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At least 19 recordsLinked to original sources
PubMedDate not supplied

Reproductive pattern in consanguineous and non-consanguineous marriages in La Cabrera, Spain.

BACKGROUND: Among determinants of the structure of human populations, consanguineous marriages can be useful in determining to what extent they influence the genetic structure of the population. This knowledge may be gained by comparing the reproductive performance of related and non-related couples. The population studied, La Cabrera, is a mountainous region in the Leon province (north-western Spain). It includes four neighbouring municipalities with similar geographic, climatic and ecological features with 37 parishes occupying an area of 784.2 km(2), of which only 1.5% is arable. The number of inhabitants remained stable from 1887 (9526 inhabitants) to 1960 (8984), when due to emigration it began to decrease. The average inbreeding coefficient (alpha) for the period 1880-1989 (up to third degree) was 4.82 x 10(-3). AIM: The objective of this analysis was to evaluate the possible factors determining the differences between consanguineous and non-consanguineous families residing in La Cabrera during the period 1880-1959. SUBJECTS AND METHODS: To study the structure and dynamics of each couple, families were reconstituted for the period 1880-1959 using demographic data corresponding to births, deaths and marriages obtained from parish registers. This procedure provided information on each couple's reproductive history through information concerning the mates' deaths as well as their offspring's births and deaths. Principal component analysis revealed the existence of three factors that together explained 57% of the reproductive pattern variability of La Cabrera. RESULTS: The first component (eigenvalue: 3.56) correlated positively with the number of live births and with the duration of the reproductive period. Component II (eigenvalue: 1.54) had a positive correlation with variables describing the beginning of the marital union, and with those indicating its ending. Finally, component III (eigenvalue: 1.13) reflected a negative association with the two variables that described neonatal and post-neonatal mortality. The above demographic variables represented the reproductive process in La Cabrera adequately. Fertility was the variable that offered the best explanation of the reproductive pattern (32%), followed by the marital structure (13%) and the infant mortality (12%). CONCLUSION: In the La Cabrera population, consanguinity appears as a socio-cultural process that affects the reproductive dynamic. Consanguinity modifies the marital structure and alters the reproductive pattern, prolonging the reproductive period, which results in a greater number of offspring. Lower infertility rates and the possible compensatory effect for infant mortality may reflect biological factors making the wife's reproductive period more efficient.

Adult↗
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↗
PubMed2007

Influence of consanguinity on the pattern of familial aggregation of congenital cardiovascular anomalies in an outpatient population: studies from the eastern province of Saudi Arabia.

BACKGROUND: Familial aggregation of congenital heart disease (CHD) has been well described in different populations, in particular those with a high consanguinity rate. Extensive genetic study of affected families has improved the understanding of basic genetics of different cardiac lesions. OBJECTIVE: To identify the role of consanguinity as a risk factor among familial cases of CHD in a stable outpatient population of a tertiary care center in the Eastern Province of Saudi Arabia. METHODS: All familial cases of CHD seen over 5 years (1996-2000) in the Division of Pediatric Cardiology were identified. The presence or absence of parental consanguinity (first cousin marriage) was defined in each of these families. RESULTS: Ninety-three cases were identified in 37 families. Twenty-three (62%) families resulted from consanguineous marriages. In 4 families where there were 2 marriages, the affected children came from the consanguineous marriage in 3 of these families. Discordant lesions occur only among non-consanguineous cases, while all consanguineous cases were concordant. Five sets of twins of the same sex (one set are monozygotic by DNA analysis) occurred among consanguineous marriages, in 3 of these both twins were affected with the same disease. Affected parents were seen in 2 families with consanguineous marriage and none in the non-consanguineous marriages. The prevalence of dilated cardiomyopathy was much higher among consanguineous cases (26 vs. 2). CONCLUSIONS: Familial aggregation of congenital heart disease is common in our population. Consanguinity is common in these families, and the distribution of congenital heart disease differs in this subgroup compared to the rest of the familial cases. Further genetic studies of these families may help to shed more light on basic genetics and the specific pathogenetic mechanisms involved.

Cardiovascular Abnormalities↗
PubMed2004

Consanguinity in Kahramanmaras city, Turkey, and its medical impact.

OBJECTIVE: Recently, several studies have indicated the rate of consanguinity ranging between 20 and 70% in the Middle East. Turkey is one of the countries with a high rate of consanguineous unions in the Middle East. In this study, the rate of consanguinity and its effects on spontaneous abortus, stillbirth, infant mortality, and birth defects were investigated in a population sample residing in Kahramanmaras city, Turkey. METHODS: For this study, 1000 randomly selected women, aboriginals of Kahramanmaras city, Turkey, were interviewed at their home and the concerning information was obtained by administration of a questionnaire between April 2002 and March 2003. RESULTS: The prevalence of consanguinity was found to be 30.6% with the mean inbreeding coefficient of 0.015373. The most common type of consanguineous mating was first cousin marriages with the frequency of 22.6%. The family pressure and love were the main reasons for marrying with a relative. The mean age at marriage of women and men were lower in consanguineous marriages than that of non-consanguineous unions. There was a negative correlation between the consanguinity and educational level of both sexes. The results revealed differences between consanguineous and non-consanguineous matings, in terms of stillbirth, infant mortality and birth defects whereas the rate of spontaneous abortus was found to be the same in 2 kinds of marriages. CONCLUSION: The incidence of consanguinity and of first cousin marriages is found to be very high in the Kahramanmaras city. A reduction of consanguinity rate is necessary for the health quality of the population.

Abortion, Spontaneous↗
PubMed2006

[Spectrum of congenital malformations observed in neonates of consanguineous parents].

INTRODUCTION: Consanguineous unions occur in all populations around the world. Couples related as second cousins or closer have been observed with deleterious effect. Among the clinical effects of parental consanguinity, the incidence of offspring with congenital malformations (CM) increases approximately two-fold. MATERIALS AND METHODS: A hospital database of neonates with CM was searched to select neonates with parental consanguinity and two control groups. One control group consisted of healthy neonates and the other control group consisted of neonates with CM but without parental consanguinity. Both control groups consisted of the first neonate of the same sex to be born after a consanguineous neonate with CM. Family, sociodemographic and anthropometric variables, as well as the severity of the malformations, were compared between the two groups with CM. Neonates with CM were grouped into five categories: Major multiple CM, minor multiple CM, isolated major CM, isolated minor CM, and specific diseases. The indigenous Mayan subpopulation was also analyzed. RESULTS: Among 1117 neonates with CM, parental consanguinity was found in 21. Parental consanguinity was also found in 8 neonates in the group of healthy controls (OR 2.4 [1.05-5.95]). The most common form of consanguinity was between second cousins and was more frequent in the Mayan subpopulation. Major multiple CM were more frequent among consanguineous than among nonconsanguineous couples. No association was found between the severity of CM and the degree of relationship. CONCLUSIONS: The prevalence of consanguinity found in neonates with CM and healthy controls (1.9 % and 0.8 %) was similar to that found in other Latin populations. A higher prevalence was found in the Mayan population. Mayor multiple CM were more frequent among the neonates of consanguineous than among nonconsanguineous couples.

Congenital Abnormalities↗
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↗
PubMedDate not supplied

Consanguinity in two Uruguayan cities: historical evolution and characteristics (1800--1994).

BACKGROUND: Information about consanguinity in Uruguay is scarce and limited to the end of the 20th century. AIM: To determine the frequency and characteristics of consanguineous marriages, as well as chronological trends, in two Uruguayan cities over almost two centuries. SUBJECTS AND METHODS: We analysed 28,393 Roman Catholic Church marriage records and Diocesan consanguinity dispensations belonging to the cities of Melo (Northeast), and Montevideo (South), for the period 1800--1994. RESULTS: 633 (2.23%) marriages were consanguineous. Among them, first cousin marriages were the most common (58.8% of all consanguineous marriages, including double consanguineous), especially those where the bride and groom were related through their maternal side. During the first decades of the 19th century both regions showed low levels of consanguinity. Consanguinity reached its maximum during the mid-1800s and decreased significantly throughout the 20th century. The overall mean coefficients of inbreeding were moderate in both cases, being greater in the Northeast (alpha=0.00165) than in the South (alpha = 0.00089). CONCLUSIONS: The low level of consanguinity as well as the structure of consanguineous marriages (distribution by degrees) is similar to that found in other southern South American countries. Temporal trends are similar to those found in industrialized regions in Europe, with maximum inbreeding levels during the middle-late 19th century; however, the clear predominance of first cousin unions, differs from most of the data for European countries. Small differences between the two cities can be related to diverse facts, such as socio-economic conditions, ethnic origin, immigration, and sampling.

Consanguinity↗