We report the results of a comprehensive and systematic clinical study of 324 patients with hereditary hemorrhagic telangiectasia, selected from a total of 1,270 cases recruited by epidemiological survey. In 94% of the cases, familial occurrence suggested autosomal dominant inheritance; maximum penetrance for at least one manifestation was 97%. Epistaxis was reported by 96% of the patients and, in more than 50%, developed before age 20. Heavy and frequent bleeding occurred mainly in middle-aged patients. Telangiectasia was documented in 74% of cases, half of whom were younger than 30 years. The frequency of involvement of the hands and wrists was 41%, and for the face, 33%. Visceral involvement was present in 25% of patients, with affected lungs and CNS in the young and gastrointestinal tract and liver in older patients. Symptomatic urinary tract involvement was seen in only two/324 patients. Involvement of other internal sites was not observed.

An important concentration of patients with Rendu-Osler disease occurs in the Valserine valley of the French Jura. A study of marriages shows that, in spite of its somewhat remote location, the valley cannot be considered an isolate, but that, on the contrary, exogamy is widely practised. Only 17.8% of the genes of inhabitants during the present century can be traced to the original population, but persons affected with the disease belong to a subgroup of the population which has lived in the villages for more than 10 generations. All the patients in 85 sibships are related. The smallest number of originator couples who lived at the beginning of the eighteenth century amounts to 16, the unique originator may therefore have lived approximately four generations earlier. However, as other patients who lived at the periphery of the Valserine valley do not appear to be related to any patients living in the valley, and because there has been considerable immigration into the valley, a number of hypotheses to explain the distribution of the disease in the region remain possible.

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SummaryMarriage-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.

Associé No. 223, Université de Lyon II. This article is based on "La population de la chatêllenie de Thoissey-en-Dombes, XVII e -XIX e siècles. Etude démographique, sociale et médicale" (thesis to be deremarriage and its timing, observing the distribution of remarriage according to the length of time elapsed since the death of the previous spouse.Naturally, remarriage is a predominantly sociological phenomenon, dependent on economic, social, and cultural imperatives. For this reason, parallel to the analysis of demographic characteristics, we have tried to measure the variations in the probability of remarriage based on the number of dependent children at the time of widowing and of remarriage.All historians are familiar with the considerable difficulties posed by the study of widowhood and remarriage. In principle, family reconstitution permits the study of the frequency of remarriage according to age at widowing and the interval elapsing before remarriage. But, in practice, serious difficulties arise because a number of people widowed in one parish remarry outside it. On the parish level, this problem must not be ignored, because the loss of remarriages can be considerable. ' Nevertheless, the study of the mechanism of mobility and of human relations between the small town of T'hoissey2 and the 1 For researchers working on one isolated parish, L. Henry outlined a methodology permitting the evaluation of lost remarriages (see the forthcoming Portuguese edition of the Manuel de démographie historique.2 Thoissey, second city of the principality of Dombes, is a city of very small area (134 hectares), situated one kilometer from the Saône and on the Chalaronne River, 30 kilometers north of Trévoux, 15 south of Mâcon, and 34 west of Bourg-en-Bresse.

This study uses sets of historical family reconstitutions from all of Quebec and from four villages of the Haut-Jura, France-first marriages of 2226 and 994 women, respectively-to investigate the physiological and social factors affecting age of mother at last birth before and during fertility transition. Age remained high throughout the period covered in Quebec, under 'natural' conditions, but showed a steady decline in the French material which extends to late 19th century generations practising family limitation.Age at marriage had no influence in Quebec; in France, however, women with the most surviving children at age 35 continued childbearing the latest. There was no link between biological ability to achieve a live birth, or in health status or aging rhythm, and age at last birth. Behaviour of mothers and daughters showed no relation. The variability in age at last birth thus appears to be random under natural conditions; with the onset of controls, social differences seem to influence not only the end of childbearing, but all aspects of behaviour governing final family size and child survival.

After thirty years of research into Rendu-Osler-Weber disease, the authors review the contributions of the three successive approaches used to analyse this rare genetic disorder. First, historical demography sees patients as markers of past migration and population transfers. Starting out from a cluster of cases straddling the Ain and Jura départements, the history of this population group was reconstituted on the basis of civil records, and their migration to other parts of France was studied by looking for family ties between the initial group and the cases observed elsewhere in the country. Second, epidemiology and population genetics give estimates of prevalence by département that are higher than those initially predicted in 1977, the start year of this study. They also provide statistical tools to test hypotheses of a single or multiple origin for the disease by looking for a common ancestor among affected families. Last, molecular biology provides a means to identify the genes responsible for the disease and their various mutations, thus confirming the existence of several different origins.