L'OXALOSE PRIMAIRE DE TYPE I. MISE AU POINT ET STRATEGIE THERAPEUTIQUE ACTUELLE

Primary oxalosis type I is due to an abnormal metabolism of glyoxylate at the hepatic level responsible of hyperoxaluria. In affected patients, a deposition of calcium oxalate crystals occurs throughout body tissues and the excessive urinary excretion of oxalic acid is the cause of renal and urinary tract lithiasis and nephrocalcinosis leading to renal insufficiency. As soon as the diagnostic has been established (by estimation of blood and urinary levels of oxalate, urinary metabolites and the enzyme activity on a liver biopsy), a medical treatment has to be started. The renal function has to be followed accurately as well as oxalate levels in blood and urine. In the resistant forms of the disease, liver transplantation is the only way to correct the metabolic defect. Renal transplantation may or has to be done at the same time if the renal function is impaired to improve the renal function and to accelerate the removal of oxalate.