Liver and intrahepatic bile ducts-nontumor

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Liver and intrahepatic bile ducts-nontumor

Metabolic diseases

Primary hyperoxaluria

Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 30 April 2012, last major update April 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.

General
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● Autosomal recessive, either type 1 (defect/absence of alanine-glyoxalate aminotransferase on 2q37.3; patients have variable clinical presentation from end stage renal disease to occasional kidney stones) or type 2 (absence of glyoxylate reductase activity at #9)
● Pathophysiology: oxalate is a metabolic end product normally excreted by kidneys; type 1 or 2 disease causes increased oxalate synthesis and excretion, eventually deposition of insoluble calcium oxalate in kidney, bones, heart, arteries
● In liver, deposited in portal areas and arterial media

Case reports
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● 39 year old woman with recurrent nephrolithiasis (Arch Pathol Lab Med 2002;126:1250)

Treatment
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● Increase urine volume; pyridoxine, high phosphate diet

Micro description
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● Crystals are birefringent

Micro images
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Crystals in portal areas

Oxalate crystal in hepatic parenchyma

Differential diagnosis
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● Increased Vitamin C, methoxyflurane, ethylene glycol, xylitol, chronic inflammatory bowel disease, small bowel resection, external biliary drainage

End of Liver and intrahepatic bile ducts-nontumor > Metabolic diseases > Primary hyperoxaluria

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