Hepatobiliary Flashcards
What causes Biliary atresia?
Progressive fibrosis and obliteration of extra- and intra-hepatic ducts
What can biliary atresia lead to within 2 years?
chronic liver failure within 2 years
Clinical features of biliary atresia
Jaundice (prolonged)
Pale stools
Dark Urine
Hepatomegaly
Faltering growth (red flag)
NOTE: JAUNDICE DUE POST-HEPATIC OBSTRUCTION
PACES: What red flag sign should you be worried about in biliary atresia?
Changes in weight or growth (faltering)
MASSIVE RED FLAG
Investigations for biliary atresia
Bedside
Urine dipstick (low urobilinogen)
Bloods
LFT
Split Bilirubin
Imaging & Other
Abdominal Ultrasound (triangular cord sign) –> 1st LINE
Intraoperative cholangiography
Liver Biopsy
1st line investigation for biliary atresia
Abdominal USS (see triangular cord sign)
Triangular cord sign on abdo USS
Biliary atresia
Management of biliary atresia
Kasai Hepatoporotentersotomy - involves ligating the fibrous ducts above the join with the duodenum and joining an end of the duodenum directly to the porta hepatis of the liver
NOTE: If unsuccessful, consider liver transplant
Gold standard investigation for biliary atresia
Intraoperative cholangiography
Kesai Hepatoportoenterostomy
Biliary atresia
Causes of acute liver failure in infants and young children?
Congenital infections
Metabolic diseases
Hepatitis
What are some causes of acute liver failure in older children?
Paracetamol overdose
Mitochondrial diseases
Wilson’s disease
Clinical features of liver failure
Jaundice
Confusion and drowsiness (encephalopathy)
Easy bruising and excessive bleeding (coagulopathy)
Hypoglycaemia
Investigations of acute liver failure
Bloods
LFT
Split Bilirubin
Clotting Screen
Viral Hepatitis Screen
Autoimmune Screen
Serum Ammonia
Imaging
Ultrasound
Management of acute liver failure
Prompt referral to national paediatric liver centre (may require transplantation)
Supportive Treatment
IV glucose to prevent hypoglycaemia
Prophylactic antibiotics and antifungals
Correct coagulopathy
What is A1AT deficiency? How does it present?
Autosomal recessive condition characterised by a deficiency of alpha-1 antitrypsin (neutrophil elastase inhibitor).
Neonatal jaundice (prolonged)
Hepatomegaly
Shortness of breath
NOTE: respiratory manifestations tend to occur in later life
Investigations for A1AT deficiency
Bloods
Serum A1AT concentration
Genetic testing
Imaging & Other
Liver biopsy (if concerns of liver involvement)
Management of A1AT deficiency
Advise against smoking and to avoid pollution
Advise against drinking alcohol/limiting alcohol consumption
Pulmonary manifestations are treated similarly to COPD (varying combinations of bronchodilators, ICS, pulmonary rehabilitation and vaccination)
Liver manifestations are managed similarly to other liver diseases (may include monitoring for coagulopathy, diuretics for ascites, OGD to detect/manage varices, liver transplantation)
What is a choledochal cyst? How does it present?
Congenital narrowing of the bile ducts resulting in neonatal jaundice.
Jaundice (prolonged)
Pale stools
Hepatomegaly
Investigations for a choledochal cyst
Bedside
Urine dipstick (low urobilinogen)
Bloods
LFT
Split Bilirubin
Imaging & Other
Abdominal Ultrasound
Cholangiogram
Liver Biopsy
Management of choledochal cyst
Surgical intervention is recommended ASAP (ideally within the first 60 days of life)
Kasai hepatoportoenterostomy - involves ligating the fibrous ducts above the join with the duodenum and joining an end of the duodenum directly to the porta hepatis of the liver
Liver transplantation is considered if the Kasai procedure is unsuccessful
PACES: Management of complications of choledochal cysts
Complications: growth failure, portal hypertension, cholangitis, ascites
Ursodeoxycolic acid promotes bile flow
During the first year of life, either breast milk or medium-chain triglyceride-enriched formula is given with monthly monitoring and nutritional status
Fat-soluble vitamins are given to all children with the condition - levels should be monitored and the dose adjusted accordingly
All patients within the first year of life should receive prophylactic antibiotics to prevent cholangitis (usually co-trimoxazole)
What is galactossaemia? How does it present?
Autosomal recessive disorder characterised by an inability to metabolise galactose resulting in the accumulation of galactose in body tissues.
Jaundice
Vomiting
Failure to thrive
Hepatomegaly
In Later Life
Liver failure
Developmental delay
Cataracts
Management of galactosaemia
Eliminate lactose and galactose from diet
NOTE: this may not prevent chronic complications
