Cancer risk among patients with cystic fibrosis and their first-degree relatives

Patients with cystic fibrosis (CF) are at increased risk of some cancers. Little is known about the cancer risks among carriers heterozygous for the CF mutation and it is hypothesized this may be associated with reduced cancer risk. Using Swedish general population-based registers, we identified 884 patients with CF from 1968 to 2003 and 3,033 of their first-degree relatives The subjects were followed from birth of index persons or 1958, whichever came later, until death, emigration or 2003, whichever came first. Cancer risks were compared with the general Swedish population using standardized incidence ratios (SIR) with 95% confidence intervals (CI). Patients, followed for an average of 21 years, were at a higher overall risk of cancer. Some 26 cancer diagnoses, after excluding multiple diagnoses of nonmelanoma skin cancer in one man, produced an overall SIR of 3.2 (95%CI 2.1-4.6).We found statistically significantly increased risks for kidney, thyroid, endocrine, lymphoma and nonmelanoma skin cancer. There was no modification of cancer risk among parents and siblings, with an average of 21 years of follow-up. This study did not identify a heterozygote advantage for CF gene mutations in relation to cancer risk. © 2009 UICC

Cystic fibrosis gene mutations and gastrointestinal diseases

Background: This study examined if CF mutation heterozygosity is associated with diseases of gastrointestinal epithelial barrier function. Design and methods: Swedish registers identified 865 patients with a diagnosis of CF between 1968 and 2003 and matched with 8101 individuals without CF. Gastrointestinal disease risk was examined among 1534 biological parents and 1396 siblings of CF patients, compared with 15,526 parents and 15,542 siblings of individuals without CF. Results: First-degree relatives of CF patients were not at lower risk of the gastrointestinal diseases, in contrast with a raised risk among CF patients. Conclusion: Heterozygosity for CF gene mutations does not protect against gastrointestinal diseases where impaired barrier function may be relevant. © 2010 European Cystic Fibrosis Society

Prevalence of paediatric inflammatory bowel disease in Sweden: a nationwide population-based register study

Summary of ICD codes used for ulcerative colitis and Crohn’s disease. (PDF 36 kb

Biologics registers in RA: methodological aspects, current role and future applications

The beginning of the 21st century saw a biopharmaceutical revolution in the treatment of inflammatory rheumatic diseases, particularly rheumatoid arthritis. The fast-evolving use of biologic therapies highlighted the need to develop registers at national and international levels with the aim of collecting long-term data on patient outcomes. Over the past 15 years, many biologics registers have contributed a wealth of data and provided robust and reliable evidence on the use, effectiveness and safety of these therapies. The unavoidable challenges posed by the continuous introduction of new therapies, particularly with regard to understanding their long-term safety, highlights the importance of learning from experience with established biologic therapies. In this Perspectives article, the role of biologics registers in bridging the evidence gap between efficacy in clinical trials and real-world effectiveness is discussed, with a focus on methodological aspects of registers, their unique features and challenges and their role going forward

Static stretching of the hamstring muscle for injury prevention in football codes: a systematic review

Purpose: Hamstring injuries are common among football players. There is still disagreement regarding prevention. The aim of this review is to determine whether static stretching reduces hamstring injuries in football codes. Methods: A systematic literature search was conducted on the online databases PubMed, PEDro, Cochrane, Web of Science, Bisp and Clinical Trial register. Study results were presented descriptively and the quality of the studies assessed were based on Cochrane’s ‘risk of bias’ tool. Results: The review identified 35 studies, including four analysis studies. These studies show deficiencies in the quality of study designs. Conclusion: The study protocols are varied in terms of the length of intervention and follow-up. No RCT studies are available, however, RCT studies should be conducted in the near future

Necrotising fasciitis of the shoulder in association with rheumatoid arthritis treated with etanercept: a case report

<p>Abstract</p> <p>Introduction</p> <p>Necrotising fasciitis is a severe infection characterised by the fulminant destruction of tissue with associated systemic signs of sepsis and toxicity. Etanercept is a fully human fusion protein that inhibits tumor necrosis factor and the inflammatory cascade. It is effective in the treatment of many disorders but concerns regarding severe life threatening infections have been raised in multiple reports.</p> <p>Case presentation</p> <p>We present the case of a 39-year-old Caucasian man, who presented with sudden onset of severe and progressive neck and left shoulder pain, with a two-year history of seronegative rheumatoid arthritis treated with azathoprine and etanercept. On examination the left side of his neck and his left shoulder were oedematous, tender with an erythematous rash and his active range of movement was limited. Magnetic resonance imaging of his shoulder showed extensive oedema of the subcutaneous and intramuscular fat of the left lower neck consistent with fasciitis. He was treated medically and made a good recovery.</p> <p>Conclusion</p> <p>Our patient, while having a pre-existing increased mortality risk, had a serious infection which responded well to optimum medical treatment without the need for surgery. As anti tumor necrosis factor agents are frequently associated with infection, including tuberculous infection, this case highlights the need for a high index of suspicion for other severe bacterial infections in patients on immunosuppressants.</p