[Prevalencia de la Telangiectasia Hemorrágica Hereditaria: estudio de base poblacional en la Comunitat Valenciana (España).].

Objective: The Hereditary Haemorrhagic Telangiectasia (HHT) is a low prevalence disease which presents heterogeneous signs and symptoms and just few population-based epidemiological studies are available. The aims of this paper were to describe the sociodemographic characteristics of people affected by HHT in the Valencian Region (VR), to determine its prevalence and mortality rate, and to analyse the sources of recruitment and verification tests used by the Rare Diseases Information System of the VR (SIER-CV).

Methods: Cross-sectional observational epidemiological study of HHT prevalent cases between 2010-2019 in SIER-CV was performed.

[Updating of the official statistics of deaths causes: "Let's get stronger after the COVID-19 pandemic".].

The National Spanish Institute of Statistics (INE) website is the main means of communication for official statistics in Spain. The INE publishes the annual statistics of deaths according to the cause of death. This vital statistic has improved a lot in recent years; also, thanks to the collaboration from the Autonomous Communities.

[Epidemiological surveillance of congenital heart defects: the Tetralogy of Fallot in the Valencian Region, 2007-2017.].

Objective: Tetralogy of Fallot is characterized by the presence of four congenital heart defects. Objective: to describe the temporal trend and distribution of Tetralogy of Fallot, in children under one year in the Valencian Region.

Methods: Cases with Tetralogy of Fallot (code Q21.

[Do women in the Valencian Community accept self-sampling as a form of cervical cancer screening?].

Objective: The use of new devices such as the self-sampling for the determination of the Human Papilloma Virus (HPV), could facilitate the implementation of population screening programs and increase the participation of women. The objective of the study was to find out the women´s knowledge and attitudes towards cervical cancer screening as well as the acceptability of self-sampling.

Methods: Analytical cross-sectional study by telephone survey carried out on a random sample of 389 women from 35 to 65 years of age.

[Wilson disease: The look of medical staff, patients and family members].

Objective: Wilson disease (WD) is a rare congenital disease that causes hepatic, neurological or lenticular degeneration due to the accumulation of copper. Sometimes it is incapacitating with implications in the quality of life of those affected and their families. The objective of this work was to identify the needs of medical staff and the social and emotional needs of patients with WD and their families.

[Perinatal mortality assessment in native and immigrant women: influence of exhaustiveness and quality of the registries].

Objective: To analyze the exhaustiveness and reliability of the data on perinatal mortality in two Spanish registries, namely, the National Statistics Institute and the Perinatal Mortality Registry of the Valencian Community and to calculate and compare the perinatal mortality rate (PMR) and its components in native and immigrant women, based on the cases reported to both registries in 2005 and 2006.

Methods: Perinatal mortality and its components were defined according to the World Health Organization's criteria. The magnitude of underreporting was calculated by taking into account the frequencies and percentages of deaths not declared for 2005-2006.

[Guide to the principles and methods of health sentinel networks in Spain].

Health sentinel networks are being increasingly used in the study of health-related problems. The present article aims to provide a methodological guide - designed by regional sentinel network managers and based on the results of a Delphi study - that can be used to set up and develop a health sentinel network. The main topics in the guide are the following: definition of a health sentinel network; network description: aims and structure; methods for the selection of sentinel participants; description of health processes suitable for study through this methodology: incidence, case definition, exclusion and inclusion criteria; description of the target population: the denominator for incidence rates estimates; quality indicators; periodicity of data collection; dissemination of the information: periodicity and methods, and incorporation of the participants in the decision-making process through multidisciplinary commissions.

[Health sentinel networks in Spain. Consensus for a guide of principles and methods].

Since the eighties, health sentinel networks have been active in our country, but with a variety of objectives, methods and different development levels in the autonomous communities. The necessity of standardising the management and harmonising the indicators has concluded in a research project on the Spanish health sentinel networks, one of whose objectives is to establish a guide for work principles and methods. A panel of 23 experts has made a study using the Delphi method to agree, in three consecutive phases, a definition of sentinel network, the objectives and the management principles and other aspects related to the organisation and functioning.

[Disease registries in the epidemiological researching of rare diseases in Spain].

Background: The use of tools such as disease registries poses a problem in the case of rare diseases. This study is aimed at describing the current situation concerning rare disease registries in Spain.

Methods: The information provided by two Spanish health registries directories prepared by the Spanish Health Assessment Technologies Agencies are employed, a descriptive cross-sectional study being conducted.