Boker L K, Blumstein T, Sadetzki S, Luxenburg O, Litvak I, Akstein E, Modan B
Department of Clinical Epidemiology, Chaim Sheba Medical Center, Ramat Gan, Israel.
Int J Cancer. 2001 Sep 1;93(5):741-4. doi: 10.1002/ijc.1383.
Epidemiologic data have confirmed the high susceptibility of persons with Down syndrome (DS) to leukemia. The question of proneness to other kinds of cancer is still open. In this study we reassessed the incidence rates of leukemia and other malignancies in Israeli DS subjects, based on the total population. The target population consisted of all DS subjects in Israel in the period of 1948--1995. Due to incompleteness of data, the target population was not fully achieved, thus the study population was divided into 2 subgroups: subjects born in Israel between 1979 and 1995 (registry group) and currently or past-institutionalized subjects born before 1979 (institution group). The cohort was linked with the Cancer Registry, and cancer cases that had been diagnosed through December 1995 were subsequently identified. Observed incidence rates were compared with expected rates in the general population. Standardized incidence ratios (SIR) and 95% confidence intervals (CI) were computed for each disease category. Analyses were performed separately for each subgroup of the study population. In the registry group, 7 cancer cases were observed, compared with 1.5 expected (SIR = 4.67, 95% CI 1.9--9.6), all leukemia cases. For the institution group a total of 17 cancer cases were observed, compared with 12.8 expected. These included 4 cases of leukemia (SIR = 6.90, 95% CI 1.90--17.70). An excess of gastric cancer in male subjects (SIR = 11.9, 95% CI 1.3--42.9) was also observed. Significant excess of leukemia in DS population in Israel is in accordance with previously published data. An excess of gastric cancer in DS male subjects born before 1979, which has not been reported before, should be further explored.
流行病学数据已证实唐氏综合征（DS）患者极易患白血病。至于他们是否易患其他类型癌症的问题仍未明确。在本研究中，我们基于总人口重新评估了以色列DS患者中白血病和其他恶性肿瘤的发病率。目标人群为1948年至1995年期间以色列所有的DS患者。由于数据不完整，未能完全涵盖目标人群，因此研究人群被分为两个亚组：1979年至1995年在以色列出生的患者（登记组）以及1979年以前出生且目前或过去曾在机构中生活的患者（机构组）。该队列与癌症登记处建立了关联，随后确定了截至1995年12月已确诊的癌症病例。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（CI）。对研究人群的每个亚组分别进行了分析。在登记组中，观察到7例癌症病例，预期为1.5例（SIR = 4.67，95%CI 1.9 - 9.6），均为白血病病例。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 1.90 - 17.70）。还观察到男性患者中胃癌发病率过高（SIR = 11.9，95%CI 1.3 - 42.9）。以色列DS人群中白血病发病率过高与先前发表的数据一致。1979年以前出生的DS男性患者中胃癌发病率过高这一情况此前未见报道，应进一步探究。
