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Primary hyperoxaluria affecting the liver

A 39-year-old African American woman with a history of primary hyperoxaluria and recurrent nephrolithiasis underwent bilateral nephrectomies and consequently a combined kidney-liver transplantation as a means to cure her disease. Her posttransplantation course was complicated by hepatic artery thrombosis and secondary bile duct necrosis and bilioma formation. The bilioma was drained, and the patient is currently doing well.

Oxalate is an end product of metabolism that is normally excreted by the kidneys. Primary hyperoxaluria is a rare autosomal recessive disease with two variants: type I and type II. Primary hyperoxaluria type I (PHI) results from a functional defect or absence of ...

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