Bone mineral density in children with primary hyperoxaluria type I

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 w as performed in 10 children with PH 1 (mean chronological age 9 +/- 3.1, me an skeletal age 8.3 +/- 3.0 years): seven were on conservative treatment (C T) including one patient after pre-emptive liver transplantation (PH1-CT) a nd 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/cm(3); P < 0.002 and P < 0.007, respective ly). while cortical bone density (CBD) was elevated to a lesser degree (517 +/- 23 vs 348 +/- 81 vs 385 +/- 113 mg/cm(3); P < 0.02 and P < 0.04, respe ctively). 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) an d plasma oxalate levels (r = 0.86 and r = 0.94. P < 0.001 and P < 0.0001. r espectively). In children with PH 1 and normal glomerular function, both CB D and TBD were comparable with healthy controls. Conclusion. These preliminary data suggest that in PH 1 BMD is significantl y increased in ESRD. probably due to oxalate disposal. Measurement of BMD m ay be a valuable and non-invasive tool in determining and monitoring oxalat e burden in this disorder.