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1.
OBJECTIVES: At present, surveillance of premalignant small bowel polyps in hereditary polyposis syndromes has a number of limitations. Capsule endoscopy (CE) is a promising new method to endoscopically assess the entire length of the small bowel. METHODS: We prospectively examined 40 patients with hereditary polyposis syndromes (29 familial adenomatous polyposis (FAP), 11 Peutz-Jeghers syndrome (PJS)). Results were compared with push-enteroscopy (PE) results in FAP and with esophagogastroduodenoscopy, PE, (MR)-enteroclysis, and surgical specimen in PJS patients. RESULTS: A total of 76% of the patients with FAP with duodenal adenomas (n = 21) had additional adenomas in the proximal jejunum that could be detected by CE and PE. Moreover, 24% of these FAP patients had further polyps in the distal jejunum or ileum that could only be detected by CE. In contrast, in FAP patients without duodenal polyps (n = 8), jejunal or ileal polyps occurred rarely (12%). CE detected polyps in 10 of 11 patients with PJS, a rate superior to all other reference procedures employed. Importantly, the findings of CE had immediate impact on further clinical management in all PJS patients. CONCLUSIONS: Our results suggest that CE may be of clinical value in selected patients with FAP, whereas in PJS, CE could be used as first line surveillance procedure.  相似文献   

2.
BACKGROUND: Duodenal cancer and ampullary cancer are major causes of death after a prophylactic colectomy in patients with familial adenomatous polyposis (FAP). Forward-viewing endoscopy and side-viewing endoscopy are recommended in patients with FAP for surveillance of periampullary and duodenal polyposis. The study of polyps distal to the duodenum in FAP is limited. A capsule endoscopy (CE) allows visualization of the mucosa of the entire small bowel. OBJECTIVE: The objective was to detect whether CE has clinical value or any utility for the surveillance of small-bowel polyps in patients with FAP and to evaluate whether there are genotypic factors that predict which patients are at a lower risk of small-bowel polyps. SETTING: Two Italian tertiary-referral centers. PATIENTS: Twenty-three patients with FAP who presented for a CE. MAIN OUTCOME MEASUREMENTS: Patients with FAP were examined by CE to assess the location, size, and number of small-bowel polyps. Patient age at CE, sex, years of observation after surgery, type of surgery, duodenal adenomas, and colorectal cancer at surgery were analyzed. All patients were selected for mutation analysis, and the germline adenomatous polyposis coli (APC) gene mutation was detected. RESULTS: Eleven of 23 patients with FAP had duodenal polyps. During CE, jejunal-ileal polyps were detected in 7 of 23 FAPs, with a total number of 15 polyps in the ileum. The presence of duodenal adenomas was the only clinical feature predictive of small-bowel polyps. Identification of the ampulla of Vater was not achieved with CE; duodenal polyps were only seen in 4 of 11 patients identified endoscopically, with an underestimation of polyp numbers. APC mutations between codons 499 and 805 were associated with the absence of small-bowel polyps. CONCLUSIONS: CE is useful and safe for the surveillance of jejunal-ileal polyps in selected patients with FAP. CE is not useful in the surveillance of the duodenum where the majority of small-bowel cancers occur.  相似文献   

3.

Purpose  

Familial adenomatous polyposis (FAP) and Peutz–Jeghers syndrome (PJS) are hereditary polyposis syndromes with a high risk for benign small-bowel polyps and cancer. The aim of this study was to assess the prevalence of small-bowel polyps beyond the duodenum in patients with FAP and PJS and to examine the clinical value and the optimal interval of capsule endoscopy (CE) for the surveillance of small-bowel polyps in patients with FAP.  相似文献   

4.
AIM: To detect the prevalence of small bowel polyps by wireless capsule endoscopy (WCE) in patients with familial adenomatous polyposis (FAP). METHODS: We examined prospectively 14 patients with FAP to assess the location, size and number of small-intestinal polyps. Patients' age, sex, years of observation after surgery, type of surgery, duodenal polyps and colorectal cancer at surgery were analyzed. RESULTS: During WCE, polyps were detected in 9/14 (64.3%) patients. Duodenal adenomatous polyps were found in nine (64.3%) patients, and jejunal and ileal polyps in seven (50%) and eight (57.1%), respectively. The Spigelman stage of duodenal polyposis was associated with the presence of jejunal and ileal polyps. Identification of the ampulla of Vater was not achieved with WCE. Importantly, the findings of WCE had no immediate impact on the further clinical management of FAP patients. No procedure-related complications were observed in the patients. CONCLUSION: WCE is a promising noninvasive new method for the detection of small-intestinal polyps. Further investigation is required to determine which phenotype of FAP is needed for surveillance with WCE.  相似文献   

5.
Hereditary syndromes cause approximately 5 to 15% of overall colorectal cancer (CRC) cases. Hereditary CRC is conventionally divided into two major categories: hereditary non-polyposis colorectal cancer (HNPCC) and those related to polyposis syndromes including familial adenomatous polyposis (FAP), Peutz-Jegher syndrome (PJS), and juvenile polyposis (JP). The screening for the cancer and methods of treatment applied to patients with hereditary CRC are quite different from those applied to the general population. The genes responsible for these syndromes has recently identified, as a result, genetic testing has become the most important determining factor in clinical decisions. Germ-line mutation of the APC gene induces FAP, an autosomal dominant disorder, characterized by the development of hundreds to thousands of colonic adenomas. CRC appears in almost all affected individuals by the time they are 50 years of age. An affected individual should undergo colectomy by his/her late teens. Furthermore, according to the findings of genetic testing, at-risk family members also need endoscopic surveillance and surgery. Recently, a mutation on the MYH gene is increasingly being investigated in patients with multiple polyps, and autosomal recessive MYH polyposis is considered to be a new category of polyposis. More common than FAP, HNPCC is caused by germ-line mutations in DNA mismatch repair genes, mainly MLH1 and MSH2. Although there is no polyposis, polyps seem to be more villous and dysplastic and appear to grow rapidly into CRCs. The aggregate lifetime risk of CRC is about 80% for mutation carriers. The risk for other types of cancer, such as endometrial, ovarian, small bowel, and transitional cell cancer, is also increased. The Amsterdam criteria and Bethesda guidelines are the best-known tools for diagnosis and genetic testing, and colectomy followed by endoscopic follow-up is the standard treatment. PJS and JP are reported to be characterized by hamartomatous polyps throughout the GI tract and germ-line mutations in the STK11 gene (PJS) and the DPC4/BMPR1A gene (JP).  相似文献   

6.
BACKGROUND: Duodenal cancer is one of the leading causes of death in familial adenomatous polyposis (FAP) patients. An endoscopic surveillance programme was therefore initiated in 1988, the outcome of which is described in this paper. METHODS: We report the 10 year follow up of 114 patients with FAP who were prospectively screened for the presence and severity of duodenal adenomas. RESULTS: Six of 114 patients (median age 67 years) developed duodenal adenocarcinoma. Four of these were from 11 patients who originally had Spigelman stage IV disease (advanced duodenal polyposis), which gives a 36% risk within this group of developing cancer. One case of duodenal cancer arose from 41 patients who originally had stage III disease (2%) and one cancer arose from 44 patients with original stage II disease (2%). All six patients have died: five were inoperable and one had recurrence three years after a pancreaticoduodenectomy. There was no association between duodenal cancer and site of germline mutation of the APC gene. CONCLUSIONS: Surveillance for duodenal adenocarcinoma and subsequent early referral for curative surgery has not been effective. Selection of patients with advanced but benign (Spigelman stage IV) duodenal polyposis for prophylactic pancreaticoduodenectomy should therefore be considered and can now be justified on the basis of these results. More comprehensive endoscopic surveillance of high risk (stage III and IV) patients is needed in an attempt to avoid underestimating the severity of duodenal polyposis, and to evaluate the role of endoscopic therapy in preventing advanced disease.  相似文献   

7.
Familial adenomatous polyposis (FAP) is a hereditary disorder caused by Adenomatous Polyposis Gene mutations that lead to the development of colorectal polyps with great malignant risk throughout life. Moreover, numerous extracolonic manifestations incorporate different clinical features to produce varied individual phenotypes. Among them, the occurrence of duodenal adenomatous polyps is considered an almost inevitable event, and their incidence rates increase as a patient’s age advances. Although the majority of patients exhibit different grades of duodenal adenomatosis as they age, only a small proportion (1%-5%) of patients will ultimately develop duodenal carcinoma. Within this context, the aim of the present study was to review the data regarding the epidemiology, classification, genetic features, endoscopic features, carcinogenesis, surveillance and management of duodenal polyps in patients with FAP.  相似文献   

8.
Adenomas of the duodenum have been described in patients with familial adenomatous polyposis (FAP). Patients with FAP are at high risk for the development of periampullary cancer. The aim of our study was to evaluate if endoscopic visualization of small polyps, often overlooked at standard endoscopic examination, was improved by chromoendoscopy. Ten patients with FAP and previous colectomy underwent upper gastrointestinal endoscopy. Two skilled endoscopists were involved for each endoscopy. Evaluation of number and diameter of polyps was made before and after staining. After staining we detected a larger number of duodenal polyps than found at the standard endoscopic examination, the difference being statistically significant. This result seems to suggest that chromoendoscopy may improve diagnostic yield of endoscopy. Further studies are needed to suggest the best surveillance program and the appropriate therapeutic modality for these patients.  相似文献   

9.
Nonampullary duodenal adenomas are relatively common in familial adenomatous polyposis(FAP), but nonampullary sporadic duodenal adenomas(SDAs)are rare. Emerging evidence shows that duodenal adenomas, regardless of their anatomic location and whether they are sporadic or FAP-related, share morphologic and molecular features with colorectal adenomas. The available data suggest that duodenal adenomas develop to duodenal adenocarcinomas via similar mechanisms. The optimal approach for management of duodenal adenomas remains to be determined. The techniques for endoscopic resection of duodenal adenoma include snare polypectomy, endoscopic mucosal resection(EMR), endoscopic submucosal dissection(ESD), and argon plasma coagulation ablation. EMR may facilitate removal of large duodenal polyps. Although several studies have reported cases of successful ESD for duodenal adenomas, the procedure is technically difficult to perform safely because of the anatomical properties of the duodenum. Although current clinical practice recommends endoscopic resection of all large duodenal adenomas in patients with FAP, endoscopic treatment is usually insufficient to guarantee a polypfree duodenum. Surgery is indicated for FAP patients with severe polyposis or nonampullary SDAs or FAPrelated polyps not amenable to endoscopic resection. Further studies are needed to develop newer endoscopic techniques to guide diagnostic and therapeutic decisions for future management of nonampullary duodenal adenomas.  相似文献   

10.
BACKGROUND: The extra colonic manifestations, like upper gastrointestinal tract polyps and duodenal cancer are disorders that affect long-term morbidity and mortality of patients with familial adenomatous polyposis, after rectocolectomy. AIM: To describe the frequency of those disorders in patients with familial adenomatous polyposis and to review efficacy of upper gastrointestinal endoscopic surveillance. METHODS: Between 1984 and 2005, 62 patients with familial adenomatous polyposis after rectocolectomy, were studied retrospectively, by Coloproctology Group, Medical Sciences Faculty, State University of Campinas, SP, Brazil. It was possible to analyze 53 patients (85,5%) in this study. RESULTS: Twenty seven (50,9%) of 53 patients in follow-up had upper gastrointestinal polyps. Eight (15,4%) had gastric adenomatous polyps, 14 (27%), duodenal polyps and 5 (9,6%) duodenal and gastric polyps. Two patients (3,8%) had adenomatous duodenal polyps with severe dysplasia, and one (1,9%) had adenocarcinoma of the duodenal papilla. CONCLUSION: The upper gastrointestinal endoscopic surveillance has importance and the aim is to detect as early as possible the occurrence of duodenal adenocarcinoma and upper gastrointestinal polyps with severe dysplasia.  相似文献   

11.
Background: In familial adenomatous polyposis and juvenile polyposis, polyps can occur throughout the gastrointestinal tract.Methods: We report seven patients with familial adenomatous polyposis and two patients with juvenile polyposis who underwent small bowel enteroscopy at the time of exploratory celiotomy either for colectomy or other pathology.Results: Polyps in the jejunum and/or ileum were noted in five of nine (56%) patients at enteroscopy. In three of nine (33%) patients these polyps were adenomatous. Two of these patients had polyps in the jejunum and in the ileum, whereas one patient had jejunal adenomas alone. These polyps were from 3 mm to 30 mm in size. The remaining two patients with polyps had lymphoid hyperplasia in the ileum. All three patients who had adenomas at intraoperative small bowel enteroscopy had duodenal adenomas at esophagogastroduodenoscopy. At the age of 14 years, one patient had an intramucosal carcinoma in a small bowel juvenile polyp.Conclusion: Baseline small bowel enteroscopy should be considered at the time of surgical exploration in patients with asymptomatic familial adenomatous polyposis and juvenile polyposis. In patients with duodenal polyps, enteroscopy should be performed at the time of surgery. Biopsy and/or excision of larger polyps should be performed because these polyps may harbor a carcinoma. (Gastrointest Endosc 1995;42:560-4.)  相似文献   

12.
BACKGROUND: Familial adenomatous polyposis (FAP) is a hereditary cancer syndrome that includes gastro-duodenal involvement, polyposis, and a propensity to adenocarcinoma necessitating endoscopic surveillance. There are few data describing pediatric upper gastrointestinal FAP resulting in conflicting screening recommendations. OBJECTIVES: To characterize pediatric gastroduodenal FAP and to investigate the association between symptoms at endoscopy and APC mutation analysis with endoscopic-histologic findings warranting surveillance. METHOD: A retrospective chart review was performed, including all children with FAP who underwent upper endoscopy (EGD) at two institutions; (UNMC: 1992-2002, JHH: 1983-2002), all biopsies were reviewed and the APC mutations present in the cohort of patients were correlated to the pattern of severity of endoscopic findings and the frequency of APC mutations identified through commercially available testing for FAP (Labcorp: 1998-2002). RESULTS: Twenty-four patients from 21 families underwent 49 EGDs. Eighty-three percent were asymptomatic at the time of endoscopy. The most common finding was fundic gland polyposis (FGP) (51%), of which 42% and 15% harbored dysplasia and changes indefinite for dysplasia, respectively. Periampullary duodenal adenomata were present in 41% of patients with one patient necessitating ampullectomy. Symptoms at endoscopy were not predictive of premalignant changes. In 15 patients where the APC mutation was known patients with dysplastic FGP, gastric, or duodenal adenoma were more likely to harbor a mutation between codons 1225-1694 than the reference population (p= 0.006). CONCLUSIONS: All pediatric patients with FAP warrant upper gastrointestinal screening and surveillance endoscopy from the time of initial colonoscopy irrespective of referable symptoms. Patients with APC mutation between codon 1225-1694 may be more susceptible to aggressive gastroduodenal involvement in FAP.  相似文献   

13.
Opinion statement Patients with familial adenomatous polyposis (FAP) have a cumulative lifetime risk of over 90% for developing duodenal adenomas, which are the precursor lesions for duodenal adenocarcinoma. Consequently, these patients have a 5% to 10% lifetime risk of periampullary or duodenal adenocarcinoma, making this the leading cause of cancer death in FAP patients who have had prophylactic colectomies. The increased relative risk of duodenal carcinoma in FAP patients and the poor outcomes associated with the treatment of advanced duodenal cancer have led to the development of prevention strategies for this cancer in the setting of FAP. It is generally accepted that surveillance for duodenal adenomas and adenocarcinomas should be included in the management of patients with FAP, although there are few data from clinical trials that demonstrate the effectiveness of surveillance strategies or chemoprevention for the prevention of death from duodenal cancer. Prospective case series have shown that endoscopic surveillance with endoscopic or surgical treatment of high-risk lesions in the duodenal or periampullary region can be performed with successful removal of the at-risk lesion(s). Surveillance should begin at about 21 years of age and should be performed using both an end-viewing and a side-viewing upper endoscope. An interval of 3 to 5 years between examinations appears to be adequate if no polyposis is evident. Once polyposis develops, an interval of 1 to 3 years between screenings for mild polyposis is appropriate. Patients with denser polyposis or larger adenomas are recommended to undergo examination every 6 to 12 months because of their increased risk of developing duodenal adenocarcinoma. Nonsteroidal anti-inflammatory drug therapy with sulindac, a nonselective cyclooxygenase (COX) inhibitor, or celecoxib, a COX-2 selective inhibitor, may be of benefit after the development of duodenal polyposis by inducing the regression or stabilization of the polyposis, although there is limited evidence from randomized, controlled trials to support its routine use. Almost all cases of adenocarcinoma occur in patients with advanced polyposis (Spigelman stage IV disease), and approximately 33% of this group will go on to develop adenocarcinoma if left untreated. The most definitive procedure for reducing the risk of adenocarcinoma is surgical resection of the ampulla and/or duodenum. Pancreaticoduodenectomy or pancreas-sparing duodenectomy are appropriate surgical therapies that are believed to substantially reduce the risk of developing periampullary adenocarcinoma. However, these procedures are associated with significant morbidity and mortality, including the risk of inducing desmoid tumor formation in FAP patients.  相似文献   

14.
BACKGROUND: Duodenal adenomas are extremely common in patients with familial adenomatous polyposis. However, it is uncertain whether patients with duodenal adenomas without familial adenomatous polyposis are at greater risk for colorectal neoplasia and, therefore, should routinely undergo surveillance colonoscopy. The aim of this study was to determine whether there is a correlation between non-papillary duodenal adenoma without familial adenomatous polyposis and colorectal adenoma. METHODS: Twenty-five patients with non-papillary duodenal adenomas without familial adenomatous polyposis, seen from January 1990 to April 2003, were retrospectively evaluated. RESULTS: Non-papillary duodenal polyps were diagnosed by endoscopy in the 25 patients. Of these, 21 underwent colonoscopy and one underwent proctoscopy. The mean age of these 22 patients (12 women, 10 men) was 69 years (range 50-83 years). Sixteen of the 22 patients (72.7%) with duodenal adenomas had associated colorectal adenomas. A total of 38 adenomas and one colorectal cancer were detected. The mean size of the polyps was 6.2 mm (range 3-15 mm). The adenomas were removed by snare excision or with a biopsy forceps. CONCLUSIONS: Based on the results of this uncontrolled, retrospective study, the frequency of colorectal adenomas in patients with duodenal polyps without familial adenomatous polyposis appears to be increased compared with the general population. All patients with duodenal polyps should undergo surveillance colonoscopy for colorectal adenomas. A prospective study to definitively establish the frequency of colorectal adenomas in these patients is warranted.  相似文献   

15.
Duodenal adenomatosis in familial adenomatous polyposis   总被引:14,自引:0,他引:14  
BACKGROUND: The prevalence of duodenal carcinoma is much higher in familial adenomatous polyposis (FAP) than in the background population, and duodenal adenomatosis is found in most polyposis patients. AIMS: To describe the long term natural history of duodenal adenomatosis in FAP and evaluate if cancer prophylactic surveillance of the duodenum is indicated. METHODS: A prospective five nation study was carried out in the Nordic countries and the Netherlands. PATIENTS: A total of 368 patients were examined by gastroduodenoscopy at two year intervals during the period 1990-2001. RESULTS: At the first endoscopy, 238 (65%) patients had duodenal adenomas at a median age of 38 years. Median follow up was 7.6 years. The cumulative incidence of adenomatosis at age 70 years was 90% (95% confidence interval (CI) 79-100%), and of Spigelman stage IV 52% (95% CI 28-76%). The probability of an advanced Spigelman score increased during the study period (p<0.0001) due to an increasing number and size of adenomas. Two patients had asymptomatic duodenal carcinoma at their first endoscopy while four developed carcinoma during the study at a median age of 52 years (range 26-58). The cumulative incidence rate of cancer was 4.5% at age 57 years (95% CI 0.1-8.9%) and the risk was higher in patients with Spigelman stage IV at their first endoscopy than in those with stages 0-III (p<0.01). CONCLUSIONS: The natural course of duodenal adenomatosis has now been described in detail. The high incidence and increasing severity of duodenal adenomatosis with age justifies prophylactic examination, and a programme is presented for upper gastrointestinal endoscopic surveillance.  相似文献   

16.
背景: Peutz-Jeghers综合征(PJS)患者的小肠多发息肉可引起出血、肠梗阻、肠套叠、息肉恶变等严重并发症,传统外科手术治疗创伤大、术后并发症多,且多次小肠切除术可致短肠综合征。目前关于小切口开腹术中内镜治疗PJS患者小肠息肉的报道较少。目的: 探讨小切口开腹术中内镜治疗PJS患者小肠多发息肉的疗效和可能优势。方法: 回顾性分析南通市第一人民医院1998年9月~2008年10月7例接受小切口开腹术中内镜治疗的PJS患者的息肉切除情况、手术相关并发症和长期随访结果。结果: 7例PJS患者术中共切除929枚小肠息肉,直径≤10mm 492枚,11~30mm 377枚,≥30mm 60枚,最大者45mm×38mm。术后2例患者分别出现肠功能障碍和少量便血,1例术后1年内腹部偶有隐痛不适,经治疗后均好转,无严重并发症和死亡病例。随访1~8年,无患者出现需考虑外科手术治疗的病情变化。结论: 小切口开腹术中内镜治疗能安全有效地切除小肠多发息肉,可反复治疗而无须切除肠段,对PJS患者具有重要临床应用价值。  相似文献   

17.
AIM: To assess the usefulness of the balloon assisted enteroscopy in preventing surgical intervention in pa-tients with Peutz-Jeghers syndrome (PJS) having a small bowel large polyps. METHODS: Seven consecutive asymptomatic pts(age 15-38 years) with PJS have been collected; six under-went polypectomy using single balloon enteroscopy(Olympus SIF Q180) with antegrade approach using push and pull technique. SBE system consists of the SIF-Q180 enteroscope, an overtube balloon control unit(OBCU Olympus Balloon Control Unit) and a dispos-able silicone splinting tube with balloon(ST-SB1). All procedures were performed under general anesthesia. Previously all pts received wireless capsule endos-copy(WCE). Prophylactic polypectomy was reservedmainly in pts who had polyps 15 mm in diameter. The balloon is inflated and deflated by a balloon control unit with a safety pressure setting range from-6.0 kPa to +5.4 kPa. Informed consent has been obtained from pts or parents for each procedure.RESULTS: Six pts underwent polypectomy of small bowel polyps; in 5 pts a large polyp 15 mm(range 20-50 mm in diameter) was resected; in 1 patient with WCE negative, SBE was performed for previous surgi-cal resection of gastrointestinal stromal tumors. In 2 pts endoscopic clips were placed due to a polypectomy. No surgical complication have been reported. SBE with resection of small bowel large polyps in PJS pts was useful to avoid gastrointestinal bleeding and emergency laparotomy due to intestinal intussuscep-tions. No gastrointestinal tumors were found in sub-sequent enteroscopic surveillance in all seven pts. In order surveillance, all pts received WCE, upper en-doscopy, ileocolonoscopy every 2 years. No pts had extraintestinal malignant lesions. SBE was performed when WCE was positive for significant polyps( 15 mm).CONCLUSION: The effective of prophylactic polyp-ectomy of small bowel large polyps( 15 mm) could be the first line treatment for conservative approach in management of PJS patients.  相似文献   

18.
INTRODUCTION: It is uncertain whether patients with duodenal adenomas without familial adenomatous polyposis (FAP) are at greater risk for small bowel neoplasia. We therefore conducted a study to determine the frequency of small bowel polyps in patients with non-papillary duodenal adenomas using capsule endoscopy for small bowel examination. PATIENTS AND METHODS: 14 patients (8 women, 6 men; mean age 67 +/- 10 years; range: 49 - 77 years) with non-papillary duodenal adenomas without FAP were included. All patients underwent wireless capsule endoscopy. The results were compared with an age- and sex-matched cohort of patients undergoing capsule endoscopy for suspected small bowel disease. RESULTS: Overall, 15 polyps ranging between 1 and 8 mm in diameter were detected in eight patients of the study group, whereas no polyps could be identified in the control group. Natural excretion of the capsule within 24 hours was always reported and no complications were reported by any of the patients. Other pathological findings were multiple angiodysplasias in two patients of the study group. In the control group capsule endoscopy detected angiodysplasias in 5 patients with the indication obscure gastrointestinal bleeding, and inflammatory lesions in 2 patients with suspected Crohn's disease. CONCLUSIONS: Based on the results of this prospective study, the frequency of small bowel polyps in patients with duodenal adenomas without familial adenomatous polyposis appears to be increased compared with a control group undergoing capsule endoscopy for other reasons. In none of the patients was the management altered. Follow-up data of these patients will be needed.  相似文献   

19.
BACKGROUND: Patients with familial adenomatous polyposis are not only at high risk of developing adenomas in the colorectum but a substantial number of patients also develop polyps in the duodenum. Because treatment of duodenal polyps is extremely difficult and it is unknown how many patients ultimately develop duodenal cancer, the value of surveillance of the upper digestive tract is uncertain. AIMS: (1) To assess the cumulative risk of duodenal cancer in a large series of polyposis patients. (2) To develop a decision model to establish whether surveillance would lead to increased life expectancy. METHODS: Risk analysis was performed in 155 Dutch polyposis families including 601 polyposis patients, and 142 Danish families including 376 patients. Observation time was from birth until date of last contact, death, diagnosis of duodenal cancer, or closing date of the study. RESULTS: Seven Dutch and five Danish patients developed duodenal cancer. The lifetime risk of developing this cancer by the age of 70 was 4% (95% confidence interval 1-7%) in the Dutch series and 3% (95% confidence interval 0-6%) in the Danish series. Decision analysis showed that surveillance led to an increase in life expectancy by seven months. CONCLUSIONS: Surveillance of the upper digestive tract led to a moderate gain in life expectancy. Future studies should evaluate whether this increase in life expectancy outweighs the morbidity of endoscopic examination and proximal pancreaticoduodenectomy.  相似文献   

20.
Peutz-Jeghers综合征(Peutz-Jeghers syndrome,PJS)以皮肤黏膜色素斑、胃肠道错构瘤息肉和遗传性为临床特征.PJS胃肠道息肉可产生梗阻、出血、套叠、恶变等严重并发症,目前其临床治疗以手术和内镜治疗为主,其中双气囊电子小肠镜对于PJS胃肠道息肉的诊断和治疗具有重要的临床意义.随着转化医学...  相似文献   

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