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
Features of Hep C
common in IVDU
co-transmission w/ HIV very common
NOTIFIABLE DISEASE
Causes of acute liver failure in children <2
Infection (HSV) seronegative hepatitis haemochromatosis inborn error of metabolism drug induced
causes of acute liver failure in children >2
Infection (hsv) seronegative hep paracetamol overdose AI hepatitis wilson's disease
Ix for acute liver failure
bili may be normal transaminases massively elevated ALP high deranged clotting ammonia high hypoglycaemia
Mx acute liver failure
IV dextrose to keep BM >4
Vit K to help clotting
Mannitol and fluid restricition to stop cerebral oedema
BSA to prevent sepsis
NB - may need transplant (PT is best assessor for liver function)
How do sclerosing cholangitis and AI hepatitis present
chronic or acute liver disease
AI symptoms - haemolytic anaemias, rashes
ix -
gammaglobulinaemia
autoab
C4 DEFICIENCY
Mx of sclerosing cholangitis
urseodeoxycholic acid
mx of AI hepatitis
azathioprine and prednisolone
presentation of wilson’s
neuropsychiatric development at 10-20
liver disease
renal tubular dysfunction
ricketts
mx of wilson’s
zinc to block intestinal Cu2+ absorption
pyridoxine to prevent peripheral neuropathy
Complications of chronic liver disease
Nutrition - can’t absorb fats
vitamins - supplement ADEK
protein metabolism - only protein restrict if encephalopathic
Pruritus - keep nails short, give cholestyramine to absorb bile salts
Mx of hepatic encephalopathy
tx precipitant cause (haemorrhage, renal failure etc.)
review mental status
reduces nitrogenous load:
protein restriction
lactulose reduces portein absorption from gut
Mx of ascites
it’s compounded by sodium retention
fluid + sodium restrict
diuretics
Mx of oesophageal varices
Acute bleed - transfusion + h2 antagonist
persistent - vasopression analogue, octreotide to reduce portal pressure, endoscopic band lifation
