Stiripentol for the treatment of primary hyperoxaluria and calcium oxalate nephropathy

2020 ◽  
Vol 97 (1) ◽  
pp. 17-19 ◽  
Author(s):  
Christina M. Wyatt ◽  
Tilman B. Drüeke
2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Jorge Lamarche ◽  
Reji Nair ◽  
Alfredo Peguero ◽  
Craig Courville

Although a multitude of syndromes have been thoroughly described as a result of vitamin deficiencies, over consumption of such substances may also be quite dangerous. Intratubular crystallization of calcium oxalate as a result of hyperoxaluria can cause acute renal failure. This type of renal failure is known as oxalate nephropathy. Hyperoxaluria occurs as a result of inherited enzymatic deficiencies known as primary hyperoxaluria or from exogenous sources known as secondary hyperoxaluria. Extensive literature has reported and explained the mechanism of increased absorption of oxalate in malabsorptive syndromes leading to renal injury. However, other causes of secondary hyperoxaluria may also take place either via direct dietary consumption of oxalate rich products or via other substances which may metabolize into oxalate within the body. Vitamin C is metabolized to oxalate. Oral or parenteral administration of this vitamin has been used in multiple settings such as an alternative treatment of malignancy or as an immune booster. This article presents a clinical case in which ingestion of high amounts of vitamin C lead to oxalate nephropathy. This article further reviews other previously published cases in order to illustrate and highlight the potential renal harm this vitamin poses if consumed in excessive amounts.


2012 ◽  
Vol 138 (suppl 1) ◽  
pp. A002-A002
Author(s):  
Nadja Burns ◽  
Brian Castillo ◽  
Aditya Gupta ◽  
Brandy McKelvy ◽  
Sozos Papasozomenos

2016 ◽  
Vol 4 (1) ◽  
pp. 33-35
Author(s):  
Rajat Das Gupta ◽  
Debashis Datta ◽  
Debashis Datta ◽  
Suranjan Kumar.

Background: The concentrated juice made from Averrhoa bilimbi is rich in oxalic acid. It can cause acute oxalate nephropathy by blocking the tubules with calcium oxalate crystals. Case: An elderly woman was admitted to the hospital with a history of swelling of the legs, facial puffiness, and abdominal distention. Her biochemical study revealed features of acute renal failure. She gave history of taking half liter of bilimbi juice. Renal biopsy confirmed it was a case of acute oxalic nephropathy, which made it the second case of acute oxalic nephropathy due to ingestion of bilimbi juice ever reported from Bangladesh. Conclusion: It is not safe to consume high oxalate-containing fruits in large quantities.


Author(s):  
Steven L. Daniel ◽  
Luke Moradi ◽  
Henry Paiste ◽  
Kyle D. Wood ◽  
Dean G. Assimos ◽  
...  

Oxalobacter formigenes , a unique anaerobic bacterium that relies solely on oxalate for growth, is a key oxalate-degrading bacterium in the mammalian intestinal tract. Degradation of oxalate in the gut by O. formigenes plays a critical role in preventing renal toxicity in animals that feed on oxalate-rich plants. The role of O. formigenes in reducing the risk of calcium oxalate kidney stone disease and oxalate nephropathy in humans is less clear, in part due to difficulties in culturing this organism, and the lack of studies which have utilized diets controlled in their content of oxalate. Herein, we review the literature on the 40 th anniversary of the discovery of O. formigenes , with a focus on its biology, its role in gut oxalate metabolism and calcium oxalate kidney stone disease, and potential areas of future research. Results from ongoing clinical trials utilizing O. formigenes in healthy volunteers and in patients with Primary Hyperoxaluria Type 1 (PH1), a rare but severe form of calcium oxalate kidney stone disease, will also be discussed. Information has been consolidated on O. formigenes strains and best practices to culture this bacterium, which should serve as a good resource for researchers.


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