Mohsen A. F. El-Hazmi

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.

Blood Proteins C and S in Sickle Cell Disease

Proteins C and S are vitamin K-dependent proteins with an essential anti-coagulant function. Protein C exists in an inactive form and is activated by a thrombin-thrombomodulin complex. Protein S combines with protein C and forms a stoichiometric complex which regulates coagulation in the presence of calcium. As patients with sickle cell disease (SCD) bear a high risk of developing thrombo-embolic disorders, we studied the coagulation derangement in 100 patients and 40 normal age- and sex-matched controls. The patients were clinically assessed and classified into sickle cell homozygotes (Hb SS), Hb S heterozygotes (Hb AS) and double heterozygotes for Hb S/beta 0-thalassaemia based on haematological parameters, red cell indices, Hb A2 and F levels and genetic studies. The proteins C and S were estimated and related to the type of the gene defect. The results showed significantly reduced levels of proteins C and S in SCD patients with the highest prevalence of deficiency in patients with a severe disease and frequent episodes of crisis. However, no significant differences were encountered in the level of proteins C and S in the same patients during the steady state and during episodes of crisis. It was concluded that the lower protein C and S levels in SCD is either due to decreased production or increased consumption though this reduction does not seem to play a role in producing thrombo-embolic disorders.

Endocrine functions in sickle cell anaemia patients.

In this study, 80 male and female sickle cell patients, aged 4-50 years, with mild (severity index, SI < 6) and severe (SI > or = 6) forms of the disease were investigated). The levels of luteinizing hormone (LH), follicle stimulating hormone (FSH), testosterone, cortisol, growth hormone (GH), free thyroxine (T4), and free triiodothyronine (T3) were determined. The results were evaluated and the mean +/- 2 SD values were compared with those obtained in age- and sex-matched normal controls. The findings indicated gonadal hypofunction in the sickle cell patients, but with varied deviations from the mean results. Patients with the severe form of the sickle cell disease showed more frequent abnormalities of LH, FSH, cortisol and testosterone in comparison with the patients with a mild disease. The LH, FSH, cortisol and testosterone levels were lower, while T3 and T4 did not show significant differences between patients and the controls. The results suggest that the sickle cell gene abnormality has an adverse effect on endocrine functions. Follow-up and appropriate management of endocrine dysfunctions are advocated in such patients.

Clinical and Haematological Diversity of Sickle Cell Disease in Saudi Children

Sickle cell disease (SCD) exhibits itself in a broad spectrum of clinical behaviour ranging from a mild disease to an incapacitating condition. In this study, we have attempted to investigate the clinical diversity of SCD in different regions of Saudi Arabia. The results of haematological parameters and clinical manifestations in 41 children with SCD from the eastern province where the disease is mild, were compared with results obtained in 51 children from the south-western province (SWP), where the disease has been shown to be more severe. The severity index (SI) of patients from the eastern province ranged from 2 to 11 with a mean of 4.5 and in patients from the south-western province, the SI ranged from 2 to 18 with a mean of 9.5. In addition, the occurrence of hand and foot syndrome, vaso-occlusive crisis, and increased frequency of requirement of blood transfusion and hospitalization differentiated the clinical presentation of SCD in the patients from south-western province from those in the eastern province. Red blood cell level, total haemoglobin and packed cell volume were lower in the SCD children from the south-western province. Haemoglobin A2 level was significantly higher, while haemoglobin F, packed cell volume (PCV), mean corpuscular volume (MCV), and mean corpuscular haemoglobin concentration (MCHC) did not show any significant differences. HbF level did not influence the haematological parameters significantly in the SWP patients. It is concluded that the SCD in Saudi population is heterogeneous clinically and haematologically.

Normal reference values for hematological parameters, red cell indices, HB A2 and HB F from early childhood through adolescence in Saudis.

BACKGROUND Values of hematological parameters are affected by factors such as age, sex, ethnic background and social, nutritional and environmental factors. The objective of this study was to determine the values of hematological parameters, red cell indices and Hb A2 and Hb F levels in normal Saudi children, living in the Central Province of Saudi Arabia. MATERIALS AND METHODS The study was carried out on 1526 apparently healthy children, with ages ranging from 1-15 years, and selected during a household screening program. Hematological parameters, red cell indices, and hemoglobin types (Hb A2 and Hb F) were estimated, and the children were divided into 15 groups depending on the age. Male and female children were separated, and the mean and standard deviation of each parameter was calculated for each age group. RESULTS No significant differences were observed in the red cell count in the male and female children. White blood cells gradually decreased from 2 years onwards, while hemoglobin and hematocrit levels increased significantly from 2 to 15 years. Mean cell volume and mean cell hemoglobin also showed slight increases, while mean cell hemoglobin concentration remained more or less constant. Hemoglobin A2 and Hb F showed slight but nonsignificant fluctuations. Comparison of the results with those reported in the literature shows that Saudi children have some values similar to Caucasians, while others have values which are intermediate between Caucasians and African children. CONCLUSION The values reported in this study can be used as normal reference values for Saudi children and adolescents.

Heterogeneity and Variation of Clinical and Haematological Expression of Haemoglobin S in Saudi Arabs

Sickle cell haemoglobin (Hb S) occurs at a high frequency in the Eastern (EP), South-Western (SWP) and North-Western (NWP) Provinces of Saudi Arabia and the presentation of the Hb S is believed to exhibit clinical diversity in the different regions. Three areas of Saudi Arabia were screened to determine the frequency of Hb S and alpha- and beta-thalassaemias and glucose-6-phosphate dehydrogenase deficiency genes. Furthermore, in an attempt to investigate and compare the clinical and haematological presentation of sickle cell disease (SCD) in the different regions of Saudi Arabia, the individuals identified as Hb S homozygotes were investigated further. The patients were further classified on the basis of whether there was associated alpha- or beta-thalassaemia. A severity index (SI) was calculated for each patient and the clinical presentations and laboratory findings were compared. The results showed significantly variable severity of SCD in patients from different regions. The patients from the EP generally had a mild clinical presentation, while in the SWP and NWP majority of the patients suffered from a severe disease as judged by the SI. No correlation could be established between Hb F level and SI, though the WBC level correlated positively with the SI. The lowest SI values were encountered in patients with associated alpha-thalassaemia who also had the lowest WBC count and MCV and the highest RBC count and packed cell volume.

Hydroxyurea for the treatment of sickle cell disease.

In this study 21 adults with severe form of sickle cell disease (SCD; sickle cell anaemia, n = 15; Hb S/beta degree-thal, n = 6) were treated with hydroxyurea (HU) to assess the effectiveness of the drug in managing SCD. The individual dose was selected for each patient. The dose selection was based on the HU clearance study. Thereafter, the patients received daily doses of 15-20 mg/kg body weight. An evaluation data form was filled out at the monthly visit. The severity index (SI) of the disease was determined and haematological parameters including red cell indices, platelet counts, reticulocyte counts, irreversibly sickled cells, red cell deformability, Hb F, Hb F cells, total and direct bilirubin levels were measured prior to treatment, at follow-up intervals during treatment and after cessation of treatment. The trial period lasted 3 months. Statistically significant improvement was observed in the clinical presentation, haematological and biochemical parameters. Hb F level and F cells showed a significant increase in most patients, but to a variable degree. A major resultant effect was an increase in mean cell volume. Our experience shows that HU can be used for the treatment of severe forms of SCD with no major side effects, provided that the doses are monitored and that laboratory investigations are regularly undertaken.

G6PD DEFICIENCY, DISTRIBUTION AND VARIANTS IN SAUDI ARABIA: AN OVERVIEW

BACKGROUND The first report of glucose-6-phosphate dehydrogenase (G6PD) deficiency in the Saudi population of the Eastern Province paved the way for extensive investigations to determine the distribution and molecular pathogenesis of G6PD deficiency in Saudis in different parts of the country. MATERIALS AND METHODS During a national study lasting from 1982 to 1993, 24,407 Saudis in 31 different areas of Saudi Arabia were screened for G6PD deficiency using spectrophoretic estimation of the enzyme activity and electrophoretic separation of the phenotypes. RESULTS The results in the males and females were separately analyzed, and showed a statistically significant difference in the frequency in the male (0.0905) and female (0.041) population (P<0.05). The frequency in the male varied from 0 to 0.398, and in the female from 0 to 0.214. The phenotypes identified included G6PD-A(+), G6PDA-Mediterranean and G6PD-A(-), and G6PD-Med-like with G6PD-B(+) as the normal phenotype in all areas. CONCLUSION This study shows that G6PD deficiency is a frequently identified single-gene disorder in Saudi Arabia and G6PDA-Mediterranean is the major variant producing the severe deficiency state in this population.

The prevalence of diabetes mellitus and impaired glucose tolerance in the population of Riyadh.

This study was conducted in Riyadh to determine the prevalence of diabetes mellitus and impaired glucose tolerance. Blood samples (3981) were collected from Saudi male and female adults (2402) and children (1579) during a household screening program conducted over a period of two years from September 1991 to September 1993. Fasting blood samples were collected and each individual was given an oral glucose load. Two-hour postprandial blood glucose was estimated and the World Health Organization (WHO) criteria were used to diagnose diabetes and impaired glucose tolerance. The diabetic patients were further grouped into noninsulin-dependent diabetes mellitus (NIDDM) and insulin-dependent diabetes mellitus (IDDM) depending on the age of onset and mode of treatment. The overall prevalence of diabetes mellitus (IDDM and NIDDM) was 4.76% in males and 4.10% in the females in the two-to 70-year age group. Ten individuals of the 3981 were suffering from IDDM, giving an incidence of two to three per 1000. When the children <14 years were removed, the prevalence increased to 8.235% and 6.476% in the males and females respectively, while in the >30 year old group, the prevalence increased to 16.0% and 12.34% in the males and females. Further significant increase in NIDDM and impaired glucose tolerance (IGT) was observed with age (P<0.05). NIDDM was more frequent in males while IGT was more frequent in females. This paper presents the results of screening in Riyadh and stresses the need for diabetes mellitus awareness programs in the Saudi population.

A Comparative Study of Hyperglycemia in Different Regions of Saudi Arabia

ABSTRACT A pilot study was conducted in different regions of Saudi Arabia to estimate the frequency of fasting hyperglycemia in the Saudi population. In Khaybar, Hofuf and surrounding villages, Al-...