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At least 19 recordsLinked to original sources
PubMed1989

Consanguinity in the Archbishopric of Toledo, Spain, 1900-79. I. Types of consanguineous mating in relation to premarital migration and its effects on inbreeding levels.

This study, the first of a series on consanguinity in the Archbishopric of Toledo from 1900 to 1979, analyses in detail the types of consanguineous mating in a population of considerable size, some 650,000 persons, in Central Spain. During the period covered, very important transformations occurred in Spanish society. The data relate to 21,464 consanguineous marriages. They were divided into two groups, according to the premarital migration of the couple. Significant differences in the frequencies of the types of consanguineous matings, and their secular evolution, are found for the two groups. The expected trend in inbreeding in the total area shows two different tendencies: constancy till the end of the 1950s, followed by a fast decline during the last two decades.

Catholicism↗
PubMed1983

Consanguinity studies in Wisconsin I: secular trends in consanguineous marriage, 1843-1981.

Over 920,000 Roman Catholic marriages have taken place since the Archdiocese of Milwaukee was established in 1843. Most of these records are extant, and all have been examined to ascertain consanguineous marriages. The changing average population coefficient of consanguinity has been calculated, by year and by decade, showing a clear downward trend since the turn of the century. The data are compared with reports of consanguineous marriage incidence from around the world, and in particular with all available previous reports from the United States.

Catholicism↗
PubMed2002

Breast cancer, consanguinity, and lethal tumor genes: simulation of BRCA1/2 prevalence over 40 generations.

Marriage between biological relatives is a social custom with a long history in many parts of the world. Today, hundreds of millions of individuals live in consanguineous families. The offspring of consanguineous parents are more likely to have the same two alleles (homozygosity) by descent. In consanguineous family with BRCA1/2 gene mutations, an offspring is more likely to be BRCA1/2 homozygous. The consequences of BRCA1/2 mutation homozygosity in humans are unknown. In knockout mice, BRCA1 or BRCA2 homozygotes die as embryos. Because tumor suppressor genes are conserved and less species-specific than other genes, human BRCA1/2 homozygotes are likely to be biologically non-viable and are unknown to exist. Among the conceptuses of consanguineous couples, there are excess deaths (abortions, stillbirths, perinatal and early-childhood deaths) as well as a decreased risk of breast cancer, especially in younger females. It has been suggested that, in part, the excess deaths are due to BRCA1/2 and other still undiscovered tumor gene homozygotes. To examine the consequences of the long-term practice of consanguineous marriage on the prevalence of lethal cancer genes, we simulated, by computer, the mating of non-consanguineous and consanguineous populations over 40 generations. The program was developed in Basic for a Macintosh computer. The input comprised the rates of consanguineous marriage types and the output parameter was the rate of heterozygotes (carriers) in each generation. The combined prevalence of BRCA1/2 mutation of 1% was used as a starting reference point. Absence of spontaneous mutations and gene flow were assumed. In a randomly mating population, the BRCA1/2 carrier rate decreases on average 0.0035% every 25 years. In a highly consanguineous population, the carrier rate decreases on average 0.022% every 25 years, or six times faster than in a non-consanguineous population. There is a worldwide trend of decreasing breast cancer incidence with an increasing consanguinity rate. In conclusion, the BRCA1/2 and possibly other undiscovered tumor gene carrier rates are significantly lower in consanguineous than in non-consanguineous populations. Gene frequency in a population depends on the rate of inbreeding and length of consanguineous practices. A drift phenomenon may exert a major effect on the carrier rate. Consanguinity may explain part of the worldwide variation of breast cancer incidence.

Breast Neoplasms↗
PubMed2001

Consanguinity decreases risk of breast cancer--cervical cancer unaffected.

Marriages between third-degree and more distant relatives are common in many parts of the world. Offspring of consanguineous parents have increased morbidity and mortality related to recessive gene disorders. In a population with a high frequency of consanguinity, we examined the frequency of breast cancer (related in part to tumour genes) and cervical cancers (related to virus infection) among offspring of consanguineous and non-consanguineous parents. Study was done prospectively in the United Arab Emirates. Selected were married female citizens, ages 40-65, who attended 12 primary health care clinics for whatever reason. In a face-to-face interview, subjects were asked: (a) about consanguineous marriages in family; (b) if they have or have had breast or cervical cancer; (c) about family history of cancer, cancer screening and other parameters. Tumour diagnosis was confirmed by review of medical records. Of 1750 women invited into study, 1445 (79%) could be used in analysis. Among 579 (40%) women of consanguineous and 866 (60%) of non-consanguineous parents there were 24 and 54 with breast cancer, respectively (RR = 0.66, CI 0.42 - 1.06). In the 40 to 50 age group, breast cancer reported 13 of 446 women of consanguineous and 37 of 633 of non-consanguineous parents (RR = 0.50, Cl 0.27 - 0.93). Cervical cancer had 15 women in consanguineous and 32 in non-consanguineous group (RR = 0.70, Cl 0.38 - 1.28). Number of families with history of breast cancer in consanguineous and non-consanguineous group was 21 and 23, respectively (P = 0.29). The cancer screening rates and other variable values had fairly balanced distribution between the 2 groups. Having consanguineous parents decreases the risk of breast cancer especially in younger women, risk of cervical cancer being unaffected.

Adult↗
PubMed1997

A study of possible deleterious effects of consanguinity.

The aim of the study was to determine whether consanguineous marriages result in reproductive wastage and an increased incidence of illness in the offspring in a community with a long history of inbreeding and an expected high rate of consanguineous marriage. A representative sample of 2200 women aged > or = 15 years from Dubai and Al Ain, two cities in the United Arab Emirates, representing on the one hand a modern metropolis and on the other a traditional society, were studied. A questionnaire, which included questions on age, parity, gravidity, number of stillbirths, number of abortions, number of children alive, neonatal deaths and specific illnesses in children, was administered by nurses in antenatal and gynaecological clinics in the two cities. The rate of consanguineous marriage was 50.5% and parity, gravidity, ages and number of children were similar in consanguineous and non-consanguineous groups. There was no significant difference in rates of abortion, stillbirth and neonatal death between the two groups. Overall, there was statistically significant higher reproductive wastage in consanguineous couples, but when the category of less than second cousins was excluded from the consanguineous group no difference was found in reproductive wastage between consanguineous and non-consanguineous marriages. Children born to consanguineous unions also had significantly higher incidences of illnesses (37.1%) than those of non-consanguineous unions (29%). The occurrence of malignancies, congenital abnormalities, mental retardation and physical handicap was significantly higher in offspring of consanguineous than non-consanguineous marriages. In conclusion, consanguinity did not result in reproductive wastage, but was found to be an important factor in the causation of specific illnesses in offspring.

Abortion, Spontaneous↗
PubMed2003

Consanguinity-associated kidney diseases in Lebanon: an epidemiological study.

Consanguineous marriages are common in many countries of the Middle East including Lebanon. Their impact on the repartition of kidney diseases and on the risk for familial nephritis is not known. We surveyed all of the dialysis centers in Lebanon. Nine hundred and twenty-five (925) patients and their private physicians were asked to answer a questionnaire. More than half of the hemodialysis (HD) patients had an unknown etiology of their kidney disease. Diabetes, polycystic kidney disease (PKD), chronic pyelonephritis and nephrosclerosis (NS) were the most commonly documented diagnoses. Consanguinity was present in 26% of the total HD population. More consanguineous patients with unknown renal etiology were diagnosed with their kidney diseases and initiated on dialysis before the age of 30 when compared with their non-consanguineous counter-parts (45% versus 33%, P<0.02 and 42% versus 27%, P<0.01), respectively. Similarly, consanguineous polycystic patients were diagnosed and started earlier on dialysis when compared with the non-consanguineous population (34% versus 12%, P<0.05 and 28% versus 8%, P<0.05), respectively suggesting a different disease pattern. Furthermore, the risk for family history of kidney disease was noticeable in the non-consanguineous population and significantly higher among the consanguineous patients (12% versus 18%, P<0.04). Consanguinity-associated kidney diseases affected all religious communities, in particular the Muslim and the Druze (36 and 39%), respectively versus 17% of the Christian community. Certain geographical areas were more involved than others such as the North, South and the Bekaa with the highest percentage (40%) in the latter. Socio-economical level was not a contributing factor. We conclude that the documentation of the underlying etiology in end-stage renal diseases (ESRD) seems to be deficient. Furthermore, consanguinity is prevalent in the Lebanese dialysis patients population, in particular the Muslim and the Druze communities. Consanguinity-associated kidney diseases pattern seems to differ from that of the general HD population by disease diagnosis and initiation at a younger age and a significantly higher risk for familial renal disease. It is a cultural phenomenon prevalent predominantly in the rural areas. We recommend a multi-approach including educational, informative and probably legislative strategy in order to limit and hopefully discourage consanguineous marriages.

Adult↗
PubMed2001

Consanguinity and family history of cancer in children with leukemia and lymphomas.

BACKGROUND: In native population of the United Arab Emirates (UAE), the rate of consanguineous marriages is 50.5%. This study was designed to determine whether the rates of consanguinity and family history of cancer among the families of children with lymphoid malignancy are different from those in the general population. METHODS: The study comprised 117 patients from the whole of the country with acute lymphocytic leukemia (ALL) and Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL), ranging in age from 2 to 14 years. The consanguinity rate in the study group was compared with the rate in the general population. To study family history of cancer, the authors matched patients with 117 controls. In a telephone interview, each mother was asked to provide data regarding the biologic relationship between her and her husband as well as that between both sets of grandparents; each was also asked whether any family relative had a cancer and, if so, of what type. RESULTS: Among the 69 ALL cases, 80% of families were consanguineous and 20% were nonconsanguineous. Among the 26 NHL and 22 HL cases, each group included 3 consanguineous families, 12% and 14%, respectively. The consanguinity rates for ALL, NHL, and HL were all significantly different from the 50.5% consanguinity rate in the UAE population (all three P values < 0.0001). The family history of cancer was more often positive in ALL patients than in controls (odds ratio, 2.14; confidence interval, 1.01--4.53). Overall and for each lymphoid malignancy, there was no difference in family history of cancer between consanguineous and nonconsanguineous groups of cases. CONCLUSIONS: The consanguinity rate in the families of patients with ALL is significantly higher and in those with NHL and HL significantly lower than that in the UAE population. The family history of cancer is more often positive among ALL cases than controls--consanguinity having no effect.

Adolescent↗
PubMedDate not supplied

Evolution of consanguinity in the Bishopric of Lugo (Spain) from 1900 to 1979.

BACKGROUND: The levels of consanguinity in human populations are known to be influenced by demographic and geographical factors, and by socio-cultural customs that govern spouse choice. Within the general model applicable to the populations of Western Europe, Galicia (north-west Spain) is a region that is especially interesting, due to its geographical and historical peculiarities that could have conditioned its behaviour pattern with respect to consanguinity. PRIMARY OBJECTIVE: The object of this paper is to analyse the evolution of the consanguinity structure levels in the rural population of the Bishopric of Lugo (Galicia) during the period 1900-1979. SUBJECTS AND METHODS: The study covers a total of 752 parishes included in 52 municipalities of the rural area of the Bishopric of Lugo. Of a total of 117,583 marriages in the period studied, 6701 were between biologically related individuals up to the level of second cousins. The percentage of the different types of consanguineous marriages, the consanguinity rates, and the average coefficient of inbreeding of the population was calculated. Also, for the analysis of the temporal variation of consanguinity, version 3.01 of Jandel Scientific's Table Curve program was used. MAIN OUTCOMES AND RESULTS: The consanguinity rate for the whole of the period is 5.70% and the average coefficient of inbreeding is 2.2477 x 10(-3), values which fall within the observed range for Spanish populations. As for the consanguinity structure, the most frequent types of consanguineous marriages are between second cousins (2.65% of all marriages), but the high proportion of marriages between uncles/nieces or aunts/nephews (0.16%) and first cousins (2.12%) with regard to the total number of marriages should be emphasized. The average value of the M22/M33 ratio is 0.80, and throughout the study period it was above the 0.25 expected in conditions of panmixia. Regarding the evolution of the average coefficient of inbreeding, its annual values fit a polynomial regression curve with one ascending and one descending branch and an inflection point in the year 1919. CONCLUSIONS: As in other rural areas of Galicia, the structure of consanguinity in the Bishopric of Lugo is characterized by the high proportion of marriages between uncles/nieces or aunts/nephews and first cousins, a phenomenon which appears to be generalized in the rural populations of northern Spain. It is suggested that the high proportion of uncle/niece marriages that have been found in the population studied are very probably due to the important migratory phenomenon which took place in this region at the end of the 19th century and in the first third of the 20th century.

Consanguinity↗
PubMed1999

Parental consanguinity as a cause for increased incidence of births defects in a study of 238,942 consecutive births.

The risk for birth defects in the offspring of first-cousin matings has been estimated to increase sharply compared to non consanguineous marriages. As a general decline in the frequency of consanguineous marriages was observed in this century, one wonders whether consanguinity is still a factor in the appearance of birth defects in developed countries. Based on our registry of congenital anomalies we tried to answer to this question. In the population studied in North-Eastern France a consanguineous mating was known in 1.21% of the cases with congenital anomalies, vs. 0.27% in controls, (p < 0.001). The frequency of the malformations recorded paralleled the degree of consanguinity: out of 89 malformed children, 51 were seen in first-cousins mating (10.3 times more frequent than in offspring of non consanguineous couples), 17 in second-cousins marriages and 18 in more distant relatives mating. Three were uncle-niece marriage. Excluding known mendelian conditions these numbers were 73, 36, 17 and 17 respectively and the corresponding relative risk were 3.68, 3.01, 3.41 and 4.89 respectively. Therefore there is a negative dose-response effect between level of inbreeding and risk of congenital malformations. Consanguineous mothers were more often pregnant than non consanguineous mothers (p < 0.01) and they had more stillbirths than non consanguineous mothers. These results show that consanguinity is still a factor of birth defects and they must be taken into account for genetic counseling of inbred marriages, in developed countries.

Congenital Abnormalities↗
PubMed2000

Consanguinity, fertility, reproductive wastage, infant mortality and congenital malformations in Jordan.

OBJECTIVES: Consanguinity is a wide spread practice in Jordan. The objective of this study is to explore the health effects of consanguinity, in particular fertility, reproductive wastage, infant mortality and congenital malformations. METHODS: A stratified 2 stage cluster sample of 1867 married couples, representative of all population groups and all geographic locations of Jordan were randomly selected. A questionnaire was specially designed to explore each of the objectives set for the study and was field tested. A group of field workers were thoroughly trained on the implementation of this instrument. All 1867 couples were interviewed by these field workers and completed questionnaires were reviewed before data entry. Data analysis was carried out using SPSSX statistical package. Significance tests were performed wherever appropriate. RESULTS: The study showed that fertility, as measured by the number of pregnancies, taking into consideration marriage duration, was not affected by consanguinity. Twin pregnancies and abortions did not show any significant difference between consanguineous and non-consanguineous marriages. Consanguineous marriages showed significantly higher rates of still births and infant mortality in general. Within the consanguineous group, female infant mortality rates were significantly higher than those of males. Congenital malformations as reported by mothers of consanguineous marriages were significantly higher than those reported by mothers of non-consanguineous marriages. CONCLUSION: This study showed that consanguinity has a detrimental effect on many aspects of reproductive health.

Abortion, Spontaneous↗
PubMed1983

Age correlation between mates and average consanguinity in age-structured human populations.

When population geneticists wish to determine the genetic consequences of some aspect of mating behavior, it is often necessary to compare observed levels of consanguinity to the level expected when mating is random with respect to the factor being studied. Expectations under random mating are often derived from discrete generation models that ignore age structure. Observed frequencies of consanguineous matings are due to processes that are continuous in time and are affected by variables that are functions of age structure. The extent to which this discrepancy between the models and reality might distort conclusions drawn from comparisons of observed and expected behavior has received insufficient attention. One potential source of error is the tendency for people to choose mates whose ages differ from their own by a certain amount; this tendency influences the frequency of consanguineous matings. Age correlation between mates should therefore also affect average consanguinity between mates and average inbreeding in the population. Expected levels of consanguinity might then differ depending on whether or not age structure has been taken into account. Hajnal developed a model that predicts the frequencies of consanguineous matings in age-structured populations. Hajnal's model is extended here to include relationships that are defined by lineal descent as well as by common ancestry, and to allow calculation of the frequencies of consanguineous matings in the absence of age correlation between mates. The extended model is then used to determine the effect of age correlation between mates on average consanguinity under various sets of conditions. The magnitude of this effect depends on the degree to which the increased frequency of some types of consanguineous matings is offset by the decrease in other types of matings. There is a fairly wide range of conditions under which this compensation is nearly complete and therefore under which the overall effect of age correlation is small. But the size of this effect is sensitive to many factors, especially the distribution of age differences between mates and the variances of ages at maternity and paternity. Under some conditions, age correlation between mates will have a substantial effect on average consanguinity.

Age Factors↗
PubMed1998

Consanguineous marriage in an urban area of Saudi Arabia: rates and adverse health effects on the offspring.

The objective of this cross-sectional study was to determine the pattern and time trend of consanguineous marriage and its adverse health effects on the offspring in Dammam city, Eastern Province, in the Kingdom of Saudi Arabia. This city is known to attract Saudis from different parts of the country because it is in the heart of this industrial region. Five primary health care centers were randomly selected from different sectors of the city in addition to the city's only Maternity and Children's Hospital. For inclusion in the study a wife must have at least one pregnancy that terminated in either full term liveborn baby, still birth, or abortion. A total of 1307 ever-married Saudis completed a pre-structured questionnaire during an interview. The rate of consanguineous marriage was 52.0% with an average inbreeding coefficient of 0.0312. First-cousin marriages were the commonest (39.3%) of all matings. The consanguineous groups had a significantly higher number of pregnancies. The mean birth weight of the offspring of consanguineous couples was not statistically significant being less than that of the non-consanguineous. However, within the consanguineous groups the more closely related couples had smaller babies on average. No significant differences were noted for the rates of inherited diseases and reproductive wastage. The rate of consanguineous marriage in this city was high and so was the inbreeding coefficient. These figures place this nation among the countries with a high rate of consanguineous marriages. A nationwide study to determine accurately the relationship between consanguinity and inherited diseases has much to commend it.

Abortion, Spontaneous↗
PubMedDate not supplied

An insight into recent consanguinity within the Basque area in Spain. Effects of autochthony, industrialization and demographic changes.

BACKGROUND: The importance of studying the genetic kinship of those human groups characterized by a deeply rooted ethnicity has traditionally been and still is an interesting goal of anthropological and population genetic studies. However, only a few surveys have aimed to learn about the impact of industrial development on the consanguinity of these populations and even those have concentrated on industrialized regions. This approach is worth analysing in Spain, where industrialization was late in relation to other western European countries. AIM: In this work we analyse the characteristics of inbreeding in Guipúzcoa from 1951 to 1995. This Basque province underwent industrial and tourist development earlier than other Spanish regions. It has the highest density of Basque speakers and has always occupied a central position within the map of distribution of the Basque language. Guipúzcoa is geographically placed in the core of the Basque area. SUDJECTS AND METHODS: Data on consanguineous marriages recorded in the province of Guipúzcoa between 1951 and 1995 were taken from Roman Catholic dispensations stored in the Diocesan Archives of San Sebastián, the province's capital city. Over the whole time period, a total of 1152 consanguineous marriages were registered. RESULTS: The high frequencies of first cousin (M22) (F = 1/16) and uncle-niece, aunt-nephew (M12) (F = 1/8) consanguineous marriages distinguish Guipúzcoa from the rest of Iberian populations. The M22/M33 ratio (with M33 being second cousins) has never dropped below 0.67, which represents a significant deviation from the expectation value of 0.25. When consanguineous marriages are classified according to marriage partner birthplaces interesting results emerge. Provincial endogamy shows the highest consanguinity rates (57%) and the proportion of M22/M33 is also rather high (0.63). However, a major contribution to the consanguinity levels and mean inbreeding coefficient recorded in Guipúzcoa over recent decades has been made by immigrant relative groups coming from other geographical areas of Spain. In this segment of population the observed M22/M33 rates are 1.44. CONCLUSIONS: This study shows again how important preferentiality (or avoidance) is in human consanguinity, and also how human groups, in spite of being spatially settled in the same territory, present differential attitudes for given consanguinity patterns.

Consanguinity↗