepatic biliary ducts

7

Incidence of biliary atresia

1 in 14,000 live births

8

Signs of biliary atresia x6

Normal birthweight but failure to thrive
Mildly jaundice
Pale stools
Dark urine
Hepatomegaly
Splenomegaly following portal hypertension

9

Liver function tests in biliary atresia

Little use diagnostically

10

Radioisotope scan in biliary atresia

with TIBIDA shows good uptake by liver but no excretion into bowel - showing obstructive problem

11

Treatment of biliary atresia and when does it need to be done

Kasai procedure - hepatoportoenterostomy - if performed before age of 60 days - 80% success rate

12

What is often needed with biliary atresia?

Liver transplant

13

What are choledochal cysts?

Cystic dilatations of the extrahepatic biliary system causing obstructive jaundice

14

Signs of choledochal cysts in adults and children

Jaundice and cholestasis in children
Adults abdominal pain, palpable mass and jaundice/cholangitis

15

Treatment of choledochal cysts

Surgical excision of the cyst - formation of a Roux-en-Y anastomosis to biliary duct

16

Future complications of choledochal cysts

Cholangitis and 2% risk of malignancy in any part of the biliary tree

17

What happens in neonatal hepatitis syndrome?

Prolonged neonatal jaundice and hepatic inflammation
Born with IUGR and hepatosplenomegaly

18

Causes of neonatal hepatitis syndrome?
x 10

Often no cause found
Congenital infection
Inborn errors of metabolism
Alpha 1 antitrypsin
Galactosaemia
Tyrosinaemia
Errors of bile acid synthesis
Progressive familial intrahepatic cholestasis
Cystic fibrosis
Intestinal failure associated liver disease associated with long-term parenteral nutrition

19

Inheritance and incidence of alpha-1 antitrypsin deficiency?

Autosomal recessive
1 in 2000-4000 in UK

20

Presentation of children with alpha-1 antitrypsin x4

Prolonged neonatal jaundice or less commonly bleeding due to vit k deficiency
Also have hepatomegaly
Splenomegaly develops

21

What % of children with alpha-1 have good prognosis

50%
Others develop liver disease and may require transplantation

22

What is galactosaemia

Inability to digest galactose or lactose therefore it builds up in body and causes widespread damage

23

Incidence of galactosaemia

1 in 40,000 - therefore rare

24

Presentation of galactosaemia x4

Poor feeding, vomiting, jaundice and hepatomegaly when fed milk

25

What is inevitable if galactosaemia is untreated? x3

Liver failure
Cataracts
Developmental delay

26

What may occur with untreated galactosaemia?

Rapidly fatal course with shock, haemorrhage and DIC with gram-negative sepsis

27

Diagnosis for galactosaemia

Measuring enzyme galactose-1-phosphate-uridyl transferase in red cells

28

What is treatment for galactosaemia and what does it lead to

Galactose free diet prevents progression of liver disease
But ovarian failure and learning difficulties may occur later

29

How do you detect errors of bile acid synthesis

If neonatal cholestasis and normal GGT - screen for elevated cholenoic bile acids in urine

30

Treatment of errors of bile acid synthesis

Ursodeoxycholic acid