J Pfeilschifter1, R Ziegler. 1. Abteilung für Innere Medizin I,Endokrinologie und Stoffwechsel, Universitätsklinikum Heidelberg.
Abstract
BACKGROUND: Patients with fibrous dysplasia and the McCune-Albright syndrome which is characterized by additional endocrine dysfunctions, such as pubertas precox, suffer from a regional impairment in the differentiation of osteoblasts that is acquired during early embryogenesis and results in fibrous bone lesions. These lesions may cause bone deformities, fractures and chronical pain in the affected skeletal regions. We here report about our experience with a systemic treatment of the bisphosphonate pamidronate. PATIENTS: We treated 3 patients with fibrous dysplasia and 5 patients with McCune-Albright syndrome over a cumulative period of 37 patient-years. RESULTS: In all patients who suffered from painful lesions, intravenous infusions of 60 mg pamidronate resulted in an improvement of pain that lasted up to 6 months. In 2 patients we also observed a reduction in the size of some of the osteolytic lesions. Side effects were limited to asymptomatical fever and a small decline in total serum calcium within the physiological limits during the infusions. CONCLUSION: Pamidronate appears to be an effective and well tolerable treatment option for patients with fibrous dysplasia.
BACKGROUND:Patients with fibrous dysplasia and the McCune-Albright syndrome which is characterized by additional endocrine dysfunctions, such as pubertas precox, suffer from a regional impairment in the differentiation of osteoblasts that is acquired during early embryogenesis and results in fibrous bone lesions. These lesions may cause bone deformities, fractures and chronical pain in the affected skeletal regions. We here report about our experience with a systemic treatment of the bisphosphonatepamidronate. PATIENTS: We treated 3 patients with fibrous dysplasia and 5 patients with McCune-Albright syndrome over a cumulative period of 37 patient-years. RESULTS: In all patients who suffered from painful lesions, intravenous infusions of 60 mg pamidronate resulted in an improvement of pain that lasted up to 6 months. In 2 patients we also observed a reduction in the size of some of the osteolytic lesions. Side effects were limited to asymptomatical fever and a small decline in total serum calcium within the physiological limits during the infusions. CONCLUSION:Pamidronate appears to be an effective and well tolerable treatment option for patients with fibrous dysplasia.
Authors: T Yamamoto; K Ozono; S Kasayama; K Yoh; K Hiroshima; M Takagi; S Matsumoto; T Michigami; K Yamaoka; T Kishimoto; S Okada Journal: J Clin Invest Date: 1996-07-01 Impact factor: 14.808
Authors: C D Malchoff; G Reardon; D C MacGillivray; H Yamase; A D Rogol; D M Malchoff Journal: J Clin Endocrinol Metab Date: 1994-03 Impact factor: 5.958