Galbraith H J, Evans E C, Lacey J
Postgrad Med J. 1977 Jan;53(615):33-9. doi: 10.1136/pgmj.53.615.33.
An unselected series of 285 patients with Paget's disease of bone has been studied, of whom 54% were male and 46% female. The youngest was aged 25 years at diagnosis and the oldest 95. Of the patients 109 (38-2%) had never suffered serious symptoms. These diagnosed cases certainly represent only a small proportion, perhaps 10-15%, of all those with Paget's disease in the community from which they came. Few of the undiagnosed cases are likely to be suffering from serious symptoms or complications so it is apparent that the majority (probably over 80 or 90%) of those with the pathological state are relatively untroubled by their disease. 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 of the 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 proportion in control series. There is evidence to suggest that the degree of severity of Paget's disease does not vary, once it has developed in any particular individual, and that the more severe and extensive forms may have a stronger genetic basis.
对285例未经挑选的骨Paget病患者进行了研究，其中男性占54%，女性占46%。诊断时最年轻的患者为25岁，最年长的为95岁。109例（38.2%）患者从未出现过严重症状。这些已确诊的病例肯定仅占其所在社区所有骨Paget病患者的一小部分，可能为10% - 15%。未确诊的病例中很少有严重症状或并发症，因此很明显，大多数（可能超过80%或90%）处于病理状态的患者相对未受疾病困扰。最常见的并发症（8.8%）是病理性骨折。骨折累及股骨干的占62%，在骨Paget病患者中该部位骨折的发生率几乎是普通人群的7倍。由于社区中有大量未确诊病例，所有骨Paget病患者中病理性骨折和骨肉瘤的实际发生率可能分别仅为1%和0.1%。对128例患者同时进行了血清碱性磷酸酶和血沉（ESR）的检测。结果表明，血沉作为疾病活动度或范围的指标毫无价值。在许多患者中，血清碱性磷酸酶水平在数年期间变化很小。在三个家族中发现有一个或多个近亲患有该病的病史，其发生率低于先前记录研究的预期。对215例患者进行了ABO血型测定；50%为A型，而对照组为46%。两组受试者血型分布的差异很小，完全在偶然范围内。对138例患者的分泌状态进行了研究；77%为分泌型。这与对照组的比例相似。有证据表明，骨Paget病一旦在任何特定个体中发生，其严重程度不会改变，而且更严重和广泛的形式可能有更强的遗传基础。
