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
2
Q
How do children with choledochal cysts present?
A
- Jaundice
- Hepatomegaly
- Acholic stools
Often difficult to distinguish from biliary atresia
3
Q
What are some of the complications of choledochal cysts?
A
- Cholangitis
- Pancreatitis
- Rupture of the cyst
- Cholangiocarcinoma
4
Q
How do we treat choledochal cysts?
A
We usually remove them
5
Q
How do we diagnose them?
A
- Clinically
2. On sonar or MRI
6
Q
What are the causes of hepatitis and subsequently jaundice in children (particularly older children and teens)?
A
- Infections
- viral-hepatitis, CMV, EBV, Herpes, rubella
- bacterial
- parasitic-toxoplasma gondii, biharzia
- gallstones - Burns
- Drugs
- All TB drugs
- ARV(NVP and Zidovudine)
- anti-convulsants (phenobarbitone)
7
Q
What is hepatitis?
A
-inflammatory process where ther is varying damage to the hepatocytes
8
Q
How is HAV spread?
A
Faecal oral route (food and water)
9
Q
Why do children by the age of 6 have HAV antibodies?
A
-inadequate sewerage disposal
10
Q
What is the incubation period for HAV?
A
2-4 weeks
11
Q
What do we see on serology when testing HAV?
A
Acute infection: IgM Antobodies
Chronic infection: IgG antibodies
12
Q
What are the typical symptoms of HAV in children?
A
- Fever
- Nausea
- Vomiting
- Diarrrhea
- Right upper quadrant pain
- Jaundice after a couple of days
BUT IT IS USUALLY ASYMPTOMATIC(95%)
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
14
Q
When does the jaundice and other symptoms of HAV resolve?
A
After 2 weeks
15
Q
How do we manage HAV?
A
-supportive treatment at home if it is uncomplicated and make sure child receives adequate fluids
16
Q
What immunization do travelers to endemic areas have to get?
A
- Human immunoglobulin that provides passive immunity for 3 months at 0,02-0,04mg/kg
- Active vaccination is given at two doses 6 months apart to children over 2 years
17
Q
What is the route of transmission for HBV?
A
- Vertical(from the mom)
- Parenteral
- Sexual
- Horizontal (from child to child during preschool)
18
Q
What is the most common cause of acute hepatitis in children?
A
Hepatitis A
19
Q
What would you ask in history in acute hepatitis?
A
- Family history-ask for contacts
2. Any drugs and toxins
20
Q
What is the peak age for hepatitis B?
A
Between 2-11 years
21
Q
What is the incubation period for hepatitis B?
A
60-180 days
22
Q
What are some complications of HBV?
A
- Glomerulonephritis
- Nephrotic syndrome
- Arthritis
- Pericarditis may also present
23
Q
What is the treatment of HBV?
A
- Mostly supportive but we can also give interferon alpha and DNA-polymerase inhibiting agents
Lamiduvidine! in children
24
Q
How is HCV spread?
A
- Sexual contact
2. IV drug use
25
Which hepatitis does HDV need to exist?
HBV
26
What age group is HEV common in?
15-40 year olds
27
What can HEV cause in endemic regions?
Fulminant hepatitis
28
Why do children with hepatitis have a prolonged INR?
They have vitamin K deficiency
29
What specific treatment would we give to these patients with hepatitis?
1. If Herpertic treat with acyclovir
2. Autoimmune cause use steroids
3. Bacterial- antibiotics
30
What is chronic liver disease?
Inflammation of the liver for more than 6 months and capable of progressing into cirrhosis
31
What is galactossemia?
A metabolic disease that prevents conversion of galactose to glucose
Usually children under 3 years
32
How do we treat galactossaemia?
By putting the child on galactose free diet
33
What are some causes of childhood cirrhosis?
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
What are the complications of chronic liver disease (cirrhosis)?
1. Portal hypertension
- oesophageal varices, ascites, hypersplenism
2. Hepatic encephalopathy
3. Jaundice
35
What is cirrhosis?
It is destruction of the normal liver architecture which leads to scarring and fibrosis
36
What is the toxic metabolite that can build up in the blood if the liver does not detoxify blood?
Ammonia
37
What is portal hypertension?
Defined as portal pressure of more than 10mmHg
38
How can we categorize portal hypertension?
1. Pre-hepatic
2. Intra-hepatic
3. Post-hepatic
39
What are the prehepatic causes of portal hypertension?
Portal or splenic vein obstruiction
| Stenosis of the portal vein
40
What are the intra-hepatic causes of portal hypertension?
Cirrhosis
| Schistosomiasis
41
What are the post-hepatic causes of portal hypertension?
Right sided heart failure
| Budd-chiari syndrome
42
If a child presents with unexplained significant gastrointestinal bleeding and splenomegaly what should you think of?
Portal hypertension
43
What are the typical causes of portal hypertension?
1. Gastrointestinal bleeding from the oesophageal varices and the gastric varices
2. Splenomegaly
3. Ascites
4. Echymosis
5. Captured medusae
6. Rectal varices
44
Which causes between pre-hepatic, intra-hepatic and post hepatic causes lead to hepatomegaly?
Intra-hepatic and post-hepatic
45
What is often the presentation of a child with oesophageal varices?
Hemetemesis and melena
46
What should be the management of a child with portal hypertension?
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
What can we do to prevent continued bleeding?
Balloon tamponade
48
What is ascites?
The pathological accumulation of fluid in the peritoneal cavity seen clinically by abdominal distension , shifting dullness to percussion and palpable fluid thrill
49
What does nitric oxide cause?
It causes vasodilation
50
What diagnostic test is done for ascites these days?
1. Diagnostic paracentesis to determine the serum ascitic albumin gradient
51
What does a serum ascitic albumin gradient level of >1,1g/dl mean?
It means that the child has ascites caused by portal hypertension
52
What does a serum ascitic albumin gradient of <1,1g/dl mean?
It means that the ascites is caused by non-portal hypertension causes
53
What is the management of ascites?
1. Bed rest
2. Fluid and sodium restriction
3. Potassium sparring diuretics-spironolactone
54
What is a complication of ascites in children?
Bacterial peritonitis which has high mortality if not treated soon enough
55
What is hepatic encephalopathy?
Neuropsychiatric manifestation in children with cirrhosis
| -usually graded fro 0 to 4
56
How does hepatic encephalopathy usually present in children?
From personality changes, intellectual deterioration to altered level of consciousness
-asterixis(flapping tremor) is the most common
57
What is autoimmune hepatitis?
It is a chronic type of hepatitis with unknown aetiology usually affecting older children and girls
58
What is alpha 1 anti-trypsin deficiency?
Autosomal recessive disorder associated with reduction in alpha 1 anti-trypsin serum concentration that causes premature pulmonary emphysema and and chronic liver disease
59
What is Wilson’s disease?
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
When does Wilson’s disease usually occur?
After 3 years
61
What does Wilson’s disease usually present with?
1. Jaundice
2. Anaemia
3. Keiyser Fleisher rings
4. Portal hypertension
62
What is the treatment of Wilson’s disease?
Giving copper chelating agents(penicillamine)
63
Which schistosomes organism frequently causes bilharzia in school going children?
Schistosoma mansoni
64
What is the intermediate host of schistosoma mansoni?
Snail
65
What organisms are released from the snail?
Cercaria which penetrate the skin of the human and deposit eggs which causes an inflammatory responses
66
How do we diagnose schistosomiasis?
Ova in the stool and rectal biopsy
67
What is the treatment for schistosomiasis?
Praziquantel (40mg/kg)
68
How does schistosomiasis commonly present?
Splenomegaly which is incidentally found
Hepatomegaly
Portal hypertension
69
What is acute liver failure?
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
The causes of liver failure are categorized into?
1. Infectious
2. Metabolic
3. Autoimmune
3. Drugs and toxins
71
What are the causes of drug induced hepatotoxicity?
- herbal remedies
| - drug overdose
72
Which hepatitis is the most common cause of acute liver failure in SA?
Hepatitis A
73
What are the presenting symptoms of acute liver failure?
1. Jaundice
2. Ascites
3. Enlarged or shrunken liver
4. hypoglycaemia
5. Bleeding
6. Encephalopathy
74
What are the complications of acute liver failure?
1. Gastrointestinal bleeding
2. Sepsis
3. Renal failure
75
What should you do with a baby with acute liver failure?
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
What is the main cause of death in children with acute liver disease?
Cerebral oedema with brain death
