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Disease relevance of Hyperoxaluria


High impact information on Hyperoxaluria

  • Slc26a6-null mice have significant hyperoxaluria and elevation in plasma oxalate concentration that is greatly attenuated by dietary oxalate restriction [6].
  • Our findings suggest that the degree of hyperoxaluria in this disorder may be only slight or moderate if the patient has been ingesting a pyridoxine-rich diet or multivitamin tablets containing small amounts of pyridoxine [7].
  • Thus, the colon is both the site of and required for increased oxalate absorption in enteric hyperoxaluria [1].
  • All seven CF patients colonised with O. formigenes had normal urinary oxalate levels, but 19 (53%) of 36 patients not colonised with O. formigenes were hyperoxaluric, with the most severe hyperoxaluria occurring in young patients [2].
  • The high urinary oxalate excretion in phosphate-supplemented XLH may be seen as a special type of enteric hyperoxaluria, in which the conditions of calcium-oxalate crystal precipitation could be reached even at normal levels of urinary calcium excretion [8].

Chemical compound and disease context of Hyperoxaluria


Biological context of Hyperoxaluria


Anatomical context of Hyperoxaluria


Gene context of Hyperoxaluria

  • To determine the importance of OPN in vivo, hyperoxaluria was induced in mice targeted for the deletion of the OPN gene together with wild-type control mice [23].
  • Hyperoxaluria is discussed relative to mutations in AGXT and GRHPR [24].
  • Application of this procedure in a recently developed Agxt-gene-deleted mouse model of PH1 resulted in marked amelioration of hyperoxaluria [25].
  • Partial deletion of the AGXT gene (EX1_EX7del): A new genotype in hyperoxaluria type 1 [26].
  • Studies have suggested possible mechanisms for the association, including hyperuricosuria, hyperoxaluria, primary defects in calcium handling caused by mutation of the CF transmembrane regulator (CFTR), hypocitraturia, and lack of colonization with O formigenes, an enteric oxalate-degrading bacterium [27].

Analytical, diagnostic and therapeutic context of Hyperoxaluria


  1. Importance of the colon in enteric hyperoxaluria. Dobbins, J.W., Binder, H.J. N. Engl. J. Med. (1977) [Pubmed]
  2. Absence of Oxalobacter formigenes in cystic fibrosis patients: a risk factor for hyperoxaluria. Sidhu, H., Hoppe, B., Hesse, A., Tenbrock, K., Brömme, S., Rietschel, E., Peck, A.B. Lancet (1998) [Pubmed]
  3. The abnormal red-cell oxalate transport is a risk factor for idiopathic calcium nephrolithiasis: a prospective study. Gambaro, G., Marchini, F., Piccoli, A., Nassuato, M.A., Bilora, F., Baggio, B. J. Am. Soc. Nephrol. (1996) [Pubmed]
  4. Direct correlation between hyperoxaluria/oxalate stone disease and the absence of the gastrointestinal tract-dwelling bacterium Oxalobacter formigenes: possible prevention by gut recolonization or enzyme replacement therapy. Sidhu, H., Schmidt, M.E., Cornelius, J.G., Thamilselvan, S., Khan, S.R., Hesse, A., Peck, A.B. J. Am. Soc. Nephrol. (1999) [Pubmed]
  5. Pyridoxamine lowers kidney crystals in experimental hyperoxaluria: a potential therapy for primary hyperoxaluria. Chetyrkin, S.V., Kim, D., Belmont, J.M., Scheinman, J.I., Hudson, B.G., Voziyan, P.A. Kidney Int. (2005) [Pubmed]
  6. Calcium oxalate urolithiasis in mice lacking anion transporter Slc26a6. Jiang, Z., Asplin, J.R., Evan, A.P., Rajendran, V.M., Velazquez, H., Nottoli, T.P., Binder, H.J., Aronson, P.S. Nat. Genet. (2006) [Pubmed]
  7. Response to a physiologic dose of pyridoxine in type I primary hyperoxaluria. Yendt, E.R., Cohanim, M. N. Engl. J. Med. (1985) [Pubmed]
  8. Evidence suggesting hyperoxaluria as a cause of nephrocalcinosis in phosphate-treated hypophosphataemic rickets. Reusz, G.S., Latta, K., Hoyer, P.F., Byrd, D.J., Ehrich, J.H., Brodehl, J. Lancet (1990) [Pubmed]
  9. Pyridoxine treatment of type 1 hyperoxaluria. Alinei, P., Guignard, J.P., Jaeger, P. N. Engl. J. Med. (1984) [Pubmed]
  10. Letter: Vitamin-C-induced hyperoxaluria. Harris, A.B. Lancet (1976) [Pubmed]
  11. Calcium oxalate crystal adherence to hyaluronan-, osteopontin-, and CD44-expressing injured/regenerating tubular epithelial cells in rat kidneys. Asselman, M., Verhulst, A., De Broe, M.E., Verkoelen, C.F. J. Am. Soc. Nephrol. (2003) [Pubmed]
  12. Effect of angiotensin II receptor blockage on osteopontin expression and calcium oxalate crystal deposition in rat kidneys. Umekawa, T., Hatanaka, Y., Kurita, T., Khan, S.R. J. Am. Soc. Nephrol. (2004) [Pubmed]
  13. Effect of sodium chenodeoxycholate on oxalate absorption from the excluded human colon--a mechanism for 'enteric' hyperoxaluria. Fairclough, P.D., Feest, T.G., Chadwick, V.S., Clark, M.L. Gut (1977) [Pubmed]
  14. Reduction of oxaluria after an oral course of lactic acid bacteria at high concentration. Campieri, C., Campieri, M., Bertuzzi, V., Swennen, E., Matteuzzi, D., Stefoni, S., Pirovano, F., Centi, C., Ulisse, S., Famularo, G., De Simone, C. Kidney Int. (2001) [Pubmed]
  15. Ethnic background has minimal impact on the etiology of nephrolithiasis. Maloney, M.E., Springhart, W.P., Ekeruo, W.O., Young, M.D., Enemchukwu, C.U., Preminger, G.M. J. Urol. (2005) [Pubmed]
  16. Lipid peroxidation in ethylene glycol induced hyperoxaluria and calcium oxalate nephrolithiasis. Thamilselvan, S., Hackett, R.L., Khan, S.R. J. Urol. (1997) [Pubmed]
  17. Changes in renal hemodynamics and urodynamics in rats with chronic hyperoxaluria and after acute oxalate infusion: role of free radicals. Huang, H.S., Ma, M.C., Chen, J., Chen, C.F. Neurourology and urodynamics. (2003) [Pubmed]
  18. Expression of nuclear pore complex oxalate binding protein p62 in experimental hyperoxaluria. Sivakamasundari, P., Sakthivel, R., Kalaiselvi, P., Selvam, R., Varalakshmi, P. Nephron Exp. Nephrol. (2004) [Pubmed]
  19. Oxalate toxicity in LLC-PK1 cells, a line of renal epithelial cells. Scheid, C., Koul, H., Hill, W.A., Luber-Narod, J., Jonassen, J., Honeyman, T., Kennington, L., Kohli, R., Hodapp, J., Ayvazian, P., Menon, M. J. Urol. (1996) [Pubmed]
  20. Effect of gamma-glutamyl carboxylation of renal microsomes on calcium oxalate monohydrate crystal binding in hyperoxaluria. Angayarkanni, N., Selvam, R. Nephron (1999) [Pubmed]
  21. Limitation of apoptotic changes in renal tubular cell injury induced by hyperoxaluria. Sarica, K., Erbagci, A., Yağci, F., Bakir, K., Erturhan, S., Uçak, R. Urol. Res. (2004) [Pubmed]
  22. New concepts in the diagnosis and treatment of lysosomal and peroxisomal disorders. Moser, H.W. Current opinion in neurology and neurosurgery. (1992) [Pubmed]
  23. Osteopontin is a critical inhibitor of calcium oxalate crystal formation and retention in renal tubules. Wesson, J.A., Johnson, R.J., Mazzali, M., Beshensky, A.M., Stietz, S., Giachelli, C., Liaw, L., Alpers, C.E., Couser, W.G., Kleinman, J.G., Hughes, J. J. Am. Soc. Nephrol. (2003) [Pubmed]
  24. The molecular basis of kidney stones. Langman, C.B. Curr. Opin. Pediatr. (2004) [Pubmed]
  25. Feasibility of hepatocyte transplantation-based therapies for primary hyperoxalurias. Guha, C., Yamanouchi, K., Jiang, J., Wang, X., Roy Chowdhury, N., Santana, A., Shapiro, L.J., Salido, E., Roy-Chowdhury, J. American journal of nephrology. (2005) [Pubmed]
  26. Partial deletion of the AGXT gene (EX1_EX7del): A new genotype in hyperoxaluria type 1. Nogueira, P.K., Vuong, T.S., Bouton, O., Maillard, A., Marchand, M., Rolland, M.O., Cochat, P., Bozon, D. Hum. Mutat. (2000) [Pubmed]
  27. The association of nephrolithiasis with cystic fibrosis. Gibney, E.M., Goldfarb, D.S. Am. J. Kidney Dis. (2003) [Pubmed]
  28. Complex pathogenesis of hyperoxaluria after jejunoileal bypass surgery. Oxalogenic substances in diet contribute to urinary oxalate. Hofmann, A.F., Laker, M.F., Dharmsathaphorn, K., Sherr, H.P., Lorenzo, D. Gastroenterology (1983) [Pubmed]
  29. Live donor/split liver grafts for adult recipients: when should we use them? Neuhaus, P. Liver Transpl. (2005) [Pubmed]
  30. The determination of oxalate in haemodialysate and plasma: a means to detect and study 'hyperoxaluria' in haemodialysed patients. Wolthers, B.G., Meijer, S., Tepper, T., Hayer, M., Elzinga, H. Clin. Sci. (1986) [Pubmed]
  31. The effect of different diets on urine composition and the risk of calcium oxalate crystallisation in healthy subjects. Siener, R., Hesse, A. Eur. Urol. (2002) [Pubmed]
  32. Ethylene glycol intoxication and xylitol infusion--metabolic steps of oxalate-induced acute renal failure. Meier, M., Nitschke, M., Perras, B., Steinhoff, J. Clin. Nephrol. (2005) [Pubmed]
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