Consanguinity analysis of congenital deafness in Northern Israel.

Consanguinity analysis of heterogeneous populations was performed on a group of 82 Israeli Jewish families with congenitally deaf probands, including 37 multiplex families with normal parents, 10 multiplex families with deaf parents, and 35 simplex families with deafness of unknown cause. Representative gene frequency was estimated as .0198, with two to four major gene loci per ethnic group. In both the simplex families and those with deaf parents, the only significant etiology found was homozygosity for pathologic recessive genes. Comparison of these findings in Israeli isolates with those in panmictic populations seems to imply that the genetic loci are not identical in the various isolates.     

Consanguinity and prereproductive mortality in the Utah Mormon population

To test the effects of parental consanguinity on mortality among offspring, inbreeding coefficients were estimated for 303,675 members of the Utah Mormon population who were born between 1847 and 1945. Although consanguinity has been relatively rare in this population, the large sample size permitted the identification of more than 3,500 inbred offspring. Among the offspring of unrelated parents, 13.2% died before the age of 16. Significant elevations in prereproductive mortality were seen among the offspring of first-cousin marriages (22%) and among the offspring of closer unions (32%). The cor- responding relative risks are 1.70 (95% confidence limits = 1.52, 1.91) and 2.41 (95% confidence limits = 1.59, 3.41), respectively. Other categories of relationship did not produce significant elevations in offspring mortality. Similar results were obtained when a case-control approach was used to remove the effects of socioeconomic variation. Consistent with many other studies of populations with low consanguinity rates, this population experienced a relatively high absolute increase in mortality among the offspring of first-cousin marriages (9%). Preliminary evidence is offered for the hypothesis that mortality differentials are larger in populations with low inbreeding and low mortality because nongenetic causes of death do not obscure the effects of consanguinity.     

Gc subtypes in the middle east: Report on an Arab Moslem population from Israel

Gc subtypes were determined by isoelectrofocusing and immunofixation on 342 blood samples from an Arab Moslem population in Israel. Observed allele frequencies were: Gc^1F 0.2120, Gc^1S 0.6023, and Gc² 0.1857. Those are similar to formerly reported frequency data for other Middle Eastern populations. A discriminant analysis, performed on data from 35 populations, resulted in a satisfactory classification of population groups related through geographic and racial origin.     

Effects of polygyny and consanguinity on high fertility in the rural Arab population in South Jordan

Based on the authors' interview survey for 608 randomly selected women of the rural Arab population in the South Ghor district of Jordan, this paper examined the effects of polygyny and consanguinity on high fertility, which was recognized as natural fertility. The prevalence of polygynous and consanguineous marriages was 28.0% and 58.1%, respectively, largely reflecting the population's traditional marriage customs. The findings highlighted a significantly higher total marital fertility rate (TMFR) in the monogamous wives (10.5) than in the senior polygynous (8.1) and junior polygynous wives (8.6); the TMFR did not significantly differ among the wives of non-consanguineous, first-cousin and second-cousin marriages. The formation of polygynous marriage was decided by the husband, mostly as a result of his senior wife's infecundity or sub-fecundity, and the age of the husband at marriage to his junior polygynous wife was high in many cases, leading to a decline in this wife's fecundity.     

Selective angiocardiography in congenital heart disease

Angiocardiography has come to be a highly specialized procedure and there are definite indications for doing it. In specific cases it offers clear-cut advantages. Technical improvements permit a single injection of a small amount of contrast substance. Selective angiocardiography is advised where the particular detail of a certain region in the heart is desired.     

Two different mutations are responsible for Krabbe disease in the Druze and Moslem Arab populations in Israel

Infantile Krabbe disease is a severe, fatal autosomal recessive disorder resulting from the deficiency of galactocerebrosidase (GALC) activity. It is relatively common in two separate inbred communities in Israel. In the Druze community in Northern Israel and two Moslem Arab villages located near Jerusalem the incidence of Krabbe disease is about 1 in 100–150 live births. With our cloning of the GALC gene, mutation analysis of these populations was undertaken. The Moslem Arabs were homozygous for two mutations in the GALC gene; a T-to-C transition at cDNA position 1637 (counting from the A of the initiation codon), which is considered a polymorphism, and a G-to-A transition at position 1582, which changes the codon for aspartic acid to one for asparagine. The Druze patients are homozygous for a T-to-G transversion at position 1748, which changes the codon for isoleucine to one for serine. Expression studies confirmed the deleterious nature of these mutations. The development of a simple polymerase chain reaction (PCR) amplification and restriction enzyme digestion method to identify these alleles will lead to accurate carrier testing and improved genetic counseling for interested individuals in these communities.     

Inheritance of congenital heart disease

Congenital heart defects (CHD) are the most common developmental anomalies and are the leading noninfectious cause of mortality in newborn babies. It has been estimated that between four and ten live-born infants per 1000 have a cardiac malformation (0.4 to 1.0%), 40% of which are diagnosed in the first year of life. The European Registration of Congenital Anomalies (EUROCAT) reported a prevalence of 58.9/10,000 live births in the northern part of the Netherlands (0.6%). Hoffman estimated that the true prevalence of CHD may be as high as 53 per 1000 pregnancies (5.3%), including a 20% occurrence of heart defects in spontaneous abortion, a 10% occurrence in stillbirth, and a 1% occurrence in live birth.     

Medicine Among New Immigrants and Arab Minorities in Israel

The reaction to modern medicine and the general health of the new immigrants and Arab populations of Israel are described. The material was gathered during a threemonth visit to Israel where the author participated in the medical care of these persons. While these peoples still share many of the traditional medical superstitions and practices, the new immigrants have progressed much more during the 16 years of Israel''s existence. At present over 99% of Jewish women give birth in hospital, whereas only 65% of Israeli Arabs do. The infant mortality rate among the Jews in 1963 was about 21 deaths per 1000 live births, about one-half the rate for Arabs. The importance of understanding the cultural background and social conflicts of these people as a preliminary to the provision of proper medical care is stressed.     

Arab football in Israel as an 'integrative enclave'

Over the last two decades, football (soccer) has become a major institution within the popular culture of the Arab-Palestinian citizens of Israel. The centrality of football has given different identity agents opportunities to impose different meanings on the sport, depending on the agents' definitions of collective identity. This article utilizes ethnographic observation in the football stadium and coverage by Arab and Hebrew sports media to illustrate and analyse this battle over meaning. The Hebrew sports media, the Arab sports press, and the Arab audience are three different agents that attach divergent meanings to the notable presence of Arab players in the Israeli football leagues. The article argues that the overlapping interests of the Hebrew sports media on the one hand and the Arab football fans, players, and bureaucrats on the other lead to the construction of the football sphere as an integrative enclave in the general Israeli public sphere. The article considers the relevance of Gramsci's theory of hegemony to explain the production of the integrative meaning of football, and it suggests integrating this explanation with other recent theories regarding the tensions between different discourses of citizenship.     

Genetic blood markers in Arab Druze of Israel

A sample of 153 individuals from a Druze village, in northern Israel, was typed for the following genetic markers--ABO, MNSs, Rh, P, Kell, and Duffy in the blood groups AcP, AK, ADA, EsD, GL01, ICD, LDH, G6PD, PGM 1 & 2, PHI, PGD and peptidases A, B, C, and D in the red cell enzymes and for the serum proteins Hp and GC subtypes. Rare variants were observed in the following systems: PGD, a new slow variant, PGM, type 8-1; Pep A, types 2-1 and 3-1, Pep B, type 2-1; Pep D, types 3-1 and 3-3; and type GC, 2-V. Significant deviations from Hardy-Weinberg expectations were observed for MNSs and Duffy because of increased homozygosity, which was also observed in three other systems. Gene frequencies compared well with those of Arab Druze and Moslems in Lebanon and of Israeli Moslems in most of the systems, except for the lower frequencies of blood group B, the NS chromosome, the cde haplotype, and the AcPA allele in the present sample. A considerably lower frequency of the Fy allele was found in the Druze compared with Arab Moslems. It may be due to the Druze having been less exposed to inflow of African genes, to their being highlanders, and, therefore, less exposed to Plasmodium vivax malaria, or to both of the above.     

Clues in diagnosing congenital heart disease.

A number of practical office and bedside clues to cardiac disease in infants and children have been passed on through the years. They relate to the history, to the inspection and palpation components of the physical examination, and to knowledge of the specific cardiac defects that are likely to be associated with certain clinical syndromes. With the possible exception of coarctation of the aorta, the clues are not diagnostically specific. In many instances, however, they serve to narrow a broad array of diagnostic possibilities to 2 or 3 and, with the aid of other clues and auscultation, they can often be distinguished from one another. When a primary care physician is confronted with a child who has an incidental murmur that is "probably" innocent but could be organic, useful clues favoring an organic murmur are a history of congenital heart disease in a first-degree relative; a history of maternal rubella syndrome, alcohol use, or teratogenic drug use during pregnancy; a history of inappropriate sweating; a history of syncope, chest pain, or squatting; maternal diabetes mellitus; premature birth; birth at a high altitude; cyanosis; abnormal pulsations; recurrent bronchiolitis or pneumonia; chronic unexplained hoarseness; asymmetric facies with crying; and a physical appearance suggestive of a clinical syndrome.