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Paediatrics > Liver disease > Flashcards

Liver disease Flashcards

1
Q

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

A

Prolonged aka persistent jaundice

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2
Q

What is the definition of prolonged neonatal jaundice

A

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

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3
Q

What is the cause of normal neonatal jaundice

A

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

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4
Q

What sort of jaundice is caused by liver disease?

A

Raised conjugated bilirubin

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5
Q

Signs of neonatal liver disease

A

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

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6
Q

What is biliary atresia?

A

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

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7
Q

Incidence of biliary atresia

A

1 in 14,000 live births

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8
Q

Signs of biliary atresia x6

A 
Normal birthweight but failure to thrive
Mildly jaundice
Pale stools
Dark urine
Hepatomegaly 
Splenomegaly following portal hypertension
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9
Q

Liver function tests in biliary atresia

A

Little use diagnostically

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10
Q

Radioisotope scan in biliary atresia

A

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

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11
Q

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

A

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

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12
Q

What is often needed with biliary atresia?

A

Liver transplant

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13
Q

What are choledochal cysts?

A

Cystic dilatations of the extrahepatic biliary system causing obstructive jaundice

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14
Q

Signs of choledochal cysts in adults and children

A

Jaundice and cholestasis in children

Adults abdominal pain, palpable mass and jaundice/cholangitis

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15
Q

Treatment of choledochal cysts

A

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

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16
Q

Future complications of choledochal cysts

A

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

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17
Q

What happens in neonatal hepatitis syndrome?

A

Prolonged neonatal jaundice and hepatic inflammation

Born with IUGR and hepatosplenomegaly

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18
Q

Causes of neonatal hepatitis syndrome?

x 10

A 
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
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19
Q

Inheritance and incidence of alpha-1 antitrypsin deficiency?

A

Autosomal recessive

1 in 2000-4000 in UK

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20
Q

Presentation of children with alpha-1 antitrypsin x4

A

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

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21
Q

What % of children with alpha-1 have good prognosis

A

50%

Others develop liver disease and may require transplantation

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22
Q

What is galactosaemia

A

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

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23
Q

Incidence of galactosaemia

A

1 in 40,000 - therefore rare

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24
Q

Presentation of galactosaemia x4

A

Poor feeding, vomiting, jaundice and hepatomegaly when fed milk

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25
Q

What is inevitable if galactosaemia is untreated? x3

A

Liver failure
Cataracts
Developmental delay

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26
Q

What may occur with untreated galactosaemia?

A

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

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27
Q

Diagnosis for galactosaemia

A

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

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28
Q

What is treatment for galactosaemia and what does it lead to

A

Galactose free diet prevents progression of liver disease

But ovarian failure and learning difficulties may occur later

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29
Q

How do you detect errors of bile acid synthesis

A

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

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30
Q

Treatment of errors of bile acid synthesis

A

Ursodeoxycholic acid

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31
Q

Sign of intrahepatic cholestasis

A

Pruritus

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32
Q

What is Alagille syndrome?

A

Rare autosomal dominant condition
Triangular facies, skeletal abnormalities, congenital heart disease, renal tubular disorders, defects in eye and intrahepatic biliary hypoplasia - severe pruritus and failure to thrive

Prognosis variable

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33
Q

What is liver problem in downs syndrome

A

Intrahepatic biliary hypoplasia therefore pruritus

34
Q

Clinical features of viral hepatitis x8

A 
Nausea and vomiting
Abdominal pain 
Lethargy 
Jaundice (30-50% of children don't get jaundice)
Tender hepatomegaly 
30% splenomegaly 
Liver transaminases are elevated 
Coagulation is normal
35
Q

Features of HAV (Hep A virus)

A

Faecal-oral transmission
Children have mild illness and recover 2-4 weeks
No chronic liver disease
No treatment but close contacts vaccinated

36
Q

Features of HBV

A

Major cause of acute and chronic hepatitis
Perinatal transmission can occur (or other blood routes for transmission)
Infants contracting HBV are asymptomatic but 90% become chronic carriers

37
Q

Features of chronic hep B

A

30-50% of children get chronic HBV liver disease and 10% get cirrhosis
Long-term risk of HCC

38
Q

Treatment of chronic HBV x2

A

Interferon successful in 50% of children infected horizontally and 30% infected perinatally
Antiviral therapy effective in some but limited by development of resistance

39
Q

Prevention of HBV

A

Vaccinate all babies born to HBsAg +ve mothers

Give to mothers if they are e antigen +ve

40
Q

Features of HCV

A

Can be transmitted vertically - more common is there is co-infection with HIV
Seldom causes acute infection
Majority become chronic carriers
20-25% lifetime risk of cirrhosis or HCC

41
Q

Treatment of HCV

A

Pegylated interferon and ribavirin
Effective 90% of children cases
Not taken before 4 years as may resolve spontaneously

42
Q

What is Non-A to G hepatitis

A

Presents similarly to Hep A and when viral aetiology of hepatitis is suspected but not identified

43
Q

What is acute liver failure/fulminant hepatitis in children?

A

Development of massive hepatic necrosis with subsequent loss of liver function
With or without hepatic encephalopathy

44
Q

Most common causes of acute liver failure in children x3

A

Paracetamol overdose
Non-A to G Hepatitis
Metabolic conditions

45
Q

How do children with acute liver failure present x5

A

Within hours or weeks

With jaundice, encephalopathy, coagulopathy, hypoglycaemia and electrolyte disturbance

46
Q

Complications of acute liver failure x4

A

Cerebral oedema
Haemorrhage from gastritis or coagulopathy
Sepsis
Pancreatitis

47
Q

Diagnosis of acute liver failure x5

A 
Bilirubin may be normal in early stages 
Transaminases very high 
ALP increased 
Coagulation is v.abnormal 
Plasma ammonia elevated
48
Q

Management of acute liver failure x4

A

Blood glucose maintenance
Antibiotics to prevent sepsis
IV vit k, plasma - prevent haemorrhage
Fluid restrict and mannitol diuresis for cerebral oedema

49
Q

What is Reye Syndrome?

A

Acute non-inflammatory encephalopathy

Microvesicular fatty infiltration of liver

50
Q

What is Reye syndrome associated with?

A

Aspirin therapy - has virtually disappeared since stopped giving aspirin to children

51
Q

Common causes of chronic liver disease/hepatitis

A

Hepatitis virus (B or C)
Autoimmune hepatitis
Always need to exclude Wilson disease

52
Q

Mean age of presentation of autoimmune hepatitis

A

7-10 years

53
Q

Gender occurrence of autoimmune hepatitis

A

More common in girls

54
Q

Presentation of autoimmune hepatitis

A

Can either present as acute hepatic failure, acute hepatitis or chronic liver disease with extra autoimmune features (skin rash, lupus, arthritis etc)

55
Q

What else can children with autoimmune hepatitis have?

A

Inflammatory bowel disease, coeliac disease or other autoimmune disease

56
Q

Treatment of autoimmune hepatitis

A

90% of children respond to prednisolone and azathioprine

57
Q

Occurrence of liver disease in cystic fibrosis

A

2nd most common cause of death after respiratory disease

58
Q

What is the most common liver abnormality in cystic fibrosis

A 
Hepatic steatosis (fatty liver) 
May be associated with protein energy malnutrition or micronutrient deficiencies
59
Q

Progression of fatty liver in cystic fibrosis patients

A

Does not generally progress and treatment involves ensuring optimal nutritional support

60
Q

More serious liver complications in cystic fibrosis

A

Thick tenacious bile - progressive biliary fibrosis

Cirrhosis and portal hypertension in 20% of children by mid-adolescence

61
Q

What is Wilson disease?

A

Autosomal recessive disorder
Reduced synthesis of caeruloplasmin (copper-binding protein) with defective excretion of copper in bile
Therefore copper accumulates in liver, brain, kidney and cornea

62
Q

Age of presentation of wilson disease and type of presentation

A

Rarely under age 3
Presentation in childhood - liver presentation is most likely
In 2nd decade - neuropsychiatric features more common

63
Q

Liver presentation of wilson disease

A

Can present with virtually any liver disease

64
Q

Other than liver and neuropsychiatric what else happens in wilson disease x3

A

Renal tubular dysfunction
Vit D resistant rickets
Haemolytic anaemia

65
Q

When are Kayser-Fleischer rings seen in wilson disease?

A

Not before age 7

66
Q

Diagnosis of Wilson disease x3

A

Low serum caeruloplasmin and copper is characteristic but not universal
Urinary copper excretion is increased - increases further with chelating agent pencillamine
Dx with elevated hepatic copper on liver biopsy or identification of gene mutation

67
Q

Treatment of wilson disease

A

Penicillamine or trientine

Promote urinary copper excretion

68
Q

What is given in wilson disease to reduce copper absorption

A

Zinc

69
Q

What is given in wilson disease to prevent peripheral neuropathy

A

Pyridoxine - improvement may take 12 months

70
Q

Features of non-alcoholic fatty liver disease in children

A 
Often obese
Asymptomatic but may have vague RUQ pain or lethargy 
Often detected incidentally
Rarely get cirrhosis 
Treat with weight loss
71
Q

Features of liver cirrhosis x5

A 
Diminished hepatic function 
Portal hypertension and splenomegaly 
Varices
Ascites 
HCC may develop
72
Q

How can cirrhosis be in children

A

If compensated, liver function may be normal and features will only show when decompensate

73
Q

How best to diagnosis oesophageal varices

A

OGD

Barium swallow may miss small ones

74
Q

Treatment of oesophageal varices x5

A

Acutely - blood transfusion and H2 blockers or omeprazole

If persists - octreotide infusion, vasopressin analogues, sclerotherapy or band ligation

75
Q

Pathophysiology of ascites x4

A

Hypoalbuminaemia
Sodium retention
Renal impairment
Fluid redistribution

76
Q

When suspect spontaneous bacterial peritonitis?

A

Undiagnosed fever, abdominal pain, tenderness
or
unexplained deterioration in hepatic or renal function

77
Q

Malnutrition in liver disease x4

A

Protein malnutrition
Fat malabsorption
Anorexia
Fat soluble vitamin deficiency (vit a, d, e k)

78
Q

Treatment of malnutrition in liver disease

A

High-protein, high-carb diet
50% more calories than recommended
Can give medium chain triglycerides if cholestasis to provide fat but long chain triglycerides are required for fatty acids

79
Q

What does Vit a deficiency cause

A

night blindness in adults and retinal changes in children

80
Q

What does Vit e deficiency x 3 cause

A

Peripheral neuropathy, haemolysis and ataxia

Paediatrics (16 decks)

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