Cystinosis-Associated Metabolic Bone Disease Across Ages and Chronic Kidney Disease (CKD) Stages 1 to 5D/T
An erratum to this article was published (J Clin Endocrinol Metab, 21 Jan 2025 ↗)
Authors' conclusion
Bone health in cystinosis deteriorates with age, which is associated with increased osteoclast activity despite counter-regulation of osteoblasts via OPG/RANKL, which in conjunction with elevated sclerostin levels and persistent rickets/osteomalacia, may promote progressive bone loss.
Study context
- Population
- Patients with cystinosis (61% children) with CKD stages 1 to 5D/T at hospital clinics61% children
- Participants
- 103 patients
- Outcome
- Skeletal complications and bone markers
Methodological detailsbinational, multicenter, Ten key bone markers evaluated
- Binational
- Multicenter
- Ten key bone markers evaluated
Main finding
As reported in the abstract
- Skeletal complications occurred in two-thirds of patients
- Adults had a 5-fold increased risk of skeletal complications vs children
- CKD stages 1–3: reduced z-scores for serum phosphate and calcium, suppressed FGF23 and PTH, elevated bone-specific alkaline phosphatase
- Serum phosphate associated with eGFR, combined phosphate and active vitamin D treatment, and native vitamin D supplementation; serum calcium associated with age and active vitamin D dosage
- Sclerostin generally elevated in children; associated with age, FGF23, and treatment with active vitamin D and GH
- Osteoclast marker tartrate-resistant acid phosphatase 5b increased; associated with age and active vitamin D treatment
- sRANKL/osteoprotegerin (OPG) ratio decreased; associated with phosphate and active vitamin D (calcitriol) levels
- Changes only partly corrected after transplantation
Observational data: associations, not effects.
Original publication
Cystinosis-Associated Metabolic Bone Disease Across Ages and CKD Stages 1 to 5D/T. J Clin Endocrinol Metab. Published 25 July 2024. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →