Paget's disease of bone in New Zealand: Continued decline in disease severity
Paget's disease of bone in New Zealand: Continued decline in disease severity
复制标题
DOI：
10.1007/s00223-004-0281-z
复制
发表时间：
2004-11-01
影响因子：
4.2
通讯作者：
Cundy, T
中科院分区：
文献类型：
--
作者：
Cundy, HR;Gamble, G;Cundy, T
我们之前曾报道过，1973 年至 1993 年间，严重佩吉特骨病在我们中心已不再常见。来自多个国家的数据支持这样的观点，即佩吉特骨病的患病率和严重程度存在重要的长期趋势。在本文中，我们描述了新转诊的佩吉特病患者的人口统计学的最新趋势，以确定病情较轻的长期趋势是否仍在继续。检查了所有新转诊的佩吉特病患者（n = 1487）（1973 年至 2002 年，30 年）的数据库。其中，832 名受试者 (56%) 接受了闪烁扫描。血浆总碱性磷酸酶（总ALP）活性、闪烁扫描上的疾病程度以及派生骨的平均ALP活性的衍生指数被用作严重程度的指数。自 1994 年起，新转诊佩吉特病的人数急剧下降，目前仅为 20 年前的一半，而平均就诊年龄每十年增加 4 岁 (P < 0.0001)。诊断时的总 ALP、闪烁扫描的疾病程度以及涉及的骨骼数量均与出生日期 (P < 0.0001) 和就诊年份 (P < 0.0001) 呈负相关，这表明最近出生和就诊的受试者的骨病严重程度要轻得多。页面骨的平均活动与就诊年份仅有微弱相关，但与出生年份或就诊年龄无关。尽管存在许多潜在的偏差，但这些数据与骨骼受累较少的老年受试者的持续长期趋势以及佩吉特病患病率下降是一致的。
We have reported previously that severe Paget's disease of bone had become less common at our center between 1973 and 1993. Data from several countries support the view that there are important secular trends in the prevalence and severity of Paget's disease. In this paper we describe recent trends in the demography of newly referred patients with Paget's disease to determine if the secular trend toward milder disease has continued. A database of all newly referred patients (n = 1487) with Paget's disease (1973 to 2002 inclusive, 30 years) was examined. Of these, 832 subjects (56%) had scintiscans. Plasma total alkaline phosphatase (total ALP) activity, disease extent on scintiscan, and a derived index of average ALP activity of pagetic bone were used as indices of severity. The number of new referrals with Paget's disease declined sharply from 1994 onward and is currently at half the rate seen 20 years earlier, while the mean age at presentation has progressively increased by 4 years per decade (P < 0.0001). Total ALP at diagnosis, disease extent on scintiscan, and the number of bones involved were all negatively correlated with both date of birth (P < 0.0001) and year of presentation (P < 0.0001), indicating that more recently born and presenting subjects had substantially less severe bone disease. The average activity of pagetic bone was only weakly correlated with year of presentation, but not with year of birth or age at presentation. Although there are a number of potential biases, these data are consistent with a continued secular trend to presentation in older subjects with less extensive skeletal involvement, and a declining prevalence of Paget's disease.