Liver and intrahepatic bile ducts-nontumor
Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 30 April 2012, last major update April 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.
● 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
● 39 year old woman with recurrent nephrolithiasis (Arch Pathol Lab Med 2002;126:1250)
● Increase urine volume; pyridoxine, high phosphate diet
● Crystals are birefringent
Crystals in portal areas
Oxalate crystal in hepatic parenchyma
● 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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