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Query: UMLS:C0020500 (hyperoxaluria)
 912 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 45-year-old man underwent a jejunoileal shunt procedure for obesity. Twenty months later he developed severe oxalosis and chronic renal failure, which required maintenance hemodialysis. The sequential observation of two biopsy specimens and the necropsy (over a span of 39 months) suggests that oxalate deposition caused tubular obstruction and destruction with subsequent atrophy of nephrons. This indicates that patients undergoing intestinal bypass are at risk for developing irreversible renal failure due to enteric hyperoxaluria.
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PMID:Oxalosis and chronic renal failure after intestinal bypass. 83 9

A patient who underwent jejunoileal bypass for morbid obesity developed servere renal failure associated with hyperoxaluria and renal oxalosis. Renal function improved and oxalate excretion decreased following hemodialysis and restoration of gastrointestinal continuity.
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PMID:Acute reversible renal failure following jejunoileal bypass for morbid obesity: a clinical and pathological (EM) study of a case. 91 50

A case is presented in which primary hyperoxaluria and oxalosis in a 14-year-old Caucasian female were diagnosed. Generalized root resorption resulted in a remarkable mobility of her maxillary central and lateral incisors, although no bone loss was noted. The management of the patient's dental concerns in this rare heritable metabolic disorder consisted of removing the maxillary incisor teeth and placing two sequential prostheses, which the patient tolerated well. A history of trauma to the maxillary incisors was ruled out, so this case adds previously unreported information to our knowledge about the effect of oxaluria on teeth and oral tissues.
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PMID:Primary hyperoxaluria in a pediatric dental patient: case report. 130 27

The following main conclusions concerning the transplantation strategies to be adopted in primary hyperoxaluria type 1 (PH1) were drawn from the data collected from 22 patients who received combined liver-kidney grafts and 2 patients who received isolated liver grafts in Europe from June 1984 to March 1990. In end-stage renal failure due to PH1 liver-kidney transplantation yields better results than conventional renal transplantation. An isolated liver graft should be planned in patients with GFR between 25 and 60 ml/min/1.73 m2 whereas a combined liver-kidney graft is to be recommended as soon as the GFR falls below 25 ml/min/1.73 m2. Such patients should not be maintained on dialysis for more than a few months since they would unavoidably develop oxalosis with the risk of disabling lesions in the skeleton and cardiovascular system. Besides, oxalosis would be regularly followed by long-standing hyperoxaluria, with the risk of damage to the kidney graft, despite the correction of the enzyme deficit brought up by the liver graft.
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PMID:[Which transplantation strategies in primary hyperoxaluria type 1?]. 192 55

The detection of type I primary hyperoxaluria is based on the finding of exceedingly high oxalate excretion which is associated with increased glycolate excretion. The differential diagnosis of this disease may become a difficult task once end-stage renal disease (ESRD) and anuria have supervened. The various procedures thus far proposed to obviate this circumstance are complex, inaccurate or not reproducible. In this paper we propose the accurate liquid chromatographic determination of glycolate in blood and dialysate as a means to detect type I primary hyperoxaluria in patients on maintenance hemodialysis (RDT). The method is based on the enzymatic conversion of glycolate to glyoxylate coupled with alpha-keto acid derivatization with phenylhydrazine. The resulting phenylhydrazone is then resolved by high-performance liquid chromatograph (HPLC). With this method, plasma glycolate in 12 healthy controls was 7.8 +/- 1.7 mumol/liter, almost twentyfold less than previously reported. Five dialysis patients with high serum oxalate, of whom four with primary hyperoxaluria and one with Crohn's disease and presumed enteric oxalate hyperabsorption, were checked by this method and compared to nine patients with oxalosis-unrelated ESRD. The patients with hyperoxalemia were also evaluated for their response to pyridoxine therapy. The measurement of glycolate in blood drawn prior to and at the end of the dialysis session as well as in the dialysate soundly discriminated the patients with type I hyperoxaluria from all the other dialysis patients. Glycolate measurement was shown to be much more powerful than oxalate in that patients with oxalosis-induced ESRD exhibited an almost two hundred and fiftyfold increase compared to the oxalosis-unrelated patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Glycolate determination detects type I primary hyperoxaluria in dialysis patients. 200 28

Primary hyperoxaluria type I (PH I) is characterized by an excessive endogenous production and excretion of oxalic and glycolic acid. Prognosis of this "inborn error of metabolism" is not favorable due to calcium-oxalate depositions in kidney and other organs. Vitamin B6 administration and/or renal transplantation can greatly improve the prognosis, as reported in literature. In this article our experience with 5 patients with vitamin B6 resistant hyperoxaluria is reported. Symptomatology and progression of the primary disease are described. The results of treatment interfering with oxalate production and calcium-oxalate crystallization are given. Three patients underwent renal replacement therapy. In these, oxalosis developed during hemodialysis and progressed following transplantation; a disabling bone disease was the most severe complication. Outcome of transplantation was disappointing. In two out of three patients, there was recurrence of the primary disease in the graft. In only one of them long-term graft function was satisfying. However, even this good function could not prevent disabling symptoms of oxalosis. Therefore, evaluation of the results of transplantation should not only include data related to graft function and survival, but also the complications due to calcium-oxalate depositions in various organs. To prevent oxalosis, kidney transplantation should be performed before end stage renal disease is achieved in patients with vitamin B6 resistant PH I.
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PMID:Vitamin B6 resistant primary hyperoxaluria type I. Report of 5 cases. 270 72

Type I hyperoxaluria results from reduced activity of alpha-ketoglutarate: glyoxylate carboligase, which is necessary for the synergistic decarboxylation of glyoxylate and alpha-ketoglutarate to alpha-hydroxy-beta-keto-adipate. Since thiamine pyrophosphate is a cofactor in the reaction, thiamine deficiency might be expected to result in tissue oxalosis. However, there was no significant increase in the incidence of renal oxalosis in 15 patients with Wernicke's encephalopathy at necropsy compared with controls. It is possible that hyperoxaluria was present in these thiamine-deficient patients but at a urine concentration below that necessary for calcium oxalate deposition. It is also possible that the severity of the thiamine deficit required for hyperoxaluria exceeds that for the neuronal and cardiac manifestations.
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PMID:Thiamine deficiency and oxalosis. 437 Dec 54

The radiological changes in the kidneys and skeleton in primary oxalosis in a patient are described. Crystalline calcium oxalate deposits, which are a secondary phenomenon, are responsible for the findings, which are of great significance both functionally and prognostically. Hyperoxalaemia is due to an enzyme defect, which leads to hyperoxaluria; this causes a reduction in renal function and can lead to rapid progression of the condition.
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PMID:[Primary oxalosis in childhood. Plain film findings during the course of the disease (author's transl)]. 621 32

In a patient with long-standing Crohn's disease and bowel resection, acute reversible renal failure associated with hyperoxaluria developed. The renal biopsy specimen demonstrated marked oxalosis. Acute renal failure, although rare, is a complication of enteric hyperoxaluria of Crohn's disease. This type of renal failure may be reversible if recognized and treated early, since remission of symptoms occurred following hemodialysis.
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PMID:Oxalate-induced acute renal failure in Crohn's disease. 742 52

In three patients with end-stage renal failure due to primary hyperoxaluria type 1, successful combined liver-kidney transplantation enabled us to assess the insoluble oxalate pool, which was compared with the histopathological changes observed in iliac crest biopsy specimens. Good correlation was observed between the histopathological grade of bone oxalosis and the estimated oxalate content of the body. In the end-stage of oxalate bone disease, hyperparathyroidism does not play a significant role in bone resorption, which appears to be the consequence of the granulomatous reaction induced by oxalate deposition. Combined liver-kidney transplantation should be performed long before this stage. Early hepatorenal grafting in uremia secondary to primary hyperoxaluria type 1 would avoid the deleterious clinical consequences of systemic oxalosis and shorten the duration of postransplant hyperoxaluria, which may compromise the course of kidney graft.
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PMID:Combined liver-kidney transplantation in primary hyperoxaluria type 1. Bone histopathology and oxalate body content. 760 40


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