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- hepatic fibrosis
- intrahepatic biliary ducts
- Caroli’s disease
- autosomal recessive polycystic kidney disease
- congenital hepatic fibrosis
A 21 year old Asian female presented to our clinic with impaired renal function (creatinine level 217 μmol/l). There was no history of note. Physical examination elicited hepatosplenomegaly. There were no other positive findings. Urine analysis was unremarkable. Laboratory investigations demonstrated a microcytic anaemia, with depressed total white cell and platelet counts, consistent with hypersplenism. Normal synthetic liver function was noted. Alkaline phosphatase levels were raised (401 iu/l).
Past medical notes (initially not available) described hepatosplenomegaly since birth, first noted in Pakistan. Liver biopsy performed in the UK at the age of two years demonstrated increased collagen deposition around the bile ducts. The patient had been lost to follow up.
Abdominal ultrasound demonstrated normal sized kidneys with hepatosplenomegaly (normal liver echotexture) and a dilated extrahepatic common bile duct (1.1 cm). The gall bladder appeared normal with no calculi. Computed tomography of the abdomen confirmed these findings.
A magnetic resonance cholangiopancreatography scan was performed fig 1.
What do these scans show and what is the unifying diagnosis?
See page 1331 for answer
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