Boker Lital Keinan, Merrick Joav
Julius Center for General Practice and Patient Oriented Research, University Medical Center Utrecht, The Netherlands.
Downs Syndr Res Pract. 2002 Mar;8(1):31-6. doi: 10.3104/reports.128.
The purpose of this study was to assess the incidence rates of leukaemia and other malignancies in persons with Down syndrome in Israel. The target population consisted of all persons with Down syndrome in the period of 1948-1995 and the study population was divided into two subgroups: (1) Persons born in Israel between 1979-95 (registry group) and (2) Persons currently or past-institutionalised, born before 1979 (institution group). The study population was linked to the Cancer Registry and cases that had been diagnosed through December 1995 were subsequently identified. The observed incidence rates were compared to expected rates in the general population. Standardised Incidence Ratios (SIR) and 95% confidence intervals were computed for each disease category. Analyses of results were performed separately for each subgroup of our study population. In the registry group seven cancer cases were observed as compared to 1.5 expected (SIR = 4.67 95% CI 1.9-9.6), all of which were leukaemia cases. For the institution group a total of 17 cancer cases were observed compared to 12.8 expected. These included four cases of leukaemia (SIR = 6.90 95% CI 1.9-17.7). An excess of gastric cancer in males, based on two cases (SIR = 11.9 95% CI 1.3-42.9) was also observed. The significant excess of leukaemia in the Down syndrome population in Israel is in accordance with other international studies. The excess of gastric cancer in males with Down syndrome, which has not been reported before, should be further explored.
本研究的目的是评估以色列唐氏综合征患者白血病及其他恶性肿瘤的发病率。目标人群包括1948年至1995年期间所有的唐氏综合征患者,研究人群分为两个亚组:(1)1979年至1995年在以色列出生的人(登记组);(2)1979年以前出生、目前或曾经在机构中生活的人(机构组)。研究人群与癌症登记处进行了关联,并确定了截至1995年12月已确诊的病例。将观察到的发病率与普通人群的预期发病率进行比较。计算了每种疾病类别的标准化发病率(SIR)和95%置信区间。对研究人群的每个亚组分别进行了结果分析。在登记组中,观察到7例癌症病例,而预期为1.5例(SIR = 4.67,95% CI 1.9 - 9.6),所有病例均为白血病。对于机构组,共观察到17例癌症病例,预期为12.8例。其中包括4例白血病(SIR = 6.90,95% CI 1.9 - 17.7)。还观察到基于2例男性胃癌病例的超额发病率(SIR = 11.9,95% CI 1.3 - 42.9)。以色列唐氏综合征人群中白血病的显著超额发病率与其他国际研究结果一致。唐氏综合征男性中胃癌的超额发病率此前未见报道,应进一步探究。