The primary hyperoxalurias are rare, inherited disorders, characterized by marked increases in urine oxalate excretion of 2 to 6 times the upper limit of normal. Primary hyperoxaluria can cause kidney failure, severe systemic disease (oxalosis), and death if not adequately treated. Primary hyperoxaluria disorders have clinical and biochemical similarities that make differentiation difficult, and without careful testing, patients may be misclassified. The consensus algorithm that was developed at the November 2003 Oxalosis and Hyperoxaluria meeting in Annapolis, Md. to assist in diagnosis was recently updated and is included in this issue.
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