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Paediatrics > Acute and Chronic Liver Disease > Flashcards

Acute and Chronic Liver Disease Flashcards

1
Q

What are choledochal cysts?

A

-rare entities characterised by single or multiple dilatations of the intra-hepatic or extra-hepatic biliary tree

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2
Q

How do children with choledochal cysts present?

A
  1. Jaundice
  2. Hepatomegaly
  3. Acholic stools
    Often difficult to distinguish from biliary atresia
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3
Q

What are some of the complications of choledochal cysts?

A
  1. Cholangitis
  2. Pancreatitis
  3. Rupture of the cyst
  4. Cholangiocarcinoma
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4
Q

How do we treat choledochal cysts?

A

We usually remove them

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5
Q

How do we diagnose them?

A
  1. Clinically

2. On sonar or MRI

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6
Q

What are the causes of hepatitis and subsequently jaundice in children (particularly older children and teens)?

A
  1. Infections
    - viral-hepatitis, CMV, EBV, Herpes, rubella
    - bacterial
    - parasitic-toxoplasma gondii, biharzia
    - gallstones
  2. Burns
  3. Drugs
    - All TB drugs
    - ARV(NVP and Zidovudine)
    - anti-convulsants (phenobarbitone)
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7
Q

What is hepatitis?

A

-inflammatory process where ther is varying damage to the hepatocytes

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8
Q

How is HAV spread?

A

Faecal oral route (food and water)

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9
Q

Why do children by the age of 6 have HAV antibodies?

A

-inadequate sewerage disposal

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10
Q

What is the incubation period for HAV?

A

2-4 weeks

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11
Q

What do we see on serology when testing HAV?

A

Acute infection: IgM Antobodies

Chronic infection: IgG antibodies

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12
Q

What are the typical symptoms of HAV in children?

A
  1. Fever
  2. Nausea
  3. Vomiting
  4. Diarrrhea
  5. Right upper quadrant pain
  6. Jaundice after a couple of days
    BUT IT IS USUALLY ASYMPTOMATIC(95%)
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13
Q

What would you find on examination of patients with HAV?

A

Tender hepatomegaly and splenomegally in 30% of patients

Sometimes there is pale stools and dark urine if cholestasis is present

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14
Q

When does the jaundice and other symptoms of HAV resolve?

A

After 2 weeks

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15
Q

How do we manage HAV?

A

-supportive treatment at home if it is uncomplicated and make sure child receives adequate fluids

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16
Q

What immunization do travelers to endemic areas have to get?

A
  1. Human immunoglobulin that provides passive immunity for 3 months at 0,02-0,04mg/kg
  2. Active vaccination is given at two doses 6 months apart to children over 2 years
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17
Q

What is the route of transmission for HBV?

A
  1. Vertical(from the mom)
  2. Parenteral
  3. Sexual
  4. Horizontal (from child to child during preschool)
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18
Q

What is the most common cause of acute hepatitis in children?

A

Hepatitis A

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19
Q

What would you ask in history in acute hepatitis?

A
  1. Family history-ask for contacts

2. Any drugs and toxins

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20
Q

What is the peak age for hepatitis B?

A

Between 2-11 years

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21
Q

What is the incubation period for hepatitis B?

A

60-180 days

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22
Q

What are some complications of HBV?

A
  1. Glomerulonephritis
  2. Nephrotic syndrome
  3. Arthritis
  4. Pericarditis may also present
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23
Q

What is the treatment of HBV?

A
  1. Mostly supportive but we can also give interferon alpha and DNA-polymerase inhibiting agents
    Lamiduvidine! in children
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24
Q

How is HCV spread?

A
  1. Sexual contact

2. IV drug use

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25
Q

Which hepatitis does HDV need to exist?

A

HBV

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26
Q

What age group is HEV common in?

A

15-40 year olds

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27
Q

What can HEV cause in endemic regions?

A

Fulminant hepatitis

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28
Q

Why do children with hepatitis have a prolonged INR?

A

They have vitamin K deficiency

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29
Q

What specific treatment would we give to these patients with hepatitis?

A
  1. If Herpertic treat with acyclovir
  2. Autoimmune cause use steroids
  3. Bacterial- antibiotics
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30
Q

What is chronic liver disease?

A

Inflammation of the liver for more than 6 months and capable of progressing into cirrhosis

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31
Q

What is galactossemia?

A

A metabolic disease that prevents conversion of galactose to glucose
Usually children under 3 years

32
Q

How do we treat galactossaemia?

A

By putting the child on galactose free diet

33
Q

What are some causes of childhood cirrhosis?

A
  1. Choledochal cyst
  2. Biliary atresia
  3. Cystic fibrosis
  4. Ascending cholangitis
  5. Post-hepatic-neonatal hepatitis, viral hepatitis, drugs, toxins
  6. Galactosemia
  7. Alpha 1 anti-trypsin deficiency
34
Q

What are the complications of chronic liver disease (cirrhosis)?

A
  1. Portal hypertension
    - oesophageal varices, ascites, hypersplenism
  2. Hepatic encephalopathy
  3. Jaundice
35
Q

What is cirrhosis?

A

It is destruction of the normal liver architecture which leads to scarring and fibrosis

36
Q

What is the toxic metabolite that can build up in the blood if the liver does not detoxify blood?

A

Ammonia

37
Q

What is portal hypertension?

A

Defined as portal pressure of more than 10mmHg

38
Q

How can we categorize portal hypertension?

A
  1. Pre-hepatic
  2. Intra-hepatic
  3. Post-hepatic
39
Q

What are the prehepatic causes of portal hypertension?

A

Portal or splenic vein obstruiction

Stenosis of the portal vein

40
Q

What are the intra-hepatic causes of portal hypertension?

A

Cirrhosis

Schistosomiasis

41
Q

What are the post-hepatic causes of portal hypertension?

A

Right sided heart failure

Budd-chiari syndrome

42
Q

If a child presents with unexplained significant gastrointestinal bleeding and splenomegaly what should you think of?

A

Portal hypertension

43
Q

What are the typical causes of portal hypertension?

A
  1. Gastrointestinal bleeding from the oesophageal varices and the gastric varices
  2. Splenomegaly
  3. Ascites
  4. Echymosis
  5. Captured medusae
  6. Rectal varices
44
Q

Which causes between pre-hepatic, intra-hepatic and post hepatic causes lead to hepatomegaly?

A

Intra-hepatic and post-hepatic

45
Q

What is often the presentation of a child with oesophageal varices?

A

Hemetemesis and melena

46
Q

What should be the management of a child with portal hypertension?

A
  1. Beta blockers(propanol) to reduce the blood pressure and prevent rebleeding
  2. If bleeding acutely- RESUS
  3. Transfuse the baby
  4. Give antibiotics
  5. Vasoconstrictor to be used to decrease blood flow
47
Q

What can we do to prevent continued bleeding?

A

Balloon tamponade

48
Q

What is ascites?

A

The pathological accumulation of fluid in the peritoneal cavity seen clinically by abdominal distension , shifting dullness to percussion and palpable fluid thrill

49
Q

What does nitric oxide cause?

A

It causes vasodilation

50
Q

What diagnostic test is done for ascites these days?

A
  1. Diagnostic paracentesis to determine the serum ascitic albumin gradient
51
Q

What does a serum ascitic albumin gradient level of >1,1g/dl mean?

A

It means that the child has ascites caused by portal hypertension

52
Q

What does a serum ascitic albumin gradient of <1,1g/dl mean?

A

It means that the ascites is caused by non-portal hypertension causes

53
Q

What is the management of ascites?

A
  1. Bed rest
  2. Fluid and sodium restriction
  3. Potassium sparring diuretics-spironolactone
54
Q

What is a complication of ascites in children?

A

Bacterial peritonitis which has high mortality if not treated soon enough

55
Q

What is hepatic encephalopathy?

A

Neuropsychiatric manifestation in children with cirrhosis

-usually graded fro 0 to 4

56
Q

How does hepatic encephalopathy usually present in children?

A

From personality changes, intellectual deterioration to altered level of consciousness
-asterixis(flapping tremor) is the most common

57
Q

What is autoimmune hepatitis?

A

It is a chronic type of hepatitis with unknown aetiology usually affecting older children and girls

58
Q

What is alpha 1 anti-trypsin deficiency?

A

Autosomal recessive disorder associated with reduction in alpha 1 anti-trypsin serum concentration that causes premature pulmonary emphysema and and chronic liver disease

59
Q

What is Wilson’s disease?

A

An autosomal recessive condition characterised by an abnormal storage of copper in the liver leading to hepatocellulkar injury, central nervous system dysfunction and haemolytic anaemia

60
Q

When does Wilson’s disease usually occur?

A

After 3 years

61
Q

What does Wilson’s disease usually present with?

A
  1. Jaundice
  2. Anaemia
  3. Keiyser Fleisher rings
  4. Portal hypertension
62
Q

What is the treatment of Wilson’s disease?

A

Giving copper chelating agents(penicillamine)

63
Q

Which schistosomes organism frequently causes bilharzia in school going children?

A

Schistosoma mansoni

64
Q

What is the intermediate host of schistosoma mansoni?

A

Snail

65
Q

What organisms are released from the snail?

A

Cercaria which penetrate the skin of the human and deposit eggs which causes an inflammatory responses

66
Q

How do we diagnose schistosomiasis?

A

Ova in the stool and rectal biopsy

67
Q

What is the treatment for schistosomiasis?

A

Praziquantel (40mg/kg)

68
Q

How does schistosomiasis commonly present?

A

Splenomegaly which is incidentally found
Hepatomegaly
Portal hypertension

69
Q

What is acute liver failure?

A

It is a rare multi-system disorder that causes dysfunction of the liver function with or without encephalopathy which leads to mortality in 70% of children if left untreated

70
Q

The causes of liver failure are categorized into?

A
  1. Infectious
  2. Metabolic
  3. Autoimmune
  4. Drugs and toxins
71
Q

What are the causes of drug induced hepatotoxicity?

A
  • herbal remedies

- drug overdose

72
Q

Which hepatitis is the most common cause of acute liver failure in SA?

A

Hepatitis A

73
Q

What are the presenting symptoms of acute liver failure?

A
  1. Jaundice
  2. Ascites
  3. Enlarged or shrunken liver
  4. hypoglycaemia
  5. Bleeding
  6. Encephalopathy
74
Q

What are the complications of acute liver failure?

A
  1. Gastrointestinal bleeding
  2. Sepsis
  3. Renal failure
75
Q

What should you do with a baby with acute liver failure?

A
  1. Refer to tertiary hospital
  2. Get airway access
  3. Surveillance of infection
  4. Good nutrition
  5. Administer N-acetylcysteine can decrease fulminant hepatic failure
76
Q

What is the main cause of death in children with acute liver disease?

A

Cerebral oedema with brain death

Paediatrics (40 decks)

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