...
  • 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>World Journal of Gastroenterology >Clinical analysis of eight kindreds of familial adenomatous polyposis
【24h】

Clinical analysis of eight kindreds of familial adenomatous polyposis

机译：八类家族性腺瘤性息肉病的临床分析

获取原文
获取原文并翻译 | 示例
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

AIM: To study the early diagnosis and management of familial adenomatous polyposis (FAP). METHODS: Eight pedigrees of FAP were collected and their pedigree trees were protracted. Clinical characteristics and treatment outcomes of FAP patients in these kindreds were analysed. RESULTS: A total of 157 members were investigated in eight kindreds and 25 patients with FAP were diagnosed. The ratio of male patients and female patients was 16:9 and the average age at onset was 38 years. Among them, six patients died of cancer with a mortality rate of 28%, and 36% (9/25) FAP patients were diagnosed as synchronous colorectal cancer on the basis of FAP. A proband was diagnosed as synchronous colorectal cancer with liver metastasis and died 11 mo later after partial colectomy and hepatic metastatic lesion biopsy. The other seven probands received total abdominal colectomy and rectal mucosectomy with ileal pouch-anal anastomosis (IPAA), and one of them was diagnosed as synchronous colon cancer on the basis of FAP and was still alive after 7.5 years follow-up. Among the other seven patients with synchronous colorectal cancer on the basis of FAP underwent total abdominal colectomy with ileorectal anastomosis (IRA), one underwent total remnant rectum resection and ileostomy for recurrent carcinoma in the retained rectum 2.5 years later after the IRA and was still alive, while the others all died of recurrence with a median survival time of 4.6 years. Through close follow-up and termly endoscopic surveillance, three FAP patients were detected before presenting symptoms at the age of 18, 20 and 23 years, respectively. Prophylactic IPAA was performed and results were satisfactory after the patients were followed-up for 6,1, and 8 years, respectively. CONCLUSION: Pedigree investigation, close follow-up and termly endoscopic surveillance are very important for early detection of FAP. Prophylactic IPAA can give satisfactory results to FAP patients.
机译：目的：研究家族性腺瘤性息肉病（FAP）的早期诊断和治疗。方法：收集了8个FAP家系，并对其家系树进行了拉长。分析了这些亲属中FAP患者的临床特征和治疗结果。结果：共对8个家庭的157名成员进行了调查，并诊断出25例FAP患者。男性和女性患者的比例为16：9，平均发病年龄为38岁。其中，有6名患者死于癌症，死亡率为28％，而在FAP的基础上，有36％（9/25）FAP患者被诊断为同步性结直肠癌。一个先证者被诊断为具有肝转移的同步性大肠癌，并在部分结肠切除术和肝转移性病变活检后11 mo死亡。其他七个先证者接受了全肠结肠切除术和直肠粘膜切除术并伴有回肠袋-肛门吻合术（IPAA），其中一个根据FAP被诊断为同步性结肠癌，并且在7.5年的随访后仍然活着。在基于FAP的其他7例同时发生大肠癌的患者中，接受了全肠结肠切除术并进行了回肠直肠吻合术（IRA），其中1例在IRA术后2.5年在保留的直肠中进行了全残直肠切除和回肠造口术，以保留复发性直肠癌，并且仍然活着，而其他所有患者均死于复发，中位生存时间为4.6年。通过密切随访和定期的内窥镜监测，分别在三名FAP患者被发现之前分别出现了18、20和23岁的症状。对患者进行了分别为期6,1和8年的随访，进行了IPAA预防性治疗，结果令人满意。结论：家谱调查，密切随访和定期的内窥镜监测对于FAP的早期发现非常重要。预防性IPAA可使FAP患者获得满意的结果。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号