title: Alpha thalassemia among sickle cell anaemia patients in Kampala, Uganda.
abstract: BACKGROUND
  Sickle cell anaemia is prevalent in sub Saharan Africa. While α+-thalassaemia is known to modulate sickle cell anaemia, its magnitude and significance in Uganda have hitherto not been described.
  
  
  OBJECTIVE
  To determine the prevalence of α+thalassaemia among sickle cell anaemia patients in Mulago Hospital and to describe the clinical and laboratory findings in these patients.
  
  
  METHODS
  A cross sectional study was carried out on patients with sickle cell anaemia in Kampala. Dried blood spots were used to analyze for the deletional α+ thalassaemia using multiplex polymerase chain reaction.
  
  
  RESULTS
  Of the 142 patients with sickle cell anaemia, 110 (77.5%) had the αα+thalassaemia deletion. The gene frequency of (-α) was 0.425. Ninety one percent (100/110) of those with α+thalassaemia were heterozygous (αα/α-). Amongst the patients older than 60 months, 15 (83.3%) of those without αα+thalassaemia had significant hepatomegaly of greater than 4 cm compared to 36 (45.6%) of those with α+thalassaemia (p=0.003).
  
  
  CONCLUSIONS
  The gene frequency of (-α) of 0.425 noted in this study is higher than that reported from many places in Africa. Concurrent alpha thalassemia might be a protective trait against significant hepatomegaly in sickle cell anaemia patients more than 60 months of age at Mulago hospital.
authors.affiliation: Makerere University College of Health Sciences, Paediatrics and Child Health.
authors.forename: Irene
authors.initials: I
authors.lastname: Lubega
authors.affiliation: Makerere University College of Health Sciences, Paediatrics and Child Health.
authors.forename: Christopher M
authors.initials: CM
authors.lastname: Ndugwa
authors.affiliation: Makerere University College of Health Sciences, Paediatrics and Child Health.
authors.forename: Edison A
authors.initials: EA
authors.lastname: Mworozi
authors.affiliation: Makerere University College of Health Sciences, Paediatrics and Child Health.
authors.forename: James K
authors.initials: JK
authors.lastname: Tumwine
mesh_terms: D000368:Aged; D000755:Anemia, Sickle Cell / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics; D003430:Cross-Sectional Studies; D005260:Female; D005787:Gene Frequency; D006801:Humans; D008297:Male; D015995:Prevalence; D011795:Surveys and Questionnaires; D014454:Uganda / Q000453:epidemiology; D017085:alpha-Thalassemia / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics
keywords: Alpha thalassemia; Kampala; Uganda; sickle cell anaemia patients