Cystinosis-associated metabolic bone disease across ages and CKD stages 1-5D/T.
Johannes LahringMaren Leifheit-NestlerAnnika EwertNadine HerzigChristian KöpplVeronika PottJun OhAnja BüscherJulia ThumfartLutz T WeberKlaus ArbeiterBirgit Acham-RoschitzBurkhard TönshoffMiroslav ZivicnjakKatharina HohenfellnerDieter HaffnerPublished in: The Journal of clinical endocrinology and metabolism (2024)
Bone health in cystinosis deteriorates with age, which is associated with increased osteoclast activity despite counterregulation of osteoblasts via OPG/RANKL, which in conjunction with elevated sclerostin levels and persistent rickets/osteomalacia may promote progressive bone loss.