[Paget's deforming osteodysttophy]

The present clinical aspects of Paget's osteodystrophy are reviewed. The nosological definition, localiztion, natural course and signs are described and the recent description of "rheumatoid manifestations" in Paget's disease by FRANCK et al. is mentioned. The same authors revealed a positive correlation between the grade of extenstion of Paget's disease in the whole skeleton and the concentration values for alkaline phosphatase and uric acid in the serum. Among the complications of Paget's disease the orthopedic, neurological, haemodynamic, oncologic, hematological and dermatological are reviewed X-ray of the involved skeleton, which in most cases is diagnostic, may be supported by isotope scanning with 18F or 87mSr and bone biopsy for establishment of diagnosis. Current drug therapy is confined to diphosphonate and calcitonin. The antibiotic mithramycin, which is cytotoxic, reduces bone turnover and may improve the course in Paget's disease. However, toxic side effects on kidney, liver and hemopoiesis do not allow its further therapeutic use in this disease. A case is described which demonstrates that a spontaneous or traumatic fracture in the area of osteodystrophy exhibits almost the same potential for conso lidation as normal bone tissue following both conservative and osteosynthetic treatment of the fracture. In a further instance corrective osteotomy with osteosynthesis (plate) because of serious varisation and antecurvature of the femur due to Paget's disease were performed sucessfully without assisting drug therapy. A third patient displayed extensive osteodystrophy of the whole pelvic skeleton, which was discovered by X-ray as rehabilitation following CVA failed to progress due to severe bilateral reduction of hip joint function. In view of the age and general status of the patient and the absence of pain, no medication or surgical therapy was performed in this case.