title: Sickle cell disease in western Sudan: genetic epidemiology and predictors of knowledge attitude and practices.
abstract: OBJECTIVE
  To investigate the epidemiology of sickle cell disease (SCD) and determinants of knowledge, attitudes and practices (KAP) towards SCD in western Kordofan State, Sudan.
  
  
  METHODS
  A community-based, descriptive, cross-sectional study was conducted in three towns. Three hundred and seventy-two households were polled, and blood samples for haemoglobin phenotyping were collected from 1116 individuals. Sociodemographic, socio-economic and KAP data were collected using investigator-administered questionnaires. Descriptive, frequency distribution and multiple regression analyses were performed.
  
  
  RESULTS
  About 50.9% of the study population were Misseriya tribes. Consanguineous marriages were reported by 67.5% of the households. The highest percentage of homozygous SCD was 2.8% among children under 5 years of age. About 24.9% were carriers of HbS allele (HbAS). HbS allele frequency was highest in children aged 5-11 years (18.3%, CI: 13.7-22.9%) and lowest in males >15 years old (12.0%, CI: 6.1-17.9%). The average HbS frequency across all age groups was 14.5% (95% CI: 12.2-16.8%). The most frequent β-globin gene cluster haplotype was the Cameroon (30.8%), followed by the Benin (21.8%), the Senegal (12.8%) and the Bantu (2.2%) haplotypes. About 17.0% of all-cause child deaths were due to SCD. The estimated change in log odds of having the SS genotype per year increase in age was (-) 0.0058 (95% CI -0.0359, 0.0242). This represents a non-statistically significant 2.9% increase in 5-year mortality for individuals with the SS genotype relative to those with AS and AA genotypes. About 46.9% of the households had poor knowledge, 26.1% had satisfactory knowledge, and 26.9% had good knowledge about sickle cell disease. Mothers' and fathers' educational levels were significant predictors of good knowledge about SCD (P < 0.05). About 48.0% had a satisfactory attitude towards sickle cell disease while 30.7% had poor attitude and only 21.3 showed good attitudes. Poor knowledge about SCD and low socio-economic status were the strongest positive predictors of poor attitude and practices towards SCD (P < 0.01).
  
  
  CONCLUSIONS
  Sickle cell disease is a major health problem in West Kordofan, Sudan. Knowledge, attitude and practices towards the disease are not satisfactory. The development of public health programs is highly recommended to control and manage SCD in western parts of Sudan.
authors.affiliation: Department of Global Health and Population, Harvard University, Boston, MA, USA.|Department of Medical Biochemistry, Faculty of Medicine, University of Khartoum, Khartoum, Sudan.
authors.forename: Ahmed A
authors.initials: AA
authors.lastname: Daak
authors.affiliation: Department of Community Medicine, School of Medicine, Ahfad University for Women, Khartoum, Sudan.
authors.forename: Elfatih
authors.initials: E
authors.lastname: Elsamani
authors.affiliation: Lipidomics and Nutrition Research Centre, Faculty of Life Sciences and Computing, London Metropolitan University, London, UK.
authors.forename: Eltigani H
authors.initials: EH
authors.lastname: Ali
authors.affiliation: School of Nursing Sciences, University of Khartoum, Khartoum, Sudan.
authors.forename: Fatma A
authors.initials: FA
authors.lastname: Mohamed
authors.affiliation: Department of Statistics, Faculty of Mathematical Sciences, University of Khartoum, Khartoum, Sudan.
authors.forename: Manar E
authors.initials: ME
authors.lastname: Abdel-Rahman
authors.affiliation: School of Pharmacy and Biomedical Sciences, University of Portsmouth, Portsmouth, UK.
authors.forename: Abozer Y
authors.initials: AY
authors.lastname: Elderdery
authors.affiliation: Department of Biostatistics, Harvard University, Boston, MA, USA.
authors.forename: Octavious
authors.initials: O
authors.lastname: Talbot
authors.affiliation: Department of Biostatistics, Harvard University, Boston, MA, USA.
authors.forename: Peter
authors.initials: P
authors.lastname: Kraft
authors.affiliation: Lipidomics and Nutrition Research Centre, Faculty of Life Sciences and Computing, London Metropolitan University, London, UK.
authors.forename: Kebreab
authors.initials: K
authors.lastname: Ghebremeskel
authors.affiliation: Department of Medical Biochemistry, Faculty of Medicine, University of Khartoum, Khartoum, Sudan.
authors.forename: Mustafa I
authors.initials: MI
authors.lastname: Elbashir
authors.affiliation: Department of Global Health and Population, Harvard University, Boston, MA, USA.
authors.forename: Wafaie
authors.initials: W
authors.lastname: Fawzi
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000755:Anemia, Sickle Cell / Q000453:epidemiology* / Q000235:genetics / Q000401:mortality; D001772:Blood Cell Count / Q000379:methods; D002423:Cause of Death; D002648:Child; D046688:Child Mortality; D002675:Child, Preschool; D016000:Cluster Analysis; D004522:Educational Status; D005260:Female; D006239:Haplotypes; D007722:Health Knowledge, Attitudes, Practice; D006801:Humans; D007223:Infant; D007407:Interviews as Topic; D000067010:Literacy / Q000706:statistics & numerical data; D008297:Male; D017720:Molecular Epidemiology; D010290:Parents; D015995:Prevalence; D012923:Social Class; D013397:Sudan / Q000453:epidemiology; D011795:Surveys and Questionnaires; D055815:Young Adult
keywords: Anemia falciforme; Sudán Occidental; actitudes y prácticas (CAP); attitudes et pratiques (CAP); connaissances; conocimientos; drépanocytose; epidemiología genética; genetic epidemiology; haplotipos; haplotypes; knowledge attitude and practices; ouest du Soudan; sickle cell disease; western Sudan; épidémiologie génétique