Home   Chapter Home   Jobs   Conferences   Fellowships   Books



Advertisement

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
=========================================================================

● 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
=========================================================================

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

Treatment
=========================================================================

● Increase urine volume; pyridoxine, high phosphate diet

Micro description
=========================================================================

● Crystals are birefringent

Micro images
=========================================================================



Crystals in portal areas

   
Oxalate crystal in hepatic parenchyma

Differential diagnosis
=========================================================================

● 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


This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).