Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020500 (hyperoxaluria)
 912 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The medical history of a 42-year-old patient with primary hyperoxaluria type I is presented. Primary hyperoxaluria was suspected after renal transplantation, when oxalate deposits were found in a biopsy of the kidney graft. Diagnosis of type I hyperoxaluria was confirmed by the finding that significantly increased amounts of glycolic acid and oxalic acid were excreted. Treatment of the patient with 500 mg pyridoxine daily resulted in a decrease of the excretion of oxalate to normal values.
Nephrol Dial Transplant 1989
PMID:Recurrence of nephrocalcinosis after renal transplantation in an adult patient with primary hyperoxaluria type I. 249 56

The probability of being a stone former (PSF) was calculated according to the method of Robertson in three groups of idiopathic calcium stone formers (normocalciuria (NCa), dietary hypercalciuria (DH) and idiopathic hypercalciuria (IH] during four conditions: on a free diet; on a calcium and oxalate restricted diet for four days and after an oxalate load (200 g of spinach) while on a calcium unrestricted or calcium restricted diet. Combined calciuria (Ca) and oxaluria (Ox) restriction significantly decreased PSF only in NCa and DH whereas the decrease was not significant in IH because of a concomitant significant increase in oxalate excretion. Increase of PSF with the oxalate load was significantly greater on calcium restricted than on calcium unrestricted diets in all groups of patients (4-6-12 times greater in NCa, DH and IH respectively). This shows the critical role of oxalate restriction when calcium is restricted in order to decrease the PSF. Combined restriction is not sufficient in idiopathic hypercalciuric patients to decrease their probability of stone formation.
Proc Eur Dial Transplant Assoc 1983
PMID:Critical role of oxalate restriction in association with calcium restriction to decrease the probability of being a stone former: insufficient effect in idiopathic hypercalciuria. 665 62

Nine male healthy volunteers were examined during a control period, during an oral glycine load (45 g/day, 600 mmol) and oral methionine (6 g/day, 40 mmol). Glycine caused a significant increase of urinary oxalate above baseline (from 644 to 797 mumol/day) without change in calciuria (4.74 vs 4.84 mmol/day). In contrast methionine caused no change of oxaluria, but a significant increase in calciuria (from 4.74 to 6.9 mmol/day). Alterations of lithogenic ions in urine after protein ingestion are mediated by different amino acids. The particular lithogenic risk of animal protein may be related to its high methionine/cystine and glycine content.
Proc Eur Dial Transplant Assoc 1983
PMID:Different effects of oral glycine and methionine on urinary lithogenic substances. 665 63

Primary hyperoxaluria (PH) type 1 and type 2 are autosomal recessive defects of oxalate metabolism resulting from glyoxylate accumulation which occurs by two distinct pathways. PH1 is associated to glycolic aciduria; PH2 to L-glyceric aciduria. Because hyperoxaluria leads to nephrolithiasis or nephrocalcinosis in both, they can be differentiated only through detection of the associated acidurias. However, glycolate and L-glycerate assays are not widely available and, in the setting of ESRF, diagnosis is hampered by a number of misleading events. At any stage of the disease diagnosis is crucial because there are differences between the two forms in clinical behaviour, long-term prognosis, and treatment. In this paper we outline diagnostic criteria for identification of PH2 in two patients, one with maintained renal function and one with ESRF on CPD, based on the use of a novel HPLC assay of L-glycerate in different body fluids. With the routine application of this procedure PH2 has been identified in two of 23 patients fulfilling criteria for diagnosis of PH. This suggests that the type 2 variant of PH may occur more frequently than so far suspected, and should be tested for even in the setting of ESRF.
Nephrol Dial Transplant 1995
PMID:Detection of primary hyperoxaluria type 2 (L-glyceric aciduria) in patients with maintained renal function or end-stage renal failure. 853 30

We report two cases of acute renal failure in patients with arteriosclerosis obliterans treated by intravenous infusion of naftidrofuryl oxalate. At renal biopsy the histological lesions were identical with those found in ARF due to hyperoxaluria of other causes, revealing tubular epithelial necrosis and massive intratubular precipitation of calcium oxalate monohydrate (C1) crystals. A second study was then conducted in four other patients with arteriosclerosis obliterans to evaluate serum and urinary levels of oxalate, and crystalluria during the intravenous administration of 800 mg of naftidrofuryl oxalate per day for 10 days. During the course of treatment, the serum and urinary oxalate levels were found to increase substantially, with the gradual onset of massive C1 crystalluria. These results indicate that naftidrofuryl oxalate was responsible for the acute renal failure in the first two patients. High intravenous doses of naftidrofuryl oxalate must be used cautiously, with close surveillance of renal function.
Nephrol Dial Transplant 1995
PMID:Potential nephrotoxicity of intravenous infusions of naftidrofuryl oxalate. 880 2

Estimating calcium oxalate saturation (beta CaOx) in body fluids is proposed as a simple and reproducible procedure to assess the risk of systemic oxalosis in several clinical conditions associated with oxalate retention. beta CaOx was computerized from the measured concentrations of main serum ions. Accurate assay of serum oxalate was crucial for reliability of beta CaOx estimates. However, beta CaOx also depended upon changes of calcium and magnesium concentrations. Patients with end-stage renal failure (ESRF) due to primary or enteric hyperoxaluria had beta CaOx greater than saturation, whereas this happened in only 10 of 25 and two of 24 of those with oxalosis-unrelated ESRF. Bony content of oxalate measured in some of these patients was consistent with these results. In patients with maintained renal function beta CaOx was inversely related to glomerular filtration rate, but the slope was steeper in patients with than in those without hyperoxaluria and beta CaOx reached saturation at earlier stages of renal insufficiency.
Nephrol Dial Transplant 1995
PMID:The clinical significance of assessment of serum calcium oxalate saturation in the hyperoxaluria syndromes. 859 17

Most cases of primary hyperoxaluria are due to deficiency of hepatic peroxisomal alanine:glyoxylate aminotransferase [i.e. primary hyperoxaluria type 1 (PH1), McKusick 259900] and several hundred examples have been described since the original report in 1925. By contrast, primary hyperoxaluria type 2 (PH2, McKusick 260000) is very rare indeed with only 22 patients recorded since the original description in 1968. PH2 is characterized by hyperoxaluria and L-glyceric aciduria and is caused by deficiency of D-glycerate dehydrogenase/glyoxylate reductase. In comparison with PH1 much less is known about PH2 and considerable uncertainties remain about its frequency, clinical course and optimum management.
Nephrol Dial Transplant 1995
PMID:Primary hyperoxaluria type 2. 859 29

Hyperoxaluria is a frequent complication of inflammatory bowel diseases, ileal resection and Roux-en-Y gastric bypass and is well-known to cause nephrolithiasis and nephrocalcinosis. The associated prevalence of chronic kidney disease and end-stage kidney disease (ESKD) is less clear but may be more consequential than recognized. In this review, we highlight three cases of ESKD due to enteric hyperoxaluria following small bowel resections. We review current information on the pathophysiology, complications and treatment of this complex disease.
Nephrol Dial Transplant 2016 Mar
PMID:Enteric hyperoxaluria: an important cause of end-stage kidney disease. 2570 16

Hyperoxaluria after Roux-en-Y gastric bypass (RYGB) increases the risk for kidney injury. Medical therapies for hyperoxaluria have limited efficacy. A 65-year-old female was evaluated for acute kidney injury [AKI, serum creatinine (Cr) 2.1 mg/dl, baseline Cr 1.0 mg/dl]. She did not have any urinary or gastrointestinal symptoms or exposure to nephrotoxic agents. Sixteen months prior to this evaluation, she underwent RYGB for morbid obesity. Her examination was unremarkable for hypertension or edema and there was no protein or blood on urine dipstick. Kidney biopsy revealed acute tubulointerstitial nephritis with oxalate crystals in tubules. The concurrent finding of severe hyperoxaluria (urine oxalate 150 mg/day) confirmed the diagnosis of oxalate nephropathy. Despite medical management of hyperoxaluria, her AKI worsened. Laparoscopic reversal of RYGB was performed and within 1 month, her hyperoxaluria resolved (urine oxalate 20 mg/day) and AKI improved (Cr 1.7 mg/dl). Surgical reversal of RYGB may be considered in patients with oxalate nephropathy at high risk of progression who fail medical therapy. Physicians need to be aware of the possibility of oxalate nephropathy after RYGB and promptly treat the hyperoxaluria to halt further kidney damage.
Case Rep Nephrol Dial
PMID:Reversal of Gastric Bypass Resolves Hyperoxaluria and Improves Oxalate Nephropathy Secondary to Roux-en-Y Gastric Bypass. 2778 Dec 7