Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Hoppner, J; Kornak, U; Hogler, W; Obermayer-Pietsch, B; Rutsch, F; Oheim, R; Grasemann, C.
Hypophosphatemic Rickets: Diagnostic Approach and current Treatments in Childhood.
OSTEOLOGIE. 2020; 29(04): 293-301.
Doi: 10.1055/a-1257-8108
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- Obermayer-Pietsch Barbara
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- Abstract:
- Rickets is caused by an undersupply of calcium and/or phosphate to the growing body. The calcium- phosphate metabolism is regulated by the secosteroid hormone calcitriol, parathyroid hormone (PTH) and fibroblast growth factor 23 (FGF23). Excessive FGF23 action results in reduced renal phosphate reabsorption, and subsequent hypophosphataemia, undermineralization of bone, teeth and growth plates and the phenotype of hypophosphatemic rickets ( HR)/osteomalacia. The most common form of FGF23-dependent HR is X-linked hypophosphatemic rickets (XLH), which was traditionally treated via supplementation of phosphate and active vitamin D only. Since 2018, a monoclonal antibody against FGF23, Burosumab, is available for the therapy of XLH in children. The new therapy has the potential to improve long-term complications, growth and quality of life; such data are currently being collected in registries. Support groups for German-speaking people affected by XLHinclude Phosphatdiabetes e.V. and www.phosphatdiabetes.at.
- Find related publications in this database (Keywords)
- Phosphate metabolism
- FGF23
- hypophosphatemic rickets
- burosumab