primary hyperoxaluria

hyperoxaluria

 [hi″per-ok″sah-lu´re-ah] an excess of oxalates in the urine, which can lead to formation of kidney stones. Called also oxaluria.enteric hyperoxaluria formation of calculi" >calcium oxalate calculi in the urinary tract, occurring after extensive resection or disease of the ileum, due to excessive absorption of oxalate from the colon.primary hyperoxaluria an autosomal recessive disorder characterized by urinary excretion of oxalate, with nephrolithiasis, nephrocalcinosis, early onset of renal failure, and often a generalized deposit of calcium oxalate.

primary hyperoxaluria

Oxalosis An AR condition characterized by crystallization in tissues Clinical Calcium oxalate–CO is ↑↑↑ in urine, resulting in CO supersaturation crystalluria, stone formation, and deposition of CO in the renal parenchyma, leading to renal dysfunction and CO deposition in multiple organs–oxalosis Treatment Orthophosphate, pyridoxine Prognosis With therapy, actuarial ESRD-free survival at 20 yrs is ± 75%; sans therapy, 80% have ESRD by the 3rd decade of life; renal transplants do not survive well; liver transplants may be used to correct the enzyme defect

primary hyperoxaluria

An inherited metabolic disease caused by a defect in glyoxalate metabolism. This causes an increased secretion of oxalate in the urine, renal calculi, renal failure, and generalized deficit of oxalate crystals in tissues. See also: hyperoxaluria