FREE NEJM E-TOC    HOME   |   SUBSCRIBE   |   CURRENT ISSUE   |   PAST ISSUES   |   COLLECTIONS   |   Search Term  Advanced Search

Sign in | Get NEJM's E-Mail Table of Contents — Free | Subscribe

Previous Volume 331:1553-1558 December 8, 1994 Number 23 Next

	Full Text Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited PubMed Citation

ABSTRACT

Background The prognosis for patients with primary hyperoxaluria has been ominous, with the expectation of renal failure, poor results with transplantation, and early death.

Methods We studied the long-term effects of orthophosphate and pyridoxine therapy in 25 patients with primary hyperoxaluria who were treated for an average of 10 years (range, 0.3 to 26). Their mean age at the start of treatment was 12 years (median, 6; range, 0.5 to 32). We also studied the effect of orthophosphate and pyridoxine on urinary supersaturation with calcium oxalate, crystal inhibition using a seeded growth system, and crystal formation using scanning electron microscopy in 12 patients during three-day stays in the clinical research center.

Results The mean (±SD) glomerular filtration rate at the start of treatment was 91 ±26 ml per minute per 1.73 m². The median decline in glomerular filtration rates was 1.4 ml per minute per 1.73 m² of body-surface area per year. The actuarial survival free of end-stage renal disease was 96, 89, 74, and 74 percent at 5, 10, 15, and 20 years, respectively. Treatment with orthophosphate and pyridoxine reduced urinary supersaturation with calcium oxalate from 8.3 ±3.0 to 2.1 ±1.7 kJ per mole at 38 °C (P<0.001), increased the inhibition of calcium oxalate formation from 63 ±11 to 108 ±10 inhibitor units per 24 hours (P<0.001), and improved the crystalluria score from 2.6 ±0.3 to 0.6 ±0.1 (P<0.001).

Conclusions Treatment of patients with primary hyperoxaluria with orthophosphate and pyridoxine decreases urinary calcium oxalate crystallization and appears to preserve renal function.

Source Information

From the Division of Nephrology, Departments of Internal Medicine (D.S.M., D.M.W., L.H.S.) and Pediatrics (D.S.M.), and the Section of Biostatistics (J.T.E., E.J.B.), Mayo Clinic, Rochester, Minn.

Address reprint requests to Dr. Milliner at the Division of Nephrology, Mayo Clinic, Rochester, MN 55905.

Full Text of this Article

This article has been cited by other articles:

• Ladwig, P. M., Liedtke, R. R., Larson, T. S., Lieske, J. C. (2005). Sensitive Spectrophotometric Assay for Plasma Oxalate. Clin. Chem. 51: 2377-2380 [Full Text]  
• van Woerden, C. S., Groothoff, J. W., Wanders, R. J. A., Davin, J.-C., Wijburg, F. A. (2003). Primary hyperoxaluria type 1 in The Netherlands: prevalence and outcome. Nephrol Dial Transplant 18: 273-279 [Abstract] [Full Text]  
• Ames, B. N, Elson-Schwab, I., Silver, E. A (2002). High-dose vitamin therapy stimulates variant enzymes with decreased coenzyme binding affinity (increased Km): relevance to genetic disease and polymorphisms. Am. J. Clin. Nutr. 75: 616-658 [Abstract] [Full Text]  
• Yamauchi, T., Quillard, M., Takahashi, S., Man, N.-K. (2001). Oxalate removal by daily dialysis in a patient with primary hyperoxaluria type 1. Nephrol Dial Transplant 16: 2407-2411 [Abstract] [Full Text]  
• C. Karakousis, P., E. Tomaszewski, J. (2001). Ulcerating subcutaneous nodules and advanced renal failure: is it time for a new liver?. Nephrol Dial Transplant 16: 2095-2096 [Full Text]  
• LEUMANN, E., HOPPE, B. (2001). The Primary Hyperoxalurias. J. Am. Soc. Nephrol. 12: 1986-1993 [Full Text]  
• Cochat, P., Basmaison, O. (2000). Current topic: Current approaches to the management of primary hyperoxaluria. Arch. Dis. Child. 82: 470-473 [Full Text]  
• van Buren, M. (2000). A stony history. Nephrol Dial Transplant 15: 551-553 [Full Text]  
• CURHAN, G. C., WILLETT, W. C., SPEIZER, F. E., STAMPFER, M. J. (1999). Intake of Vitamins B6 and C and the Risk of Kidney Stones in Women. J. Am. Soc. Nephrol. 10: 840-845 [Abstract] [Full Text]  
• Santos-Victoriano, M., Brouhard, B. H., Cunningham, R. J. III (1998). Renal Stone Disease in Children. CLIN PEDIATR 37: 583-599 [Abstract]  
• Somach, S. C., Davis, B. R., Paras, F. A., Petrelli, M., Behmer, M. E. (1995). Fatal Cutaneous Necrosis Mimicking Calciphylaxis in a Patient With Type 1 Primary Hyperoxaluria. Arch Dermatol 131: 821-823 [Abstract]