Biliary Atresia | Cheat Sheet - Edubirdie

University:
Western Sydney University
Course:
MEDI 2003 | Clinical Sciences 2
Academic year:

2024
Views:

70

Pages:

2
Author:

Mariam A. A.

Biliary Atresia cel MOST COMNON CAUSE OF PAEDIATRIC ATRESIA * Progressive libro-abdillerative disease of thee extra hepatic ducts usually manifests in 2-8 wlcs * Aethiology/RF Comery be associaled be with genetic alonormalities unclear possibly = viral infechiamseg. cur L autoimmune Le developmental may also somehmes include the intra-hepatic ducts of the billiay system WOT typically inherited genetic * Pathophysiology Bile duct fuil to form properly or inflamed or scared after birth persistent jaundice Clinical features acholic (pale) stods, dask vine, hepatomegaly, failure to thrive bile cannot be drained intra-hepatic bile stasis ↓ inflammation, scaung Cholestasis, bile accumulahon liver damage *Ix direct bilirubin → elevated cholestasis uls small or absent gallbladde gallbladde bowel HIDA scan no excelion inte suggest obstruchion live biopsy confims bile duct proliferation & fibrosis →intra-operative cholangiogram → GOLD STANDARD * Management →Kasai Procedure / Hepatoportenteostamy → removal of atrenic ducts → connects the liver into the small intestine → success. outcomes are best if performed within 25% the 1st 60 days 50% good bile flowe of life 25% fuil partial improvement liver transplant if kasai fails or liver disease progresses most children will eventually need one even afte inhial kasai success Complications *Chodangihs heeds IV ABx Tx bacterial infection post kasai * Cirrhosis portal HITN * mulabsorpticon of fat soluble vitamins ADEK

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