ISSN 1662-4009 (online)

ey0021.5-4 | Advances in Clinical Practice | ESPEYB21

5.4. Serum phosphorus as a driver of skeletal morbidity in fibrous dysplasia

Hasan Gun Zubeyir , Osamor Charles , Taylor Jocelyn , Li Xiaobai , Szymczuk Vivian , Boyce Alison M.

In brief: This retrospective study reports on the relationship between serum phosphorus and fibrous dysplasia-related skeletal complications (fractures, orthopaedic surgery, and scoliosis) in a large cohort of patients (n=240).Commentary: Fibrous dysplasia (FD) / McCune-Albright syndrome (MAS) is a rare mosaic disorder caused by postzygotic gain-of-function mutations in Gαs, resulting in continuous receptor activation. FD can range from an isolated,...

ey0021.5-8 | Novel Treatments | ESPEYB21

5.8. Management of RANKL-mediated disorders with denosumab in children and adolescents: a global expert guidance document

Vanderniet Joel A. , Szymczuk Vivian , Hogler Wolfgang , Beck-Nielsen Signe S. , Uday Suma , Merchant Nadia , Crane Janet L. , Ward Leanne M. , Boyce Alison M. , Munns Craig F.

In brief: This article reviews the evidence and provides expert opinion on the safe and appropriate use of denosumab in children and adolescents with RANKL-mediated disorders such as giant cell bone tumours, fibrous dysplasia and juvenile Paget’s disease.Commentary: Receptor activator of nuclear factor κB ligand (RANKL) is expressed by osteogenic cells and induces osteoclast differentiation by binding to RANK on osteoclast precursors. Excessive...