4.1 Liver Disease - Paeds Flashcards
Jaundiced neonate approach?
Family history Examine baby Urine and stools for colour -pale stools -dipstick for bilirubin Check newborn screen FBE+film,(haemolysis), TFTs Urine culture
What are the new born screen diseases that can cause jaundice?
CF
Galactosaemia
Phenylketonuria
Hypothyroidism
Prolonged unconjugated tested hyperbilirubinaemia in neonate definition?
Persistence of jaundice beyond 2nd weeks of life
- total bilirubin raised
- direct fraction <30umol/l, <20% total
Prolonged unconjugated tested hyperbilirubinaemia in neonate 2 main causes
Increased production
Decreased excretion
Prolonged unconjugated tested hyperbilirubinaemia in neonate causes of increased production
Haemolysis
-thesis, aBO, G6PD, -Pyruvate kinase—thalassemia
Extra vascular blood: cephalohematoma
Polycythemia
Prolonged unconjugated tested hyperbilirubinaemia in neonate kinds of reduced excretion?
-hypothyroidism UTI Dehydration Delayed meconium Familial Critter-Najarro syndrome Breast-milk jaundice
Breast milk jaundice, how to diagnose?
Diagnosis of exclusion
- usually serum bilirubin is not >340
- reassure
- no role of ceasing breast feeding or phenobarbitone
Conjugated bilirubinaemia/cholestasis is dangerous?
Always pathological
Cholestasis in the newborn, causes?
Infections
Metabolic diseases
Biliary diseases
Neonatal/giant cell hepatitis
Cholestasis in the newborn, which infections?
TORCHS Toxoplasmosis Rubella CMV Herpes Syphilis
Echo, Coxsackie
UTI
Septicaemia
Hep B,C(rare)
2 Most common metabolic disorders causing Cholestasis in the newborn?
Alpha-1-antitrypsin deficiency
Galactosaemia
CF
Neonatal iron storage disease
Biliary diseases causing Cholestasis in the newborn?
- Biliary Atresia
- Alagille’s syndrome (intrahepatic biliary hypoplasia)
- Choledochal cyst
- Pigment stone obstruction
Cholestasis workup?
LFTs U/S DISIDA scan (for biliary atresia) Exclude infectious/metabolic causes Cholangiogram Liver biopsy
Biliary atresia cause?
Unknown, not inherited
?environmental/infective
Epidemiology of biliary atresia
1 in 10 000
Treatment for biliary atresia
- Porto-enterostormy (Kasai procedure)
- liver transplant
Prognosis with Kasia procedure?
1/3 do well
1/3 well but complications
1/3 need liver transplantation
Jaundice in order child, what to ask for in history?
Viral? Contact Hx Fix Shellfish, oysters/hep A Recent blood produces, six, injections Recent ABx Wild mushrooms?
DDx of hepatitis in child?
-Infections
-Drugs: sulphur, antiepileptics, halothane, ecstasy, +++paracetamol
-Toxins: amanita phylloides
-Wilson’s
Autoimmune chronic hepatitis
-budd-chiari
Infectious causes of hepatitis?
Hep, A,B,C,E EBV CMV HHV6 VZV, HSV Echo, coxsackie
Worry about Hep E where?
Pregnant women
How to test of a child is in acute liver failure? Best:
Coags
What do you do if child in liver failure and coags are abnormal?
Give Vit. K
Reassess
?transplantation
What do you do if child in liver failure and coags are normal?
Viral serology
U/S - choledochal cyst
What do you do if child in liver failure and coags are normal and viral serology is negative? 2 things
Wilsons’s disease -copper - caerulospamin -KF rings Urinary Cu -biopsy
Autoimmune hepatitis
Common causes of chronic liver disease in paeds?
Alpha-1-antitrypsin deficiency Wilson’s Chronic hep B,C Autoimmune Sclerosing cholangitis Biliary obstruction CF Budd chiari Haemochromatosis(rare)
Management of chronic liver disease in paeds?
- Portal HT: varicella bleeding, asictes, hyperslenism
- nutrition: vit, ADEK, albumin
- sepsis, immunocompromised
- renal, encephalopathy, pulmonary
- itch
Most common cause of liver transplantation in paeds?
50% biliary atresia
Chronic liver disease
Acute liver failure
Enzyme replacement
What is the syndrome that can cause benign mild jaundice?
Gilbert’s Syndrome: 3% of pop
-partial deficient to of UDP glucuronyl transferase
DDx of Gilbert syndrome?
Haemolysis
