Liver disorders

Question 1

Biliary atresia

Answer 1

Ix: bili, LFTs, abdo USS, cholangiogram, liver biopsy
Surgery ASAP
Karsai hepatoportoenterstomy
Liver Trx
Mx of Cx

Question 2

Cx of biliary atresia

Answer 2

growth failure, portal htn, cholangitis, ascites
Ursodeoxycholic acid aids bile flow
In first year of life: breast milk or medium chain triglyceride enriched formula (monthly nutrition r/v)
Fat soluble vit supplements
Proph. cotrim to prevent cholangitis

Question 3

Neonatal hepatitis syndrome

Answer 3

nutrition and fat soluble vitamins
Some req. liver trx
most survive to adult life

Question 4

Alpha-1 anti-trypsin deficiency

Answer 4

avoid smoking and pollution
limit alcohol consumption
Manage pulmonary w/bronchodil. ICS, vaccination
Liver manifestations: monitor like any other liver Dx (coagulation, ascites, ?varices)

Question 5

Galactosaemia

Answer 5

Ix: galactose in urine, galactose-1-phosphate-uridyl transferase in RBC
Galactose free diet prevents liver Dx
ovarian failure and learning difficulties may occur later

Question 6

Hepatitis A

Answer 6

Supportive
Close contacts vaccincated within 2wks of onset
Unvacc. pts. w/recent exposure to hepatitis A should have normal IM IG or hep A vaccine

Question 7

Hepatitis B

Answer 7

Acute:
Supportive, anti-viral therapies +/- liver trx (lamivudine, entacavir, tenofovir)
Chronic:
Supportive (usually asymptomatic), IFN or antiviral monotherapy (entacavir, PEGifnalpha, tenofovir, lamivudine) in some
Prevention: ALL pregnant women should have AN screening for HbsAG
babies ofHBsAGpositive mothers have HBV vaccine
HBV IG given also given if mother is HBeAG positive
Vaccinate all famly

Question 8

Hepatitis C

Answer 8

Based on genotype of HCV
PEG-ifn
ribavirin
new drugs e.g. sofosbuvir
NB Mx not undertaken in first 3y of life because many vertical cases will resolve spontaneously

Question 9

Acute liver failure

Answer 9

Early referral to national paediatric liver centre
steps to stabilise:
- glucose > 4 w/IV dextrose
- prevent sepsis w/abx and afx
- prevent haemmorhage with IV vit K and H2 agonists/PPI
Mx is based on cause of liver failure
Poor prognosis:
-shrinking liver
-rising bili
- rising transaminases
- worsening coagulopathy
- coma

Question 10

Wilson’s Dx

Answer 10

Zn (blocks intestinal copper absorption)
Trientine - increases urinary copper excretion
Sx Mx for tremor, dystonia, speech impediment
Pyroxidine (B6) to prevent peripheral neuropathy (may take 12m to work)
Liver trx in end stage liver dx

Question 11

Non alcoholic fatty liver disease

Answer 11

wt loss/bariatric surgery
Treatment of insulin resistance and diabetes
statins
vit C+E
Ursodeoxycholic acid

Question 12

Hepatic encephalopathy

Answer 12

Supportive (freq. monitoring)
Identify and correct precipitating factors (GI bleed, infection, electrolyte disturbance, drugs)
Reducing nitrogenous load (dietary protein restriction but no malnut.)
(reduce gut load
wtih nonabsorable disaccharides eg lactulose, or rifaximin

Question 13

Cirrhosis and portal htn

Answer 13

Sodium and fluid resus
Diuretics
refractory ascites: albumin infusion, paracentesis