Paget’s disease of bone: epidemiology, pathogenesis and pharmacotherapy

Interleukin-6 trans-signaling and pathological low back pain in patients with Paget disease of bone

“…All enrolled patients with PD were treated with Zol (single intravenous infusion of 5 mg for at least 15 minutes in an ambulatory setting) or Ner (100 mg dissolved in 500 mL of saline solution administered intravenously for 2 consecutive days), and cholecalciferol supplementation was recommended based on pretreatment vitamin D status. 20,44 All patients with PD were revaluated 6 months after the Zol or Ner infusion based on the proposed criteria. 21,51 Response to aminobisphosphonate treatment was defined as a consistent decrease of at least 25% compared with pretreatment values of tALP; remission was defined as a decrease in serum tALP values to within the laboratory reference range (98-275 U/L); relapse was defined as a consistent and progressive increase in tALP activity of at least 25% from the nadir values; and complete relapse was defined as an increase in tALP to the last pretreatment values.…”

Enfermedad ósea de Paget

“…More studies are still required to determine the association of the different genes, as well as the importance of environmental factors which influence the development of PDB with these genetic alterations [11][12][13][14][15][16] . Some mutations of SQSTM1 may act as predisposing factors but are not sufficient to induce PDB, with additional factors (genetic or environmental) possibly being necessary [10][11][12]17,18 . Mutations of this gene are the most common cause of familial PDB.…”

“…Transverse studies indicate that 80% of the carriers of SQSMT1 mutations develop PDB in the eighth decade of their lives. There are data which show that the age of onset of the disease in families with PDB in the current generation, in those with SQSMT1 mutation, is delayed in comparison with their parents' generation [10][11][12]15 . This emphasises the importance of environmental factors in triggering the disease.…”