Paget's disease of bone—a clinical and genetic study

Abstract

The most common complication (8.8%) was pathological fracture. The fracture involved the shaft of the femur in 62%, occurring in this section of the bone almost seven times more frequently in the patients with Paget's disease than in the general population.Because of the large number of undiagnosed cases in the community, the true incidence of pathological fracture and of sarcoma of bone in all patients with Paget's disease is probably only 1% and 0-1% respectively.Simultaneous estimates of the serum alkaline phosphatase and ESR were made in 128 ofthe patients.It was shown that the ESR is valueless as a measure of activity or extent of the disease.In many patients the level of the serum alkaline phosphatase changed very little over periods of several years.A history of the disease in one or more close relatives was found in three families, a lower incidence than was expected from previously recorded studies. The ABO blood groups were determined in 215 of the patients; 50% were of group A compared with 46% in a control group. The difference in distribution of blood groups in the two series of subjects is small and well within chance limits. The secretor status of 138 patients was investigated; 77% were secretors. This is similar to the DroDortion in control series.

Demographic and Clinical Features Related to a Symptomatic Onset of Paget’s Disease of Bone

“…A widely accepted figure estimates that symptomatic disease could account for 5% of the total population of patients with PDB 7 . As reported by others, the age at diagnosis was not different between asymptomatic patients diagnosed by chance and patients with a clinical diagnosis, suggesting that the disease does not become symptomatic with age 14 . Even if the disease extent were not greater in men than in women, a significantly older age at diagnosis was found in women, in keeping with other series 4,15 .…”

Osteosarcoma complicating familial Paget’s disease

“…Thus Schmorl (1932) noted a 10% incidence in persons aged > 90 years. The incidence of the disease under 40 years of age is not certain, but Galbraith, Evans and Lacey (1977) had only 3 such patients in their series of 285 with Paget's disease and only 12 under the age of 50 years. The surviving patient described here was probably suffering from Paget's disease of the pelvis when he developed osteoarthrosis of the left hip aged 38 years.…”

“…Galbraith, Evans and Lacey (1977) suggested that a positive family history is more likely to be associated with severe polyostotic disease. A very early age of onset of Paget's disease in a familial situation was noted by Irvine (1953) in 2 affected daughters of an affected male patient who subsequently developed an osteoclastoma.…”

The Significance of Hepatobiliary Enzymes for Differentiating Liver and Bone Diseases: A Case Control Study from Manipal Teaching Hospital of Pokhara Valley

“…The above findings concurred with Trifan 28 . Number of males were more in cases of Paget's disease than females 29 .…”