liver disorders Flashcards
when to consider intensifying phototherapy in
If bili rising more than 8.5 micromol/hour
bili within 50 micro of exchange transfusion boundary
bili hasn’t reduced after 6 hours
How does biliary atresia present
mild jaundice and pale stools (which get paler)
failure to thrive in child that was born fine
hepatomegaly
splenomegaly
Diagnosis of biliary atresia
Cholangiogram (shows absent biliary tree)
biopsy shows neonatal hepatitis
Mx biliary atresia
Kasai hepatoportoenterostomy within 60 days of life
liver transplant
give urseodeoxycolic acid for bile flow
nutritional supplements
fat soluble vitamins
prophylactic Abx for cholangitis
what is choledochal cyst
cystic dilation of extrahepatic biliary tree
How does neonatal hepatitis syndrome present
prolonged neonatal jaundice in newborn
in older children can see LBW and faltering growth
Mx for neonatal hepatitis syndrome
give fat soluble vitamins
some require liver transplants
How does alpha 1 antitrypsin present
Prolonged jaundice
bleeding (due to vitamin K deficiency)
hepatomegaly
pulmonary disease later in life
Symptoms of galactosemia
When fed milk: vomiting hepatomegaly jaundice if left untreated get liver failure, cataracts etc.
Ix for galactosemia
galactose in urine
galactose-1-phosphate-uridyl transferase in RBC
Clinical features of viral hepatitis
fever, vomiting, abdo pain
not always jaundiced
hepatomegaly
ELEVATED TRANSAMINASES, NORMAL CLOTTING
Ix + Mx of hep A
anti-Hep A IgM
Mx supportive
vaccinate close contacts in 2 weeks]
NOTIFIABLE DISEASE
What happens to children who get hep B
most resolve spontaneously
1-2% get fulminant disease
5-10% become chronic carriers
Ix for Hep B
IgM HBcAb - Ab against core antigen, suggests acute infection
HBsAg - hep b surface antigen, suggest ongoing infection
Mx Hep B
Acute- supportive, sort out itch
may need transplant
chronic - interferon or antivirals
prevention - all mothers screened for HBsAg
NOTIFIABLE DISEASE
