What is new in primary hyperoxaluria?

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Abstract
Although the majority of renal stones both in children and in adults are formed by calcium oxalate (CaOx), few patients actually suffer from a primary metabolic defect leading to excessive endogenous production of oxalate. Nevertheless, there is considerable interest in primary hyperoxaluria (PH)—the prototype and model of hyperoxaluria—since a better understanding of the pathogenic mechanisms is also expected to help the much larger group of patients with urolithiasis not suffering from PH. Two recent symposia (NIH Workshop, 8–9 December 1998, Bethesda, USA and 5th Workshop on PH, 12–13 March 1999, Zurich, Switzerland) have dealt with these problems [1]. It has become obvious that PH is a highly complex disorder, consisting of two well-described forms (PH1 and PH2) and of further conditions.