Hyperoxaluria in children with hepatic and intestinal dysfunction. J. P. K. McCollum, S. Packer, J. Manning, and J. T. Harries. The Hospital for Sick Children, Great Ormond Street, and Institute of Child Health, London, W.C.1. The association of hyperoxaluria and renal oxalate stones has recently been reported in adults after small intestinal resection; the hyperoxaluria may be related to distu...

Renal failure can occasionally be caused by oxalate nephropathy. Glyoxylate metabolism abnormalities and particular hepatic enzyme deficits are the causes of primary hyperoxaluria (PH). Increased intestinal absorption, an excessive diet or increased intake precursors all result in secondary hyperoxaluria. A 13-month old male child with high blood creatinine, low sodium, calcium levels, uric aci...

The following is a case report of a patient with type I hyperoxaluria who received a living related renal transplant for end-stage renal disease. The deficiency or complete absence of the hepatic enzyme alanine glyoxalate aminotransferase characterizes type I hyperoxaluria. When deficient, an overproduction of oxalate occurs, which then can be deposited in multiple end-organ systems. When perfo...

1. Danpure CJ. Primary hyperoxaluria. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic and Molecular Bases of Inherited Disease. New York: McGraw-Hill, 2001:3323–67. 2. van Woerden CS, Groothoff JW, Wanders RJ, Davin JC, Wijburg FA. Primary hyperoxaluria type 1 in The Netherlands: prevalence and outcome. Nephrol Dial Transplant 2003;18:273–9. 3. van Woerden CS, Groothoff JW, Wijb...

Oxalic acid is an end product of human metabolism and does not appear to be needed for any process in the body. Under normal conditions the daily load of oxalate deriving from endogenous production and intestinal absorption is fully excreted by the kidneys. Up to a certain extent renal oxalate excretion may even keep pace with an elevated oxalate load, yet at the expense of hyperoxaluria, a maj...

BACKGROUND Urinary oxalate excretion is an important contributor to calcium oxalate stone formation. Methods of reducing oxalate excretion are not wholly satisfactory, and no controlled trials using them have been performed to prevent stone recurrence. Some lactic acid bacteria can degrade oxalate in vitro. This study sought to reduce urinary oxalate excretion in calcium stone formers with idio...

Primary hyperoxalurias are rare recessive inherited inborn errors of glyoxylate metabolism. They are responsible for progressive renal involvement, which further lead to systemic oxalate deposition, which can even occur in infants. Primary hyperoxaluria type 1 is the most common form in Europe and is due to alanine-glyoxylate aminostransferase deficiency, a hepatic peroxisomal pyridoxin-depende...

Secondary hyperoxaluria is due either to increased intestinal oxalate absorption or to excessive dietary oxalate intake. Certain intestinal diseases like short bowel syndrome, chronic inflammatory bowel disease or cystic fibrosis and other malabsorption syndromes are known to increase the risk of secondary hyperoxaluria. Although the urinary oxalate excretion is usually lower than in primary hy...