Mortality associated with neurofibromatosis type 1: A study based on Italian death certificates (1995-2006)

<p>Abstract</p> <p>Background</p> <p>Persons affected by neurofibromatosis type 1 (NF1) have a decreased survival, yet information on NF1-associated mortality is limited.</p> <p>Methods/Aim</p> <p>The National Mortality Database and individual Multiple-Causes-of-Death records were used to estimate NF1-associated mortality in Italy in the period 1995-2006, to compare the distribution of age at death (as a proxy of survival) to that of the general population and to evaluate the relation between NF1 and other medical conditions by determining whether the distribution of underlying causes of NF1-associated deaths differs from that of general population.</p> <p>Results</p> <p>Of the nearly 6.75 million deaths in the study period, 632 had a diagnosis of NF1, yet for nearly three-fourths of them the underlying cause was not coded as neurofibromatosis. The age distribution showed that NF1-associated deaths also occurred among the elderly, though mortality in early ages was high. The mean age for NF1-associated death was approximately 20 years lower than that for the general population. The gender differential may suggest that women are affected by more severe NF1-related complications, or they may simply reflect a greater tendency for NF1 to be reported on the death certificates of young women. Regarding the relation with other medical conditions, we found an excess, as the underlying cause of death, for malignant neoplasm of connective and other soft tissue and brain, but not for other sites. We also found an excess for obstructive chronic bronchitis and musculoskeletal system diseases among elderly persons.</p> <p>Conclusion</p> <p>This is the first nationally representative population-based study on NF1-associated mortality in Italy. It stresses the importance of the Multiple-Causes-of-Death Database in providing a more complete picture of mortality for conditions that are frequently not recorded as the underlying cause of death, or to study complex chronic diseases or diseases that have no specific International Classification of Diseases code, such as NF1. It also highlights the usefulness of already available data when a surveillance system is not fully operational.</p

Review of Health Examination Surveys in Europe

Information on the health, health risks and use of health services and medicines of the population are needed for planning and evaluating health policies and health care. Typical data sources for health information are various registers, such as mortality registers for total and cause-specific mortality and population surveys, which provide a cross-section of health and its determinants in the population. Different data sources provide information on different aspects of health, and therefore they are largely complementary

Merkel cell carcinoma: a population-based study on mortality and the association with other cancers

Few population-based epidemiological data are available on Merkel cell carcinoma (MCC), a rare lethal non-melanoma skin cancer. We analysed multiple-cause-of-death records to describe MCC mortality and trends and the association with other primary cancers. We reviewed all 6,713,059 death certificates in Italy (1995-2006) to identify those mentioning MCC. We evaluated the association with other primary cancers by calculating the ratio of observed to expected deaths, using a standardized mortality ratio (SMR)-like analysis. We also evaluated the geographic distribution of deaths. We identified 351 death certificates with the mention of MCC. The age-adjusted mortality was 0.031/100,000, with a significant trend of increase and a slight north-south gradient. There was a significant deficit for solid cancers (SMR = 0.15) and a non-significant excess for lymphohematopoietic malignancies (SMR = 1.62). There were significant excesses for chronic lymphocytic leukemia (SMR = 4.07) and Waldenstrom's macroglobulinemia (SMR = 27.2) and a non-significant excess for chronic myeloid leukemia (SMR = 5.12). The increase in MCC mortality reflects the incidence trend in the literature. The association with chronic lymphocytic leukemia confirms the importance of immunologic factors in MCC. Regarding Waldenstrom's macroglobulinemia, an association with MCC has never been reported

Guidelines to Implement or Improve Syndromic Surveillance Systems

The Triple-S project (Syndromic Surveillance Survey, Assessment towards Guidelines for Europe) is a 3-year European project, which main objective is to produce guidelines for implementing or improving a syndromic surveillance (SyS) system in animal and human health. The major expectation was the guidelines to be user-friendly, practical and based on concrete examples of existing systems in Europe

Guidelines to Implement or Improve Syndromic Surveillance Systems

The Triple-S project (Syndromic Surveillance Survey, Assessment towards Guidelines for Europe) is a 3-year European project, which main objective is to produce guidelines for implementing or improving a syndromic surveillance (SyS) system in animal and human health. The major expectation was the guidelines to be user-friendly, practical and based on concrete examples of existing systems in Europe