Enfermedad de Paget en Chile: una serie de 15 pacientes / Paget disease of bone in Chile: report of 15 cases

RESUMO

BACKGROUND: Paget disease of bone (PD) is a localized disorder of bone remodeling, which leads to bone fragility and deformity. In Chile PD is uncommon. AIM: To study clinical and demographic characteristics of patients with PD seen in the Clinical Hospital of the Catholic University. PATIENTS AND METHODS: Patients with typical radiological and clinical features of PD referred to our institution during the last decade were included in this review. RESULTS: We obtained data from 15 patients with PD (ten males, eight Chilean, six European and one Asian), eleven of them were diagnosed during the last 3 years. The mean age at diagnosis was 68.7 +/- 11.1 years old. No one had first degree relatives with PD. Bone pain was the main complaint in 13 patients and elevated total alkaline phosphatases in the other two. The average duration of the symptoms prior to diagnosis was 38.8 months. Eight patients had monostotic lesions; the most commonly involved sites were the pelvis, spine and femur. Radiological evaluation disclosed sclerotic changes in all patients as well as bone deformity and osteoarthritis in eight patients. Total alkaline phosphatases were elevated in 14 cases (mean 4 times over the upper normal limit). CONCLUSIONS: When compared to series of the Northern hemisphere, PD in Chile is characterized by an older age at diagnosis, a higher frequency of symptomatic presentation, advanced radiological involvement and greater proportion of complications. PD should be suspected in every patient, Chilean or foreigner, with bone pain or elevated alkaline phosphatases.