ABSTRACT
Antecedentes y objetivo La osteogénesis imperfecta (OI) es una enfermedad genética que cursa con fragilidad ósea. Varios trabajos han demostrado la eficacia de los bisfosfonatos. El objetivo de este estudio es evaluar la evolución de la densidad mineral ósea (DMO) y parámetros bioquímicos de metabolismo óseo, en pacientes adultos con OI tratados con ácido zoledrónico iv. Material y métodos Estudio prospectivo no aleatorizado con pacientes adultos con OI con osteoporosis u osteopenia, con T-score <2, e intolerancia o contraindicación de bisfosfonatos orales, a los que se administró ácido zoledrónico iv cada 6 meses. Material y metodosSe realizó densitometría basal y cada año. Se determinaron basal y anualmente calcio total (Ca), fósforo (P), paratirina intacta (PTHi), 25 hidroxivitamina D (Vit D) y marcadores de remodelado óseo: fosfatasa alcalina ósea (FAO), telopéptido carboxiterminal del colágeno tipo I (ß cross-laps [CTX] y deoxipiridolina urinaria (DOP). Se registraron los efectos secundarios y la aparición de nuevas fracturas.ResultadosSe trataron 10 pacientes, 2 hombres y 8 mujeres. Resultados La DMO medida en columna lumbar mostró un aumento significativo a los 24 (0,738±0,141 vs 0,788±0,144g/cm2; p=0,048) y a los 36 meses (0,720±0,139 vs 0,820±0,128) con respecto a la basal. En cuello femoral se evidenció un incremento significativo de la DMO a los 24 meses: 0,677±0,121 vs 0,703±0,122g/cm2; p<0,016.ResultadosNo se evidenciaron cambios significativos en las concentraciones de Ca, P, FAO y CTX a lo largo de seguimiento. La concentración de PTHi aumentó y la de Vit D descendió a los 36 meses. La excreción de DOP disminuyó significativamente a los 24 meses. Siete pacientes presentaron un cuadro pseudogripal leve en las primeras 24h tras la primera infusión. No se evidenciaron efectos secundarios graves.(..) (AU)
Effects of zoledronic acid in adults with osteogenesis imperfectajueves, 01 jul 2010Background and objective Osteogenesis imperfecta (OI) is a genetic disorder that results in bone fragility. Several studies have demonstrated the effectiveness of bisphosphonate therapy. The aim of this study was to evaluate the effects of intravenous zoledronic acid on bone mineral density (BMD) and biochemical markers of bone turnover in adults with OI.Material and methodsWe carried out a prospective non-randomized study in patients with osteoporosis or severe osteopenia (T score (..) (AU)
Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Bone Density Conservation Agents/therapeutic use , Diphosphonates/therapeutic use , Imidazoles/therapeutic use , Osteogenesis Imperfecta/drug therapy , Prospective StudiesABSTRACT
BACKGROUND AND OBJECTIVE: Osteogenesis imperfecta (OI) is a genetic disorder that results in bone fragility. Several studies have demonstrated the effectiveness of bisphosphonate therapy. The aim of this study was to evaluate the effects of intravenous zoledronic acid on bone mineral density (BMD) and biochemical markers of bone turnover in adults with OI. MATERIAL AND METHODS: We carried out a prospective non-randomized study in patients with osteoporosis or severe osteopenia (T score < -2) related to OI and intolerance or contraindication to oral bisphosphonates. The patients were treated with a zoledronic acid infusion every 6 months. Densitometry was carried out annually. Calcium (Ca), phosphate (P), intact parathormone (PTH), 25 hydroxyvitamin D and biochemical markers of bone turnover [bone alkaline phosphatase (BAP), beta-cross-laps (CTX) and urinary deoxypyridoxine (DOP)] were measured every year. Adverse events and new fractures were registered. RESULTS: Ten patients (2 men and 8 women) were treated. Treatment increased BMD measured in the lumbar spine after 24 (0.738+/-0.141 vs 0.788+/-0.144 g/cm(2); p=0.048) and 36 months (0.720+/-0.139 vs 0.820+/-0.128; p=0.01). Significant increases in BMD were also observed after 24 months in the femoral neck (0.677+/-0.121 vs 0.703+/-0.122 g/cm(2); p<0.016). Serum Ca, P, BAP and CTX concentrations remained unchanged. PTH concentrations increased and vitamin D concentrations decreased after 36 months of treatment. DOP excretion decreased significantly after 24 months. Seven patients had mild influenza-like symptoms occurring within the first 24 h after the first infusion. No severe adverse events were observed. None of the patients had new fractures. CONCLUSION: Zoledronic acid seems to be a safe and effective treatment option in adults with osteoporosis related to OI.
Subject(s)
Bone Density Conservation Agents/therapeutic use , Diphosphonates/therapeutic use , Imidazoles/therapeutic use , Osteogenesis Imperfecta/drug therapy , Adolescent , Adult , Female , Humans , Male , Middle Aged , Prospective Studies , Young Adult , Zoledronic AcidABSTRACT
The long-term effects of bisphosphonate treatment in children with osteogenesis imperfecta (OI) are unknown. The aim of this study was to evaluate whether treatment with bisphosphonates interferes with the healing of fractures in a group of children with OI. Seven subjects (6 boys), aged 11.4 +/- 5.95 years, were followed for 2.5 +/- 0.84 years after the start of treatment with intravenous pamidronate (9 mg/kg/y) and/or oral alendronate (5 or 10 mg/d). Orthopaedic surgery of 24 bones was performed after 2.33 +/- 4.14 months of treatment, with 1.6 +/- 0.84 osteotomies per bone. Ambulation was started after 26.1 +/- 32.28 days. Reoperation was required in 8% of the bones due to fracture below primary fixation. Pseudoarthrosis was seen in one fracture, an osteotomy of the proximal femur (14% of the patients, as expected in an OI population). These results suggest that treatment with bisphosphonates at the administered doses does not interfere with fracture healing. Larger and longer studies are warranted.
