Haptoglobin gene polymorphisms in sickle cell disease patients with different βS-globin gene haplotypes.
OBJECTIVE
To investigate the prevalence of haptoglobin (Hp) gene alleles in Kuwaiti sickle cell disease (SCD) patients, who generally have a mild phenotype, and compare the pattern to Nigerian SCD patients whose SCD phenotype is more severe.


METHODS
Hp genotyping was carried out in a group of 82 and 54 SCD patients from Kuwait and Nigeria, respectively, and appropriate Hb AA controls. The Hp genotyping was done using a PCR technique followed by agarose gel electrophoresis.


RESULTS
The frequency of the Hp-2 allele was 73.8% among Kuwaiti SCD patients, while the Hp-1 allele predominated among Nigerian patients (60.7%). However, the differences were not significant (p > 0.05) when the allele distributions were compared between Kuwaiti SCD and their AA counterparts or between Nigerian SCD and their AA controls. There was no association of Hp-2 allele with frequent vaso-occlusive crisis among the Kuwaiti SCD patients.


CONCLUSIONS
The distribution of Hp alleles appears to follow ethnic and geographical trends. Their role in the pathophysiology of pain crisis is not clear.
Adekile
A D
AD
Department of Paediatrics, Faculty of Medicine, Health Sciences Centre, Kuwait University, Kuwait. adekile@hsc.edu.kw
Haider
M Z
MZ
D000755:Anemia, Sickle Cell / Q000208:ethnology / Q000235:genetics*; D004587:Electrophoresis, Agar Gel; D005787:Gene Frequency; D006239:Haplotypes; D006242:Haptoglobins / Q000235:genetics*; D006801:Humans; D007730:Kuwait / Q000453:epidemiology; D009549:Nigeria / Q000453:epidemiology; D016133:Polymerase Chain Reaction; D011110:Polymorphism, Genetic; D055544:beta-Globins / Q000235:genetics*
D006242:Haptoglobins; D055544:beta-Globins