MANAGEMENT OF PRIMARY HYPEROXALURIA - EFFICACY OF ORAL CITRATE ADMINISTRATION

被引:92
作者
LEUMANN, E
HOPPE, B
NEUHAUS, T
机构
[1] University Children's Hospital, Zurich, CH 8032
关键词
HYPEROXALURIA; OXALATE; CITRATE; NEPHROCALCINOSIS; UROLITHIASIS; CALCIUM OXALATE SATURATION;
D O I
10.1007/BF00864405
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
The prognosis of primary hyperoxaluria (PH) is not only related to endogenous oxalate production and the response (if any) to pyridoxine (in type I), but is greatly influenced by extrarenal factors like dehydration. The earlier the diagnosis of PH, the better the chances of improving the prognosis in individual patients. Measures to enhance the solubility of calcium oxalate are important. Besides ensuring at all times a generous fluid intake (>2 l/m2), administration of alkali citrate (0.15 g/kg), which has not been advocated so far in PH, appears very promising. We studied the effect of sodium citrate in six patients with PH. Mean urinary citrate excretion (mmol/day per 1.73 m2) Without oral citrate was very low (0.57) and rose to 2.49 with citrate administration. This was accompanied by a significant decrease in the calcium oxalate saturation (calculated by equil 2) from 11.7 to 6.9 (P <0.05). Treatment in five patients over 10-36 months resulted in improved (1) or stabilized (4) renal function and reduced passage of stones. Additional measures include restriction of salt and of oxalate-rich food. We conclude that long-term administration of alkali citrate is beneficial in patients with PH.
引用
收藏
页码:207 / 211
页数:5
相关论文
共 27 条
[1]  
Danpure C.J., Jennings P.R., Watts R.W.E., Enzymological diagnosis of primary hyperoxaluria type I by measurement of hepatic alanine: glyoxylate aminotransferase activity, Lancet, 1, pp. 289-291, (1987)
[2]  
Seargeant L.E., deGroot G.W., Dilling L.A., Mallory C.J., Haworth J.C., Primary oxaluria type 2 (l-glyceric aciduria), a rare cause of nephrolithiasis in children, J Pediatr, 118, pp. 912-914, (1991)
[3]  
Wharton R., D'Agati V., Magun A.M., Whitlock R., Kunis C.L., Appel G.B., Acute deterioration of renal function associated with enteric hyperoxaluria, Clin Nephrol, 34, pp. 116-121, (1990)
[4]  
Noel C., Sault M., Dhont J., Gosselin B., Leclet H., Marie A., Sebert J.L., Lemaguer D., Lelievre G., Primary hyperoxaluria diagnosed after renal transplantation, J Nephrol, 1, pp. 43-45, (1989)
[5]  
Leumann E.P., Dietl A., Matasovic A., Urinary oxalate and glycolate excretion in healthy infants and children, Pediatr Nephrol, 4, pp. 493-497, (1990)
[6]  
Barratt T.M., Kasidas G.P., Murdoch I., Rose G.A., Urinary oxalate and glycolate excretion and plasma oxalate concentration, Arch Dis Child, 66, pp. 501-503, (1991)
[7]  
Schaumburg H., Kaplan J., Windebank A., Vick N., Rasmus S., Pleasure D., Brown M.J., Sensory neuropathy from pyridoxine abuse, N Engl J Med, 309, pp. 445-448, (1983)
[8]  
DeZegher F.E., Wolff E.D., van der Heijden J., Sukhai R.N., Oxalosis in infancy, Clin Nephrol, 22, pp. 114-120, (1984)
[9]  
Yendt E.R., Cohanim M., Response to physiologic dose of pyridoxine in type I primary hyperoxaluria, N Eng J Med, 312, pp. 953-957, (1985)
[10]  
Reusz G.S., Latta K., Hoyer P.F., Byrd D.J., Ehrich J.H.H., Brodehl J., Evidence suggesting hyperoxaluria as a cause of nephrocalcinosis in phosphate-treated hypophosphataemic rickets, Lancet, 335, pp. 1240-1243, (1990)