Alain Bideau

Epidémiologie et constitution d'un registre de population à propos d'une concentration géographique d'une maladie héréditaire rare

Plauchu Henri y Bideau Alain. — Epidemiologie de la concentracion geografica de una enfermedad hereditaria rara y constitucion de un registre de poblacion. Los autores presentan los resultados de un estudio realizado a partir del hallazgo de una concentracion geografica de una enfermedad hereditaria rara (la genopatia de Rendu- Osier) en los montes de Jura del departamento de Ain. Se trata de un estudio doble : constitucion de un banco de datos en torno a las cuatro aldeas que constituyen el foco de la enfermedad y enseguida el estudio de la difusion espacial del gene a partir de ese foco. La constitucion del registro de enfermedades amplia el enfoque puramente epidemiolo- gico y permite el estudio de numerosos aspectos comunes de los datos demograficos y geneticos recolectados en las cuatro aldeas bajo observacion. El aspecto original del estudio epide- miologico esta caracterizado por la metodologia empleada y especialmente por la encuesta postal que se efectuo para incluir en forma exhaustiva todos los casos pertinentes.

Étude épidémiologique de la maladie de Rendu-Osler en France : répartition géographique et prévalence.

Bideau Alain, Plauchu Henri, Brunet Guy y Robert J.M. — Estudio epidemiologico de la enfennedad de Rendu-Osier en Francia : reparticion geografica y prevalencia. Este estudio sobre la reparticion de la enfermedad de Rendu-Osier se basa en una encuesta postal realizada en 52 provincias francesas enviada a 23 000 medicos. El punto de partida ha sido la concentracion de enfermos observada en la region Rhone-Alpes, exten- diendo progresivamente la encuesta a otras regiones. Debiera cubrirse el territorio nacional en el ano de 1989. Las 3 331 respuestas de los medicos permitieron ubicar 392 de entre ellos en una carta de Francia y sabiendo que cada medico que se proponia representaba en promedio 4 enfermos de una misma familia, pudimos estimar una « prevalencia minima » рог provincia. La media nacional es de 1/8 345, о sea 10 veces mas de lo esperado; a nivel local 3 provincias destacan claramente : Ain (1/3 375), Deux-Sevres (1/4 287), Jura (1/5 062). Estudios complementarios han sido llevados a cabo sobre 7 provincias. Las desigualdades son grandes entre provincias continguas, incluso entre los distritos de una misma provincia. En consecuencia este estudio plantea una interrogacion sobre la difusion de la enfermedad en Francia : i existen diversos focos originales, o el gene patogeno se ha diseminado a partir de un tronco unico ?

Intervals between marriage and first birth in mothers and daughters

Marriage-first birth intervals are examined in two historical populations, Quebec (1608-1765) and Haut-Jura (1689-1980), comparing intervals in mothers and daughters, and in sister-sister pairs. The results point to a weak relationship between intervals of mothers and daughters, though it does not attain significance. Shared environment does not seem to be responsible since there is no association between pairs of sisters from the same populations.

Fécondité et mortalité après 45 ans. L'apport des recherches en démographie histórique.

Bideau Alain. — Fecundidad y mortalidad despues de los 45 aňos de edad. El aporte de los estudios de demografia historka. A traves del presente estudio se intenta comprender los mecanismos y las relaciones que puedan existir entre la fecundidad y la mortalidad despues de los 45 aňos de edad. A partir de resuitados originales y de una coleccion de resultados publicados en el campo de la demografia historica, se ha replanteado el problema de saber si existe o no una tendencia a la prolongacion de la vida en funcion del numero de hijos tenidos. Al termino de este analisis se puede concluir finalmente que la esperanza de vida a los 45 aňos depende poco del numero de hijos tenidos. Incluso parece ser que las mujeres mas fecundas son las que tienen una longevidad mayor que las otras. Para las otras, de 0 a 1 1 hijos, los resultados son variables de un estudio a otro y no es seguro de que el numero de hijos tenidos pueda tener una influencia cualquiera sobre la edad al morir de las mujeres que han alcanzado 45 aňos de edad. Puede decirse con seguridad que la fecundidad no es sino que uno de los componentes de la vitalidad femenina.

La consanguinité, révélateur de la structure de la population. L'exemple de la vallée de la Valserine du XVIIIe siècle à nos jours

Bideau (Alain), Brunet (Guy), Heyer (Evelyne), Plauchu (Henri). - La consanguinidad, reveladora de la estructura de la poblacion. El ejemplo del valle de Valserina desde el siglo XVIII hasta nuestros dias Los demografos-historiadores calculan tasas de matrimonios consanguineos utilizan- do generalmente las declaraciones de exencion incluidas en las actas de matrimonio. La confrontacion de esta fuente con otras (registro del Obispado y corpus genealogico) muestra las imprecisiones de dicha medida. El analisis del corpus genealogico de los cinco municipios del valle de Valserina, desde finales del siglo XVII hasta nuestros dias, permite establecer el coeficiente medio de consanguinidad de la poblacion. Las genealogias posibilitan ademas la inclusion de la consanguinidad lejana (mas alla de la cuarta generacion). De este modo se percibe la existencia de sub-poblaciones con comportamientos especificos en lo referente a eleccion del conyuge y a movilidad. La consanguinidad es una caracteristica propia de un grupo restringido de fa- milias presentes en estos mismos municipios a lo largo de los tres siglos estudiados.

Surnames: History of the family and history of populations

A meeting on surnames was recently held in Lyons, gathering searchers from different scientific horizons such as sociology, history, anthropology, demography and population genetics. We selected the six following papers because of their major interest to history of the family and history of populations. Surnames appear as an important and convenient tool for the study of marriage patterns or of geographical mobility. We also provide some general comments about the different uses of surnames in the current works in the field of historical demography and population genetics. We also mention some examples revealing the social significance of surnames in some specific historical context.A meeting on surnames was recently held in Lyons, gathering searchers from different scientific horizons such as sociology, history, anthropology, demography and population genetics. We selected the six following papers because of their major interest to history of the family and history of populations. Surnames appear as an important and convenient tool for the study of marriage patterns or of geographical mobility. We also provide some general comments about the different uses of surnames in the current works in the field of historical demography and population genetics. We also mention some examples revealing the social significance of surnames in some specific historical context.

Migration and the life course: mobility in Haut Bugey (France) during the nineteenth and twentieth centuries.

In order to study geographical mobility at the end of the nineteenth century and the beginning of the twentieth century, we construct the biography of two cohorts of individuals born in the villages of Chezery and Forens during the periods 1871–1880 and 1901–1910. We seek to define the boundaries of emigration from that area and look for successive migrations. Movers are identified in the census schedules of a set of 580 communes surrounding the place of birth. Migration is studied according to sex, age, familial history and individual activity; and the features of movers and stayers are compared.

Trente ans d'étude de la maladie de Rendu-Osler en France: démographie historique, génétique des populations et biologie moléculaire

Rendu-Osler-Weber disease, also known as hereditary hemorrhagic telangiectasia (HHT), has been the focus of several interdisciplinary studies over the last thirty years. An initial epidemiological survey conducted in the 1980s revealed that the prevalence of this hereditary genetic disorder in France is much higher than previously thought, and brought to light several geographical clusters of HHT carriers. The subsequent analysis of the family genealogies of carriers in the main cluster and of the demographic history of the region did not confirm the thesis of a unique founder effect at national level, but provided clues for dating the occurrence of a local mutation. Last, thanks to the recent identification of the genes responsible for HHT, the considerable genetic heterogeneity of the disease has been confirmed and a large number of different mutations existing in France have been identified. Nonetheless, many carriers living in the main geographical cluster studied previously share a single mutation associated with a specific haplotype. At the end of this remarkable study, marked by its multi-disciplinary approach and its exceptional duration, the various converging strands confirm the existence of a local founder effect and provide consistent evidence for dating this mutation.

How German Lutherans became Brazilians: A methodological essay

The article reviews earlier collaborative research about German immigrants to Brazil by using data from family genealogies (the reconstruction of family histories from parish register data). These data permit the preliminary testing of a methodology to establish indicators of the groups ethnic identity. There is need to understand the groups criteria for determining and receiving members and for making inclusion and exclusion apparent in the group. The choice of baptismal names is analyzed, and these choices are considered against the background of the changes the population underwent as it continued to adapt to new social and economic circumstances. The analysis needs to be more complex and continuous cultural uniformity cannot be assumed; each cohort of the population has to be better defined as a relatively distinct cultural unit.

Orphans and their family histories: A Study of the Valserine Valley (France) during the Nineteenth and Twentieth Centuries

In this article we propose two approaches for determining the number of orphaned children in a population and for studying their life course. The first approach is based on vital registration records, the second on census data. At the end of the nineteenth century some 10 percent of children became orphans before their 10th birthday, and 13 percent before their 13th. At the beginning of the twentieth century the surviving parent normally took responsibility for the custody and guardianship of the children. When this option was not available, close relatives could either provide a home for some of the orphaned children in their own households, or join the household of the surviving parent to provide care for the children. Placing the orphans in domestic service was rare. All such arrangements tended to be temporary but, in general, orphans were cared for either by members of their family or by neighbors.