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Liver disease Flashcards

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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
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
31
Sign of intrahepatic cholestasis
Pruritus
32
What is Alagille syndrome?
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
33
What is liver problem in downs syndrome
Intrahepatic biliary hypoplasia therefore pruritus
34
Clinical features of viral hepatitis x8
``` 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
Features of HAV (Hep A virus)
Faecal-oral transmission Children have mild illness and recover 2-4 weeks No chronic liver disease No treatment but close contacts vaccinated
36
Features of HBV
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
Features of chronic hep B
30-50% of children get chronic HBV liver disease and 10% get cirrhosis Long-term risk of HCC
38
Treatment of chronic HBV x2
Interferon successful in 50% of children infected horizontally and 30% infected perinatally Antiviral therapy effective in some but limited by development of resistance
39
Prevention of HBV
Vaccinate all babies born to HBsAg +ve mothers | Give to mothers if they are e antigen +ve
40
Features of HCV
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
Treatment of HCV
Pegylated interferon and ribavirin Effective 90% of children cases Not taken before 4 years as may resolve spontaneously
42
What is Non-A to G hepatitis
Presents similarly to Hep A and when viral aetiology of hepatitis is suspected but not identified
43
What is acute liver failure/fulminant hepatitis in children?
Development of massive hepatic necrosis with subsequent loss of liver function With or without hepatic encephalopathy
44
Most common causes of acute liver failure in children x3
Paracetamol overdose Non-A to G Hepatitis Metabolic conditions
45
How do children with acute liver failure present x5
Within hours or weeks | With jaundice, encephalopathy, coagulopathy, hypoglycaemia and electrolyte disturbance
46
Complications of acute liver failure x4
Cerebral oedema Haemorrhage from gastritis or coagulopathy Sepsis Pancreatitis
47
Diagnosis of acute liver failure x5
``` Bilirubin may be normal in early stages Transaminases very high ALP increased Coagulation is v.abnormal Plasma ammonia elevated ```
48
Management of acute liver failure x4
Blood glucose maintenance Antibiotics to prevent sepsis IV vit k, plasma - prevent haemorrhage Fluid restrict and mannitol diuresis for cerebral oedema
49
What is Reye Syndrome?
Acute non-inflammatory encephalopathy | Microvesicular fatty infiltration of liver
50
What is Reye syndrome associated with?
Aspirin therapy - has virtually disappeared since stopped giving aspirin to children
51
Common causes of chronic liver disease/hepatitis
Hepatitis virus (B or C) Autoimmune hepatitis Always need to exclude Wilson disease
52
Mean age of presentation of autoimmune hepatitis
7-10 years
53
Gender occurrence of autoimmune hepatitis
More common in girls
54
Presentation of autoimmune hepatitis
Can either present as acute hepatic failure, acute hepatitis or chronic liver disease with extra autoimmune features (skin rash, lupus, arthritis etc)
55
What else can children with autoimmune hepatitis have?
Inflammatory bowel disease, coeliac disease or other autoimmune disease
56
Treatment of autoimmune hepatitis
90% of children respond to prednisolone and azathioprine
57
Occurrence of liver disease in cystic fibrosis
2nd most common cause of death after respiratory disease
58
What is the most common liver abnormality in cystic fibrosis
``` Hepatic steatosis (fatty liver) May be associated with protein energy malnutrition or micronutrient deficiencies ```
59
Progression of fatty liver in cystic fibrosis patients
Does not generally progress and treatment involves ensuring optimal nutritional support
60
More serious liver complications in cystic fibrosis
Thick tenacious bile - progressive biliary fibrosis | Cirrhosis and portal hypertension in 20% of children by mid-adolescence
61
What is Wilson disease?
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
Age of presentation of wilson disease and type of presentation
Rarely under age 3 Presentation in childhood - liver presentation is most likely In 2nd decade - neuropsychiatric features more common
63
Liver presentation of wilson disease
Can present with virtually any liver disease
64
Other than liver and neuropsychiatric what else happens in wilson disease x3
Renal tubular dysfunction Vit D resistant rickets Haemolytic anaemia
65
When are Kayser-Fleischer rings seen in wilson disease?
Not before age 7
66
Diagnosis of Wilson disease x3
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
Treatment of wilson disease
Penicillamine or trientine | Promote urinary copper excretion
68
What is given in wilson disease to reduce copper absorption
Zinc
69
What is given in wilson disease to prevent peripheral neuropathy
Pyridoxine - improvement may take 12 months
70
Features of non-alcoholic fatty liver disease in children
``` Often obese Asymptomatic but may have vague RUQ pain or lethargy Often detected incidentally Rarely get cirrhosis Treat with weight loss ```
71
Features of liver cirrhosis x5
``` Diminished hepatic function Portal hypertension and splenomegaly Varices Ascites HCC may develop ```
72
How can cirrhosis be in children
If compensated, liver function may be normal and features will only show when decompensate
73
How best to diagnosis oesophageal varices
OGD | Barium swallow may miss small ones
74
Treatment of oesophageal varices x5
Acutely - blood transfusion and H2 blockers or omeprazole | If persists - octreotide infusion, vasopressin analogues, sclerotherapy or band ligation
75
Pathophysiology of ascites x4
Hypoalbuminaemia Sodium retention Renal impairment Fluid redistribution
76
When suspect spontaneous bacterial peritonitis?
Undiagnosed fever, abdominal pain, tenderness or unexplained deterioration in hepatic or renal function
77
Malnutrition in liver disease x4
Protein malnutrition Fat malabsorption Anorexia Fat soluble vitamin deficiency (vit a, d, e k)
78
Treatment of malnutrition in liver disease
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
What does Vit a deficiency cause
night blindness in adults and retinal changes in children
80
What does Vit e deficiency x 3 cause
Peripheral neuropathy, haemolysis and ataxia

Paediatrics (16 decks)

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