Liver disease Flashcards
Name some causes of unconjugated and conjugated hyperbilirubinaemia in neonates
Unconjugated: breastmilk jaundice, physiological jaundice, infection, haemolytic anaemia, hypothyroidism, Crigler-Najjar
Conjugated: biliary atresia, IMDs (galactosaemia), CF, A1 antitrypsin deficiency, Alagille syndrome
You are seeing a neonate on the postnatal ward who has developed jaundice 12 hours after birth. What do you want to do?
History: when, progression. Current state (lethargy, feeding, fever, dehydration, tone/movements/seizures). Pregnancy and birth (booking bloods + blood type, illnesses, problems, gestation at delivery, method), FHx of jaundice/blood disorders, social
Examination: general newborn exam, including tone + reflexes, vitals
Ix: FBC + film, CRP, U+Es, LFTs, G+S, DAT
Use bilirubin threshold table to determine treatment
Describe how phototherapy works (to a layperson) and how to use it
The baby looks yellow because of a coloured substance in the blood called bilirubin
We use light therapy with a blue light to break the substance down so that it can be released in the urine
Completely safe and painless. Still be able to take him out for short breaks to feed, cuddle, etc.
Use: measure bilirubin every 4-6 hours until stable/falling, then less frequently. When >50 below the threshold, stop and monitor for a day to make sure not increasing again
What is the pathophysiology of biliary atresia? How does it present and what is the treatment?
- Progressive fibrosis + obliteration of the bile ducts
- Causes obstructive jaundice: pale stools, dark urine (bilirubin Not urobilin), faltering growth, hepatomegaly
- Dx: LFTs -> USS -> ERCP/operative cholangiogram, liver biopsy
- Treatment: Kasai’s procedure or liver transplant. Also support good nutrition, vitamin supplementation and antibiotic prophylaxis with Septrin
Name some inherited causes of jaundice/liver disease in children
- CF -> cholestasis
- Alagille syndrome (AD, triangle face, cardiac abnormalities)
- Alpha1 antitrypsin deficiency (AR, liver + lung problems)
- Galactosaemia (very rare, disorder caused by milk -> liver disease, devleopmental delay, fatal sepsis)
- Glycogen storage disorders
Describe the presentation of acute viral hepatitis. Which types cause acute hepatitis?
- Fever, N+V, RUQ pain and tenderness, jaundice, acute liver failure in severe cases
- Caused by HAV, HEV, and some HBV
What are the different types of viral hepatitis, what type of virus are they? Describe the key points of disease for each
- Hep A: RNA virus. Faecal-oral transmission from contaminated food. Children are usually asymp/mild illness that recovers in several weeks. Supportive management with vaccination of close contacts.
- Hep B: DNA virus. BBV/sexual transmission. Causes acute or chronic disease. Older kids usually clear but infants with perinatal infection often chronic carriers. Supportive Mx or antiviral therapy in chronic
- Hep C: RNA virus. BBV/sexual transmission. Link with HIV. Causes chronic disease. Treat with antivirals: Peg-IFN, ribavirin.
- Hep D: RNA. needs HBV. Causes worse chronic infection
- Hep E: RNA. faecal-oral transmission, especially wild game in Europe. Usually mild illness (except in pregnancy)
What happens in acute liver failure? How does it present? What is the management?
Massive hepatic necrosis -> loss of liver function
RUQ pain, jaundice, coagulopathy, encephalopathy, hypoglycaemia, electrolyte disturbance
Management: refer to national liver centre
-Treat the cause
-Monitor glucose, acid-base, coagulation and treat as needed
-IV dextrose
-Broad spectrum antibiotics
-Fluid restriction to prevent cerebral oedema
-IV Vitamin K and PPIs to prevent haemorrhage
Name some causes of chronic liver disease in children. How do they present? What is the specific management for each type?
All can have general signs/symptoms of chronic liver disease
- Viral hepatitis: antiviral treatment
- CF: also lung involvement, DM, pancreatic insufficiency. Ursodeoxycholic acid.
- Wilson’s: neuropsychiatric symptoms. Zinc, trientine
- Autoimmune: systemic involvement eg. rash, arthralgia. Treat with steroids/azathioprine
- NAFLD: obesity, hypertriglyceridaemia. Lifestyle changes, statins etc.
- A1 antitrypsin: lung problems
What is the pathophysiology of Wilson’s disease? How it is diagnosed?
- Gene defect causing low levels of caeruloplasmin (binding protein) and reduced excretion in bile
- Dx: low caeruloplasmin, low serum Cu, high urinary Cu (especially after giving penicillamine), liver biopsy showing Cu deposits, gene mutation testing
Describe the complications of chronic liver disease and management
- Nutrition: dietician support, vitamin supplements (ADEK). Monitor growth
- Pruritus: urso, cholestyramine
- Encephalopathy: frequent monitoring, correct precipitating factors (haemorrhage, drugs, infection), reduce protein
What is cirrhosis? What are the complications?
Cirrhosis is fibrosis of the liver with regenerative nodules
- Can be compensated with normal LFTs
- Decompensation leads to: varices, ascites (w/ possible infection SBP), encephalopathy, coagulopathy, oedema
- Manage with sodium and fluid restriction, diuretics, tap
