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Query: UMLS:C0035078 (renal failure)
 31,970 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Enteric hyperoxaluria due to malabsorption syndromes has been well documented to cause renal calculi and chronic tubulointerstitial renal damage. Rarely, in the setting of intestinal bypass operations for morbid obesity, enteric hyperoxaluria has produced acute renal failure. We report two patients who suffered acute deterioration of renal function associated with increased intestinal absorption and renal excretion of oxalate associated with steatorrhea. One patient had a large portion of his small bowel resected many years prior to the onset of the renal failure and the second patient had chronic pancreatitis causing steatorrhea. Both patients had renal biopsy documentation of the acute nature of the tubular damage produced by oxalate deposition. The mechanisms of their deterioration of renal function may relate to sudden increases in steatorrhea in association with episodes of volume depletion. Enteric hyperoxaluria may be an easily overlooked and potentially preventable etiology of acute renal dysfunction.
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PMID:Acute deterioration of renal function associated with enteric hyperoxaluria. 222 62

Primary oxalosis is a rare inborn error of oxalate metabolism. Most cases are discovered in children, but occasionally symptoms begin later in life. Since early deaths in the past were from renal failure, prolonged survival obtained with chronic dialysis allows oxalosis to develop. This paper presents a 38-year-old man with an atypical history of type-I primary hyperoxaluria, not diagnosed until after 5 years of dialysis. Bone biopsy was performed because the biochemical and radiologic features did not seem consistent with a putative diagnosis of secondary hyperparathyroidism. This case emphasizes the clinical heterogeneity of this disorder, and the need for its considerations in the spectrum of dialysis-related bone diseases. It also stresses that bone oxalosis may mimic hyperparathyroidism, especially radiologically. Differential diagnosis is therefore mandatory.
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PMID:Primary oxalosis mimicking hyperparathyroidism diagnosed after long-term hemodialysis. 224 64

Primary hyperoxaluria (oxalosis) is an autosomal recessive disorder due to an inherited deficiency of the peroxisomal alanine:glyoxylate aminotransferase characterized by increased production and urinary excretion of oxalate and glycolate resulting in renal failure due to oxalate deposition. Because of the risk of continuing oxalate deposition in the transplanted kidney, oxalosis had been considered a contraindication for transplantation. A 5-year-old boy with oxalosis, maintained on peritoneal dialysis, received a haploidentical qiving-related transplant. The preoperative management included donor-specific transfusions and daily hemodialysis to remove a maximum amount of oxalate. The immunosuppression consisted of azathioprine and prednisone. Aggressive fluid management including noncalciuric diuretics (hydrochlorothiazide) kept urine output high. Pyridoxine, magnesium, neutral phosphate and sodium benzoate were used to prevent deposition of oxalate in the transplanted kidney. Two acute rejection episodes responded to steroid boluses. A kidney biopsy during the second rejection episode confirmed the diagnosis but also revealed oxalate deposits in the transplanted kidney. More than 4 years after transplantation, the patient has catch-up growth and his serum creatinine is 1.4 mg/dl. In conclusion, oxalosis is not an absolute contraindication to renal transplantation. Transplantation can be performed successfully utilizing living-related donor kidneys and aggressive medical management. The risks of deterioration of function and oxalate deposition in the transplant kidney are offset by improvement in quality of life.
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PMID:Renal transplantation in a child with primary oxalosis. 225 46

Oxalosis, the systemic deposition of calcium oxalate crystals, may occur in several hyperoxaluric states due to increased production or absorption of oxalate. Type I primary hyperoxaluria (PH I) is a rare autosomal recessive disease caused by deficiency of the peroxisomal enzyme alanine:glyoxylate aminotransferase. Most patients with this disorder are noticed in mid-childhood or even later due to symptoms related to urinary stone disease. In this paper, we report a patient with PH I with a rapid downhill progression to renal failure and death. Oxalosis was detected by renal biopsy, and the diagnosis of PH I was confirmed by increased urinary oxalate and glycolate levels.
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PMID:Early and unusual presentation of type I primary hyperoxaluria. 225 47

Patients with chronic renal failure who undergo hemodialysis experience accelerated atherosclerosis and premature death. Since the end-metabolite, oxalic acid, accumulates in plasma in proportion to the severity of renal failure, we studied whether sodium oxalate (0 to 300 microM) is an endothelial toxin and, therefore, might enhance atherogenesis. Exposure to uremic levels of oxalate (greater than 30 microM) for 9 to 28 days depressed endothelial cell replication by 33% to 84% (mean +/- SD, 54% +/- 15.7%, n = 17 experiments, p = 0.002). In contrast, replication of fibroblasts exposed to 200 microM oxalate for 45 days was not inhibited. The inhibitory effect of oxalate on endothelial cell replication was both dose- and time-dependent (both p less than 0.0001) and was first detected 3 to 7 days after the initial exposure to oxalate. Further, the inhibitory effect was fully reversible upon removal of oxalate, but only if exposure was limited to 5 days or less. Sodium salts of other carboxylic acids (citric, succinic, glyoxylic, and malonic; 200 microM) as well as HCl (200 microM) did not suppress endothelial cell replication. Oxalate also inhibited endothelial cell migration but had no effect on basal, thrombin-induced, or arachidonate-induced prostacyclin production by endothelial cells. Exposure of endothelial cells to sodium oxalate (200 microM) for as little as 24 hours-a time period sufficient to induce delayed, transient inhibition of replication not detectable until approximately 1 week after exposure-inhibited incorporation of 3H-leucine into protein by 40% (p = 0.009). We conclude that sodium oxalate acts as a uremic toxin, inhibiting endothelial cell replication and migration, functions which may be important for constitutive inhibition of atherosclerosis.
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PMID:Uremic levels of oxalic acid suppress replication and migration of human endothelial cells. 231 57

Primary oxalosis should be considered in patients with multisystem disease of the kidneys, heart, peripheral vasculature, and skin. Crystalline deposits can lead to nephrolithiasis with kidney failure, complete heart block, peripheral vasospasm, and livedo reticularis, as in our patient. Crystals were first observed in the myocardial biopsy specimen and then identified as calcium oxalate in skin from an area of livedo reticularis.
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PMID:Clinical and histologic features of primary oxalosis. 206 63

A case of primary hyperoxaluria which presented with progressive renal failure in a 34 year old woman is reported. The patient died six years following the initial diagnosis and post mortem examination revealed widespread deposition of oxalate crystals in her tissues.
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PMID:Primary hyperoxaluria--a case report. 236 24

Results of treatment of end-stage renal failure (ESRF) in patients with primary hyperoxaluria have been generally poor, largely due to the inability of any treatment regime to match the endogenous overproduction of oxalate. The severity of the disease varies widely, as reflected by the differences in age at onset of ESRF. This variability may influence the results of treatment of ESRF in these patients. The longest reported survival on haemodialysis of a patient with primary hyperoxaluria is eight and a half years. We report a patient who survived for eleven years on haemodialysis after reaching ESRF due to primary hyperoxaluria, and suggest that this prolonged survival was due to relatively mild disease severity rather than exceptional treatment.
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PMID:Long term survival on haemodialysis in primary hyperoxaluria. 188 47

Two Arab siblings with nephrocalcinosis and renal failure secondary to primary hyperoxaluria are presented. Percutaneous renal biopsies obtained from both siblings showed marked oxalate deposition in the renal medulla. Primary hyperoxaluria should be considered in the differential diagnosis of renal failure in infancy and early childhood especially when evidence of obstructive uropathy is lacking.
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PMID:Nephrocalcinosis due to primary hyperoxaluria: case report from Saudi Arabia. 241 74

Rosette-like arrays of highly birefringent calcium oxalate crystals are commonly seen in the marrow space of bone biopsy specimens taken from patients with primary hyperoxaluria, particularly if complicated by renal failure. Similar deposits have been described in chronic hemodialysis patients with secondary forms of oxalosis. Large multinucleated histiocytes may be seen surrounding these crystal deposits. Many of these cells are histologically indistinguishable from osteoclasts. We present a patient in whom this histiocytic reaction appeared to be of sufficient magnitude to stimulate bone resorption and to cause severe osteodystrophy. This observation, and those of other investigators reviewed in the discussion, suggest that oxalate deposition within bone may contribute to the pathogenesis of uremic osteodystrophy in chronic renal failure patients with primary or secondary types of oxalosis.
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PMID:Oxalate bone disease--an emerging form of renal osteodystrophy. 259 72


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