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1 - Paeds AE > Liver Disorders > Flashcards

Liver Disorders Flashcards

1
Q

What is the main feature of liver disorders in infants?

A

Prolonged neonatal jaundice, it can be physiological but if caused by liver diseased it is characterised by a raised conjugated bilirubin.

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2
Q

What are the clinical features of liver disease in infants?

A 
Prolonged neonatal jaundice
Pale stools
Dark urine
Bleeding tendency
Failure to thrive
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3
Q

Is liver disease an important differential to consider?

A

Yes, there is an urgency to diagnose liver disease as early as possible in the neonatal period, because early diagnosis and management improves prognosis

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4
Q

What is biliary atresia?

A

A progressive disease, in which there is a destruction or absence of the extra hepatic biliary tree and tntrahepatic biliary ducts.

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5
Q

What are the clinical features of liver disease in children?

A 
Encephalopathy
Jaundice
Epistaxis
Cholestasis
Ascites
Hypotonia
Peripheral neuropathy
Rickets secondary to vit D deficiency
Varices with portal HTN
Spider nave
Muscle wasting from malnutrition
Bruising and petechiae
Splenomegaly with portal HTN
Hypersplenism
Hepatorenal failure
Liver palms
Clubbing
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6
Q

What are some causes of unconjugated prolonged neonatal jaundice?

A 
Breast milk jaundice
Infection (particularly UTI)
Haemolytic anaemia (G6PD deficiency)
Hypothyroidism
High GI obstruction
Crigler - Najjar syndrome
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7
Q

What are some causes of conjugated prolonged neonatal jaundice?

A

Bile duct obstruction - biliary atresia, choleductal cyst
Neonatal hepatitis syndrome - congenital infection, inborn errors of metabolism, alpha1 antitrypsin deficiency, galactosaemia, CF

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8
Q

How would you investigate biliary atresia?

A

A fasting abdominal US may show a contracted or absent gallbladder. A radioisotope scan with TIBIDA shows good uptake by the liver, but no excretion into the bowel. The diagnosis is confirmed at laparotomy by operative cholangiography which fails to outline a normal biliary tree.

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9
Q

How would you treat biliary atresia?

A

Surgical bypass of the fibrotic ducts, hepatoportoenterostomy, in which a loop of jejunum is anastomosed to the cut surface of the porta hepatic, facilitating bile drainage. Success decreases with age

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10
Q

What are choledochal cysts?

A

These are cystic dilatations of the extra hepatic biliary system

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11
Q

How does choledochal cysts present?

A

About 25% present in infancy with cholestasis. In oder children, abdominal pain, a palpable mass and jaundice or cholangitis.

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12
Q

How would you diagnose choledochal cysts?

A

US or radionuclide scanning

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13
Q

How would you treat choledochal cysts?

A

Surgical excision of the cyst

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14
Q

What is neonatal hepatitis syndrome?

A

Prolonged hepatic jaundice and hepatic inflammation

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15
Q

How does neonatal hepatitis syndrome present?

A

They may have IUGR and hepatosplenomegaly at birth

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16
Q

What is alpha-antitrypsin deficiency?

A

Deficiency of the protease alpha-antitrypsin, it is associated with liver disease in infancy and childhood and emphysema in adults.

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17
Q

How is alpha-antitrypsin deficiency inherited?

A

It is inherited as an autosomal recessive disorder.

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18
Q

What are the clinical features of alpha-antitrypsin deficiency?

A

Prolonged neonatal jaundice or, less commonly, bleeding due to vitamin K deficiency (haemorrhagic disease of the newborn). Hepatomegaly is present. Splenomegaly develops with cirrhosis and portal hypertension.

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19
Q

How is alpha-antitrypsin deficiency confirmed?

A

Estimating the level of alpha-1-antitrypsin in the plasma and identifying the phenotype

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20
Q

What is the prognosis of alpha-1-antitrypsin deficiency?

A

50% of children have a good prognosis, but the remainder will develop liver disease and may require transplantation

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21
Q

What is progressive familial intrahepatic cholestasis?

A

A heterogenous group of cholestatic disorders of bile transporter defects caused by recessive mutations in different genes.

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22
Q

How does progressive familial intrahepatic cholestasis present?

A

Jaundice, intense pruritus, diarrhoea with failure to thrive, rickets and a variable progression of liver disease.

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23
Q

What are the clinical features of viral hepatitis?

A

Nausea, vomiting, abdominal pain, lethargy and jaundice (30-50% do not develop jaundice). A large tender liver is common and 30% will have splenomegaly. The liver transaminases are usually markedly elevated. Coagulation is usually normal.

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24
Q

What is acute liver failure?

A

In children, it is the development of massive hepatic necrosis with subsequent loss of liver function, with or without hepatic encephalopathy.

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25
Q

What are the main causes of acute liver failure in children?

A

Paracetamol overdose, non-A to G viral hepatitis and metabolic conditions (Wilson’s disease, tyrosinaemia).

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26
Q

How does acute liver failure present in children?

A

May present within hours or weeks with jaundice, encephalopathy, coagulopathy, hypoglycaemia and electrolyte disturbance.

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27
Q

What are the signs of encephalopathy secondary to acute liver failure?

A

early signs include alternate periods of irritability and confusion with drowsiness. Older children may be aggressive and unusually difficult.

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28
Q

What are the complications of acute liver failure?

A

Cerebral oedema, haemorrhage from gastritis or coagulopathy, sepsis and pancreatitis.

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29
Q

How would you diagnose acute liver failure?

A

Bilirubin may be normal. Transaminases are greatly elevated (10-100 times normal), alkaline phosphatase is increased, coagulation is very abnormal and plasma ammonia is elevated.

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30
Q

What would you monitor during acute liver failure?

A

The acid-base balance, blood glucose and coagulation times. An EEG will show acute hepatic encephalopathy and a CT scan may demonstrate cerebral oedema.

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31
Q

How would you manage acute liver failure?

A

Maintaining the blood glucose with IV dextrose.
Preventing sepsis with broad-spectrum antibiotics and antifungals.
Preventing haemorrhage, particularly from GI tract with IV vit K, FFP and cryoprecipitate and H2-blocking drugs or PPIs
treating cerebral oedema by fluid restriction and mannitol diuresis
Urgent transfer to a specialist liver unit.

32
Q

What are the features of poor prognosis of acute liver failure?

A

A shrinking liver, rising bilirubin with falling transaminases, a worsening coagulopathy or progression to coma.

33
Q

What is Reye’s syndrome?

A

An acute non-inflammatory encephalopathy with microvesicular fatty infiltration of the liver.

34
Q

What drug is Reye’s syndrome linked with?

A

Aspirin therapy

35
Q

What are the causes of chronic liver disease in children?

A 
Chronic hepatitis - post-viral hepatitis B, C; autoimmune hepatitis; drugs (nitrofurantoin, NSAIDs); IBD; primary sclerosing cholangitis
Wilson's disease (>3 years)
Alpha-1-antitrypsin deficiency
CF
Neonatal liver disease
Bile duct lesions
36
Q

What is the clinical presentation of chronic liver disease in children?

A

It can vary from an apparent acute hepatitis to the insidious development of hepatosplenomegaly, cirrhosis and portal HTN with lethargy and malnutrition.

37
Q

What is the mean age of presentation of autoimmune hepatitis and what sex is it most common?

A

7-10 years, most common in girls

38
Q

How does autoimmune hepatitis present?

A

It may present as an acute hepatitis, as fulminant hepatic failure or chronic liver disease with autoimmune features such skin rash, SLE, arthritis, haemolytic anaemia or nephritis.

39
Q

How is autoimmune hepatitis diagnosed?

A

It is based on elevated total protein, hypergammaglobulinaemia (IgG > 20g/L); positive autoantibodies, a low serum complement (C4) and typical histology.

40
Q

How would you treat autoimmune hepatitis?

A

90% of children will respond to prednisolone and azathioprine

41
Q

What is the most common liver abnormality associated with cystic fibrosis?

A

Hepatic steatosis (fatty liver). It may be associated with protein energy malnutrition or micronutrient deficiencies.

42
Q

After hepatic steatosis, what is the potential, more significant liver disease that could occur with CF

A

Progressive biliary fibrosis due to thick tenacious bile with abnormal bile acid concentration. Cirrhosis and portal HTN develop in 20% of children by mid-adolescence.

43
Q

How would you treat chronic liver disease secondary to CF?

A

It does not generally progress and treatment involves ensuring optimal nutritional support. It could be treated with ursodeoxycholic acid if further treatment is indicated. In end-stage liver failure, liver transplantation may be considered

44
Q

What is Wilsons disease?

A

The basic genetic defect is a combination of reduced synthesis of caeruloplasmin (the copper-binding protein) and defective excretion of copper in the bile, which leads to an accumulation of copper in the liver, brain, kidney and cornea

45
Q

How is Wilson’s disease inherited?

A

It is an autosomal recessive disorder

46
Q

When does Wilson’s disease often present?

A

It does not often present below 3 years

47
Q

How does Wilson’s disease often present at younger ages?

A

In those presenting in childhood, a hepatic presentation is more likely. The may present with almost any form of liver disease, including acute hepatitis, fulminant hepatitis, cirrhosis and portal HTN

48
Q

How does Wilson’s disease often present in the second decade?

A

Neuropsychiatric features are more common including deterioration in school performance, mood and behaviour change, and extrapyramidal signs such as incoordination, tremor and dysarthria. Renal tubular dysfunction, with vitamin D-resistant rickets, and haemolytic anaemia also occur.

49
Q

What are Kayser-Fleischer rings and when are they seen?

A

They are copper accumulation in the cornea and they are not seen before 7 years of age.

50
Q

What are the investigation results in Wilson’s disease?

A

A low serum ceruloplasmin and copper is characteristic, but not universal. Urinary copper excretion is increased and this further increases after administrating the chelating agent penicillamine.

51
Q

How is Wilson’s disease confirmed?

A

Elevated hepatic copper on liver biopsy or identification of the gene mutation.

52
Q

How do you treat Wilson’s?

A

Treatment is with penicillamine or trientine. Both promote urinary copper excretion, reducing hepatic and CNS copper. Zinc can reduce copper absorption. Liver transplant is considered for severe end-stage liver disease

53
Q

What can be used to prevent peripheral neuropathy in Wilson’s?

A

Pyridoxine. Neurological improvement may take up to 12 months of therapy

54
Q

What is fibropolycystic liver disease?

A

A range of inherited conditions affecting the development of the intrahepatic biliary tree.

55
Q

How does fibropolycystic liver disease present?

A

Liver and renal disease. The liver disease may include cystic disease of the liver or the biliary tree or congenital hepatic fibrosis.

56
Q

How and when does congenital hepatic fibrosis present?

A

In children over 2 years old with hepatosplenomegaly, abdominal distention and portal HTN.

57
Q

How does congenital hepatic fibrosis differ from cirrhosis?

A

Liver function tests are normal in the early stage of congenital hepatic fibrosis

58
Q

What does histology show in congenital hepatic fibrosis?

A

Large bands of hepatic fibrosis containing abnormal bile ductules.

59
Q

What are the main pathophysiological effects of cirrhosis?

A

Diminished hepatic function and portal HTN with splenomegaly, varices and ascites. Hepatocellular carcinoma may develop

60
Q

What are the physical signs of cirrhosis?

A

Palmar and plantar erythema and spider naevi, malnutrition and hypotonia. Dilated abdominal veins and splenomegaly suggest portal HTN, although the liver may impalpable

61
Q

What are the investigations for cirrhosis?

A

Screening for known causes of chronic liver disease.
Upper GI endoscopy to detect the presence of oesophageal varices and/or erosive gastritis.
Abdo US - may show shrunken liver and splenomegaly gastric and oesophageal varices.

62
Q

What would the bloods show as cirrhosis worsens?

A

Elevation aminotransferases and alkaline phosphatase.
The plasma albumin falls.
Prothrombin time is prolonged

63
Q

When do oesophageal varices develop? Is this a quick process?

A

They are inevitable consequences of portal hypertension and may develop rapidly in children

64
Q

How do you investigate oesophageal varices?

A

Upper GI endoscopy because barium swallow may miss small varices

65
Q

How do you acutely treat oesophageal varices?

A

Acute bleeding is treated conservatively with blood transfusions and H2 blockers (ranitidine) or omeprazole.

66
Q

How do you treat oesophageal varices if the bleeding continues?

A

Octreotide infusion, vasopressin analogues, sclerotherapy or band ligation

67
Q

What are the contributing factors to ascites?

A

Hypoalbuminaemia, sodium retention, renal impairment and fluid redistribution.

68
Q

How do you treat ascites?

A

Sodium and fluid restriction and diuretics

69
Q

When should spontaneous bacterial peritonitis be considered?

A

If there is an undiagnosed fever, abdominal pain, tenderness or an unexplained deterioration in hepatic or renal function.

70
Q

What may precipitate encephalopathy secondary to end-stage liver disease?

A

GI haemorrhage, sepsis, sedatives, renal failure or electrolyte imbalance

71
Q

How is nutrition altered in liver disease?

A

High protein, high carbohydrate diet with 50% more calories than the recommended daily allowance.

72
Q

How would you treat pruritus?

A

Loose cotton clothing, avoid overheating
Emollients or evening primrose oil
Phenobarbital to stimulate bile flow; cholestyramine which is a bile salt resin; ursodeoxycholic acid, an oral bile acid

73
Q

What are the indications for liver transplant in chronic liver disease?

A

Severe malnutrition unresponsive to intensive nutritional therapy.
Recurrent complications (bleeding varices, resistant ascites)
Failure of growth and development
Poor quality of life

74
Q

What are some absolute contraindications to liver transplantation?

A

Sepsis
Untreatable cardiopulmonary disease
Cerebrovascular disease

75
Q

What are some complications of liver transplantation?

A 
Primary non-function of the liver
Hepatic artery thrombosis
Biliary leaks and strictures
Rejection
Sepsis, the main cause of death

1 - Paeds AE (24 decks)

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