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Nephrol Dial Transplant (2001) 16: 2236-2239
© 2001 European Renal Association-European Dialysis and Transplant Association

Preliminary Reports

Bone mineral density in children with primary hyperoxaluria type I

Barbara Behnke, Markus J. Kemper, Hans-Peter Kruse1 and Dirk E. Müller-Wiefel

Departments of Paediatric Nephrology and 1 Medical Osteology, University Hospital Eppendorf, Hamburg, Germany

Background. In primary hyperoxaluria type I (PH 1), hepatic overproduction of oxalate leads to its deposition in various organ systems including bone (oxalosis). To evaluate skeletal status non-invasively in PH 1 we measured bone mineral density (BMD).

Methods. Peripheral quantitative computed tomography of the distal radius was performed in 10 children with PH 1 (mean chronological age 9±3.1, mean skeletal age 8.3±3.0 years): seven were on conservative treatment (CT) including one patient after pre-emptive liver transplantation (PH1-CT) and three were studied with end-stage renal disease on peritoneal dialysis (PH1-ESRD).

Results. Mean trabecular bone density (TBD) was significantly increased in PH1-ESRD compared with both age-matched healthy and uraemic controls (65227 vs 168±63 and 256±80 mg/cm3; P<0.002 and P<0.007, respectively), while cortical bone density (CBD) was elevated to a lesser degree (517±23 vs 348±81 vs 385±113 mg/cm3; P<0.02 and P<0.04, respectively). In PH 1, CBD and, even more so, TBD were significantly correlated with serum creatinine (r=0.91 and r=0.96, P<0.0001, respectively) and plasma oxalate levels (r=0.86 and r=0.94, P<0.001 and P<0.0001, respectively). In children with PH 1 and normal glomerular function, both CBD and TBD were comparable with healthy controls.

Conclusion. These preliminary data suggest that in PH 1 BMD is significantly increased in ESRD, probably due to oxalate disposal. Measurement of BMD may be a valuable and non-invasive tool in determining and monitoring oxalate burden in this disorder.

Keywords: bone mineral density; primary hyperoxaluria type I; pQCT; skeletal oxalate deposition

Correspondence and offprint requests to: Dr Markus J. Kemper, Paediatric Nephrology, University Children's Hospital, CH-8032 Zurich, Switzerland. Email: markus.kemper{at}kispi.unizh.ch


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