Case presentation: Dr Nor Azmi

salam..

first of all dr nor azmi baik gile kot..haha.. aman damai klu ade specialist mcm die.. yg aku tgk2, klu background dr tu from oversea mesti cara diorang approach lain.. very kind hearted la..

ok, yesterday aina was presenting on Biliary atresia..i will write on the case summary & a bit discussion regarding this topic.

A 3 y/o malay girl with underlying billiary atresia which was diagnosed at the age of 3 months old; with multiple admission presented with poor oral intake & lethargy. currently, patient is in the state of chronic liver failure with impending end stage renal failure in evidence of deeply jaundice and severe ascites.

so, there are few things that can be discussed:

1. Prolonged jaundice in infant

• define as jaundice > 2 weeks of life in term baby & > 3 weeks in preterm baby(NICE guidline)
• patient must be sent for detail investigation & suspected to have Biliary atresia even it is only occur in 1/15000 baby.
• prolonged jaundice can be divided into:
1. unconjugated(ABO incompatibility, G6PD deficiency, breast milk jaundice)
2. conjugated(liver disease like hepatitis and obstruction like biliary atresia)
• this can be confirm by viewing the liver function test which the increase of conjugated bilirubin in the case of biliary atresia, pale stool & dark color urine.
• biliary atresia is actually due to obstruction of extrahepatic(intrahepatic not involve) bile duct that occur progressively as it is usually be manifested in a gradual manner.
• we can confirm biliary atresia by performing operative cholangiogram(in malaysia) which will reveal the site of obstruction. the B.A must be detected & corrected as early as possible to give a better prognosis for the patient-more than 3 & 1/2 months old is consider to have poor prognosis( this patient is operated at 139days of age.
• biliary atresia is corrected through Kasai procedure: http://www2.niddk.nih.gov/NR/rdonlyres/D7C8B34F-60B2-4410-B07E-3307FC3954C5/0/ldrb_chapter10.pdf  
• NICE guidline for Neonatal jaundice:  http://www.nice.org.uk/nicemedia/live/12986/48679/48679.pdf  

2. Chronic liver failure

stigmata of CLD:

http://www.turner-white.com/pdf/hp_jul03_stigmata.pdf


acute liver failure in children:
http://pediatrics.uchicago.edu/chiefs/picu/documents/ALFinchildren.pdf

investigation of liver disease:
http://www.sawa2006.com/slides_lectures/4th/1/Internal/Lec/Int40_Investigation%20of%20Liver%20Diseases.pdf