Risk of cancer during the first four years of life in children from The Beckwith-Wiedemann Syndrome Registry
Risk of cancer during the first four years of life in children from The Beckwith-Wiedemann Syndrome Registry
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DOI：
10.1016/s0022-3476(98)70008-3
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发表时间：
1998-03-01
影响因子：
5.1
通讯作者：
Tucker, MA
中科院分区：
文献类型：
--
作者：
DeBaun, MR;Tucker, MA
为了确定Beck-with-Wiedemann综合征（BWS）儿童癌症的发病率和相对风险（RR），对BWS儿童从出生到死亡、诊断癌症、4岁生日或随访的最后一天进行随访。共对183例BWS儿童进行了482人年的随访。研究终点为癌症的发病率、癌症的RR和与特定BWS表型特征相关的RR。13名儿童在四岁前被发现患有癌症，相比之下，根据同期一般人口的发病率，预计这一群体中的癌症不到一种。在生命的前4年，癌症的年平均发病率为每人每年0.027例癌症。肾母细胞瘤（RR = 816; 95%置信区间[CI]，359-1156）、神经母细胞瘤（RR = 197; 95% CI，22-711）和肝母细胞瘤（RR = 2280; 95% CI，928- 11，656）的RR具有统计学显著性。肢体不对称（偏侧肥大）是与癌症RR增加相关的唯一临床特征（RR = 4.6; 95% CI，1.5-14.2）。鉴于BWS患者在婴儿期和儿童早期癌症的高发病率，有必要进行前瞻性研究以解决癌症筛查的实用性。
To determine the incidence and relative risk (RR) of cancer in children with Beck-with-Wiedemann syndrome (BWS), children with BWS were followed up from birth until death, diagnosis of cancer, fourth birthday, or last day of follow-up. A total of 183 children with BWS were followed up for 482 person-years. The end points were incidence of cancer, RR of cancer, and RR associated with specific BWS phenotypic features. Thirteen children were identified with cancers before the fourth year of life in comparison with fewer than one cancer expected in this group on the basis of general population rates over the same period. The average annual incidence of cancer in the first 4 years of life was 0.027 cancer per person-year. The RR of Wilms tumor (RR = 816; 95% confidence interval [CI], 359-1156), neuroblastoma (RR = 197; 95% CI, 22-711), and hepatoblastoma (RR = 2280; 95% CI, 928-11,656) were statistically significant. Asymmetry of the limbs (hemihypertrophy) was the only clinical feature associated with an increased RR of cancer (RR = 4.6; 95% CI, 1.5-14.2). Given the high incidence of cancer in infancy and early childhood of patients with BWS, a prospective study is warranted to address the utility of screening for cancer.