The Primary Hyperoxalurias

Marangella, Martino; Petrarulo, Michele; Vitale, Corrado; Bagnis, Cristiana; Berutti, Silvia; Ramello, A; Antonio, Amoroso

doi:10.1159/000060176

Cited by 11 publications

(4 citation statements)

References 69 publications

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“…Even though men outnumber women in CaOx stone formation, there are no significant differences between the two in urinary excretion of oxalate expressed as either the mmol/ 24h or μmol/ mmol urine creatinine 21 . Results of our study presented here also show the absence of significant differences in the amounts of urinary oxalate between various groups of mice, male or female; normocalciuric or hypercalciuric on day 0.…”

Section: Discussionmentioning

confidence: 84%

Experimental Induction of Calcium Oxalate Nephrolithiasis in Mice

Khan

Glenton

2010

Journal of Urology

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Purpose Availability of various transgenic and knockout mice provides an excellent opportunity to better understand the pathophysiology of calcium oxalate (CaOx) stone disease. However attempts to produce CaOx nephrolithiasis in mice have not been very successful. We have hypothesized that CaOx nephrolithiasis in mice requires increasing the urinary excretion of calcium as well as oxalate and that experimentally induced hyperoxaluria alone is not sufficient. To provide evidence we induced hyperoxaluria by administering hyperoxaluria inducing agents to normocalciuric as well as hypercalciuric mice and investigated various aspects of nephrolithiasis. Materials and Methods Ethylene glycol (EG), glyoxylate (GOx) or hydroxyl proline (HLP) were administered through diet to male and female normocalciuric B6 mice as well as hypercalciuric Npt2a −/− mice for 4 weeks. 24 hour urine samples were collected on 0.3,7,14,21 and 28 days and analyzed for pH, creatinine, lactate dehydrogensae (LDH) calcium and oxalate. Kidneys were examined using light microscopy. Urine was examined for crystals using both light and scanning electron microscopy. Results Hypercalciuric mice on HLP did not tolerate the treatment and had to be sacrificed before 28 days. All mice receiving EG, GOx or HLP became hyperoxaluric and demonstrated CaOx crystalluria. None of the female mice, normo or hypercalciuric developed renal CaOx crystal deposits. All mice on Gox and some on EG developed CaOx nephrolithiais. Kidneys of all mice showed epithelial injury. Male mice particularly on GOx showed more renal injury and migration of inflammatory cells into the interstitium around the crystal deposits. Conclusions Results confirm that induction of hyperoxaluria alone is not sufficient for CaOx nephrolithiais in mice. Hypercalciuria is also required. Kidneys of male mice are more prone to injury than those of female mice and are susceptible to CaOx crystal deposition. Perhaps epithelial injury promotes crystal retention. Thus CaOx nephrolithiais in mice is gender dependent and requires both hypercalciuria and hyperoxaluria.

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Section: Discussionmentioning

confidence: 84%

Experimental Induction of Calcium Oxalate Nephrolithiasis in Mice

Khan

Glenton

2010

Journal of Urology

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“…The determination of plasma oxalate (P Ox ) and glycolate is a powerful tool to distinguish primary from secondary forms of hyperoxaluria (44). Normal levels of P Ox range from 0.5 to 7.5 µmol/l depending on the method used (3,45), and 10 µmol/l is the upper cutoff point.…”

Section: Plasma Oxalatementioning

confidence: 99%

Diagnostic and therapeutic approaches in patients with secondary hyperoxaluria

Höppe

Leumann²,

G³

et al. 2003

Front Biosci

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Secondary hyperoxaluria is due either to increased intestinal oxalate absorption or to excessive dietary oxalate intake. Certain intestinal diseases like short bowel syndrome, chronic inflammatory bowel disease or cystic fibrosis and other malabsorption syndromes are known to increase the risk of secondary hyperoxaluria. Although the urinary oxalate excretion is usually lower than in primary hyperoxaluria, it may still lead to significant morbidity by recurrent urolithiasis or progressive nephrocalcinosis. A clear distinction between primary and secondary hyperoxalurias is important. As correct classification may be difficult, appropriate diagnostic tools are needed to delineate the metabolic background as a basis for optimal treatment. We developed an individual approach for the evaluation of patients with suspected secondary hyperoxaluria. First, 24 h urines are examined repeatedly for lithogenic (e.g. calcium, oxalate, uric acid) and stone-inhibitory (e.g. citrate, magnesium) substances, and the patients are asked to fill in a dietary survey form. Urinary saturation is calculated using the computer based program EQUIL2, and the BONN-Risk-index is determined. The measurement of plasma oxalate and of urinary glycolate helps to distinguish between primary and secondary hyperoxalurias. If secondary hyperoxaluria is suspected, the stool is examined for Oxalobacter formigenes, an intestinal oxalate degrading bacterium, as lack or absence may lead to increased intestinal oxalate absorption. The last diagnostic step is to study the intestinal oxalate absorption using [13C2]oxalate. Depending on the results, various therapeutic options are available: 1) a diet low in oxalate, but normal or high in calcium, 2) a high fluid intake (>1.5 L/m2/d), 3) medications to increase the urinary solubility, 4) specific therapeutic measures in patients with malabsorption syndromes, depending on the underlying pathology, and 5) intestinal recolonization of Oxalobacter formigenes or the treatment with other oxalate degrading bacteria.

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“…Data of urinary oxalate in ESRF cannot be used in contrast to plasma levels of oxalate and glycolate [16]. Patients with PH have significantly higher P Ox levels (>80 mmol/l) than non-PH patients in ESRF (<60 mmol/l) [17].…”

Section: Diagnosismentioning

confidence: 99%

“…Patients with PH have significantly higher P Ox levels (>80 mmol/l) than non-PH patients in ESRF (<60 mmol/l) [17]. Plasma glycolate levels are significantly elevated in PH I patients only once they are on renal replacement therapy [16]. The finding of birefringent CaOx crystals at a bone or kidney biopsy is strongly suggestive of PH, and an eye ground examination showing crystal retinopathy is virtually diagnostic [1].…”

Section: Diagnosismentioning

confidence: 99%