Cases reported "Hyperoxaluria"

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1/15. Chronic renal failure secondary to oxalate nephropathy: a preventable complication after jejunoileal bypass.

    Enteric hyperoxaluria is a commonly seen adverse event after the jejunoileal bypass procedure. The increased concentration of urinary oxalate predisposes bypass patients to various renal complications such as nephrolithiasis and oxalate nephropathy. If not diagnosed and appropriately treated, these complications can lead to irreversible renal damage. We describe 3 patients in whom severe renal complications developed with irreversible compromise of renal function after a jejunoileal bypass. patients who undergo a jejunoileal bypass require lifelong follow-up with close monitoring of their renal function. Marked decline in renal function mandates prompt investigation and aggressive intervention, including reversal of the jejunoileal bypass if necessary. Chronic renal failure secondary to oxalate nephropathy is preventable and treatable but may require conversion of a jejunoileal bypass to a more current form of bypass.
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2/15. Conjugated bile acid replacement therapy reduces urinary oxalate excretion in short bowel syndrome.

    BACKGROUND: patients with short bowel syndrome (SBS) have steatorrhea, in part because of bile acid malabsorption that causes decreased bile acid secretion into the duodenum and consequent fat maldigestion. In SBS patients with colon in continuity, luminal calcium forms calcium fatty acid soaps rather than precipitating as insoluble calcium oxalate. Soluble oxalate is hyperabsorbed by the colon leading to hyperoxaluria and an increased risk for renal calcium oxalate stones and deposits. The authors hypothesized that oral ingestion of conjugated bile acids would increase fat absorption and thereby decrease calcium fatty acid soap formation and oxalate hyperabsorption. methods: The effect of conjugated bile acid replacement therapy (9 g/d) on fecal fat excretion and urine oxalate excretion was measured in an appropriate patient, utilizing the metabolic balance technique. The effects of chronic bile acid replacement therapy on oxalate excretion and nutritional status also were measured in a 3-month outpatient study. RESULTS: Natural conjugated bile acid replacement therapy reduced fecal fat excretion from 119 to 79 g/d (Delta40 g/d), and urinary oxalate excretion from 87 to 64 mg/d (966 to 710 micromol/d; Delta23 mg/d). Cholylsarcosine, a synthetic conjugated bile acid, had similar but less powerful effects. During a 3-month outpatient trial of natural conjugated bile acids (9 g/d), urine oxalate decreased to normal levels (27 mg/d) in association with weight gain, decreased hunger, and decreased hyperphagia. CONCLUSION: Conjugated bile acid replacement therapy reduced fecal fat excretion, reduced urinary oxalate excretion, and improved nutritional status in a patient with SBS with colon in continuity, hyperoxaluria, and oxalate nephrolithiasis.
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3/15. endocytosis of calcium oxalate crystals and proliferation of renal tubular epithelial cells in a patient with type 1 primary hyperoxaluria.

    A patient with primary hyperoxaluria who received a liver-kidney transplant is presented. A postoperative renal biopsy showed apparent tubular cell endocytosis of calcium oxalate crystals and cell proliferation, indicating that renal epithelial cells do not perceive urinary crystals as inert. Such cellular responses to crystals may have a role in nephrolithiasis.
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4/15. Always look beyond the stones: hyperoxaluria overlooked.

    We report a case which demonstrates the disastrous consequences of late diagnosis of hyperoxaluria in a 24-year-old woman with nephrocalcinosis, a staghorn calculus and recurrent urinary tract infections. Her initial management at another hospital included multiple percutaneous nephrostomies and lithropsies. Metabolic screening was not undertaken. hyperoxaluria was finally diagnosed by elevated urine oxalate (1.235 mmol/24 h) and renal biopsy, by which time there was already significant reduction of renal function. A diagnosis of hyperoxaluria type I was confirmed by liver biopsy. Despite starting pyridoxine and crystallization inhibitors, her renal function deteriorated, requiring hemodialysis and she was referred for combined liver-renal transplantation. Clinical clues of primary hyperoxaluria type I are a positive family history or presentation with severe renal stones at an unusually early age. Irrespective of the above, all patients with first presentation of renal calculi should undergo metabolic screening, including urine oxalate.
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5/15. Bilateral renal autotransplantation with pyelovesicostomy: a surgical treatment of refractory enteric hyperoxaluria.

    A 38-year-old man, with only 3 feet of small bowel remaining after multiple resections because of chronic inflammatory bowel disease, had severe symptomatic calcium oxalate nephroureterolithiasis. Because of the refractory symptoms, he was successfully treated with bilateral autotransplantation of the kidneys, totally bypassing the ureters. Anatomically effective urinary tract continuity was reestablished by means of bilateral pyelovesicostomies with concomitant rectus muscle vesicofixation to create direct stone-dumping channels into the urinary bladder. The patient is now completely without symptoms 18 months after surgery. A description and rationale for this surgical treatment is provided. Bilateral autotransplantation of the kidneys with direct drainage into the urinary bladder may be an attractive and viable therapeutic option in complicated patients with short-gut syndrome and severe refractory calcium oxalate nephroureterolithiasis.
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6/15. Treatment of enteric hyperoxaluria with calcium-containing organic marine hydrocolloid.

    An organic marine hydrocolloid (OMH) charged with calcium ('Ox-Absorb') was studied in vitro for oxalate binding and in patients with enteric hyperoxaluria to investigate oxalate excretion and the inhibitory activity on crystal formation of the urine. In-vitro experiments showed complete binding of oxalate to OMH. In clinical studies in nineteen patients with intestinal disorders and stone formation, urinary oxalate excretion was significantly lower during OMH treatment than off treatment. The activity product index of calcium oxalate was reduced on treatment. A pronounced rise in the inhibitory activity of urine was seen in two patients with very low pretreatment values. Most patients experienced virtual normalisation of bowel function, and in those with severe stone formation there was substantial clinical improvement. It is concluded that OMH has the capacity to bind oxalate in vitro and to reduce urinary oxalate excretion. These observations suggest a new promising treatment for enteric hyperoxaluria.
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7/15. Late presentation and microcrystalline arthropathy in primary hyperoxaluria.

    A 66 year-old woman was referred in 1981 because of renal insufficiency and pronounced nephrocalcinosis. The urinary oxalate excretion was elevated. Secondary hyperoxaluria was excluded. End-stage renal disease necessitated hemodialysis from late in 1982 up to her death in 1986, at the age of 71 years. During the course of the disease, an aggressive arthropathy developed in the fingers. Classical signs of oxalosis were found: deposits of calcium oxalate crystals in bone tissue, the pancreas, myocardium, subcutaneous tissue and especially in the kidneys. This rare case documents the possible occurrence of late clinical presentation and long survival in primary oxalosis.
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8/15. Post-surgical deterioration of renal function in primary hyperoxaluria.

    Primary hyperoxaluria leading to calcium oxalate urinary stones and renal deposits occurs rarely in adults. We report three cases in whom end-stage renal failure was precipitated by urological surgery. In contrast, in one case renal stones were destroyed by extracorporeal shock-wave lithotripsy and renal function was not significantly altered. This emphasises the need for early diagnosis. This may not be easy in adult patients: urolithiasis may not be severe, radiological nephrocalcinosis may be lacking, and renal failure may develop late in life.
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9/15. Type I primary hyperoxaluria associated with type I renal tubular acidosis.

    An 8-year-old boy who had suffered from recurrent stone formation since the age of 4 years, was admitted as an emergency due to anuria for a half day on November 20, 1986. kidney-ureter-bladder film showed that the urethra was obstructed by a stone, and emergent cystoscopy was performed to remove it. He is the product of consanguinous marriage, his parents being first cousins. There was no family history of renal stone. Laboratory investigations showed hypokalemic, hyperchloremic metabolic acidosis. The ammonium chloride loading test revealed inability to acidify the urine and a markedly decreased excretion of titrable hydrogen ion and ammonium ion in the urine. These results indicate that this is a case of Type I renal tubular acidosis. His 24-hour urinary excretion of oxalate and glyoxylate were also markedly increased. There were no underlying causes leading to the development of secondary hyperoxaluria. These results also establish the diagnosis of Type I primary hyperoxaluria. The patient then received regimens of Polycitra 1ml/kg/day and Vitamin B6 50mg/day for 4 months. However, urinary stone developed again in this patient 4 months later. To our knowledge, Type I primary hyperoxaluria in association with Type I renal tubular acidosis has not been previously reported.
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10/15. urolithiasis and enteric hyperoxaluria in a child with steatorrhea.

    Malabsorptive states are frequently associated with increased urinary oxalate excretion. The authors describe a 10-year-old girl with steatorrhea, hyperoxaluria, and a renal calculus in a single functioning kidney. Successful management of steatorrhea corrected both the chronic diarrhea and hyperoxaluria. Enteric hyperoxaluria is a well-known etiology of calcium oxalate urolithiasis in adults. Pediatricians caring for children with malabsorptive conditions should be aware of the risk of urinary calculus formation as a result of increased dietary oxalate absorption.
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