[Evaluation of clinical and humoral data in 148 patients with Piaget's bone disease]

Pain was present in 113 out of a total of 148 patients with Paget's bone disease who were evaluated. The symptom was originated by: a) bone, in 56.6 % of the cases; b) joints, in 32.7 %; c) skull compression in 7.1 % and spine compression in 3.5 % of the cases. Bone deformities were seen in 50.7 % of patients. Of this total, 41.4 % were localized in femorae. Pathological fractures were found in 16.9 % of patients. Of this total, 44.0 % were localized in femore and 19.2 % in the humerae. The localization of Paget's bone disease in the humerus appears to be specially risky due to the high percentage of fractures found in the present study as well as the frequency of the sarcomatous degeneration described in the literature. Two patients (1.4 %) developed an osteogenic sarcoma in bones previously affected by Paget's disease. It was localized in the tibia in one case and in iliac bone in the other. Frequent associated diseases were osteoporosis, verified in 31.8 % of the patients, arthritis deformans in 28.6 % and nephrolithiasis, verified in 31.8 % of the patients, arthritis deformans in 28.6 % and nephrolithiasis in 12.2 %. Associated neoplastic diseases were mammary cancer in 6.5 % of 77 women and colonic cancer in 2.0 of 148 patients of both sexes. The diagnosis has been made previously or during the period of the present study. The effects of the extent of Paget's bone disease as well as the effect of Paget's skull complication on serum alkaline phosphatase and urinary total hydroxyproline were investigated. Serum alkaline phosphatase and urinary total hydroxyproline were higher in polyostotic then in oligostotic patients although the difference was not significant for serum alkaline phosphatase in a group of 50 polyostotic with skull lesions versus 16 oligostotic with skull lesions. There was a significant increase of serum alkaline phosphatase in groups of 29 polyostotic without skull lesions versus 45 oligostotic without skull lesions (p < 0.001) and in groups of 50 polyostotics with skull lesions versus 29 polyostotics with skull lesions (p < 0.05), and of 16 oligostotic withh skull lesions versus 45 oligostotics without skull lesions (p < 0.02). There was a significant increase of urinary total hydroxyproline in a group of 46 polyostotics with skull lesions versus 15 oligostotics with skull lesions (p < 0.01) and of 25 polyostotics without skull lesions versus 41 oligostotics without skull lesions (p < 0.001), as well of 46 polyostotics with skull lesions versus 25 polyostotics without skull lesions (p < 0.005) and 15 oligostics with skull lesions versus 41 oligostotics without skull lesions (p < 0.02). Although the differences should be referred to a greater number of patients, the results suggest that the effects of Paget skull lesions are more evident on serum alkaline phosphatase than on urinary total hydroxyproline.