Epidemiology of Sickle Cell Disease in Saudi Arabia

Jastaniah, Wasil

doi:10.5144/0256-4947.2011.289

Cited by 35 publications

(33 citation statements)

References 36 publications

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“…The Sickle Cell Anemia affected about 100, 000 people of the United States, among them 2 million are carrier 3 however, embarked decline in the mortality 4 and the mean age of death in male is 53 years and in female about 58 years 5 . In Pakistan, 0.5-1% of populationis carriers of Hemoglobin S 6 , whereas about 5.1% of prevalence found in Karachi 7 . The prevalence of Sickle cell disease is 1.4% in some regions of Saudi Arabia 8 .…”

Section: Introductionmentioning

confidence: 98%

Sickle Cell Anemia; 3 Years Clinical Experience in Bisha, Saudi Arabia (2010-2013)

Aziz¹,

Musharraf²,

Shah³

et al. 2017

Int. j. endorsing health sci. res.

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Objective Three-year clinical experience of patients diagnosed with Sickle cell disease in Bisha, South Western region of Saudi Arabia is analyzed. Methodology A retrospective analysis was done on data recruited from 2011 to 2013 of all the patients presented with sickle cell anemia. The demographic data, clinical presentation, course of disease, complications and chronic conditions were collected. The descriptive percentage of data with years of presentation and its incidence rate per 100 person-years were also taken with the cause mortality. Results Total 386 patients were analyzed, among them 45.2% were females and 54.8% were males. According to our study, the sickle cell crises included as acute chest syndrome (26%), pulmonary hypertension (21%), Splenomegaly (7%), septicemia (11%), acute Vaso-occlusive crisis (10%), and other sickle cell crisis (2-10%) of patients. It was also found that about 48% of patients suffered from 1 or more irreversible organ damage. The survival rate of male and female are not significant, however total 82 patients died of sickle cell anemia. Conclusions Sickle cell disease represents high mortality in Saudi Arabia, comprising distinctive diseases characteristic in different regions. The qualitative and quantitative study of genetic and environmental factors in relation to disease complications need to be done.

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Section: Introductionmentioning

confidence: 98%

Sickle Cell Anemia; 3 Years Clinical Experience in Bisha, Saudi Arabia (2010-2013)

Aziz¹,

Musharraf²,

Shah³

et al. 2017

Int. j. endorsing health sci. res.

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“…The highest frequency of sickle cell disease was found in tropical regions, particularly sub-Saharan Africa, India and the Middle-East, [9] with prevalence of sickle-cell trait (HbS) of 4.2% in Saudi Arabia and 0.26% of sickle cell disease (HbSS) with the highest prevalence in the Eastern province. [10] A population-based study from Bahrain has confirmed a high prevalence of VDD, especially in females, that shows a seasonal variation. [11] In this study we assess the prevalence of VDD in patients with SCD (HbSS) in Bahrain and evaluate the clinical predictors of VDD in such patients.…”

Section: Introductionmentioning

confidence: 98%

Prevalence of vitamin D deficiency in patients with sickle cell disease in Bahrain

Bakri

Garadah²,

Jaradat

et al. 2013

IJM

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Background: Patients with sickle cell disease (SCD) had a low serum level of vitamin D. The prevalence and the impact of this observation in SCD patient's in Bahrain is not clear. Aim: To assess the prevalence of vitamin D deficiency (VDD) in homozygous patients with SCD, and to evaluate the clinical and biochemical predictors of occurrence of VDD in such patients. Methods: We evaluated the vitamin D status in 70 patients with confirmed diagnosis of SCD and compared them with an age matched control group. Serum level of vitamin D, parathormone (PTH), calcium, phosphate, alkaline phosphatase, haemoglobin (Hb), and uric acid were measured. Linear regression analysis was performed to assess the relation between PTH and vitamin D level. Multiple regression analysis was performed to assess the predictive value of gender, body mass index (BMI) >24, serum level of uric acid>400 umol/Land estimated glomerular filtration rate (eGFR) <60, for occurrence of vitamin D deficiency in patients with SCD. Results: The mean age of the study group was 28.85 ±7.21 years compared with the control of 29.91 ±4.3 years, p=0.23. There were 40 (57%) female SCD patients compared with 39 (55%) in the control. In patients with SCD, the serum level of vitamin D was sufficient, >50 nmol/L in 4 (5%) patients, insufficient > 27.5 -≤50 nmol/L in 21 (30%) patients, deficient >12.5 -≤27.5 nmol/L 35 (50%) and profoundly deficient ≤12.5 nmol/L in 10(15%). In the control group, those with sufficient level of vitamin D were 53 (75%), insufficient 21(30%) deficient of 10 (15%) and none with profound deficiency, respectively. The serum level of PTH and alkaline phospahtase were significantly higher in the SCD group compared with control group. The eGFR, Hb and were significantly lower, serum level of uric acid was significantly higher (p < 0.05 but no significant difference between serum level of calcium or phosphate. Linear regression analysis showed inverse relation between VDD (<25 nmol/L) and PTH (r = -0.34, P<0.001). Multiple regression analysis showed female gender, BMI>24, hyperurecemia and low eGFR were positive predictor of VDD in patients with SCD. Conclusion:The risk of vitamin D deficiency among subjects with SCD-SS was 3.8 folds greater than control subjects; the prevalence of VDD in SCD was 95% and 25% in the control patients without SCD. On linear regression analysis, there is a significant inverse relation between serum levels of vitamin D and PTH in SCD patients. The female gender, BMI>24, hyperurecemia>400 umol/L and low eGFR<60 were positive predictor of VDD in patients with SCD.

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“…The highest prevalence of SCD in Saudi Arabia is in the Eastern province. [28] Sickle cell diagnosis raises concerns about the affected child's life span. With appropriate clinical management supported by nurses' involvement in learning and education about their children' disease, 95 % of children will live beyond the age of 18.…”

Section: Significance Of the Studymentioning

confidence: 99%

Empowering Mothers to Overcome Sickle Cell Crisis in Their Children through Engagement and Education

El-Gawad¹

2017

AJNR

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Background: Sickle cell anemia is a common disease in Saudi Arabia. It causes major crises to children who suffering from it. Empowerment refers to measures designed, to increase autonomy and self-determination through professional support and engagement and empowerment. Mothers education and engagement are critical components of high-quality and early care of children suffering from sickle cell crises (SCC). Aims: To empower mothers to manage SCC events experienced by their children. To educate the mothers and engage them in caring for their children and to evaluate the effect of engagement and education on mother's knowledge and performance regarding SCC. Study design: A quasi-experimental design was used. Individualized interview and small group teaching with pre and post evaluation was applied. Setting: The study was conducted in Pediatric Hematological Department at Maternity and Children Hospital, at Makkah Al-Mukararma. Subjects: A purposive sample composed of 40 mothers recruited according to certain inclusion and exclusion criteria. Tools: Two tools were used to collect data including, First: Interview Questionnaire sheet was used to collect mothers' socio-demographic characteristics. Second: empowerment scale, that consisted of 34 items within three construct areas family, services system and community. Results: Nearly two thirds of the mothers had poor knowledge regarding SCA pretest compared to one fifth of them posttest. Statistical significant differences regarding was found (P= 0.00). Improved mothers' performance was recognized post-test, most of the mothers, always give immunization, and enough oxygen. Three quarters of the mothers offer plenty of fluids to their children. Conclusion: Mothers knowledge and performance were improved after education and engagement. Mothers empowerment was very true "post-test" and there was statistical significant difference pre and posttest. There were significant positive correlations between mothers' age and their education, total knowledge, and total performance. Recommendations: Empowering and engaging mothers in caring for their children with SCA. Developing educational program for mothers about SCA and SCC.

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Epidemiology of Sickle Cell Disease in Saudi Arabia

Cited by 35 publications

References 36 publications

Sickle Cell Anemia; 3 Years Clinical Experience in Bisha, Saudi Arabia (2010-2013)

Sickle Cell Anemia; 3 Years Clinical Experience in Bisha, Saudi Arabia (2010-2013)

Prevalence of vitamin D deficiency in patients with sickle cell disease in Bahrain

Empowering Mothers to Overcome Sickle Cell Crisis in Their Children through Engagement and Education

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