Brenton D P, Isenberg D A, Bertram J
Postgrad Med J. 1980 Apr;56(654):238-43. doi: 10.1136/pgmj.56.654.238.
A most unusual family with aggressive Paget's disease is reported. Three brothers out of 10 siblings developed the disease at a relatively early age. Two of these brothers developed an osteosarcoma and died. It is emphasized that very little information is available concerning the predisposition to severe polyostotic disease and even sarcomatous change when Paget's disease begins at a young age or is familial. Possibly the risk of these serious complications is then greater than when the disease is sporadic and begins later in life.
报道了一个患有侵袭性佩吉特病的非常特殊的家庭。10个兄弟姐妹中有3个兄弟在相对年轻时患上了这种疾病。其中两个兄弟发展成骨肉瘤并死亡。需要强调的是，关于佩吉特病在年轻时发病或具有家族性时，严重多骨型疾病以及肉瘤样变的易感性，目前可用信息极少。当疾病为散发性且在生命后期发病时，这些严重并发症的风险可能比佩吉特病年轻时发病或具有家族性时要小。
