ABSTRACT
Familial adenomatous polyposis (FAP) is a well‐described inherited syndrome, characterized by the development of hundreds to thousands of adenomas in the colorectum, with implications in ...children and adolescents. Almost all adult patients will develop colorectal cancer if they are not identified and treated early enough. Identifying and screening for FAP commences in adolescence. The syndrome is inherited as an autosomal dominant trait and caused by mutations in the adenomatous polyposis (APC) gene. This European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) position paper provides a guide for diagnosis, assessment, and management of FAP in children and adolescents.
This is the first position paper regarding FAP published by ESPGHAN. Literature from PubMed, Medline, and Embase was reviewed and in the absence of evidence, recommendations reflect the opinion of paediatric and adult experts involved in the care of polyposis syndromes. Because many of the studies that form the basis for the recommendations were descriptive and/or retrospective in nature, these of the recommendations are supported on expert opinion. This position paper will instruct on the appropriate management and timing of procedures in children and adolescents with FAP.
ABSTRACTFamilial adenomatous polyposis (FAP) is a well-described inherited syndrome, characterised by the development of hundreds to thousands of adenomas in the colorectum, with implications in ...children and adolescents. Almost all adult patients will develop colorectal cancer (CRC) if they are not identified and treated early enough. Identifying and screening for FAP commences in adolescence. The syndrome is inherited as an autosomal dominant trait and caused by mutations in the adenomatous polyposis (APC) gene. This European Society for Paediatric Gastroenterology, Hepatology and Nutrition (EPGHAN) position paper provides a guide for diagnosis, assessment and management of familial adenomatous polyposis in children and adolescents.This is the first position paper regarding FAP published by ESPGHAN. Literature from PubMed, Medline and Embase was reviewed and in the absence of evidence, recommendations reflect the opinion of paediatric and adult experts involved in the care of polyposis syndromes. Because many of the studies that form the basis for the recommendations were descriptive and/or retrospective in nature, these of the recommendations are supported on expert opinion. This position paper will instruct on the appropriate management and timing of procedures in children and adolescents with FAP.
Community-level policy, systems, and environmental (PSE) change strategies may offer an economical and sustainable approach to chronic disease prevention. The rapidly growing number of untested but ...promising PSE strategies currently underway offers an exciting opportunity to establish practice-based evidence for this approach. This article presents lessons learned from an evaluation of a community-based PSE initiative targeting stroke and cardiovascular disease prevention in the Mississippi Delta. Its purpose is to describe one approach to evaluating this type of PSE initiative, to stimulate discussion about best practices for evaluating PSE strategies, and to inform future evaluation and research efforts to expand practicebased evidence. The evaluation used a descriptive mixed-methods design and focused on the second year of a multisectoral, multiyear initiative. Cross-sectional data were collected in the summer and fall of 2010 using four data collection instruments: a grantee interview guide (n = 32), a health council member survey (n = 256), an organizational survey (n = 60), and a grantee progress report (n = 26). Fifty-eight PSE changes were assessed across five sectors: health, faith, education, worksite, and community/city government. PSE strategies aligned with increased access to physical activity opportunities, healthy food and beverage options, quality health care, and reduced exposure to tobacco. Results showed that grantees were successful in completing a series of steps toward PSE change and that sector-specific initiatives resulted in a range of PSE changes that were completed or in progress. Considerations for designing evaluations of community-based PSE initiatives are discussed.
Community-level strategic planning for chronic disease prevention.
To share the outcomes of the strategic planning process used by Mississippi Delta stakeholders to prevent and reduce the negative ...impacts of chronic disease in their communities. A key component of strategic planning was participants' use of the Prevention Impacts Simulation Model (PRISM) to project the reduction, compared with the status quo, in deaths and costs from implementing interventions in Mississippi Delta communities.
Participants in Mississippi Delta strategic planning meetings used PRISM, a user-friendly, evidence-based simulation tool that includes 22 categories of policy, systems, and environmental change interventions, to pose what-if questions that explore the likely short- and long-term effects of an intervention or any desired combination of the 22 categories of chronic disease intervention programs and policies captured in PRISM. These categories address smoking, air pollution, poor nutrition, and lack of physical activity. Strategic planning participants used PRISM outputs to inform their decisions and actions to implement interventions.
Rural communities in the Mississippi Delta.
A diverse group of 29 to 34 local chronic disease prevention stakeholders, known as the Mississippi Delta Strategic Alliance.
Community plans and actions that were developed and implemented as a result of local strategic planning.
Existing strategic planning efforts were complemented by the use of PRISM. The Mississippi Delta Strategic Alliance decided to implement new interventions to improve air quality and transportation and to expand existing interventions to reduce tobacco use and increase access to healthy foods. They also collaborated with the Department of Transportation to raise awareness and use of the current transportation network.
The Mississippi Delta Strategic Alliance strategic planning process was complemented by the use of PRISM as a tool for strategic planning, which led to the implementation of new and strengthened chronic disease prevention interventions and policies in the Mississippi Delta.
The undisputed optimum treatment for venous leg ulcers is compression therapy, where an external appliance (multilayer, short-stretch bandaging and compression hosiery) promotes venous return through ...graduated compression of the tissues and capillaries. However, this is not always acceptable to the patient, particularly when the patient’s social life is affected with malodour and pain associated with venous leg ulcers, thereby reducing quality of life. There is a psychosocial consideration when the bandaging system is bulky, hot and difficulty is found with the fitting of shoes. An alternative and clinically effective solution is essential if the wound is to heal. In this case study, the patient was provided with a cohesive and short-stretch compression bandage (Actico), which allowed her to continue her social life while effectively treating her ulcer.
Cytotoxic T lymphocyte antigen 4 (CTLA4), a key negative regulator of T-cell activation, is targeted by the antibody tremelimumab to release potentially useful antitumor activity.
This phase II trial ...investigated tremelimumab as a second-line treatment for patients with metastatic gastric and esophageal adenocarcinomas. Tremelimumab was given every 3 months until symptomatic disease progression. Safety, clinical efficacy, and immunologic activity were evaluated.
Eighteen patients received tremelimumab. Most drug-related toxicity was mild; however, there was a single death due to bowel perforation that complicated colitis. Four patients had stable disease with clinical benefit; one patient achieved a partial response after eight cycles (25.4 months) and remains well on study at 32.7 months. Markers of regulatory phenotype, forkhead box protein 3 and CTLA4, doubled transiently in CD4(+)CD25(high) lymphocytes in the first month after tremelimumab before returning to baseline. In contrast, CTLA4 increased in CD4(+)CD25(low/negative) lymphocytes throughout the cycle of treatment. De novo proliferative responses to tumor-associated antigens 5T4 (8 of 18 patients) and carcinoembryonic antigen (5 of 13) were detected. Patients with a posttreatment carcinoembryonic antigen proliferative response had median survival of 17.1 months compared with 4.7 months for nonresponders (P = 0.004). Baseline interleukin-2 release after T-cell activation was higher in patients with clinical benefit and toxicity.
Despite the disappointing response rate of tremelimumab, one patient had a remarkably durable benefit for this poor-prognosis disease. In vitro evidence of enhanced proliferative responses to relevant tumor-associated antigens suggests that combining CTLA4 blockade with antigen-targeted therapy may warrant further investigation.
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