BACKGROUND We report a case of domino liver transplantation using the liver harvested from a patient who underwent a combined liver and kidney transplantation for primary hyperoxaluria (PH). METHOD A cadaveric liver transplantation was performed in a 19-year-old man with PH. In a second step, the PH liver harvested from the first patient was transplanted in a 69-year-old man with hepatitis C-...

BACKGROUND Definitive diagnosis of primary hyperoxaluria type 1 (PH1) requires analysis of alanine:glyoxylate aminotransferase (AGT) activity in the liver. We have previously shown that targeted screening for the 3 most common mutations in the AGXT gene (c.33_34insC, c.508G>A, and c.731T>C) can provide a molecular diagnosis in 34.5% of PH1 patients, eliminating the need for a liver biopsy. Havi...

The objective of this study was to investigate ethnic differences in the glyoxylate reductase/hydroxypyruvate reductase (GRHPR) gene in patients with primary hyperoxaluria type 2 (PH2). GRHPR was genotyped in Japanese patients with PH2 and all GRHPR mutations described to date were reviewed in terms of geographic and ethnic association. We identified a novel mutation, a two-nucleotide deletion ...

Oxalate is a metabolic end product excreted by the kidney. Mild increases in urinary oxalate are most commonly associated with Nephrolithiasis. Chronically high levels of urinary oxalate, as seen in patients with primary hyperoxaluria, are driving factor for recurrent renal stones, and ultimately lead to renal failure, calcification of soft tissue and premature death. In previous studies others...

Environment and diet have a major role in calcium nephrolithiasis by affecting urine saturation, but this is not enough to cause lithogenesis; the crystals must adhere to the tubular epithelium (TE), but it is hard to say how environment and nutrition may be involved in this step. The hypothesis that TE damage (known to enhance crystal attachment) is lithogenic in mild hyperoxaluria was tested....

Abstract Background and Aims Oxalate nephropathy is a rare cause of renal failure which usually due to primary or secondary hyperoxaluria. Secondary hyperoxaluria results from increased intake intestinal oxalate availability (enteric hyperoxaluria), degradation, colonic permeability [1]. Little known about the burden end stage kidney disease in this group patients as most initial literature was...

doi: 10.1007/s12519-010-0214-z ©Children's Hospital, Zhejiang University School of Medicine, China and Springer-Verlag Berlin Heidelberg 2010. All rights reserved. Background: Primary hyperoxaluria type 1 is a rare disorder caused by a defect in the hepatic metabolism of glyoxylate. Cases presenting in infancy are very uncommon and often have a severe course leading to early end-stage renal fai...

Vediyuppu cheyaneer has been widely used in Siddha system of medicine for various diseases. The liquid form of Vediyuppu cheyaneer showed a significant inhibitory effect when screened at 500 mg/kg, for the invivo antiurolithiasis activity on ethylene glycol induced hyperoxaluria rats. The standard frusemide (20 mg/kg) and cystone (750 mg /kg) were used as comparison for diuretic and antiurolith...

OBJECTIVES To review the possible causes of the high incidence of urolithiasis in the oil-rich Gulf States. METHODS Data were extracted from published reports on the incidence of urolithiasis, affluence and diet in the Gulf States, various Western countries and China. RESULTS There are strong relationships: (a) between the life-expectancy of stones in men and the Gross National Income (GNI)...