Consanguinity is a deeply rooted social trend among one-fifth of the world population mostly residing in the Middle East, West Asia and North Africa, as well as among emigrants from these communities now residing in North America, Europe and Australia. The mounting public awareness on prevention of congenital and genetic disorders in offspring is driving an increasing number of couples contemplating marriage and reproduction in highly consanguineous communities to seek counseling on consanguinity. Primary health care providers are faced with consanguineous couples demanding answers to their questions on the anticipated health risks to their offspring. Preconception and premarital counseling on consanguinity should be part of the training of health care providers particularly in highly consanguineous populations.

OBJECTIVE: The present study examined mean level similarities and differences as well as correlations between mothers' and fathers' attributions regarding successes and failures in caregiving situations and progressive versus authoritarian attitudes in Jordan. DESIGN: Interviews were conducted with both mothers and fathers in 112 families. RESULTS: There were no significant main effects of gender on any of the constructs of interest. Mothers and fathers reported similar levels of attributions regarding uncontrollable success, adult-controlled failure, and child-controlled failure in the same family. Regarding attitudes, mothers and fathers reported greater progressive attitudes than authoritarian attitudes. Large, significant correlations were found for concordance between parents in the same family on all seven attributions and attitudes examined; all remained significant after controlling for parents' age, education, and possible social desirability bias. Significant positive correlations were found for mothers' and fathers' attributions regarding uncontrollable success, adult-controlled failure, child-controlled failure, perceived control over failure, progressive attitudes, authoritarian attitudes, and modernity of attitudes. CONCLUSIONS: This study concluded that in Jordan mothers and fathers hold similar levels of attributions and attitudes.

The present cross-sectional study was done in order to illustrate the prevalence and types of consanguineous marriages among Afghanistan populations. Data on types of marriages were collected using a simple questionnaire. The total number of couples in the study was 7140 from the following provinces: Badakhshan, Baghlan, Balkh, Bamyan, Kabul, Kunduz, Samangan and Takhar. Consanguineous marriages were classified by the degree of relationship between couples: double first cousins, first cousins, first cousins once removed, second cousins and beyond second cousins. The coefficient of inbreeding (F) was calculated for each couple and the mean coefficient of inbreeding (α) estimated for each population. The proportion of consanguineous marriages in the country was 46.2%, ranging from 38.2% in Kabul province to 51.2% in Bamyan province. The equivalent mean inbreeding coefficient (α) was 0.0277, and ranged from 0.0221 to 0.0293 in these two regions. There were significant differences between provinces for frequencies of different types of marriages (p<0.001). First cousin marriages (27.8%) were the most common type of consanguineous marriages, followed by double first cousin (6.9%), second cousin (5.8%), beyond second cousin (3.9%) and first cousin once removed (1.8%). There were significant differences between ethnic groups for the types of marriages (χ2=177.6, df=25, p<0.001). Tajiks (Soni) and Turkmens (also Pashtuns) showed the lowest (α=0.0250) and highest (α=0.0297) mean inbreeding coefficients, respectively, among the ethnic groups in Afghanistan. The study shows that Afghanistan's populations, like other Islamic populations, have a high level of consanguinity.

Purpose  To assess the health-related quality of life (HRQoL) of Jordanian children with thalassaemia, identify differences in HRQoL according to socio-demographic and clinical characteristics of participants, and identify factors influencing HRQoL of thalassaemic children in Jordan. Methods  The Pediatric Quality of Life Inventory and socio-demographic and clinical data forms were used to collect data from a convenience sample of 128 thalassaemic children and 83 healthy children between the ages of 8 to 18 years. Thalassaemic children were recruited from two thalassaemia units during their blood transfusion and treatment visits. Healthy children were recruited from four public schools. Results  Thalassaemic children had significantly lower HRQoL mean scores in all dimensions compared with their healthy counterparts. The lowest mean scores for thalassaemic children were reported for the school functioning and the physical functioning domains (46.71 ± 21.14 and 54.19 ± 15.10), respectively. Regression analysis showed that disease complications and family history of thalassaemia explained 8.5% of the variance in the total HRQoL. Conclusion  This study highlights the negative impact of thalassaemia on the quality of life of children, especially in terms of physical well-being and school achievement. Healthcare providers, counsellors and school teachers have very important roles in helping these children to overcome these problems and enhance their quality of life.

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It is proposed that consanguineous marriages increase the risk of primary immunodeficiency disorders (PID). The aim of this study is to review the frequency and pattern of parental consanguinity among PID patients and to determine its effects on the distribution of different PID, the patients' performance status and the risk of death. The data was obtained from the Kuwait National Primary Immunodeficiency Disorders Registry. The coefficient of inbreeding was determined for each patient and the patients' overall performance status was assessed using the Lansky Play Performance Scale and the Karnofsky Performance Scale. A total of 128 patients with PID from 99 families are reported. A family history suggestive of PID and parental consanguinity was reported in 44 and 75% of the patients respectively, while the mean coefficient of inbreeding was 0.044067. There were statistically significant associations between both a family history of PID and parental consanguinity and PID category, the risk of death and the patients' overall performance status. Evidence of autosomal recessive transmission of disease was present in 44% of the patients. CONCLUSIOns: Parental consanguinity is a risk factor for the development of PID. There is a need to increase the public awareness of the health consequences of consanguineous marriages.

BACKGROUND Despite being associated with multiple genetic problems, consanguineous marriages continue to remain extremely prevalent worldwide. Studying the variation of kin preferences in diverse inbred societies may provide some answers to this paradox. AIM To find the reasons for specific kin choice in different geographical areas of the world. METHOD We used a set of sociobiological rules (kin altruism, sexuality and inbreeding avoidance) and ecological constraints (e.g. tribal warfare, food availability) that influence human behaviour. The cumulative help that the extended family can provide to a nuclear family was calculated using the coefficient of relatedness between kin in different types of consanguineous families. RESULTS The maximum potential support for kin markedly varied between different types of consanguineous marriages. Overall, members of consanguineous families received up to two-and-half times more support than members of non-consanguineous families. In various inbred cultures, preference for a specific type of kin was determined by prevailing ecological limitations and sociobiological factors interacting in a complex manner. CONCLUSION In different inbred populations, the ideal kin for a consanguineous marriage is the one who can provide the most altruistic support; however, this choice is influenced by biological rules of behaviour and ecological constraints.

From March 2007 to March 2008 a cross-sectional study was conducted in Qatar to estimate the prevalence of consanguinity among Qataris and to assess their knowledge of the risks and their attitudes towards the practice. A secondary objective was to test the acceptability of sixteen Likert-style questions within the Qatari population. Face-to-face interviews using a 70-item structured questionnaire were conducted by three native Arabic-speaking medical students with 362 Qatari employees. Where consanguinity existed between the employee's parents, a diagram of the consanguinal relationship (phylogram) was completed. The response rate was 93%. By phylogram, 22% of participants reported a cousin relationship between their parents (consanguinal relationship) and another 15% reported that their parents were from the same tribe (affinal relationship). With respect to their own marital decision, 68% of the respondents had been married at least once. By phylogram, 35% of these reported a consanguineous relationship (first marriage), 9% reported only an affinal relationship and 56% reported that they were not married to a blood relative. Results on the sixteen Likert-style attitude questions were stratified by consanguinity status of parents and of self. In the stratification by consanguinity status of parents the top five attitudes differed by group but there appeared to be more similarity between the consanguinal and only tribal groups. Attitudinal results were stratified by sex. Results showed that the males had a stronger belief in several of the attitudes than females with the exception of causation of genetic abnormalities and health problems. The phylogram was shown to collect more detailed and explicit data than hard-coding. With respect to knowledge, the results showed that knowledge was imperfect with high proportions of participants not knowing that consanguinity has been implicated in autosomal recessive diseases such as thalassaemia, inborn errors of metabolism, deafness, anomalies of the extremities and specific congenital heart defects. Additionally, a sizeable proportion of the participants did not know that a more distant cousin marriage (e.g. third cousin) theoretically could be a less genetically risky choice to potential offspring than a closer cousin marriage (half-first cousin). These results indicate that more effort needs to be made in developing public health strategies to improve the population's understanding of the cost-benefit analysis involved in contracting consanguineous marriages given the goal of healthy offspring.

Consanguinity, the marriage between relatives, has been associated with adverse child health outcomes. The objective of the present study was to assess the effect of consanguinity on offspring weight gain from birth to 12 months after birth. Data were collected on 250 consecutive live-born singleton newborns referred to a local health centre in Shiraz (Fars province, southern Iran). Collected data covered socio-demographic characteristics (such as parental age at delivery and parental education), sex, birth order, weights from birth to 12 months after birth and consanguinity of marriages of parents. Considering the low prevalence of double first cousin, first cousin once removed, second cousin, and beyond second cousin marriages, only first cousin and unrelated marriages were included in the study. The study population consisted of a total of 207 newborns (57 offspring of first cousins, 150 offspring of unrelated marriages). Based on the results of repeated measurements analysis of variance, weight gain was associated with type of marriage (p=0.018), sex of offspring (p=0.001) and paternal education (p<0.001). There was no interaction between type of marriage and sex (p=0.831). Birth weight was not affected by type of marriage (p=0.46). There was significant interaction between inbreeding and time (p=0.034). Offspring of consanguineous marriages showed lower weight gain in comparison with those of unrelated marriages during 3-12 months after birth.

Consanguineous marriages have been practiced since the early existence of modern humans. Until now consanguinity is widely practiced in several global communities with variable rates depending on religion, culture, and geography. Arab populations have a long tradition of consanguinity due to socio-cultural factors. Many Arab countries display some of the highest rates of consanguineous marriages in the world, and specifically first cousin marriages which may reach 25-30% of all marriages. In some countries like Qatar, Yemen, and UAE, consanguinity rates are increasing in the current generation. Research among Arabs and worldwide has indicated that consanguinity could have an effect on some reproductive health parameters such as postnatal mortality and rates of congenital malformations. The association of consanguinity with other reproductive health parameters, such as fertility and fetal wastage, is controversial. The main impact of consanguinity, however, is an increase in the rate of homozygotes for autosomal recessive genetic disorders. Worldwide, known dominant disorders are more numerous than known recessive disorders. However, data on genetic disorders in Arab populations as extracted from the Catalogue of Transmission Genetics in Arabs (CTGA) database indicate a relative abundance of recessive disorders in the region that is clearly associated with the practice of consanguinity.

Although autism is a global disorder, relatively little is known about its presentation and occurrence in many developing countries, such as Saudi Arabia. This article describes the reasons for referral and clinical characteristics of a sample of 49 children (37 males and 12 females) diagnosed with an autistic spectrum disorder at a tertiary referral center in Saudi Arabia. The diagnosis of autism was based on DSM-IV criteria supplemented by information obtained from parent and child interviews, rating scales, and examination of school and hospital records. Females were older than males at the time of referral. Eleven patients had a history of seizure disorder and one patient had a chromosome abnormality. Twenty-five patients were taking psychotropic medications and 14 patients were the product of consanguineous marriages. Clinical and research implications of these findings are discussed.

Consanguineous marriage is the union of individuals having at least one common ancestor. The present cross-sectional study was done in order to illustrate the prevalence and types of consanguineous marriages in the Syrian Arab Republic. Data on consanguineous marriages were collected using a simple questionnaire. The total number of couples in this study was 67,958 (urban areas: 36,574 couples; rural areas: 31,384 couples) from the following provinces: Damascus, Hamah, Tartous, Latakia, Al Raqa, Homs, Edlep and Aleppo. In each province urban and rural areas were surveyed. Consanguineous marriage was classified by the degree of relationship between couples: double first cousins (F=1/8), first cousins (F=1/16), second cousins (F=1/64) and beyond second cousins (F<1/64). The coefficient of inbreeding (F) was calculated for each couple and the mean coefficient of inbreeding (alpha) estimated for the population of each province, stratified by rural and urban areas. The results showed that the overall frequency of consanguinity was 30.3% in urban and 39.8% in rural areas. Total rate of consanguinity was found to be 35.4%. The equivalent mean inbreeding coefficient (alpha) was 0.0203 and 0.0265 in urban and rural areas, respectively. The mean proportion of consanguineous marriages ranged from 67.5% in Al Raqa province to 22.1% in Latakia province. The alpha-value ranged from 0.0358 to 0.0127 in these two provinces, respectively. The western and north-western provinces (including Tartous, Lattakia and Edlep) recorded lower levels of inbreeding than the central, northern and southern provinces. The overall alpha-value was estimated to be about 0.0236 for the studied populations. First cousin marriages (with 20.9%) were the most common type of consanguineous marriages, followed by double first cousin (with 7.8%) and second cousin marriages (with 3.3%), and beyond second cousin was the least common type.

The union of individuals with a common ancestor may lead to serious health consequences in their offspring. Consanguinity is high in Middle Eastern communities; it was around 26% in 1988. The objective of this study was to determine the prevalence of consanguinity in Beirut and other Lebanese regions, and its associated factors in different subgroups. The cross-sectional study was performed on a convenience sample of married women in Lebanon. The women were administered a standardized questionnaire in a face-to-face interview by independent enquirers. Among 1556 women, the overall prevalence of consanguineous marriages was 35.5%, and the consanguinity coefficient was 0.020; 968 marriages (62.2%) were not consanguineous, 492 (31.6%) were first cousin, 61 (3.9%) were second cousin and 36 (2.3%) had lower degrees of consanguinity. Beirut suburb dwelling, low education subgroups, women working in the home and non-Christian religion presented the highest rates of consanguinity (p<0.05). Consanguinity is associated with couples' nulliparity and child chronic morbidity. Factors that could affect consanguinity are having consanguineous parents, having a favourable opinion towards consanguinity, choosing a spouse for religious reasons, particularly in Islam, woman having a low education, woman working in the home and women thinking that consanguinity would not lead to serious diseases. Consanguinity is therefore still a prevailing problem in Lebanon. Specific health education, and genetic counselling in particular, are suggested to explain the consequences of consanguinity to the general population and to help couples make informed choices.

This paper explores cancer family clustering in a random sample of patients registered in the Jordan National Cancer Registry for the year 1999, the most recent year that complete data were available. A special instrument was designed and data collected through personal interviews. Of the final sample of 707 cancer patients, 23% had a positive family history of cancer, 59% of which was first-degree clustering. For every proband there were 1.39 contacts. Half of them were first-degree relatives of the proband and 17% had cancer at the same site as the proband. Family clustering of cancer in Jordan appears to be of public health significance, and we recommend immediate and thorough followup of family members of cancer cases.

Summary Inbreeding patterns over the past two centuries have been studied more extensively in Spain and Italy than anywhere else in Europe. Consanguinity studies in mainland Spain have shown that populations settled along the Cantabrian cornice share inbreeding patterns that distinguish them from other populations further south. A visual representation of spatial variations of two key inbreeding variables is presented here for the first time via contour maps. This paper also analyzes time trends of mean inbreeding coefficients for X-linked (F(x)) and autosomal genes (F)(1862-1995) together with variations in F(x)/F ratios in Guipúzcoa, the most autochthonous Spanish Basque province. Because close cousin marriages are a mark of identity of the study population, we evaluated the contribution of uncle-niece/aunt-nephew (M12) and first cousin (M22) marriages to F(x) and F values and compared the frequencies of M12 and M22 pedigree subtypes and their corresponding F(x)/F ratios to those found in other Spanish populations. The mean Fx and F inbreeding levels in Guipúzcoa for the 134-year period analyzed were 1.51 x 10(-3) and 1.04 x 10(-3), respectively, and the F(x)/F ratio was seen to be very stable over time. Our findings show that major similarities exist for close consanguineous marriage subtypes between Basque and non-Basque Spanish populations, despite significant geographic variability in terms of first cousin pedigrees. The distortion seems to be caused by Guipúzcoa. The F(x)/F ratios for first cousins in Spanish populations were higher than expected (1.25), with values ranging from 1.34 to 1.48. The findings of the present study may be useful for advancing knowledge on the effects of the interaction between biology and culture and for exploring associations between mating patterns and the prevalence of certain diseases.

Arab societies are characterized by a wide range of family and social structures, religious and legal conventions, and highly variable economic resources. As might be expected under these circumstances, genetic services cannot readily be devised, delivered, and assessed according to a single model. However, in general terms, the provision of genetic services in Arab communities at all population levels is still inadequate given the prevalence and burden of genetic diseases. Improving this situation calls for major educational efforts that include increasing the genetic literacy of the general public, comprehensive courses and campaigns to familiarize primary health care workers with counseling needs and skills and with referral guidelines for high-risk families, updating medical, nursing, and paramedical curricula to incorporate information on community genetics, and training clinical and laboratory genetic specialists to meet the short- and long-term goals of genetic disease prevention and management.

This cross-sectional study was carried out to assess the association between consanguineous marriages and adverse pregnancy outcome in the north of Jordan. Women delivered in four major hospitals in the north of Jordan between April 2007 and May 2007 were included in the study. Non-Jordanian women and women with multiple pregnancies were excluded. Mothers answered a pilot-tested structured questionnaire administered by trained personnel in the maternity ward. Data regarding pregnancy outcomes were obtained from the patients' individual records. A total of 3,269 women with a mean age of 27.2 (SD 6.6) years were included. About 49% of women had consanguineous marriages. Consanguineous marriages were significantly associated with low birth weight delivery (13.9% vs. 10.1%), preterm delivery (19.9% vs. 12.3%), and births with congenital anomalies (4.1% vs. 0.8%) compared with non-consanguineous marriages. In the multivariate analysis, consanguinity was significantly associated with preterm delivery (OR = 1.5, 95% CI 1.2, 1.9), and congenital malformations (OR = 6.5, 95% CI 2.8, 15.3). In conclusions, this study supports the association between consanguinity and some adverse pregnancy outcomes.

Secondary analysis of the trends and correlates of consanguinity in the Palestinian Territories was conducted using data from two separate surveys in 1995 and 2004. The analysis was conducted on ever-married women aged 15-54 who were asked about their relation to their husband in both surveys. A total of 16,197 women in 1995 and 4971 women in 2004 were successfully interviewed. Consanguinity was found to be widely practised in the Palestinian Territories with rates of total consanguinity reaching 45% of all marriages in 2004. Analysis was conducted with the data from the two surveys combined and this indicated that consanguinity was significantly decreasing with time after controlling for other variables. Age of the women, their age at marriage, region and locality type they lived in and their standard of living were all found to be significant predictors of consanguinity. The education level of the women was not found to be significant. After controlling for the survey year, women's labour force status was also found to be a non-significant predictor of consanguinity. Although consanguinity was found to be significantly decreasing slowly with time after controlling for other variables, the future trends of consanguinity are not known due to the unstable political situation in the territories, which could have a direct effect on marriage patterns.

BACKGROUND AND OBJECTIVES There is a high rate of consanguinity in Saudi Arabia; however, information on its relationship with genetic disorders is limited. The objective of this cross-sectional study was to explore the role of consanguinity in genetic disorders. SUBJECTS AND METHODS The study sample was determined by a multistage probability random sampling procedure. Consanguinity status was obtained during household visits. Primary care physicians performed a history and physical examination of all children and adolescents younger than 19 years, and all cases of genetic diseases were recorded. The chi-square test was used to compare proportions. RESULTS During the two-year study period (2004-2005), 11 554 of 11 874 (97%) mothers answered the question on consanguinity, and 6470 of 11 554 (56%) were consanguineous. There was no significant association between first-cousin consanguinity and Down syndrome (P=.55). Similarly, there was no significant association with either sickle cell disease (P=.97) or glucose-6-phosphate dehydrogenase deficiency (P=.67) for first-cousin consanguinity. A borderline statistical significance was found for major congenital malformations (P=.05). However, the most significant association with first-cousin consanguinity was congenital heart disease (CHD)(P=.01). Finally, no significant association was found for type 1 diabetes mellitus (P=.92). For all types of consanguinity, similar trends of association were found, with a definite statistically significant association only with CHD (P=.003). CONCLUSION The data suggest a significant role of parental consanguinity in CHD. However, a relationship between consanguinity and other genetic diseases could not be established. The effect of consanguinity on genetic diseases is not uniform and this should be taken into consideration in genetic counseling.

OBJECTIVES To determine the etiologies, outcomes, and risk factors for epilepsy in infants. PATIENTS AND METHODS This retrospective study included all children who had their first afebrile seizure between 1 and 12 months of age, and who were followed in the Child Neurology Clinic at the Jordan University Hospital from January 2004 to January 2006. Medical records were reviewed to collect demographic data and the clinical data pertaining to epilepsy. An age-matched control group of healthy children in a 1:2 ratio was included to determine the risk factors for epilepsy. For statistical analysis, SPSS, Version 13, was used. RESULTS Fifty-five patients were included in the study group and 111 were in the control group. Epilepsy was classified as follows: symptomatic in 24 (43.6%) children, probably symptomatic (cryptogenic) in 28 (50.9%) children, and idiopathic in 3 (5.5%) children. The etiologies of epilepsy in the symptomatic group included hypoxic-ischemic encephalopathy (n=11), cortical malformations (n=5), neurocutaneous syndromes (n=2), metabolic disorders (n=4), leukodystrophy (n=1), and craniosynostosis (n=1). Twenty-seven patients (49%) were seizure-free at their last follow-up visit for at least the last 6 months; only six patients (10.9%) continued to have normal development at the time of their last follow-up examination. The risk factors for epilepsy included parental consanguinity (P=0.0003), a family history of global developmental delay (P=0.0002), a family history of epilepsy (P=0.010), and a positive perinatal history (P=0.011). CONCLUSION This study emphasized that afebrile convulsions in infancy are rarely benign. Furthermore, consanguinity was shown to be a major risk factor for epilepsy.