Opportunities in Primary and Enteric Hyperoxaluria at the Cross-Roads Between the Clinic and Laboratory

Abstract

Hyperoxaluria is a condition in which there is a pathologic abundance of oxalate in the urine through either hepatic overproduction (primary hyperoxaluria [PH]) or excessive enteric absorption of dietary oxalate (enteric hyperoxaluria [EH]). Severity can vary with the most severe forms causing kidney failure and extrarenal manifestations. To address the current challenges and innovations in hyperoxaluria, the 14th International Hyperoxaluria Workshop convened in Perugia, Italy, bringing together international experts for focused presentation and discussion. The objective of the following report was to disseminate an overview of the proceedings and provide substrate for further thought. The format of this paper follows the format of the meeting, addressing, "PH type 1" (PH1) first, followed by "surgery, genetics, and ethics in PH", then "PH types 2 and 3," (PH2 and PH3) and, finally, "EH." Each session began with presentations of the current clinical challenges, followed by discussion of the latest advances in basic and translational research, and concluded with interactive discussions about prioritizing the future of research in the field to best serve the need of the patients.

Figure 1

Overview of the pathways controlling oxalate homeostasis. AGT, alanine:glyoxylate aminotransferase; GO, glycolate oxidase; GR/HPR, glyoxylate hydroxypyruvate reductase; HOG, 4-hydroxy-2-oxoglutarate; HOGA1, HOG aldolase; HyPro, hydroxyproline; LDH, lactate dehydrogenase.