Sickle cell disease in Middle East Arab countries.

The sickle cell (HbS) gene occurs at a variable frequency in the Middle Eastern Arab countries, with characteristic distribution patterns and representing an overall picture of blood genetic disorders in the region. The origin of the gene has been debated, but studies using ß-globin gene haplotypes have ascertained that there were multiple origins for HbS. In some regions the HbS gene is common and exhibits polymorphism, while the reverse is true in others. A common causative factor for the high prevalence and maintenance of HbS and thalassaemia genes is malaria endemicity. The HbS gene also co-exists with other haemoglobin variants and thalassaemia genes and the resulting clinical state is referred to as sickle cell disease (SCD). In the Middle Eastern Arab countries, the clinical picture of SCD expresses two distinct forms, the benign and the severe forms, which are related to two distinct ß-globin gene haplotypes. These are referred to as the Saudi-Indian and the Benin haplotypes, respectively. In a majority of the Middle Eastern Arab countries the HbS is linked to the Saudi-Indian haplotype, while in others it is linked to the Benin haplotype. This review outlines the frequency, distribution, clinical feature, management and prevention as well as gene interactions of the HbS genes with other haemoglobin disorders in the Middle Eastern Arab countries.

Ethical issues on preventions and management of blood genetics disorders--Islamic views.

Ethical issues are of concern to all members of the health team and the community at large, particularly in relation to chronic and genetic diseases. The fear from the negative social impact, including stigmatization of the carriers of a genetic disease and the affected individual is of a particular concern to the individual and his family. Members of the health team should protect the individual autonomy, observe the ethical principles including beneficence, nonmaleficent, justice, and confidentiality, and maintain high level of professional competence. In addition, Islamic/Arab costumes and traditional issues need to be respected in these communities. This article will outline the prevention and management of common blood genetic disorders in the high of Islamic ethical teachings, where the individuals benefit is paramount.

Pre-marital examination as a method of prevention from blood genetic disorders. Community views.

The Saudi Royal Cabinet issued the Saudi Royal Decree No. 3 dated 11-7-1424, establishing the pre-marital examination as a health preventive measure for all Saudis, and requesting the 2 prospective partners (male and female) to carry out a pre-marital examination and present a certificate of pre-marital examination before the wedding. However, the prospective husband and wife are not obliged to abide by the laboratory results if they so wish. This program started officially on the 1st of Muharram, 1425. As a consequence, the community perception and views on the pre-marital examination program as a preventive measure become crucial. Therefore, this study was organized and conducted to reveal the perception of the community regarding the program, through a purpose-made questionnaire that was distributed during symposia and awareness lectures held throughout the Kingdom (Riyadh, Jeddah, Dammam, Al-Khobar, Al-Ahsa, Al-Qassim, and Madinah). The questionnaire covers the opinions of the participants in various aspects related to genetic diseases including nature of diseases, mode of transmission, affecting factors, complications, management, and prevention. In addition, the questionnaire also covers opinions regarding the pre-marital examination as a preventive measure of blood genetic diseases, and its contribution to the control of these diseases. The questionnaire also covers methods in obtaining information, whether from studies, lectures, information media, and awareness lectures. The results revealed that the majority of the participants (94.3%) were convinced that the pre-marital examination is an effective mean of prevention of blood genetic diseases. The majority (86.9%) of the participants were also convinced that the pre-marital examination should be mandatory. These are indications that the community is aware of the pre-marital examination, and the effectiveness of the awareness program is enriching the knowledge of the citizens.

Hematologic risk factors for stroke in Saudi children.

OBJECTIVE: To explore the hematologic risk factors for stroke in a cohort of Saudi children. METHODS: We evaluated children at the Division of Pediatric Neurology at King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, during the periods July 1992 to February 2001 (retrospective study) and February 2001 to March 2003 (prospective study). Investigations for suspected cases included neuroimaging, transcranial Doppler (TCD) for cases of sickle cell disease (SCD), and Duplex scan. Hemostatic assays included coagulation screening tests, tests of thrombin generation and fibrinolysis, coagulation inhibitors, and activated protein C resistance. RESULTS: During the study period, 104 Saudi children (aged one month to 12 years) with stroke were seen. The mean age of the cohort was 27.1 months (SD = 39.3 months) and median was 6 months. Ischemic strokes accounted for the majority of cases (76%). A major risk factor was identified in 93 of 104 cases of stroke (89.4%). Hematologic disorders were the most common (46.2%), followed by prothrombic disorders (31.7%); microcytic hypochromic anemia (26%); sickle cell disease (SCD), or SCbeta(0)-thalassemia, (11.5%), and factor IX deficiency (2.9%). Raised anticardiolipin antibodies (13/49, 26.5%) was the most frequent abnormality. Deficiencies of the natural anticoagulants (protein S, protein C and antithrombin III) were as follows: protein S (15/70, 21.4%); protein C (15/70, 21.4%) and combined deficiency of 2 or more inhibitors (9/70, 12.9%). Activated protein C resistance has not been detected. Contrary to the findings of previous studies from Saudi Arabia, SCD is a common risk factor and is severe, as it resulted in multiple strokes. Moyamoya syndrome was diagnosed in 2 patients with SCD, one of whom had revascularization surgery (encephaloduroarteriosynangiosis). Assessment of children with SCD at risk of stroke was helped by the introduction of TCD followed by neuroimaging, using MRI and magnetic resonance angiography. CONCLUSIONS: The study strongly highlights the importance of prothrombotic disorders and the severe phenotype of SCD as risk factors for stroke in Saudi children.

The natural history and the national pre-marital screening program in Saudi Arabia.

The genetic disorders are chronic in nature and, therefore, require continuous support and health care. Consequently, the genetic diseases cause formidable economic and psychosocial burdens on the family with negative reflection on the community at large. The genetic diseases are a heterogeneous group that result in varieties of chronic health ailment as a result of defects in the genetic material. The congenital malformations and some genetic defects may result from exposure to radiation, pharmaceutical drugs, the exposure of the mother during pregnancy to certain infectious diseases, such as rubella, toxoplasma or viruses. It may also result as a side effect of chronic diseases, including diabetes, hypertension or varieties of environmental factors, or both. The other group of genetic diseases are transmitted from parents to the offspring through a specific pattern of inheritance exemplified by recessive genetic disorders. This group includes the sickle cell gene, the thalassemias, the hemophilias, inborn errors of metabolism and red cell enzymopathies. The main etiological factors of genetic diseases and congenital malformations are 1) Genetic defects which are transmitted to offspring through carriers of affected parents. 2) Mutations in the genetic materials due to spontaneous mutations, exposure of the mother during pregnancy to infectious diseases, such as rubella and toxoplasma, receiving certain teratogenic drugs during pregnancy, exposure of the mother to ionizing radiation during pregnancy such as x-ray and chronic diseases of the mother, such as diabetes mellitus. 3) Others such as difficult labor or injury to the baby, during or after labor. This paper reviews the natural history of common blood genetic disorders and the means of prevention and control, focusing on pre-marital screening as a means of prevention.

Ethics of genetic counseling--basic concepts and relevance to Islamic communities.

Scientific advances and technical developments in the field of laboratory diagnosis and their practical applications have raised ethical issues linked to religion, beliefs, lifestyle and traditions prevailing in different communities. Some of these are pertinent to genetic screening at various stages of life, prenatal diagnosis and the right of the genetically affected fetus to live--all aspects relevant to inbreeding marriages. Of relevance are medical and ethical principles based on professional responsibility. These ideological and social aspects encounter the challenges of science and its applications in the health field, which are linked, directly or indirectly, to scientific achievements and applications related to human genetics. Analysis of the human genome and identification of its sequence, and chemical components, and theories arising from connection of human genome components in health and disease conditions, have led to global requirements to outline legal aspects and ethical principles in relation to diagnosis, prevention and health care. This paper presents basic aspects of disseminating genetic information, guiding the individual, the couple, or the concerned family through genetically induced ill health and methods of control and prevention. The paper discusses the elements and manner and presents details of the application of genetic counseling in Islamic communities in light of scientific, religious, social and legal aspects in the Islamic arena.

Leptin level in pregnant mothers at term and cord blood and the effect of newborns gender.

OBJECTIVE: To evaluate the leptin level in healthy pregnant mothers at term and in their newborns and its relationship to their body mass index (BMI) and gender of the newborn. METHODS: The leptin level was measured in serum of 187 pregnant women at term delivering at the King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia in July 2001 and their newborns. Correlation studies were made between leptin level and their growth parameters, and gender of the newborns. RESULTS: Leptin level of the mothers was significantly higher compared to their newborns. There was a significant correlation between leptin level in the pregnant women and their weight, BMI, and the newborns leptin level and weight, but not their height or BMI. A statistically significant difference was found between male and female newborns plasma leptin level. A significant correlation was found between male newborns leptin level and all their growth parameters, while in the female newborns there was no correlation between their leptin level and BMI. CONCLUSION: Leptin plays an important role in providing a growth promoting signal during pregnancy, but the fat mass does not seem to be the most important predictor of cord leptin level.

Hematological risk factors for coronary heart disease.

Coronary heart disease (CHD) is a leading cause of morbidity and mortality in the developed and developing countries. Several underlying genetic and environmental factors have been implicated in its etiology. Some of the hematological risk factors implicated in the development of coronary heart disease include antithrombin III deficiency, protein C and protein S deficiency, factor V Leiden mutation, prothrombin gene (20210A) mutation hyperhomocystinaemia, elevated factor VIII levels, plasminogen activator inhibitor type 1 and dysfibrinogenaemia. In general, these factors result in thrombosis, thus having a negative effect on the heart and blood vessels. This paper presents an overview of some of the hematological risk factors involved in the development of CHD.

The inter-relationship hematological parameters between Saudi newborns and parents.

OBJECTIVE: To determine the relationship of the hematological parameters between Saudi newborns and their parents. METHODS: This study was carried out at King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia, over a one-year period. Venous blood samples were taken from 82 healthy Saudi pregnant women in labor at full term, their husbands, and umbilical cord blood after delivery. Estimation of the hematological indices, in addition to transferrin level were performed. RESULTS: A statistically significant correlation was found between parents and cord blood with regards to red blood count, mean corpuscular volume, mean corpuscular hemoglobulin, mean corpuscular hemoglobulin concentration and packed cell volume, while no correlation was found with hemoglobulin and transferrin. CONCLUSION: Some of the hematological indices could be mainly genetically determined including red blood count, mean corpuscular volume, mean corpuscular hemoglobulin concentration, and packed cell volume as significant correlation was found between parents and their newborns while hemoglobulin and transferrin levels were not as these variables are mainly affected by environmental factors.

A comparative study of prevalence of overweight and obesity in children in different provinces of Saudi Arabia.

The aim of this study was to determine the prevalence of overweight and obesity in Saudi children from different provinces of the country and in different age groups. A total of 12,701 children (6,281 boys and 6,420 girls) with ages ranging from 1 to 18 years were enrolled during a household screening programme in different provinces of Saudi Arabia and height and weight were recorded. Body Mass Index (BMI) was calculated and applying age and sex specific cut-off points for BMI the children were grouped into overweight and obese. The overall prevalence of overweight was 10.68 and 12.7 per cent and that of obesity was 5.98 and 6.74 per cent in the boys and girls, respectively. In the different provinces the prevalence of overweight ranged from 8.8 to 27.4 per cent and from 9.3 to 27.6 per cent and obesity ranged from 4.7 to 10.4 per cent and from 4.3 to 13.8 per cent in the boys and girls, respectively. Prevalence of overweight and obesity was also calculated after grouping the children into 17 groups according to age. It is concluded that overweight and obesity occur in all provinces of Saudi Arabia although at a variable prevalence. In general, girls have a higher prevalence of both overweight and obesity compared with boys. Eastern province children have the highest prevalence and the Southern province children have the lowest prevalence of overweight and obesity. When grouped according to age, overweight and obesity tend to increase with age. Suggestions are made to prevent overweight and obesity development in Saudi children.

The prevalence of obesity and overweight in 1-18-year-old Saudi children.

OBJECTIVES: The aim of this study was to review the prevalence of overweight and obesity in Saudi children with ages ranging up to 18 years. SUBJECTS AND METHODS: The study was a cross-sectional national epidemiological household survey, and the study group included 12071 children (boys 6281; girls 6420), with ages ranging from 1-18 years. Their height and weight were measured and body mass index (BMI) was calculated. The study group was classified as obese or overweight, using age- and sex-specific cut-off points for BMI for determining overweight and obesity in children. RESULTS: The overall prevalence of overweight was 10.7% and 12.7% in the boys and girls, respectively, and obesity was 6.0% and 6.74% in the two groups, respectively. The children were grouped according to the province to which they belonged, and prevalence of obesity and overweight were calculated for each province. The highest frequency was in the Eastern Province, while the lowest was in the Southern Province. The children were further grouped into 1-6, 6-12 and 12-18-year-olds and prevalence of obesity and overweight was calculated. In addition, at yearly intervals, the prevalence of obesity and overweight was calculated. Among the boys and girls, the maximum prevalence of obesity was in the 2-3 year-olds. A decrease in prevalence was found in both males and females up to the age group of 8-13 years, and then the prevalence increased again up to the 18 years age. CONCLUSION: This epidemiological household survey shows the overweight and obesity trends in Saudi children based on the international sex-specific cut-off points for BMI. It also shows a variable prevalence in different age groups until after 13 years, when the prevalence rate increases.

Piracetam for the treatment of sickle cell disease in children- a double blind test.

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Blood groups in Saudi obstetrics patients.

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Prevalence of hypertension in adult Saudi population.

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Sickle cell gene as a multifaceted problem in Saudi Arabia.

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Diabetes mellitus as a health problem in Saudi Arabia

A total of 25 337 Saudis [11 713 males [46.2%] and 13 624 females [53.8%]] were screened for diabetes mellitus and impaired glucose tolerance using WHO criteria for diagnosis. The prevalence of insulin-dependent diabetes mellitus, non-insulin-dependent diabetes mellitus and impaired glucose tolerance in the total Saudi male population was 0.23%, 5.63% and 0.50% respectively, and in the total Saudi female population was 0.30%, 4.53% and 0.72% respectively. Differences were observed in the prevalence of diabetes mellitus and impaired glucose tolerance between the provinces. Non-insulin-dependent diabetes mellitus increased to 28.82% and 24.92% in males and females respectively over the age of 60 years, while impaired glucose tolerance increased to 1.60% and 3.56%