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Nephrol Dial Transplant (1995) 10: 2224-2227
© 1995 European Renal Association-European Dialysis and Transplant Association

research-article

Changing pattern of primary hyperoxaluria in Switzerland

N. Kopp and E. Leumann

University Children's Hospital Zürich Switzerland

Correspondence and offprint requests to: Correspondence and offprint requests to: Prof. E. Leumann. University Children's Hospital, Steinwiesstr. 75, CH-8032 Zurich, Switzerland

BACKGROUND: The clinical course of primary hyperoxaluria (PH) is greatly variable and diagnosis is often delayed. Little is known about the overall occurrence and current prognosis.

METHODS: We evaluated all known patients with PH residing and observed in Switzerland during the last 15 years with the help of a survey among Swiss nephrologists.

RESULTS: Of the 25 patients observed between 7/79 and 6/94 in Switzerland, 18 were alive in 1994—14 on conservative therapy and four on renal replacement therapy (RRT). Twenty-two patients had PH type 1; the exact type was not determined in three. The estimated prevalence of PH (type 1) is 2 per million population; the minimal incidence is 1 per 100000 live births. Diagnosis was delayed by 8 years (median) except in infants. Five patients were pyridoxine sensitive. According to life table analysis, 20% of patients were in end-stage renal failure (ESRF) and 10% had died by the age of 15 years, and 50% were in ESRF and 20% dead at 25 years. Prognosis has improved: Five of 13 patients died during the first half of the observation period as opposed to two of 20 in the second part.

CONCLUSIONS: Overall prognosis appears better than hitherto believed considering the large clinical spectrum of PH. Greater awareness of PH is needed to improve further long-term prognosis.

Keywords: oxalosis; prevalence; primary hyperoxaluria; survey; Switzerland


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