Jaundice

Question 1

CHildhood liver diseases tha tmay cuase neonatal jaundice?

Answer 1

1. biliary atresia
2. progressive familial intrahepatic cholestasis
3. alagille syndrome
4. alpha-1 antitrypsin deficiency

Question 2

Primary biliary cirrhosis?

Answer 2

Destruction of intrahepatic bile ducts
progressive cholestatic liver disease
autoimmune 
-positive AMA
-middle aged women
-elevated alkaline phosphatase -elevation total bilirubin sign of advanced disease 

Florrid duct lesion

• periductal inflammation with GRANULOMA formation and duct destruction
• portal tract with lymphocytes, plasma cells, eosinophils, macrophages and granulomas
• bile duct injury and bile ducts are infiltrated by lymphocytes

Treatment: ursodeoxycholic acid then transplant

Cholestatic symptoms

• pruitis
• fatigue
• xanthomas
• juandice

Question 3

Primary sclerosing cholanigits

Answer 3

obliterative fibrosis of intra and extra hepatic bile ducts with inflammation and dilation of preserved segments

• ERCP-beading
• associated with IBD
• cholestatic symptoms (pruritis)
• progress to end stage liver disease
• transplant
• risk of cholangiocarcinoma
• males 20-40 X2

bile duct onion skin