Impact of screening examinations on survival in familial adenomatous polyposis.
Impact of screening examinations on survival in familial adenomatous polyposis.
复制标题
筛查检查对家族性腺瘤性息肉病生存的影响。
DOI：
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复制
发表时间：
2000
影响因子：
1.9
通讯作者：
H. Järvinen
中科院分区：
文献类型：
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作者：
I. Heiskanen;T. Luostarinen;H. Järvinen
背景
预防性家庭筛查和手术改善了家族性腺瘤性息肉病（FAP）患者的预后，在很大程度上预防了结直肠癌导致的死亡。本研究比较了通过症状（先证者）或通过家庭筛查（应召）诊断的FAP患者的死亡率和死亡原因。
方法
这项研究包括了截至1998年6月底在芬兰息肉病登记处登记的所有236名FAP患者。有116名先证者和120名应召患者，诊断时的中位年龄分别为36.8岁和22.8岁，中位随访时间分别为6.3年和9.9年。计算各组的累积粗生存率和相对生存率估计值，并确定死亡原因。
结果
结肠切除术后，征召组的预期寿命明显优于先证者（P < 0.001）。在结肠切除术后18年内，召集组的生存率与一般人群中可比组的预期生存率相等。主要死亡原因是结直肠癌，占68例死亡中的54例：4例在召集组（均为直肠残端癌），50例在先证者中。上消化道癌导致4例死亡（壶腹周围癌2例，胃癌2例），2例死亡是由于术后肺栓塞。
结论
通过预防性筛查和手术治疗，FAP患者的生存率显著提高。进一步的改善可能是使用修复性直肠结肠切除术，而不是结肠切除术和回肠直肠吻合术，并定期上消化道内镜监测。
BACKGROUND
Prophylactic family screening and surgery has improved the outcome of patients with familial adenomatous polyposis (FAP) largely preventing deaths due to colorectal cancer. The present study compared the mortality rates and causes of death of FAP patients diagnosed by symptoms (probands) or by family screening (call-up).
METHODS
The study comprised all 236 FAP patients registered in the Finnish Polyposis Registry until the end of June 1998. There were 116 probands and 120 call-up patients with a median age of 36.8 and 22.8 at diagnosis and median follow-up times of 6.3 and 9.9 years, respectively. Cumulative crude and relative survival estimates were calculated for each group and the causes of death were determined.
RESULTS
The life expectancy was significantly better in the call-up group than in the probands after colectomy (P < 0.001). The survival rates of the call-up group equaled those expected for a comparable group in the general population up to 18 years after colectomy. The main cause of death was colorectal cancer accounting for 54 out of 68 deaths: four in the call-up group (all rectal stump cancer) and 50 in probands. Upper GI-tract cancer caused four deaths (periampullary cancer two, stomach cancer two) and two deaths were due to postoperative pulmonary embolism.
CONCLUSION
The survival of FAP patients is significantly improved by prophylactic screening and surgery. Further improvement may be possible by using restorative proctocolectomy instead of colectomy and ileorectal anastomosis and by regular upper GI-tract endoscopic surveillance.