Unusual morphology of calcium oxalate calculi in primary hyperoxaluria

Abstract

Primary hyperoxaluria (PH) is a severe inherited disease induced by an enzymatic deficiency responsible for high endogenous production of oxalate. Oxalate ions are excreted by the kidney where they can form an insoluble salt with calcium ions, thus inducing urinary stones, crystal deposition in the tubular lumen and renal parenchyma leading to nephrocalcinosis and renal failure. Eighty-seven calculi from 63 PH patients with primary hyperoxaluria were analyzed and compared to 24,130 calculi from unselected consecutive stone formers referred to our laboratory between January 1977 and December 1996. All stones were analyzed according to a protocol including morphological examination of both surface and cross-section, and sequential infrared identification of the crystalline phases. A typical aspect of both surface and section corresponding to morphological type Ic according to our proposed classification (Daudon et al. Scanning Microsc 1993, 7:1081-1106) was observed in all patients but two whereas only two type Ic stones were observed among patients without primary hyperoxaluria. The latter two patients suffered from severe inflammatory bowel disease and developed heavy hyperoxaluria following extensive ileal resection. We conclude that evidence of type Ic morphology is a simple, cheap and fast tool to detect diseases with heavy hyperoxaluria such as primary hyperoxaluria.