Osteomalacia inducida por tumor en inicio de tratamiento específico con Burosumab anticuerpo monoclonal anti FGF23
Tumor-induced osteomalacia (TIO) is a rare disease characterized by an overproduction of fibroblast growth factor 23 (FGF 23) by tumors,hypophosphatemia secondary to phosphaturia, and an alteration in the active vitamin D synthesis. This causes osteomalacia, bone pain, fractures,muscle weakness, and...
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| Autores principales: | , , , |
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| Formato: | Artículo revista |
| Lenguaje: | Español |
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Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
2019
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| Materias: | |
| Acceso en línea: | https://revistas.unc.edu.ar/index.php/med/article/view/25539 |
| Aporte de: |
| Sumario: | Tumor-induced osteomalacia (TIO) is a rare disease characterized by an overproduction of fibroblast growth factor 23 (FGF 23) by tumors,hypophosphatemia secondary to phosphaturia, and an alteration in the active vitamin D synthesis. This causes osteomalacia, bone pain, fractures,muscle weakness, and fatigue. When mesenchymal tumors secretory of FGF23 are completely resected, disease is cured. Medical treatment involvesphosphorus salts and calcitriol. Treatment with burosumab, monoclonal antibody anti-FGF23, is in phase II.Case report: Woman of 50 yrs. old, with evolution of the disease of more than 14 yrs., with progressive disabling symptomatology of musculoskeletalpain, functional muscle deficit, multiple fractures in spine, humerus, and shoulder, and total hip arthroplasty (right and left). Musculoskeletal pain isresistant to the analgesic. At every moment, hyperphosphaturia and hypophosphatemia (1,2 mg/dl average) did not produce a significant therapeuticresponse to the phosphorus salts and gastric intolerance to the salts, which led to interruptions and modifications of the dose depending on tolerance.The patient was also treated with calcium and calcitriol. Serum FGF-23 levels were 1102,56 pg/dl (VN: 0-134 pg/dl). Besides, a secondaryhyperparathyroidism with neither hypercalcemia nor hypercalciuria was confirmed. In 2008 a tumor was removed from left ischio-pubian region, whoseanatomopathological study showed fibromuscular and adipose fragments, and bone fragments with pathological diagnose compatible with fibrousdysplasia. After surgery, the patient remained asymptomatic for about 3 yrs. Afterwards, progressively and uninterruptedly, TIO reappeared. Numerousstudies were carried out to localize the tumor responsible for the TIO, which included biopsies of suspicious areas, with a negative result.Patient with serious tumor-induced osteomalacia, of long-term evolution, with an unsatisfactory response to conventional treatment at the beginning oftreatment with burosumab. Up to the present, this constitutes the first and only case of tumor-induced osteomalacia in the world, which has begun to betreated with burosumab, without an investigation protocol.
Keywords: tumor-induced osteomalacia; hypophosphatemia; fgf23; burosumab |
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