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Bone mineral density in children with primary hyperoxaluria type I
B Behnke1, M J Kemper, H P Kruse
1Department of Paediatric Nephrology, University Hospital Eppendorf, Hamburg, Germany.
Insights
Primary hyperoxaluria type I (PH 1) causes oxalate buildup in bones. Bone mineral density (BMD) is increased in PH 1 patients with end-stage renal disease (ESRD), suggesting BMD is a useful tool for monitoring oxalate burden.
Area of Science:
- Nephrology
- Pediatrics
- Metabolic Bone Disease
Background:
- Primary hyperoxaluria type I (PH 1) is characterized by excessive oxalate production.
- Oxalate deposition in organs, including bone (oxalosis), is a hallmark of PH 1.
- Evaluating skeletal status non-invasively is crucial for managing PH 1.
Purpose of the Study:
- To assess bone mineral density (BMD) in children with PH 1.
- To investigate the relationship between BMD and kidney function in PH 1.
- To determine if BMD measurement can serve as a non-invasive marker for oxalate burden.
Main Methods:
- Peripheral quantitative computed tomography (pQCT) of the distal radius was used.
- Ten children with PH 1 were studied, including those on conservative treatment and with end-stage renal disease (ESRD) on peritoneal dialysis.
- Bone mineral density (BMD) was compared to age-matched healthy and uraemic controls.
Main Results:
- Trabecular bone density (TBD) was significantly higher in PH 1 patients with ESRD compared to controls.
- Cortical bone density (CBD) was also elevated in PH 1 patients with ESRD.
- BMD (CBD and TBD) correlated significantly with serum creatinine and plasma oxalate levels in PH 1 patients.
Conclusions:
- Bone mineral density (BMD) is elevated in children with PH 1 and ESRD, likely due to oxalate accumulation.
- BMD measurements may be a valuable, non-invasive method for monitoring oxalate burden in PH 1.
- In PH 1 patients with normal kidney function, BMD is comparable to healthy controls.
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/cm(3); 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/cm(3); 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.