Cancer Risk in Cystic Fibrosis: A 20-Year Nationwide Study From the United States
Cancer Risk in Cystic Fibrosis: A 20-Year Nationwide Study From the United States
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DOI：
10.1093/jnci/djs481
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发表时间：
2013-01-01
影响因子：
10.3
通讯作者：
Lowenfels, Albert B.
中科院分区：
文献类型：
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作者：
Maisonneuve, Patrick;Marshall, Bruce C.;Lowenfels, Albert B.
许多囊性纤维化（CF）患者现在已经成年，此时患癌症的风险增加。本研究的目的是确定非移植和移植CF患者的癌症风险。方法从1990年到2009年，我们随访了41，188名在美国250个CF护理中心接受护理的患者，并将非移植和移植患者的癌症观察数量与美国普通人群的预期数量进行比较。结果在344名患者中，对非移植患者进行114患者-年的观察，总体癌症风险与背景风险相似（标准化发病率比[SIR] = 1.1，95%置信区间[CI] = 1.0 - 1.3）。然而，我们观察到涉及食管胃交界处、胆道、小肠和结肠的消化道癌风险升高（SIR = 3.5，95% CI = 2.6 - 4.7）。睾丸癌（SIR = 1.7，95% CI = 1.02 - 2.7）和淋巴性白血病（SIR = 2.0，95% CI = 1.2 - 3.1）的风险也增加，恶性黑色素瘤的风险降低（SIR = 0.4，95% CI = 0.2 - 0.9）。在8235患者-年的移植患者观察中，观察到26例肿瘤，而预期为9.6例（SIR = 2.7，95% CI = 1.8 - 3.9）。增加的风险是特别高的消化道癌症（SIR = 17.3，95%CI = 10.7至26.5），与大多数情况下出现在bowel.Conclusions CF患者的癌症的总体负担仍然较低，但他们有一个增加的消化道癌症的风险，特别是在移植。他们也有淋巴白血病和睾丸癌的风险增加，黑色素瘤的风险降低。J Natl Cancer Inst 2013;105：122-129
Background Many patients with cystic fibrosis (CF) now reach adulthood, at which time the risk of cancer is increased. The aim of this study was to determine cancer risks in nontransplanted and transplanted CF patients.Methods From 1990 to 2009, we followed 41,188 patients who received care at one of the 250 CF care center programs in the United States and compared the observed number of cancers in nontransplanted and transplanted patients with that expected in the general US population.Results In 344,114 patient-years of observation of nontransplanted patients, the overall cancer risk was similar to the background risk (standardized incidence ratio [SIR] = 1.1, 95% confidence interval [CI] = 1.0 to 1.3). However, we observed an elevated risk of digestive tract cancer (SIR = 3.5, 95% CI = 2.6 to 4.7) involving the esophago-gastric junction, biliary tract, small bowel, and colon. There was also an increased risk of testicular cancer (SIR = 1.7, 95% CI = 1.02 to 2.7) and lymphoid leukemia (SIR = 2.0, 95% CI = 1.2 to 3.1) and a decreased risk of malignant melanoma (SIR = 0.4, 95% CI = 0.2 to 0.9). In 8235 patient-years of observation of transplanted patients, 26 tumors were observed compared with 9.6 expected (SIR = 2.7, 95% CI = 1.8 to 3.9). The increased risk was particularly high for digestive tract cancers (SIR = 17.3, 95% CI = 10.7 to 26.5), with most cases arising in the bowel.Conclusions The overall burden of cancer in CF patients remains low; however they have an increased risk of digestive tract cancer, particularly following transplantation. They also have increased risk of lymphoid leukemia and testicular cancer, and decreased risk of melanoma. J Natl Cancer Inst 2013;105:122-129